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Contents

1 Cross;Sectional Anatomy of the Brain. . . . . . . . . . . . . . . . . . . . . . . . 1


I. Imroduclion I
II. Midsayill-al >(.'Ction I
III. Commll .st.'Ction through the op(ic chia:.m 3
IV. Coron:11 S(,'CllQll through the mamillary boJio 4
v. A:rcial inm!:,'c through Ihe Ih:llamU'i and imcm.... 1capsule 5
VI. Axial irJJ:1boe through Ihe midbrain. mamillary hodics. and 0I1'11c lr.'Cl 6
2 Meninges, Ventricles, and Cerebrospinal Fluid. . . . . . . . . . . . . . . . . . 8
l. Mcnill/,'C5 8
II. Vcnuicul:lr sy:.tcm 10
III. Cerehrospm<lllluid 11
IV. Hcml:ltion II
3 Blood Supply 15
I. The spinal cord :mu lower hrain slcm 15
II. The imerna! carOl:id SYSlcm 16
III. The \'cnchrolxlSilar system 17
IV. 1111,' hl/XXI supply of lhe iIHem:'[ c;lpsulc 18
V. Veins or till' hr..in 18
VI. Venous duml sinu.$t.'$ 18
VII. Anyiogral1hy 18
VIII. The middle 11l1'nin!:,'ca] :1T(("ry 19
4 Development of the Nervous System
I. n,C Ileur;l] wbc 24
II. Thc Ileum] crest 2S
III. Thc ;mlcrior ncuropore 26
IV. The lX>Slcrinr neuropore 26
V. Microglia 26
VI. Myelination 27
VII. uf the cord 27
VIII. The optic ncrvc and chiasma 27
IX. The hypophysis 27
X. Clllgenit;l]lll;llfonnillions of lhe eNS 27
. . . . ......... .... .. .. .. . . 24
5 Neurohistology....................... . . . . . . . . . .. 30
I. N('urons 30
II. Nbsl SUI\ililllCC 31
III. AXOTlflllranspor! 31
IV. dl'gcncrmioll 31
v. 3 I
VI. Rl'gcllcrlllionofncrvccclls 31
VII. Gliill cclls 3I
VIII. TI1C blood-hrain 1,.1rricr 32
IX. TI1C Mood-CSF harricr 32
,
vi Contents
X. Pigmcnt:> :1r1<1 inclusion, 32
XI. TIlC o( ncr\'C tillers 32
XII. Tumurs of thc CNS anJ PN$ H
XIII. Cut:ll\('Ou, )j
6 Spinal Cord 36
I. Gr..), :'111(1 whit contmuniG'ling rami 36
II. Termimuion 01 Ihe conus rneJullaris 36
III. Location o( Ih... m"jor Tl\O{o.- and senSO!1' nuclei of the spinal cord 37
IV. TIle caw" C<.Jllin" 37
V. TIl(" m\-oI:llic Tl."Hcx 37
7 Tracts of the Spinal Cord ..... "........................... 38
I. ImrtlducllOrl 38
II. DurSll colllmn-mcdialltmni.scus rr.tlhwa)' 38
III. ulICT.ll srmothal:llnic tract 40
IV. uucml cnrl:icosrinaltracl 40
V. 1-ln"Mhal:un.-.;pinal tract 44
8 Lesions of the Spinal Cord "............ 45
I. 1:>.sc:lSCS of the l1\O{or nl"\.lrons and eorticospinr.1 tracts 45
II. SctlSOr)' p:llh""ay lesions 47
III. wnhmcd mOlur "nJ senSOf')' lesions 47
IV. Peril'her.ll nervous S)"I('m (PNS) lesions 48
V. Imcn'crl:chml d1$k hemi:Uioll 48
9 Brain Stem "",,"........................................ 49
I. Overview 49
II. Cro:,.,-.....'Ctron duough the m(odulb 49
III. Cros,-,cClion the pons 51
IV. CroSS'SCClrnn Ihrough the rostral .nidhrain 52
V. C..nicobull'<lr tibers 51
10
11
12
13
Trigeminal System
t. Ov... rview 53
II. The lrigemin:ll g,rnglion 53
III. Trigcminolh,lhrmic jl:llhw<lrs 53
IV. ret1cxes 55
V. CUVo.'rnOllS sinus 57
Auditory System ....................................
I. OI'crl'i<:1I' 58
II. TI1... ilUdiwry I'ulhw:l\, 58
III. J l<:nnng defccts 58
IV. AudiwT}' tc,lS 59
Vestibular System .... " . ".. "......................
I. Ovcrvio.'w 61
II. Thc bhrrinlh 61
III. TIle vCSl:ihul:tr 62
IV. V<:"stihulil-O(:ubr reflex..." 63
Cranial Nerves ....................................
I. TIle oIfacwr'1 ne("\'e 65
II. TIle OI'l(ic nen'e 66
III. TIle OCUIOmQlo.- nen'c (CN III) 66
53
58
61
65
Contents vii
IV. TIl(" lrochlear ner e (CN IV) 67
V. TIle Iril.'Cmin:11 ncr (CN V) 67
VI. TIle :IbJucenl ru-oc (CN VI) 6S
VII. Thc facial nem.' (CN VII) 69
VIII. The ..r n..o"e (CN VIII) 71
IX. TIle glo:.:;opl\;u)"nl.'CalllCo"c (CN IX) 71
X. TIle vag:llllCO'C (CN X) 74
XI. The acCc:.soI)' nCO"C (CN XI) 75
XII. The (CN XII) 77
14 Lesions of the Brain Stem . . . . . . . . . . . . . . . . . . . . . .. 78
I. lcsions of lilt nleJull.\ 78
II. Lesions of lhe rons 79
III. lesions o( lhe midhr..in 80
IV. Acoustic nClIrllll1:l ("chw,lllnul1la) 81
V. Jugubr (O!lII1lCIl syndmmc 82
VI. "Locked in" syndrome 83
VII. Cemml p.l1lline myelinulysis 84
VIII. "Top of lhe ha,ilar" 84
IX. Subcl;lvian 84
X. TIle cerehdlol'llll1ine :mgle 84
:1.5 Ce,ebellum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 85
I. Function 85
II. Anmomy 85
III. maiO!" ccrebellar 86
IV. Cerebellar drJunction 87
V. Cerebellar synJromes and lun"lOfS 87
:1.6 Thalamus ........................................... 88
I. Imroduction
II. Major Ih:lbmic nuclei :md thcirconneclions 88
III. Blood 90
IV. The internal c;lpsule 90
17 Visual System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 91
I. Introdoction 91
II. TIle visual palhway 91
III. The pllpilbry light Teilex 93
IV. Tht pllpil);,r)' dilmion p'llh\\"<!\, 93
V. The nC;lr rdlcx ;ll\d :Kcullunud;'llon I';llhway 94
VI. ConiC<ll and subcllnic,ll centers (or ocu);tr lllotilil y 94
VII. Oinical correhllion 96
18 Autonomic Nervous System .......................... 98
I. IntroduClion 98
II. Cr:mi:tl IlCO'Cl> ""'hh componellls 98
III. Communicatlng r:11111 98
IV. NeurUllansmiucl'5 98
V. Clinical cOlTcI.lIion 101
19 Hypothalamus ...................................... 103
I. Introduclion 103
II. Functions 105
III. correl.uion 106
viii Contents
20 Limbic System """"""""""""""""""""."." .. ".............. 107
I. Imroducllon 107
II. Major components .md conn<.'Clion" 107
Ill. 111e P"pc: circuit lOS
IV. Clinical correlation 109
21 Basal Ganglia and Striatal Motor System
I. 8a,;:]1 ganglia III
II. TI,e "I ri<llal (extrapyramidal) Illotur I 12
III. Clinic;llcurrclmion 113
III
22 Neurotransm' "'ters . . . . . . . . . . . . . . . . . . .. . . . . . . .. . . ........
I. Impon;llll .uld their p,llhw;'IYS II;
II. Funnioll"l and dinic,11 cunsidcmtions 120
115
23 Cerebral Cortex .................................
I. [mroJuc[ion III
1I.111e"ix.layeredncoconcx III
Ill. Function"l area. 121
IV. Nw.....ll dcs!rocti\'c hemispheric lesions ..lid ')'tlll'tUlIlS IZ7
V. Cetl.'hr<ll d"mimlllcc 117
VI. Split.hr<lin syndrome Il8
VII. 01 her lesil)ns of the curpllS c;lll,)sulll 128
VIII. Brain ilud spin:d C<lrd tumors 128
121
133
137
Index ..............................................
24 Apmxia, Aphasia, and Dysprosody 129
I. Apr<IXi,1 IZ9
II. Aph'l.i'l 129
III. 131
Appendix .
1.
Cross-Sectional Anatomy of the Brain
I. INTRODUCTION. The illustrations in this ch:lplcr are accompanied by corresponding
m:l1.'11Clic resonance imaging (MRI) scans. Together they represent a mini-atlas of brain
slices ill the three onhogonal planes (i.e., mids..'lgittal. coronal. and axial). An inscn on
each figure shows rhe level of the slice. TI,e most commonly lested Structures an:' labeled.
II. MIDSAGITTAL SECTION (Figures 1-1, 12, and 1-3). TI,e loc:uion oflhe struc-
lures shown in the fif,oures should be known.
Galcarine sulcus
Cerebellum (vermis)
Fourth ventricle
Corpus callosum
ThaJam",
Fornix (column) Motor strip
Interventricular foramen sulcus
sensory strip
Pineal body
Superior and Inferior co4liculi
(tectum)
Septum p&llucldum
Mamillary-bodY eN III
lamina termlnalis.
Third ventricle
Anterior commissure
Medulla oblongata
Agure 1-1. MiJsaginal section of (he br.lin :md brain the SlrllClurCS surrounding rhc rhird and
fourlll vcntricles. hrnm srem includes rhc midbrnin (M), (P), and medulla oblongol('[l.
1
2 Chapter 1
Paracenlrallobole
Precuneus
Cingulale gyrus
Superiot frontal gyrus
Anterior cerebral arlery
Crista galli
Basilar artery
Sphenoid sinus
Clivis
Nasopharynx
C2
Superior sagittal sinus
Parielooccipital fISSUre
Vein of Galen
Cuneus
Cerebellar vermis
Cisterna
cerebellomedullaris
Figure 1-2. Mid$<lgill,11 Tll<ll,'1'k:tk inlaging duuugh the brain and bmin rhe
imponmu structufCli the thinl anJ fourth wlllrklc". This b a TI-weightoo image. The l;:r.IY m;.lt[er
is I,'fll)' (hYJ'Oimensc). wln'reas tile white m:lIh:r is while (hyperintense).
Corpus callosum --"'----_
lateral ventricle ---.'<:
Anterior cerebral artery __-=""..
Optic chiasm
Hypophysislinfundibulum
Mamillary body
Cerebral aqueduct
Fornix Thalamus
Vein of Galen
Pineal gland
Superior and inlerior colliculi
Fourth ventricle
Cisterna cerebellomedullaris
Spinal cord
Subarachnoid space
Figure 13. 1\,liJ....gilt",l1 ma},'!lCtlC rcs.m:lllce ima<.:ing section through the brain stem and diencephalon. Nou'
the ccrebrospin31 fluid tract: lalcr.ll ,cntndc, inren;emMculal fommen ufMonro, third ,emMele, cerchr:ll aque-
ducl, fourth n:mridc, foramen o( Mal,ocndle, cerebdlOllledull<ll)' cistern, and spin...l subarAchnoid .space. NOll.'
also the relation between the optic chIasm. infundihJlum, and hnXl(lhysis (pituitary gland).
Cross-sectional Anatomy of the Bram 3
III. CORONAL SECTION THROUGH THE OPTIC CHIASM (Figures:l.4 and :1.5).
The location of the S[nICtures shown in (he figures should be known.
.....
Oplic chiasm
-
Globus pallidus
,.....
-"
Agure 1-4. Coronal sccllon o( Ihe bmin al the lc\d o( lhe :mler;or COllllll".surelJXic chi,blll, ,mJ amn:-
dol!;l. NOIe Ihm dU' imemal c:.psule llcs hclwecn the cauJmc nucleus :mJ Ihe lentl(onn noch,'us (gl,lI'lm 1'l;:,II.Juo
and put:llllen).
seplum peltucidom _
Inlernal capsula
Amygdala
Hypophysis
Cavernous sinus
Intemerftspheric llssore
Cingulclle gyrus
Corpus callosum
Lateral ventricle
- Caudale nucleus
Third ventricle
Optic chiasm
::"'--':--': ...JL Intundibulum
Inlerior carotid arlery
Agure 1-5. Coronal m:lgnelic reson,lnce imaging seCtion through the alnygd"li., UPIIC chi:bl1l.1, m(un,lIhu-
1ll1'l1, :tnd intern:.l c:lpsule. The c:werl'!UlIS sinus encircles the turck<l ,lI'ld cont:lins the (ollowing
cranial nerl'es (eN) Ill, IV, VI. V.l, :1I'ld V2; posl/.!:mgli"l'lic sympmhet ic :mel 1110.' intern,11 c:tro1ld :mer\'.
TIlis is a Tl.weighted image.
4 Chapter 1
IV. CORONAL SECTION THROUGH THE MAMILLARY BODIES (Figures :1.6 and
1-7). The [ocalion of rhe SlfiJCtures shown in the /igurL'S should be known,
Lateral
ventfide)",,;;;;;;;:;::;')7'-
ThOd
ventricle

..
Mamillary bod".es
Fornix
caudate nucleus
pallidus
Lateral ventficle
Optic tract
InllMldibulum
Hippocampus
Agure 1-6. Coroml] SCClioll of Ihe brain <It the ]nd of the m:ullill:lry lxxlics. and hippocmnp:l]
(oml:lli(m. NOte lh;ll the intem:l] c:lpsule li ..-s belwecn tht, thalamus :lll<1 the IClUiform nucleus.
Corpus callosum
CaUdate nucleus
Putamen
Globus pallidus
Hippoeampl.ls
Crus cerebri
Thalamus
Internal capsule
Substantia nigra
Interpeduncular Iossa
Base of pons
,..::..c:E.__-,,-,\- Pyramid 01 medulla
Agure 1-7. Qmll\al m:II,'11elic fC>,()ll;lllce imaging SCClion of lhe hrain <lnd hmin Slem:11 lhe le\'c1 of the lhal-
amus, ,Ind IUPPoc:lInp;.11 formation. NOll' lhal lhe poslcrior limb of the imern:ll cal)Su]e lics lX:lwl-en Ihe lhala-
mus :m,1 the lcllo;oon Illlcl..u, (put:llnen anJ globus pallidus). This is a TI-wcighlOO posrcontrast hnagc.
Cross-Sectional Anatomy of the Brain 5
v. AXIAL IMAGE THROUGH THE THALAMUS AND INTERNAL CAPSULE
(Figures 1..s and 1-9). The location of the structmcs shown in ,he tigurN should be
known.
Internal capsule
(genu)
Internal capsule
(anleriot
Lalllflll ventricle
(lrigone)
Inferior coIlicuLrs
VISUal
Pineal gland
capsule
{posterior limb}
n
Caudate nucleus (tail)
Putamen
Globus pallidus

Third ventricle
Corpus callosum
(splenium)
Agure 1..s. Axial ,;tttKlfl of the hr.tin at the le\'c1 of tile IIllemal capsule and nasal ganglia. Noll,- th:llthc
intem..1G1psule has an "melior luub. a ..enu. and a posterior limb. NOll' al;;o that the corpus Clillosum scc;-
tioneJ through the genu and
laleral Yefltricle
Septum peuudicum __
and

Globus pallidus _
Insula
Exlernal capsule
Velum inlerpos;tum
sagiltal sinus-----'
.--- Inlernal capsule (genu)
Inlernal capsule
(posteriof limb)
Thalamus and third
ventricle
T.......
Co<po, """'"'"
(splerWum)
VISUal rlMialions
cortex
Agure 1-9. Axi:11 rcson..nce im..ging ,;tttion at Ihc le\'el of the internal G1psule and basal ganglia.
Note Ihal the caudale nucleus hull,,0e5 IIlIO,he fromal homof the l:lIcml \"emricle. In lhere
is a massiH' loss of -y-:nmnObutyric :}(iJ (GABA)ergic neurons in the caudatc nucleus that results in hydro-
ceph'::llus ex ,acoo. A lesion of the I"ocnu of the imamal capsule results in :1 commlateral wC:lk lower f:u;e with
sp.::lring of the upper face. This IS:I TI.wdghled image.
6 Chapter 1
VI. AXIAL IMAGE THROUGH THE MIDBRAIN, MAMILLARY BODIES, AND OP
TIC TRACT (Agures 1.-1.0, 1.-:1.1., 1.-1.2, and 1.-1.3). The ]oc-;Jrioll of rhe 5trucrurl'S
5hown in the figure!> !>hOllIJ be known,
Madarybody
'-"J """"""
eN III
Figure 1-10. AXial SCC{I,m of the N:lln ;II the k\"Od ulth.., 1l1lJN:lln, 1\\;ulliII:u)' hoJlc:., ,Ill.! ;lllIn......II.I. NUIe
Ih,1I dlC ..ub.clIlua llIgT;1 ,..,,,.IT;lll'!> rhl' crus c..,r..,bn fr<,lm rhl' rhe lIuJbr.lin.
C,I.t5 cerebti
Substantla nigra
- Postariot Cllrebtal arlery
0uadfigemlrIal cist&rn
Cerebella< _rnos
Figure 111.. Axi;l[ magnetic resonance imaging (MRI) Sl'Crion :1{ fhe 1e\1:!1 of (he midbnin and nlamilla!)'
boJil$. lkauS<' of (he high iron OOIllCIll, Ihe red nuclei, mamilla!)' boJIl$, aoo suhsrantia nign s1ww a r<'Juced
MRI signal in n.weighll..J imaj,.'CS. Flowing blood in the cc....bnll vessels stands OUt as a mid. Cer...
brospinal fluid proJuce!i a srrong signal in thl' \"l'ntric\cs aoo cisterns.
Cross-Sectional AIlatomy of the Btain 7
Optic nerve
Optic chiasm
Optic tracl
Mamillary bodies
Superior sagittal sinus-
---
_ ""'ygtlata
Infundibulum
Lalll'fal ventricle
(ocapital hom)
Figure 1-12. Axial magnetic rCS0llanc<' imaging secrio.:m Ilr rhe k".;:1 of the opric chiasm, mamillary Ixxli<.'S,
and midbrain. This p.1\icllI has type I and :Ul opric ner"e gHotnll. NOh: the sb: of the right OJ>
lie ncr"... The inflilldibulum is poslfix<.od. This is a TI-wdl:htcd imal:".
--
Infundibvlum
- Sphtnood sinus
- Uneul
CNt cerebri
Figure 1-13, Axi;ll tn;lgllel ic rcson;lnce im;lging seCl iun at the level of Ihe IIllc;11 incisure, oculornowr Ilcr\'c.
and inferior colliculus. b there pmhology within the orhh!
8
2
Meninges, Ventricles,
and Cerebrospinal Fluid
I. MENINGES an' thl"C'(" connective membranes thaI "urruund spinal CllrJ and
bmin.
A. n,e of rhe pia mater, arachnoid, and dura malcr.
1. The pia mater is a delicate, highly vascular !:lyer of (tlnnective riSSl.IC. It c1o:,(ly
covers the 'urt:lCC 'If rhe brain and spinal ('lrd.
2. The arachnoid is a Jclicat(. nOll\':l$Cuhu CQnll{'Cli\"c tis:.ul' lIlt.:mhr:m\.', It is located
hCl\wcn rhe Jura mater and the pi;l m:ller.
3. The dUT;1 matl'T is the oUler layer of mcninl:.'cs, It consbts 1)( J"I\s..' connl'Cti"o.:" tis-
:.lIC.
B. Mcningial spaces
1. TIle sub.uachnoid space (Figure 2-1) lies octween rhe pia ll\:lt'r ;mJ the :mlch-
noid. It terminates at the Ic\'cl of the S\.'conJ s;lcral \'ertebra. II contains the ccrc-
br<J.Spinal fluid (CSF).
2. SUbdUr..l[ SP:lCC
a. In rhl' cranium, thl' sulxlur;11 spnce is rrnverseJ by "bridging" veins.
b. In thl: spinal cord, it is n clinic;ll1y insignificnnt potcntial space.
3. Epidural space
a. Thl: cranial epidural SpiKe is ,1 potential Sp:lCC. l[ conrains rhl: IIlcllingl'al ;JT-
('crics :mJ veins.
b. The spinal cpidur.. l SPilCC contnins f;lrry areolnr and vcnUllS
1,lc",uscs. The cpidur;11 space m"y be injecred with:J l\)l:nl anesthetic 10 pro-
duce;) pnrnvcrrebr:11 ("s:1ddle") nerve block.
C. Meningial tumors
1. Meningiomas arc benign, well-circulllscribed, slow-growing rumors. They account
for 15% of primary intracraninl rumors nnd arc llIore COllllllon in wOlllen in
men (3:2). Ninety percent of mcningiol1lns arc suprarellturial.
2. Subdural and cpidur;11 hematomas
a. Subdural hematoma is caused by bccrarion of the superior cl'Rbral (bridging)
\'eins.
b. Epidural hematoma is c"llSC\1 by bcctarion of the rniddk meningeal artery.
D. Tmuma
E. Meningitis is inllammmion of the pia-arachnoid area of the bmin. tile spinal cord, or
both.
Superior silglttal S,"US

fot,men 101 MOr'IIol
Thlld venltlCle
C'Slern
CereD,a' ,)queducl
Ponl,ne CISU!'In
Pia maier
ArachnOId
Dura male,
Central tanal
Meninges. Ventricles. and Cerebrospinal Fluid 9
ArachnOId granulallon
Velum inle'pcKllum
Glealce,ebl"l
Superio, cislern
SH"ghl s,"us '"
lenlorlYm
Confluence 01 ehe
s,"uses
fou,lh ventrICle
flNINl ClSlern
Suba,achnold spate
Sutldurtl sp,ce
_ Epldur,1 space
Spinal c,slern
Conus medullMls
FIlum lermlnale
FIgure 2-1. The "ulmrachnoid ,IllJ cisterns ,,( thc br.lin ,lIld cord. Cerd'r,,,pirml tlmd " pn>.
duced in the ch, ,mid ,,(! he \'cntriclcs. II exits the fourth vcntrlcle, cireulml'" lt1 the "Uh.lfilchll(lld "pace,
,md enters superior sagiu,11 through the arachnoid granllhniollS. No!e Ih,,\ Ihe conll. ler-
mimucs at L1. Thc lutllh;1I cistl:Tn ends at 5-2. (Rel'rimed with (rum Noh,lek CR. SIf<lllllllga NL.
Dl:nwrl:st It The HUllum Nl'1I'OIIS SySlelll, 41h c<1. 11,ltinlllrl:. WilIi,lms & W,lkin", 1991,]'. 68.)
1. Bacterilll meningitis is charaeteri:eJ clinically by fever. headache, nuchal rigiJery.
and Kernig's sign. (With the parient supine. the examinl'r Ocxes the p:ltiCOl'" hip,
bm cannot extend the knee without causing pain. It b a :.ign of meningeal iTrim-
lion.) [Remember: Kernig = knee. I More lhan 70% of c.be" occur in chilJren
younger than 5 years of age. TIle disease lIlay cauS!..' cranial nerve pal:.il's anJ hy-
drocephalus.
10 Chapter 2
a. Common causes
(1) In newborns, bacleri<ll meningiris is most frequently caused by Group B
streptococci (Slre/JIOCOCCUS agalacfiae) anJ Escherichia coli.
(2) In older infants and young children, it is most frequently C:lUSl...1 by
HacmOfmillt:> influenzac.
(3) In young adults. it is most frequently c:lused by NeisSl,.'Tia mcningifidis.
(4) In older adults, it is most frequently c:lused by Sm"Jrococcl4s pllellllloniae.
b. CSF findings
(1) Numerous polymorphollucle:lr leukocytes
(2) DecreascJ glucose Icvels
(3) Increased protein lewis
2. Viral mcningitis is also known as aseptic meningitis. It is characreri:\.-d clinically
by fC\'cr, headache, nuchal rigidity, and Kemig's sign.
a. Common causes. Many viruscs arc associ:ltl..J with viml meningitis, includ-
ing mumps. echovirus. Clxs;Jckie virus, Epstcin-B:lrr \'irus, anJ herpes sim-
plcx tnle 2.
b, CSF findings
(1) Numerous Iymphoc},tes
(2) Norm:11 glucose le\'c1s
(3) Moderately increased protein le\'e1s
II. VENTRICULAR SYSTEM
A. The choroid plexus is a speciali:ed stmcture that projectS intO the lateral, thirJ, and
founh "cntrick's of the brain. It consists of infoldings of blood \'essels of the pia matcr
IIl:l! afe coverl-J by modified ciliatcJ ependymal cells. It sccrete$the CSF. TIght junc-
tillns of the choroi,1 plexus cells fonn the blood-CSF barrier.
8, Ventricles contilin CSF and choroid plexus.
1. The twO lateral ventricles communicate widl the thirJ \'entricle through tile in-
foramina of Monro.
2, The third ventricle is located between the medial walls of Ihe diencephalon. It
communicates with the fourth ventricle through the c('rchr.ll aqucducl.
3. The cerebral aqueduct connens the third and fourth vcnrricles. It has no choroid
plexus. Blockage of I he cerebral :lqUl,duet results in hydrocephalus.
4, The fourth ventricle communicates with rhe subarachnoid space through three
uurlcr ()ramina.
C, Hydroccphillus is dibtion of the cerebral ventricles c(luscd by blockllgc of the CSF
pathll'ays. It is characterized by excessive accumulation ofCSF in the ccrebr<11 ventri-
cles or subarachnoid space.
1. NoncoOlmunicilting hydrocephalus results from obstruct ion within the ventricles
(e.g.. congenital aqueducral stenosis).
2. Communicating hydrocephalus rcsulrs from blockage within the subarachnoid
space (e.g.. aJhesions after meningitis).
3, Normal-pressure hydrocephalus occurs when the CSF is nor absorbed by the
arnchnoid villi. It may occur .second<1r)' to posttraumatic meningeal hemorrhage.
Clinically, it is charneteri:ed by rhe triad of progressive dementia, araxic gait, and
urin:u)' incontinence. (Remember: wacky, wobbly, and wet.)
4. Hydrocephalus ex vacuo results from a loss o( cells in the caudate nucleus (e.g.,
Huntington's disease).
5. Pseudotumor cerehri (benign inrmcmnial hypenension) r..'Suits (rom increased
Meninges, Ventricles, and Cerebrospinal Auid 11
.................... . -
resistance to CSF outflow at tho:: arilchnoiJ villi. It occurs in obese young womcn
and is charaeteri:ed by papillcJema without mass, elevated CSF pressure, and de-
teriorating vision. The ventricles may be slit-like.
Ill. CEREBROSPINAL FLUID is a colorless acellular fluid. It (Jows through the ventricles
and into the subarachnoid space.
A. Function
1. CSF supports the central nervous syslCm (CNS) and proreets it against con-
cussive injury.
2. It transports hormones and hormone-releasing faclOrs.
3. h removes metabolic waste products through absorption.
B. Formation and absorption. CSF is fonm.'d by the choroid plexus. Absorption is pri-
marily through the arachnoid villi inlO the superior 5..'lgittal sinus.
C. The composition of CSF is clinically rele\'ant (1:1.ble 2-\).
1. The normal numocr of mononuclear cells is less than 5hd.
2. Red blood cells in the CSF indicate sub.1.rachnoid hemorrhage (e.g., caused by
trauma or a rup[Ured berry aneurysm).
3. CSF glucose levels are normally 50 to 75 mg/ell (66% of the blood glucose level).
Glucose levels are normal in patients wilh viral meningitis and decreased in pa-
tients with bacterinl meningitis.
4. Total protein levels arc normally octween 15 and 45 mg/dl in Ihe lumbar cistern.
PrOlcin levels are increased in patients with b..1.Ct"erial meningitis and normal or
slightly increased in patienrs with \'iral meningitis.
5. Normal CSF pressure in the l:ncral recumbent position r.mges from 80 to 180 mm
H
2
0. Brain tumors and meningitis elcv;lIc CSF pressure.
Table 21
Cerebrospinal Fluid Profiles in Subarachnoid Hemorrhage, B....cterial Meningitis, and Viral Encephalitis
Cerebrospinal Subarachnoid Bacterial Viral
Fluid Normal Hemorrhage Meningitis Encephalitis
Color Clear Bloody Cloudy Clear. cloudy
Cell count/mm
3
< 5 lymphocytes Red blood cells > 1000 poly 25-500 lymphocytes
present morphonuclear
leukocytes
Protein < 45 mg/dl Normal to slightly Elevated> 100 Slightly elevated
elevated mgjdl < 100 mg/d\
Glucose - 66% > 45 mgjdl Normal Reduced Normal
of blood (80-
120 mgjdl)
Cell counlS in infants < 10 cells/mm
3
; protein in infants - 20-170 mgjdl.
IV. HERNIATION (Figures 22, 2-3. 2-4, 2.5, and 2-6)
A. Transtenlorial (uncal) herniation is protrusion of the brain through the tentorial in-
cisure.
B. T.... ,lnsforaminal (tonsillar) herniation is protrusion of the bmin stem and cerebellum
through the fommen magnum.
C. Subfalcial herniation is herniation below the falx ccrebri.
12 Chapter 2
Figure 2-2. Coronal secrion of:l 1\11110r in compartment. (/) Anterior cerebral artery; (2)
subfalcial herniation; (J) shifting of \'Cntridcs; (4} posterior rebral artery (compression r<:sul" in contralateral
hemianopia); (5) uncal (translcl1!orial) herniation; (6) Kernohan's notch, Witll damaged corticospinal and oor-
tioobuloor fibers; (7) tentorium cerebelli; (8J pyramidal cells that gi\'C rise to the corticospinal rrae(; (9) tonsil-
lar (tr3osforaminal) herniarion, which damab'CS \'it:al medullary centers. (Adapted with permission from L.."<.'('h
RW, Shuman RM: Nn-ropalholDgy. New York.. Harper & Row, 1982, p. 16.)
o
\)/ '
,
,
\--,
7
Figure 2-3. Axial sed:ion through (he midbrain and (he hernialing parahippocamp31 b'Yru$. The left oculo-
motor nen-e is being stTetched (dilaled pupil). The left posterior cerebral arteT"j' is compressed, resulting in a con-
(l1lbteml hemianopia. right crus cerebri is damaged (Kernohan's nOlch) by Ihe lTec edb"C of the tentorial in-
cisure, resulting in a oon(r:l.1ateI1l1 hemiparesi$. Kernohan'$ nO{ch results in a fube locali:ing sign. The caudal
dispbcement of the brain stem c:Juses rupture of Ihe paramedian arteries of the basilar artery. into
the midbrain and rostral pontine is usually fuml (Durel hemorrhages). The posterior cerebral arteries
lie superior to the oculomotor nenl:S. (I) Parnhippocampal b'YTUS; (2J crus rebri; OJ posterior cerebral artery;
(4J optie (5) optic chiasma; oculomotor nen",; (7J free edge of tentorium; (8J Kernohan'5 notch. (Adapted
wilh pemlission from Leech RW, Shuman RM: Newropaflwloo. Nl'W York., Harper & Row, 1982, p. 19.)
Meninges, Ventricles, and Cerebrospinal Fluid 13
..................................................... . .
B
A
c
D
E
Figure 2-4. Magncrk re,on:mc(' Imaging scan showing br:lin Imum3. (A) Inlcn\31 capsule; (8) sulxlurol
helllalOllla; (C) sulxlur.ll hclllafOlIIa; (D) thalamus; (E) epiJur.ll helll:1I01ll:1. Eplduml hem:llomas may cross Jural
alIachm('nts. Sulxluml hClllafOmas do IlOl cross dum! anachment>.. TIle hypennlcnsc signals arc caused by
lIIclhemoglobin. Thi;; i ~ a TI'"'ciglll<xl i l l l : l b ~ '
A
B
c
D
Figure 2-5. Compuu:J roolUgr.lphy scan axial section showing lill imr.lparenchymal helnorrh.'1&'C in Ihe left
frOflt;llloh:. (A)lmmp".Ir('nch)n'l:ll helliorrha!.'C; (8) bter.11 \'entriclc; (Cl imcrnal capsule; (D) calcilied glomus
in rhe rngone region of Ihe larcr;ll ventricle.
14 Chapter 2
A
B
c
D
Figure 2-6. IOmograph)' axinl seclion lin cpidul1ll hCl1lillnma ,Ind ,I fmClure. (A)
Epidural hemalOnm; (IJ) fmc(lITc; (C) calcifie<1 pineal gland; (D) calciti('{1 in lhc lrIl,'unc r('gion of
lhe 1.'lel111 venlricle. epidural helll,lt,lma is ,I c1a""ic hicOllVCX, or lentiform.
3
Blood Supply
I. THE SPINAL CORD AND LOWER BRAIN STEM arc ;)upplied with bloOO through
the anterior spinal arrery (Figure 3-').
A. The anterior spinal art.:!)' supplies Ihe anh:rior two-thirds of the spinal cord.
B. In dlC nn.-dulla, th... amcriof spinal artery I>upplic:. the pyramid. medial lemniscus, and
fOOl fibers of cranial neryc (eN) XII.
carotid .
Medial striate a.--------,,.......
Ant. spinal 8
AnI. communicating 8. ----_,'
Vertebral ..--------\I
Post. Inf. cerebellar
communicating a.

III
Sup. cerebellar a. Post. cerebral a.
Basilar 8. N V
Transversa pontine 88.---<'/.,.,;:;........:'
eN VU-=======
eN VIIl-

Ant. lnl. cerebellar B-----.
Middle cerebral .-_.....
Lat. slriate
An\. chOroidal a.
ArW.. oerebfal .--------1
Figure 3-1. Arteric;s of the ba.-.e of [he hrain :tnJ hr;lin ;:,tCIII, including die arterial circle of Willis.
"
.1.6 Chapter 3
II. THE INTERNAL CAROTID SYSTEM (sec Figure 3-1) consiSl5 of Ihe in[crnal carotid
artery anJ its branches.
A. The ophthalmic artery emers the orbil wilh Ihe oplic nerve (CN 11). The cenlral
ancry of thc retina isa hranch of the ophthalmic arrery. Occlusion results in blindness.
B. The pos[crior communicating artcry irrigates the hypothalamus anJ \'emral thab-
mus. An aneurysm of this artery is Ihe second most common aneurysm of Ihe circle
of Willis. II commonly results in third-nene palsy.
C. TIle anlcrior choroidal arlery arises from the imernal carmid arrery. It is not parI of
till' circle of Willis. h pcrfuses [he lateral genicuble body, globus p.111idus. anJ poste-
rior limb of Ihe internal capsule.
D. TIle anlerior cerebral artery (Figure 3-2) supplies the medial surface of rhe hemi-
sphere from the frontal pole to [he parieto-occipital sulcus.
1. The anterior cerebral arrery irrig:Hes the paracentral lobule, which conrains the
leg-fOOl area of the mawr and sensory cortices.
2. The anterior communicaling artery connects the two anterior ccrebml aneries.
It is the !1l11st common site of aneurysm of rhe circle of Willis, which lila)' cause
bitcmporallower quadrantanopia.
3. The medial striate arteries (see Figure 3-1) arc thl' pcnNra[ing arll'ries of rhe an-
terior cerebral anl'I)'. They supply the antl'rior portion of the putamen and cau-
Jale nucleus and the ameroinferior p::lrl of Ihe imemal capsule.
E. The middle ccrebml artery (sec Figure 3-2)
1. This anel)' supplies Ihe laleral comexity of the hemisphere, including:
a. Broca's and \Vernicke's speech areas
b. The face anJ aml areas of Ihe moror and sensor)' coniccs
c. The frontal eye field
A

___IAnterior cerebral ar1ery


Agute 3-2, ConiCl.lll:rTItOfIl':i of dlC cercbr:tl ancrics. (A) uueml aspt'Cl of Ihe hemisphere. Mosl of
dll.' I.lll'F.I[ convexil\' is "up'phcJ by the nuJdlc ccrct>m.1 :lnefY. (B) Mt.Jial lUll.! in(cfior aspeclS of till.' IlI.'Illl:irl'll:fe.
TIle (IIltcnoc cere"r:11 anel)' sul'pho lhe lll<.-Jial :.\.uface 1,( Ihe henlbllhefe from Ihe hllnin.'l lennin:llb 10 lhe
CII11CU". TIll.' pOSlcnlK cercbF.II :1II(1)' <uppllC.'$ Ihe ,isual cuncx and lhe fI05lerior inie-rior surface of the le-mpo-
mll.)j'l('. (Modifi ..-J (rolll Tondur)'. as prcs.:mcd in Sobon:. J: Alias dt-r Allmami... des M...nsdll"lI. Munich, Urban &
1%1, PI'. 1)]-1 l8.)
Blood Supply 17
2. The laternl striate arteries (Figure 3-3) are the penetrating brnnches of me mid-
dle cerebral flrtery. They are the of stroke. and they supply the internal
capsule. caudate nucleus, putamen, and globus paltidus.
III. THE VERTIBROBASILAR SYSTEM (see Fil,oure 3-I)
A. The \ertebral artery is a branch of the subclavian anery. It givcs rise to the anterior
spinal artery (sec I) and the posterior inferior cerebellar artery (PICA), which sup-
plilS thc dorsolatcrnl quadrnnt of the medulla. Thisquadrnnt includes the nucleus am-
bii;uUS (eN IX, X. :md XI) and the inferiur surface of the cerebellum.
B. The basilar artery is formed by the twO V<.'rtebral arteries. It gives rise to the follow-
ing arteries.
1. The paramedian branches of the pontine arteries supply the base of the pons,
which inciuJes the corticospinnl fibers :lOd the exiting rOOt fibers of the abducent
nerve (eN VI).
2. The labyrinthine artery arises from the basilar artery in 15% of people. It arises
from the anteriur inferior cerebelbr arrery in 85% of people.
3. The anterior inferior cerebellar artery (AICA) supplies the caudal lateral pon-
tine tegmcntum. including eN VII, the spinal trigeminal tract of eN V, and the
inferior surface of the cerebellum.
4. TI,e superior cerebellar artery supplies the dorsolateral tegmentum of the rostral
Medial.1de
__ Caudal' nud8tIs
__ Internal capsule
o
o
_"Gklbus pallklus
.'
.' ,Middle
cerebrel anery
.'
--
-- ------ Amygdale
.'
.'
._- ..
Third ventricle.
"'"
Caudate nucleus ".
Anterior cerebrallll\8l'Y
Posler\(lr cereb.alllrtery -

L...;""J
o
FIgure 3-3. Coronal..ecllon through the cerebr;ll henmpherc:ll the le\'c1 o( Ihe internal Cllpsulc: and thala-
mus Ihe major \':l>cubr tcrrilOrlCS.
18 Chapter 3
pons (i.c., rostrnl [0 the mowr nucleus of eN V). the superior ccrdX'lbr peduncle.
the superior surfl.lcC o( the cerebellulll and cereocllar nuclei, and the cochlear nuclei.
5. The posterior cerebral artery (see Figures 3-1,3-2, and ).) is connected to lhe
carotid artery through the posrerior communicaring artery. It provides the ma-
jor blood supply to the midbrain. It also supplies the thalamus. lateral and me-
dial geniculate bodies, and occipitallobc (which includes the visual cortex and
the inferior surface o( the temporallobc, including ,he hippocampal (ormation).
Occlusion o( this artery rcsults in a contralateral hemianopia with macular
sparing.
IV. THE BLOOD SUPPLY OF THE INTERNAL CAPSULE comes primarily from the lat-
eral striate arteries o( the middle cerebral artery and the anterior choroidal arrery.
V. VEINS OF THE BRAIN
A. The superior cerebral ("bridging") veins drain infO the superior sagittal sinus. L'1c-
cration rCS\lhs in a subdural hematoma.
B. l1\e great cerebral "ein of Galen drains the deep cerebral \'eins into the straight sinus.
VI. VENOUS DURAL SINUSES
A. The superior sagittal sinus receives [he bridging and through the arachnoid
\'i1Ii. the cerebrospinal fluid (CSF).
B. 111ecavernous sinus conrainsCN III, IV, V-I :lIld V-2. VI, and the
p:uhcric fibers. It also contains rhe siphon of the internal carotid artery 3-4).
VII. ANGIOGRAPHY
A. Carotid angiography, Figures 3-5A and Bshow the intern:ll carotid artery. anterior
cerebrnl artery. and middle cerebral arrery.
Superior sagittal S;r1\lS
Anlerlor cerebral arlery
Cavernous pan ollCA
Petrosal pari ol ICA
Basilar arlefY
Superior cerebral veins
- Superior saglltal sinus
Branches of MeA
Straight sinus
Coo1lluenee ollhe
$lnuses
..... Transverse Sinus
verlebral artery
Figure 3-4. Magncllc resoml1lCC angiogr..llll. latcral projcction. showing (he mHJor \'cnous sinuses and aner-
ics. N()(c thc bridging \'cins entering the supcriOl'" sagirral sinus. leA = imcm:ll carouJ fJrlCry: MeA = middle:
cercbral artcl')'; PeA = poslcrior ccrebral arlel')'.
Blood Supply 19
B. Vertebral angiography. Figures 3-5C and IJ show the \'erlebral arte!)'. PICA and
AICA, b.'lsilar artery. superior cerebellar artery, and JX'Slerior cerebral anery.
C. Veins and dural sinuses. Fib'Ure 3-6 shows Ihe intcrnal cerebral \cin. surcriorc.... rebral
veins, great cerebral \'ein. superior ophthalmic vein. and major duml sinuses.
D. Digital subtraction angiography. See Figures 3-7. 3-8. 3-9. and 3-10.
VIII. THE MIDDLE MENINGEAL ARTERY. a branch of the maxillaI')' artery. enters the cra-
nium through the foramen spinosum. It supplicsmos[ ofthe dum. including ilSc.'lI\'arial por-
tion. Lacermion n.'SUhs in epidural hemorrhage (hematoma) (Fib'urcs 3-11 and 3-121.
Lislot structures:
o
1? 1,3
,
,
"17><'""l
'- ( 4
c
1. Anteriof cerebral artery
2, Anterior choroidal artery
3. Anteriof inferior cerebellar artery
4. Basilar artery
5. Calcarine artery (01 posterior cerebral artery)
6. Cailosomargioal artery (01 anterior cerebral artery)
7. Callosomarginal and pericallosal arteries
(of anterior cerebral artery)
a. Internal carotid artery
9. Lateral striate arteries (of middle cerebral artery)
10. Middle cerebral artery
11. Ophlhalmic artery
12. PericaJlosal artelY (of anterior cerebral artery)
13. Posterior cerebral artery
14. Posterior choroidal arteries
(01 posteior cerebral artery)
15. Posterior oommunicaling artery
16. Posterior inferior cerebellar artery
17. SUperior cerebellar artery
18. Vertebral artery
Agure 3-5. CA) Carolid Iater...1projt.'Clion. (8) ClroliJ .mgiogmm. amcropostcrior proj:tion.
(e) Vcrtcbnll hllcral proj:tion. (D) Venebrnl angiogmlll. ,lnlcroposlcrior projeclion.
20 Chapter 3
- Confluence of sinuses
- Straight sInus
Great cerebral vein (of Galen)
. Transverse sinus
---
Cavernous sinus
Inferior sagittal mus, .Intema! c:erebral vein


, SUperior sagittal sinus
Superior ophthalmic vein ,... "Superior cerebral veins (bridging veins)

Agure 3-6. QmlliJ ;1nl,;;Oj;r.un, \'enous ph:tSC. showin!; the cerchml veins ;1nd \'cnous sinu:>t:'S.
Callosomarginal artery
'" ACA
Pericallosal artery
ofACA
Frontopolar branch
of ACA
Ophthalmic artery ---
Cavernous ICA
Petrous ICA
Cervical ICA----.=....
J
__ Ml segment
""''-----='7'--PCoM
Figure 3-7. Carotid 'Illl,;io,;r.un, Iater.11 proje<:lioll. IJcntify lhe conicoll br.mchcs of Ihe .lIllcrior cercbr.11
:lncl)' (ACAl ;1nd middle ccrehr.11 ;1r!('ry (MCA). Follow Ihe coun;c of Ihe inteOl;11 QlfOlid .Inel)' (leA). Re
mcmber Ih31 ;1neurisms of Ihe poslwior communicating arrcry may result in Ihirdncf\e l"llsy. The l);lI";lcemr.11
lohule is irrigated b\' Ihe callosoll\<Irgin:lt .\rlel)'. Conical br.mchcs of the MCA arc designated with dots. 1'CoM
:< posterior artery.
Blood Supply 23.
Al segment of M;A

Cavernous part 01 ICA
COrlical bfanc:hes 04 MCA
lateral striate branches
olMCA
M1 segment 01 MCA
Supraclinold part or ICA
Petrous part ollCA
Cervical part or ICA
Agure 3-8. ClImid :mlliuur..lm, al\lertll'ustcrior Ickmify the mllerior ccrcbr..ll :UICry (ACA). mid-
dle cerebral anery (MCA). and imemal carvtid arlery (ICA). TIle huri:untal br.ll'Khes of'the MeA pcrfu.e thC'
basal and intemal capsule, ACoM = anterior cOllllllunlc,lIing anery.
Posterior choroidal
arteries
PeA, Pl segmenl-!-,
Thalamopertorating -l---
arteries
PCoM
Superior cerebellar artery
Basilar
Verlebral arlery'
'-''-c_--Parieto-ocdpitar
branches 04 PeA
Calcarine branches
or PCA
Hemispheric branches
olSCA
:...L---
PICA
Vertebrallll1ery
Agure 3-9. Vertebral angilll,'r.l1ll. laterJI Two "tnJClUrcs :lre fwnd benn.--en Ihe posterior cerebral
arlt:l)' (PeA) and Ihe :lUpcrior cerebdhlr :UI('I)" tbe telllurium ,md Ihe third cranial nerve, PeaM. - poslcriOf
comlllunicating "riel)'; PICA = posterior inferior cerebellar arl<'I)': SCA = superior cerebellar anel)',
22 Chapter 3
CaJcarine arlefY 01 PeA
/
PCA
Superior cereoellar ----
arlery \
Basilar arlery
PICA _------.tli;;
..
V
>--Tempo!'lll branches
of PCA
arlery
Figure 3-10. Vef!ehnll angiogrmn. mucroposrerior projeclion, Which anery supplies rhe visual cortex! The
C;IIClrine ,lIlery, a bnmch of the pClSlcrior cerebr.-I anery (PCA). Occlusioll of Ihe rcA (calcarine ariel)')
in ,I CUllImlateml homonymuus hemianopia, with 1Il;IeUI:lr PICA"" posterior inferior cerebellar arlcl1'.
Oufertable
Diploe
Du'"
Periosteum
(of inner fable)
Figure 3-U. An epiJuml hem:l!Oma results from laceralioll of Ihe middle ariel)'. Arterial bleed
ing imo Ihe cpidurol space forms:l bi((xwex dOl. The das.sic Mludd inrcnml" is seen in SO% of cases. Skull fr.te
are found. Epidunll hcm:lIomas r.ucly suturallmes. (Reprinted wilh pennission from Osburn
AG, Tung KA: Handbook of NellromdioiOlO: Bram and SkIIU. Sr. Louis, 1996. p. 191.)
Blood Supply 23
'1--\-- Arachnoid
Figure 3-12. A 5ubdural hematom;1 (SOH) re5ulu (rom lacer..lloo bridging vcim. SOHs are frequendy ac-
companied by t ~ l I m a t i c 5ubar..lChnoiJ hemorrhage>' and cor{ical contu5i0Il5. Sudden deceleration o( the head
cau:iC5 rearing of rhe superior cerebral \cins. The SOH clI:rend5 ovcr thc cre>'t of {he con\'ell:it\' imo the imer-
hcmi5phcric hssuw, but doc5 nor cro:\S the dllral ,1U<lchmellr o( the f.J1:: cerebri. The clot can be Cre5Cen{'5haped,
biconvCll:, or lllultilocul:ncd. SDHs arc more common rhan epidural hcmmornas. SOI1s alIVaY5 c,mse bl""<lin dam-
age. (Reprinted with permission (rom Q;;burn AG, Tong KA: Handbook of Neuroriu.liology: Brain lind Skllil. St.
Louis, Mosby, 1996, p. 192.)
4
Development of the Nervous System
I. THE NEURAL TUBE (Figure 4-1) gi\'cs rise ro the centr'dJ nenrous system (eNS) (i.e.,
brain and spinal cordI.
A. The brain stem and spinal com h:l\'c:
1. An alar plate thaI gh"cs rise to the sensory neurons
2. A basal plate that gi\'cs risc [0 the motor neurons (Figure 42)
B. The neural tube gh'cs rise (0 three primary vesicles, which dC\'clop infO five sec-
ondary vesides (Figure 4-3).
C. Alpha-fetoprotein (AFP) is foond in the amniotic fluid and maternal serum. It is an
indicator of ncurnl rube defects (c.g., spina bifida, anencephaly). AFP Ic\"cis are fe-
duct.'<i in mothers of (('tuses with Down syndrome.
Neural plate
" Notochord
e_..J
Neural crest
Surface ectoderm
Neural lube
... ~ Dorsal rool ganglion
"Q2=AJarplate (sensory)
Sulcus limilans
Basal plate (molor)
e
central canal
Figure 4-1. Developmcnt of thc neural tube and crest. The alar pl:uc glvcs rise 10 sensory neurons. The bastl
I1I31c gh'cs rise 10 mOtor ncurons. TI1C ncural crest givcs rise w the peripheral ner....ous ~ r ~ l c m .
24
Development of the Nervous System 2S
Semicircular
d"'"
t)-- Taste bud cell
of tongue
"f-
Skin
"
Ampullae
,':-':;---Cochlea
,,----Telachoroidea
'------smooth muscle
VISceral epithelium
Fourth ventricle ---,
SVA column ----\"-;"'IV
GVA column
GVE column
SVE column
GSEcolumn
Floor plate ---/'
Somatic striated muscle
(tongue)
Branchial striated muscle --------;
(larynx)
SSA nud,; '\-_
""\ Ajar plate ----en
GSA column ---It-
Pial blood vessels
Roof plate (epeodymallayer)
Figure 4-2. The bfilil\ stem showing the cell columns derived (rom the ..br .. oo basal platcs. The scven ern
ni..1nerve lnod.. litics ,Ire shown. GSA == b'Cnernl SOln,ll1c ,.fferem; GSE '" l.ocnernl SOlll,llic c((erent; GVA = bocn-
ernl \'iseeml afferent; GVE '" genefill \iscer-..I efferent; SSA '" speci:.1 som:uic ,liferent; SVA == special \'iscefi,1
"lferent; SVE '" special \'iscernl efferent. (Adapted with pcnnission (rom Panen 8M: HUllUm Embryology, Jrd
ed. Ne\\' YorK, McGraw-Hill. 1969. p. 298.)
II. THE NEURAL CREST (see Figure 4-1) gives rise tu:
A. The peripher:lI nervous system (PNS) [i.e., peripheral nerves and sensory and aurQ-
nomic ganglia]
B. The following cells:
1. Pscudounipolar ganglion cells of the spinal and cranial ner\'e ganglia
2. Schwann cells (which ehlborate the myelin sheath)
3. Multipolar ganglion cells of autonomic ganglia
4. Leplomeninges pi:'l-arachnoid) em'dop the brain and spinal corJ
5. Chromaffin cells of the suprarenal mcdulla (which elaborate epinephrine)
6. Pigment cells (meianocYlcs)
7. Odontoblasls (which elaborate
8. Aorticopulmonary septum of the heart
26 Chapler 4
Three primary
vesicles
Five secondary
vesicles
Adult derivatives of:
Walls Cavities
Lower part of
foorth ventricle
Lateral
ventricles
Upper part of
fourth ventricle
Cerebellum
Cerebral
hemispheres
Pons
\'--.
) t--Midbrain
\\---
I
---'\\--Midbrain ,
(mesencephalon)


--j-j--Hindbrain --==-,
(rhombencephalon)
Myelencephalon---1'f--
--;:r=t,cavilY / Telencephalon
-ttForebrain L-.DiencephaIon'---,H-
(prooeocepha""'1
Spinal cord
Figure 4-3. The bmin \'csiclcs indic<llinl; thc adult derivativcs o( their wall;; and cavilics. (Rcprimcd with
pcrmis;;ion (rom Moore KL: Thl> Det'ew,ling Hlmlllll: CUllicllll)' Orieurillg 4th cd. Phil:tdelphia. WB
Smmdcrs, 1988, p. 380.)
9. ParJ.follicular cells (calcitonin.producing C-cclls)
10, Skelelal anJ conncrli\'e tissue components of the pharyngeal arches
III. THE ANTERIOR NEUROPORE. The closure of the anterior neuroporc gh'cs rise to the
lamina terminalis. Failure to close results in anencephaly (i.e., failure of the brain to de-
velop).
IV. THE POSTERIOR NEUROPORE. Failure to close resuh's in spina binda (Figure 4-4).
V, MICROGLIA arise from the monocyles.
Dura
Spinal cord
Arachnoid
Transverse
'''''''''
Subarachnoid
'PO'"
[),,,.
Neural tissue

A. Spina blfld. occulta 8. Meningocele C. Menlngomvelocele D. Rachischisis


Agure 4-4. TIle I)'pes of spin.l hi6Ja. (RCjlrmtl-J wilh pcnnis:.ion (rom S;".Ilcr TW: UnlRJll(llt'S Med-
iCIII Embryology. 6th ed. &lrllUurC, Wilh;lllt'> & Wilklll., 1990, p. 363.)
Development of the Nervous System 27
VI. MYELINATION begins in the founh month of gestation. Myelination of the corti-
cospinal tTOlCts i,. not complcteJ until the ... nd 'If th... ,..,;cond IXhmatal year. when the tntel:>
become functional. Mydination in th... cercbr.tl a:>:>ocimion conex continues into the IhirJ
decade.
A. Myelination of the eNS i,. accompli,.he.J b\' olij:,'odendrocylcs, which are nO[ found
in lhe rdina.
B. Mydination of the PNS b accolllplishL-d by Schwalm cells.
VII. POSITIONAL CHANGES OF THE SPINAL CORD
A. In the newborn. the conu,. IllcJullaris end,. at the third lumbar vertebra (LJ).
B. In rhe adult, Ihe conus medullaris ends lit L-I.
VIII. THE OPTIC NERVE AND CHIASMA arc Jcriwd from the diencephalon. TIle optic
nerve occupy the choroid fissure. Fai!llre of this fissure to resultS in coloboma
iridis.
IX. THE HYPOPHYSIS (pituitary gland) is d<:rived from twO embryologic subSU':lla (Fig-
ures 4-5 lind 4-6).
A. Adenohypophysis is derived from an eChxh.:rlllal dh'crticulum of th" primiri\'" mouth
caviry (stomodeum). which is also calkd Rllthke's pouch. Remnanrs of Rathke's
jX)uch may give rise to a congenital qsric tumor, a cr.miopharyngioma.
B. Neurohypophysis develops from a ventral evagination of the hypothalamus (neu-
roectodeml of the neural tube).
X. CONGENITAL MALFORMATIONS OF THE CNS
A. Anencephaly (meroanencephaly) results from failure of the anterior neuropore to
close. As a result. the brain does not JL'velop. The frequency of this condition is
101000.
Third ventricle
Sphenoid bone
Pars intermedia
of anterior lobe
Neurohypophysis
(posterior lobe)

Infundibulum of hypothalamus
Diaphragma sellae
Optic chiasm
Adenohypophysis
(anterior lobe) --Ii<\ofu,+<
Pars tuberalis
of adenohypophysis -t----'iIl
Craniopharyngeal canal Remnant of Rathke's pouch
Figure 4-5. Midsagill:ll..eclion through the hypophrM" and ..ella 1lle IllCloolllg
,hc p:l!"S rubcr:aHs and p:111i UltCn1lt..J1J. lli Jenvt-d (flllil R:ldlke's pouch (oruecuxlenn). The
an.)CS frum the mfunJibulum of the hypodl:llamu:. (nCUflJl."'<:fwcnn).
28 Chapter 4
Figure 4-6. 'CCli,'n through til(' brain 3u.'"m and dIencephalon. A cr.lllioph.lTyngiom:l (llJT()(.t'S)
IIe3 3Ul'mSCIl,lr III the nllJlllll". It cOlnpn:::.scs the optic chiasm :mJ hnltJlh:lbmus. Tlus tumor b the most com-
mun SUI'T:uclll"naltumur Ihm ,JCcurs in childhooJ and the most CnmmQfl olhYI'OI'iWit;ITism in chilJren.
b:l TI-wclghll.'d m.lj;netlc rCotlfl,lllCe im.ll,'illg sc:m.
' .
, ,
, .'
.
,
2 ,
,
.....
B
"
.
"
..
FOfsmen magnum
, ,
,',
A
.
"
,
"
'.
.,
.. '

"'
Figure 4-7. Arn"ld-Oll,lri m,lllonn,1I inn. Mid'>:Igimll secli"n. (A) Noon;ll cerehelium, founh n:mride, :mJ
1'r;lln 31CIll. (B) Ahn"rm:l] cerehcllum, i"'unh vClllridc, and 11min 31CI1I thc common COllb-cllll<l1 :morn-
alll"; (I) hcakmg "f the tl'CI.,1 p1.ue, (2) :ll/Uloduct:ll slcno>is, (3) kinking :mtllr.msftlramlll.ll hemi;uion of th..
l1wJutI,llIIltllhe \'endmll canal. anJ (4) lK'mi,t(ion anti unrol1mg of Ihc cerehclbr inlt) the \'cncbrnl
C:ln;l1. An :lCCOmpan)'lIlg meningomyelocele i3 common. (Rcl'nnwJ wllh rcnni""ion from Fix jI} BRS
Neuroanmomy. J\;lltil))nn, Willi,lIn.> & Wilkins. 1996, p. n.)
Corpus callosum
Lateral ventricle
Massa intermedia
Third ventricle --"'1!
..
Pons --"8>0\+-
Development of the NelVOus System 29
Polymicrogyria
Superior saginal sinus
Straigtlt sinus
c---Confluence 01
sinuses
f---------Jift- Cerebellar vermis
---'--;0,.'--- Posterior fossa cyst
Figure 4-8. I}.mdy- W"lkcr 1Il;llform:ltion. Midsagill:11 so.:ction. An CI'lOrt\lOl.lS dil"l[ion of the (ourth vemricle
n..'SUh:s (rom ("ilure of I!)t." for::nnm:l of Lu.chb lInd to open. This (ondilion is associated ..... lth ()((ipi-
ml menin!,'ocelt, de\'adon of the confluence of tile sinu:>cs (IOreu!:lr Hemphili). agenesis of the cerebellar \'er-
mis, and splenium o( the corpus cllllosom. (Reprillloo pcnnilorl (rom Dudek RW, Fix JQ BRS Embryology.
&lhillwre, Williams & Wilkins. 199i, p. 9i.)
B. Spina biflda resulu from failure of the posterior neuropore to fom\. The defect usually
occurs in the sacrolumbar region. The frcquenC)' of spina bifida occulra is 10%.
C. Cranium bifidum results from a defect in the occipital bone through which meninges.
cerebellar tissure, and the fourth ventricle may herniate.
D, ArnoldChiari m:llformation (type 2) has a frequency of I: 1000 (Figure 4 7).
E. Dandy. Walker lIlalfOrln:ltion has il frequency of I :25000. It may result from riboflavin
inhibitOrs, posrcrior fossa trauma. or viral in(ection (Figure 4-8).
F. Hydrocephalus is most commonly caU:K'(1 by srenosis of rhe cerebral aqueduct during
dc\"(lopmcnr. Excessiw cerebrospinal nuid Olccurnubtes in rhe ventricles and sub-
arachnoiJ space, This condilion Illay result from malernal infection (cytomegalovirus
and lOxoplaslllosis). The frequency is I: I(X)().
G. Feral alcohol syndrome is the most COllllnon cause of mental retardalion.1t includes micro-
and conl,oeniml hcan: diset;c; hoioplOiCnccphaly is [he Ill()';[ se....ere m:mifestation.
H. Holoprosencephaly resuhs from f<lilurc of midline cleavage of lhe embyonic forebrain.
Tl,e cOnl<lins a singular \'cntricular caviry; is seen is trisomy 13 (Palau
syndrome); the corpus c:lllosum may be absent; holoprosencephal)' is rhe most severe
m<lnifcsmrion of lhe feral <llcohol s)"ndrome.
I. Hydranencephaly rcsulufrom bilateral hemispheric infarclionsecondary lOocclusion
o( lhe carQ{id arterieS. TllC hemispheres arc replaced by hugely dilated vennicles.
5
Neurohistology
I. NEURONS arc classified by the number o( processes (Fi1.'Urc 5-1).
A. neurons are IOGHcd in the spinal dors.,1 rom ..angli;1 anJ sensory gan-
glia of cr.lOial nc.....cs (C ) Y, VII. IX, and X.
Nissl substance
Schwann cell
Motor neuron
Nerve endings in muscle (myoneural junction)
Myelin sheath
Collateral branches
Auditory
Sensory (receptor) neurons
Olfactory
T
Telaxon
"
Synaptic endings (boulons terminaux)
Nod.
01 Aanvier
Axonorigin _
Dendrite zone
Figure TI'I:lCl> o( nerve cdb. OIt;tCl'If)' neurons :uc bipobr ;lnd unmyc1in:u('(1. Auditory neurons ;uc hipo-
\:IT :lnd rnyclin:lIl-d. ll...,rs;.l TIl()( gangllOll cdb (cUran('olls) arc plicudounirohu and mrclimucd. MO{()T neurons
:IT(' multipular and mrdinalC<1. AlTOU's inJicatc input Through the ;I)(ons of mhcr IlCurollS. Nc.....c cells arc Ch;lf-
oclcri:cd b).he prc..encc oiNIS>.1 Sllhslflnce :md rough endoillflsmic rclicuhnn. (/I.loc!ilied with pcnnission irom
C'lrpeiller MB. 5U1in): Illllnlm NClfTOOlllIIOlII)". Baltunore. Wllli;uns & 1983, p. 92.)
3.
Neurohistology 31
B. Bipolar neurons found in Ihe cochlear anJ \'estibular &oanglia ofCN VIII. in the
olfactory ner\'e (C I). and in the retina.
C. Multipolar neurons an' the Iarg\.'S1 populalion ofnerve cells in the nervoussysu.m. This
gruup includes motor neurons. neurons of the auronomic nenOlIs SySICIIl, intem<.'UfOns,
p)'ramidal cells uf the cercbml conex, and Purkinje's cdls of the cerebellar concx.
D. 11ll"re arc approximmcly 1011 neurons in Ih(' bmin anJ approximmely lOll) nt"urons in
Iht" n('oconcx.
II. NISSl SUBSTANCE is characleristic uf neurons. It cunsistsof rosenes of polysomcs and
rough n::liculum; therdore, il has;l rule in prolcin synlhesis. Nissl subslance
isfounJ in the nerve cdl body (perikaryon) ami dendrites, nor in Ihe axon hillock 1)1' axon.
III. AXONAL TRANSPORT lIleJiates rill' illlracelllllar Jisrribmion of secrelury proteins. or-
g:mellcs, :'lnJ cyruskdef:ll <"lemcnts. II is inhibiled by culchicine, which depolYllll:ri:es mi-
crorubules.
A. Fasl anterograde axonal transport is respl lIlsihle for transporring :111 newly synt hesizeJ
membranous organelles (vesicles) and preCllrSllrs (>f ncuro[r:lllsmitters. This process
OCCurS:l the fate of 200 [0 400 lIlm/d:'ly. II' mediall,J by neurotubules and kincsin.
(Fast rr:lllSrX11't is neurorubule-dep<.'ndent.)
B. Slow :llllerograde tr-dnsporl is responsible for lr:msl'0ning fibrilbr C\,toskdef:J1 and
protoplasmic clemenls. This pnx{'ss occurs at Ihe rate of I 105 mm/day.
C. Fast relrogr-.lde lrdnsporl returns used materials from the axon (enninal ro the cell
for Jegnkl:lI ion anJ recrcling:Jl :1 nlte of 100 to 200 mm/da)'. It It:lllspons nerve
growth factor. neurotropic viruses, and toxins, such as herpes simplex, rabies. p0-
liovirus, and telanus toxin. It is mcJi:ucJ by neurorubules and dynein.
IV. WALLERIAN DEGENERATION is amerogmde degenl:ration characleri:cJ by th.... dis-
appearance ofaxons and myelin sheaths anJ the secondary prolifer:llion ofSchwann cells.
IT occurs in Ihe central nervous syslem (CNS) and the peripheral nervOllS system (PNS).
V. CHROMATOLYSIS is the result of retrograde degellet::ltion in the neurons of lhe eNS
and PNS. There is a luss ofNissl substance after axolOlIly.
VI. REGENERATION OF NERVE CELLS
A. eNS. Effective reg..::n..::r:ttiun Joes not uccur in the CNS. For ex:nnple, there is no re-
generation of th..:: optic nerve. which is:1 <)f the diencephalon. There arc no base-
ment membr:mes or endoneural investments surrounding the axons of the CNS.
B. PNS. Regcncr:uioll Joes occur in the PNS, The proximal tip of a severed axon grows
illlO the endoneuml tube. which cunsists of Schwann cell basement mcmbr:me and
endoneurium, The axon sprout grows at the r:lle of 3 nun/day,
VII. GLIAL CELLS :Ire lhl' nonneurnl cdls of (he n('rmus sySll"Ill.
A. Macroglia consist of aSlrocytes and oligodendroc}'les.
1. ASlrocyte:; perfurm the following function3:
a. 11ley prtljl.'C1 foot processes Ihal em'clup Ihe basement membmne of c:lpillar-
ics. neurons. ;md synapses.
b. Th(')' fonn the extt'rnal and intcrnal glial-limiling m{'mbranes of the CNS.
c. TIle)' playa role in the melabolism of cenain neurolransmillers le.g. 1-
aminOOutyric aciJ (GABA), scrotonin, glulmnmel.
32 Chapter 5
d. Onley buffer the potassium concenrralion of rhe extnlcellular space.
e. TIley form glial scars in damaged areas of the br.lin (i.e., asnogliosis).
f. They contain glial fibrillary acidic protein (GFAP), which ;s a marker for as-
trocytes.
g. TIley conwin glutamine synrhetase. another biochemiclll marker for astrocytes.
h. May be idemifiL-d with monoclonal antibodies (c.g., AzB;).
2. Oligodendrocytes are the myelin-forming cells of fhe eNS. One olib'Odendrocytc
can my"linate as many as 30 axons.
B. Microglia arise from monocytes and function as the sca\'cnb'Cr cells (phago.::ytcs) of
the eNS.
C. Ependymal cells ,If(' ciliated cells that line the central canal and \'entricles of rhe
brain. TIley also line the luminal surface of the choroid plexus. T h ~ cells produce
cerebrospinal fluid (CSF).
D. Tanycytes are modified ependymal cells thm contact clpillarics and neurons. TIleY meJi-
ate cellular trallSpOfT between dlC vemricles and r!lc IleulUpil. TIlC)t project to hyporhal-
amic nuclei th.1t "1,'lIlate dlC release ofgon..-mropic hormone /Tom t!lc adenohypoph)'sis.
E, Schwann cells are derh"ed from the neural crest. TIley are Ihe myelin-forming cells of
Ihe PNS. One Schwann cell can myelinale ani)' one intemooe. Schwann cells i m ~ t
all myelinaled and unmyclinated a..'<onsof the PNS and arc separated from each orher
b)' the nodes of Ran\'ier.
VIII. THE BLOOD-BRAIN BARRIER consists of rhe tight junctions of nnnfenesnared en-
dothelial cells; some authorities include rhe asuocytic foot processes. Infarction of brain
tissue dCSTroys the tight junctions of endothelial cells and rC$ulu in vasogenic edema,
which is an infiltratc of plasma into the extracellular space.
IX. THE BlOOD-CSF BARRIER consists of the right junctions between the cuboidal cp-
idlelial cells of the choroid plcxus. TIle barrier is permeable to some circulating peptidcs
(e.g., insulin) and plasma protcins (e.g., prealbumin).
x. PIGMENTS AND INCLUSIONS
A, Lipofuscin granules <lrc pigmented cytoplasmic inclusions that commonly aCClllIlU-
hnc with aging. They ,ITC considered residual bodies thar arc deth'eJ from lysosolllcs.
B. Melanin (neuromelanin) is blackish intracytoplOlsmic pigmcnt found in the substan-
tia nigra lmd locus cocruleus. It disappears from nigral neurons in patients who have
Parkinson's Jisease.
C. Lewy bodies arc neuromll inclusions that are char.lcteristic of Parkinson's disease.
D. Negri bodies are intracytoplasmic inclusions that are pathOl,'110monic of rabies. TIley are
found in the pyramidal cells of the hippocampus and rhe Purkinjc cells ofthe cerebellum.
E. Hirano bodies arc intrlillcuronal, eosinophilic, rodlike inclusions that arc found in the
hippocampus of patients with Ahheimer's disease.
F. Neurofibrillary tangles consist of intracytoplasmic degenerated ncurofilaments. They
arc seen in pmicnts with Al:heimer's disease.
G. Co\\'dr)' type A indusion bodies are intranuclear inclUSions that are found in neu-
rons and glia in herpes simplex encephalitis.
XI. THE CLASSIFICATiON OF NERVE FIBERS is shown in Table )-1.
Neurohistology 33
Table 5-1.
ClassificiUion of Nervc Fibers
Fiber
Diameter
holm) *
Conduction
Velocity
(m/sec) Function
Sensory axons
la (A-o)
Ib (A-o)
II (AP)
III (A-S)
IV (C)
Motor axons
Alpha (A-ul
Gamma {A-y}
Preganglionic autonomic fibers (BI
Postganglionic autonomic fibers (C)
Myelin sheath included if present.
12-20 70-120 Proprioception. muscle spindles
12-20 70-120 Proprioception. Golgi tendon organs
5-12 30-70 Touch. pressure. and vibration
2-5 12-30 Touch. pressure. fast pain. and
temperature
0.5-1 0.5-2 Slow pain and temperature,
unmyelinated fibers
12-20 15-120 Alpha motor neurons of ventral horn
(innervate extrafusal muscle fibers)
2-10 10-45 Gamma motor neurons of ventral horn
(innervate intrafusal muscle fibers)
<3 3-15 Myelinated preganglionic autonomic
fibers
1 2 Unmyelinated postganglionic
autonomic fibers
XII. TUMORS OF THE CNS AND PNS arc in Figure 5-2.
A. Onethird of brain tumors arc I11Clastatic, and two-thirds are primary. In metastatic
tumors, the primary site of malignancy b the lung in 35% of cases. the breast in 17%.
in the gastrointestinal U:'lCt in 6%. mdanomil in 6%. and the kidney in 5%.
B. Brain Imllors ate classified as glial (50%) or nonglial (50%).
C. According to national board questions, the five IIlUSt common brain tumors are:
1. Glioblastoma muhiforme, thl' most common and lllost (awl type
2. Meningiolllll, a benign llonitll'asive IUlllur of the falx ,mel the convexity of the
hemisphere
3. Schwannoma, a benign periphcrnlllllllur derived (rom Schwann cells
4. Ependymoma, which is found in till' velltricles and accounts for 60% ofspinal cord
gliomas
5. Medulloblastoma, which the second must common posterior fossa tumor seen
in children and melastasi:e through the CSF tmcts
XIII. CUTANEOUS RECEPTORS (Fi!,"urc 5-J) are JiviJ(od into t.....o large groups: free nerve
endings and encapsulated endings.
A. Free nerve endings nociceph>rs (p,ain) and (cold and heat).
B. Encapsul:.lh:d endings arc touch rccepwTS (Melssner's corpuscles) and pressure and vi-
br:uion rt."Ceptors (Pacinian COll>uscI6).
C. Merkel disks an: Unenl""llpsulatoo light touch receptors.
34 Chapter 5
)--"r-- GlloblaslorN1 multilotme
represer'llS 55% 01 gliomas
malignant; rapicJy !alai
astrocytic tumor
commonty in the I.ontal and
temporal lobes and ba.saI gangia
Irequently aosses the mdne Yia the
corpus caloslm (buIIerfly glioma)
fflOSf common prionary brain tumor
NsklIogy: pseudopaJisar;I
perivaso.oIar pseudoro$elles
Oligodendrogliomas
represenl 5% 01 al
!he gliomas
!7OW slowly and are
relaliYely benign
most common in !he kontallobe
caJcilicalion in 01 cases
cells look eggs' (perinu::lea.l\alos)
Meningiomas
derived lrom aracMokl cap cells and lepresenlthe second most
common primary intracranial brain lUmot after aSl.oeylomas (15%)
are not invasive: they indent the brain: may produce hyperostosis
padlology: concenlric: whor1s and calcified psammoma bodies
location: parasagittal and convexiry
gender; females> men
associated with neurolibromalOSis'2 (NF2)
Astrocytoma,
represent 2O'llo 01 the gliomas
histologically benign
lM1usety inIi1trale the hemispheric ..tIite malte<
most convnon glioma bond in !he posterior
Ependymomas tossa 01 children
A
B
Germlnomas
germ ceM tumors tha' are commonly
seen rn the pineal regron (>50%)
overlie the tectum ot \he midbrain
cause obstruclNe hydrocephalus due to
aQl,le(luclal stenosis
\he common cause of Paunaud's syndromeO_-'2f'l,=,
B,ain .bscltSRS /r-_
may resull lrom sinusilrs,
mastoiditis,
hema1ooenous spread
Iocaton: lrontal and
tempofallobes. cerebelum
organrsms: streptoeoe:ei.
staphlocooci. and
"'"""""'"
'esul in cerebral edema
and hemiallon
Colloid eyslS ollhlrd Yflltricle
comprise 2"110 oIl'llraerarnaJ gliomas
are 01 ependymal origin
Iot.Wtd at the JoraminIa
ventricular obslnJcbon resuhs I'l increased
intracranl31 p1'essd"e, and may cause
posiIionaI headaches, "drop altacb:
Of sudden tleattl
Brain stem glioma
u$l,lally a benign pilocylic: astroeyloma
usually causes cranial nerve palsies
may cause the "lodled-in" syndrome
C,anlopharynglomas
represent 3% 01 primary brain tumors
derived Irom epitheliaf 'e<TWlllIlts 01 Ra1hl<e's pouch
location: suprasellar arad inlerior to !he opOc chiasma
cause bilemporaf hemranopl8 and hypopiluilarism
calcitieation is common
Piluitary (PA)
most common lumors of the pltuilary gland
prolactinoma is !he most common (PA)
derived Irom lhe stomodeum
(Rathke's pouch)
repreS8f'l1 8% ot primary brain tumors
may cause hypopituilarism, visual
lield delects (bitemporal hemianopia
and cranial nerve palsies CNN III, IV,
VI, V, and V2, and postganglionic
sympalhetlc fibers to the dilalor
musclo of tho iris)
Schwannomas (acoustic neuromas)
consist of Schwann celfs and arise hom the
vestibular division 01 CN VIII
comprise approx, 8% 01 Intracranial neoplasms
pathology: Antoni A and B tissue and Verocay bodies
bilaleral acousllc neuromas are diagnoslic 01 NF2
gender: female> mon
Choroid plellus papillomas
histology: benign: no necrosis Of inYasiIIe leatures
represent 2% ot the gliomas
one ot \he most common brain tUmoB in patients" 2 of age
occur in decreasing Irequency: lourtto, lateral. and third ventricle
CSF owrproduclion may cause hydrocephalus
Cerebellar aslrcx:ytomas
benJgn tumors 01 childhood with good prognosis
most common pediatric: intracrarvaltumot
contain pllocylic: astroeyles and Rosenthal libers
Medulloblastom,s __
rllpfesent 7% ot primary brain tumors
repr&S6f'lt a primiliye
neuroectodermal turTlOf (PNET)
second moSI common posterior lossa
tumor in children
responsible for the posterior vermis syndrome
can metastasize via Ihe CSF tracts
highly radiosenSitive
HemangloblllSlomas
characterized by abundanl capillary blood vessels
<lI'Id foamy cells: moSt Ol1on found in the celebellum
when found in the cerebellum and retina,
may represent a part 01 the von HippelLindau syndrome
2% of primary intf8C'anial lumors: 100'. of posterior fossa
,,-
Int,asplnat tumo., Ependymomas
Schwannomas 30% represent ot the gliomas
Meningiomas histology: benign, epen<lymaltubules.
Gliomas perivascular pseodoroseltes
Sarcomas are supratentoriat: 60% are inlralentoriaf (posterior lossa)
Ependymomas represent most common spWlal cord glioma (60%)
of intrame<lUllary gliomas thwd most common posterior fossa 'umor In chifdren and adolescents
Figure 5-2. Pc' Ih( celllmi :mJ tle,nlU> (AI (B) Inir.lICll
ll'rI..1 ,1l,,1 In chilJrl'll. 70'''' (II tUII"'''''' ,If.' In ....lull,;;, 70% of
lUn"'11i an: eN "" Ct:ltl",1 ncrn.'; CSF = c('rchn"'I'IIl:llllui,l.
Neurohislology 35
Free nerve endings Meissner corpuscles Merkel cells
Deri'TVs
Merkel disk
...."",", "",m'",'''' } Epido,mi,
Pacinian corpuscles
Adipose tissue
o
A-P fiber
o
Cutaneous nerve
Agure $-3. Foor imporlam r.x:cptofS. Free ne.....e endings m... .Jiate rain and lempermure sensalion.
Meissner oorpusck'S of Ihe Jemlal p::lpillae mediate maile ",'O-point discrimination. Paccinian corpuscles of the
dermis m... -diatc tOllch. pressure and \ibrntion sensation. Merkel disks mooiate light looch.
6
Spinal Cord
I. GRAY AND WHITE COMMUNICATING RAMI
A. Grny communicating rnmi contain unmydin:uN ['OStl,oanglionic sympmhcric flbers.
They arc founJ ;u alllcn-is of the spinal corJ.
B. White communicating Tami coma;n myl'limm'd prcl,oanglionic liymly,uhclic fil'CTS.
They arc (0\111(1 fmm T-J til L-l (thC' CXICIll of the Imcml hom and th.:- inH'm,roiob,-
ernl cell column).
II. TERMINATION OF THE CONUS MEDULLARIS (sec Figure 2-1) occurs in the new-
born m rhe !(>H" of rhe body of ,he thir<llumhar \"cncrm (L}). [n ,Ill' aJulr. if occurs at
,he lc\'c1 of til<.' lo\\,{'r burder of rhe firSI lumhar n'nchra (l!).
Prevertebral ganglion
Motor endplate
GSE fiber
Alpha motor neuron
of ventral hom (GSE)
GSA fibers
Interneuron
GVA fiber
Sweat gland
Dorsal root
ganglion
Peripheral
nerve
Postganglionic, \, \ Preganglionic
fibers '. .\ fibers (GVE)
, -4' ramus - (4 " Postganglionic
Gray ramus ,neuron
Blood vesser Digestive tube
Paravertebral ganglion (f
(sympathetic trunk)
Figure 6-1. TIle (ulir (lI11Cli"nal cnml'onetlls n( the Illllr.,cic sllinal n.... rn.': /;cncr.tl d:\Cer.tl (GVA),
g.... ncrnl somllfic affercnl (GSA). Jlcncml Sl'mali( elierent (0SE). :tnJ I,ocllernl \'isccrnl cf(.... n.'tlI (GVE). Prol'rlO-
ccpli\'e, cUl:tneous. :md visn'ml rdl...x :on:;i :Irc muwn. TIll' musclc slrclch (myoralic) reHex lhc lllllsde
spindl.... GSA ,Iorsal n'lOl Jlangfinll cell. GSE \,('nlml hum m'lIor nellH'n, :ull.! ,kdelJll muscle.
3.
Spinal Cord 37
Table 6-1.
The Five Most Commonly Tested Muscle Stretch Reflexes
Muscle Stretch Reflex
Ankle jer1<
Knee jer1<
Biceps jer1<
Forearm jerk
Triceps jerk
Cord Segment
5-1
L-2-L-4
C-5 and C-6
C-5-e<;
C-7 and C-8
Muscle
Gastrocnemius
Quadriceps
Biceps
Brachioradialis
Triceps
III. LOCATION OF THE MAJOR MOTOR ANO SENSORY NUCLEI OF THE
SPINAL CORO
A. The ciliospinal center of Budge, from C-B (Q T-Z, mediates the sympathetic innerva-
tion of the eye.
B. The intermediolateral cell column, from C-B to L3, mediates the entire sympathetic
innervation of the body.
C. The nucleus dorsalis of Clark, from CB ro L3, gives rise to the dorsal spinocerelx'l-
lar tract.
D. The parasympathetic nucleus, from 5-Z to 5-4
E. 111e spinal accessory nucleus, from C-I ro C6
F. The phrenic-nucleus, from C-3 to C-6
IV. THE CAUDA EQUINA. Motor and sensory rootS (L.Z to Co) that are found in the sub-
arachnoid space below the conus medullaris fonn the cauda equina. They exit the \'enc-
bral canal through the lumlxlr interwnebrnl and sacral foramina.
V. THE MYOTATIC REflEX (see Figure 6-1) is a monosynaptic and ipsilateral muscle
stretch reflex (MSR). Like all reflexes, the myotatic reflex has an afferent anJ an efferent
limb. Interruption of either limb results in areflexia.
A. The afferent limb includes a muscle spindle (receptor) and a dorsal rOOt ganglion neu-
ron and its la fiber.
B. 11,c efferent limb includes a ventral horn mOtor neuron that innervates striated mus-
clc (effector).
C. The fivc most commonly tcsted MSRs arc listed in Table 6-1.
7
Tracts of the Spinal Cord
I. INTRODUCTION. Figure 7-1 sh"ws the ascending and descending (met;; of the spinal
cOTel. This chapteT c, weTS four of the major tracts.
II. DORSAL COLUMN-MEDIAL LEMNISCUS PATHWAY (Figure 7.2; sec also Figure
8-1 )
A. Function. The c'llumn-mcJiallemniscus pathway mediates tactile discrimina-
tion. vibration S('nsariun. form rcclJgnition. and joint and musclescnsarion (conscious
rropri()C("prion).
B. Receptors inciuJe Pacini's and Meissner's tactile corpuscles. join! receplOrs. muscle
spindles, and Gol).!i 1.'llllon org:ms.
C. First-order neurons aTC' located in the dorsal root ganglia ;It alllc\'c1s. They projeCT
,owns TO the spinal curd rhruuj.!h Ihe medial rOOT cnny zone. First-order neurons gh'c
TIl;
1. The J.!mcilc fasciculus fmm the lower exrremiry
Ascending tracts
Gracile lasciculus
Cuneate lasciculus
Dorsal spinocerebellar tract
Lateral spinothalamic tract
Ventral spinothalamic tract
o
Descending tracts
Lateral corticospinal tract
Hypothalamospinal tract
Rubrospinal tract
Vestibulospinal tract
Ventral corticospinal tract
Figure 7-1. The major ascending :lnd .IcscenJinj.: p;uhwars (>f Ih,' spin:ll conI. The usc,'nding sensory IT:lCU
;Ire .nnwn ...n tilt: kff. and th,' de-<ccndlng :lrc shown (>11 lhe ng#z,.
38
Tracts of !he Spinal Cord 39
Pons
Medulla
')";''r--Arm area
++-- Spinal trigeminal nucleus
'4----:I----Mediallemniscus
Midbrain
Decussation of
medial lemniscus
Medal lemniscus
';!>------Mediallemniscus
Internal capsule ----!t,.,....ff-

""'-Head area

area
Ventral posterolateral

nucleus ollhatamus
Lentiformnucleus:'" (neuron III)
Postcentral gyrus
"'C;::::------Legarea
area
Nucleus gracilis
Nucleus
Internal arcuate libers
(neuron II) -------1".:::::..----
Cuneate fasciculus
Dorsal root Gracile fasciculus
(neuron I) - Cuneate fasciculus
Cervical cord

Gracile fasciculus
Mel,,",,', - ,
:/
__ - -.... Lumbosacral cord
Pacinian
corpuscle --,...,"'''-__'
=
Figure 7-2. The Jorsal column-mooiallemniscus pathlOo'll}. Impulses conducted by rnc..J.;IlC
Cr1min:ltory tactile sense (e.g. tooch...jhration, P'fessurel and kmc"lhellC !lCIlSC (c.g. iJ'.biIlOIl. The
dors..'11 column syStem l",,--diates conscious proprioception. (Ad'll'lloo wuh irum Sulm
J: Human Nellr(lmlaloln:Y. Baltimore, Williams & Wilkin 1983. p. 266.)
40 Chapter 7
2. The Cllncare fasciculus rhc 1I1'f'<'r extTl'll1ily
3. The fur reflexes (c.g., lll,oraric reflex)
4. TIlle' aXI'ns thai ;L'"cnd in rhe dnT"al and tennin:l\c in dw ,Il:T;icilc ;llld
cuneate nucle; of Ih(' caudal nwdlilb
D. Sl"Cond-order neurons ;'rc Incated in the }!mcil(' allli CUllcat(' nucl('i of thc cauJal
Illl-Julla. They ghc rise I" axon" and ;nt('rnal arcuate fil'Crs Ihm dl"CllSSo11(' and fnrm a
cUlIlpacl fil'Cr hunJle (i.e.. m{"o.!iallemlliscus). The Illediallclllni.sclis m:cell1.!S Ihrnugh
the ;;;tl"m and H'rm;nares in Ihe \('llTral rustl'rLlbteral (VPL) nu-
cleus of Ihe Ihalamus.
E. Third-order neurons are l"cal('(1 in tilt' VPL nucl{'us ,)f the thalamus. TIw)' rrujl"'Ct
Ihrnut:h thl' r"-rcri,,r lilllh of Ihe illtem;)1 clpsule III the I'llslccnlml ,Il:ynlS, which is
Ihe rrimary "'um:l1. >SCIlSl.ry nlrtcx (I'I<'l!tn,lIl1l's areas 3. I. anJ 2),
F. Transection of the dors-11 eolumn-media!lcmniscus trael
1. Above the sensory d{'Cussalion, Ir:105(crin r(';;;\lhs in cumml:ul'mllossnf the Jor-
s.,1 cI,llImn 1ll....Ia!ilics.
2. In the spinal cord, tmnR"1.:li"n r("<lIlts in luss of the JOTS.11 culumn
lIlllllal it ics,
III. LATERAL SPINOTHALAMIC TRACT (Figllre 7- >: "'C'l' also Figur(' 8-1)
A. Funclion. Th.. Imeml spillOlhalalllic tmcl IllNialCS pain :mJ tl'lllpemlllTe senS."1Iion.
B. Receplors arl' fflX' Ol'f\C l'nJing-s. TIll' lareT;11 spin,'thalamie Imct receh'('S inp\II frum
fast- ;Illd pain fil'Crs (i.e.. A &,md C, r('1>f'L'Cli\c1y).
C. Firslorder neurons ;.re f"lInJ in Ih.. dilf"al r.....Jt J:::tn.t:lia:u allle\'cls. Thq prujo.:."Ct ax-
ons II' til(' spin:ll cnrd dlTllugh rh1. d.II"ulall'r..l trael ,-.f Li:-';;;;-l\ll'r (I'lIer.11 rLll11 ('nTT)'
:nnc) II) n('urons.
D. Second-order ncuronll ;.rl' ft'lIlld in rill' dorsal hurn. They givc rise to axons that .1("-
cms;uc in dw \cntral while commissur.. and as{'("nd in lhe c.mtrabllr:.1 bteml fu-
niculus. Theil axnns termin,l(c in the VPS llucleus ,-,f Ihe thalamus.
E. Third-order ncur'lIls arc found in the VPL nucleus o( the thalamus, They prujcct
I'hTClllgh the I'ostel ;,)r limh of till' internal G1l'sulc [, 1 111(' prinwry ..... ,ry cor-
t.. x (Brodmann's 3. I. :md l).
F. Tr,lIlSl'ction of the lateral spinothalamic Iracl results in c, \Il1T01lateral ,f pain and
tClllpcralur(' h'lo\\' Ihe lesinn.
IV. LATERAL CORTICOSPINAL TRACT (Fij,lllre 74: see Figure 8-1)
A. Funclion. TIll' Iater:.l cnnicospillnl Ir,K"1 mcdin[l's \<nluluary skille.1 mOIOl aClivity,
primarily, 'f Ih.. ul'lx'r liml's. It is 11'" fully myC"linmc.I'llllilt he end of 1he seo.:.l[\L! ye;.r
(B:lhinski's :oign).
B. Fiber caliber, AI' )TLlximatC"ly 90% of lhe fil1Crs lie hetween I :md 4""111. and 4% lie
:11)(11"(' lOJ.L1Il (frol 1 til(' gianr cells o( &'1:),
C. Origin and termination
1. Origin. Till' 1:1I.'r;11 ctlnicospinallmcl arises frlllll layt:r V of th(" cer{"hml conex
from lIun' conic:ll :ueas in equal aliqull1:>:
a. TIl(' prcmotor corlex (BruJm:lnn's an,'a 6)
b. TIle prim:.ry corlex (Br,,,lmann's aTl'a 4)
Tracts of the Spinal Cord 41
Ventral posterolateral
nucleus (VPl)
Neuron III
cerebral cortex
(postcentral gyrus)
.7,'7'::le-- Axons of neurons
in posterlor limb of
internal capsule
Crus cerebri --'
"""'"'
Corpus callosum
Intemal capsule
Mediallemni$CUs --------JI----{21
Thalamus ....___'\
Medulla
CJ 0 0
Neuron I
(dorsal root ganglion cell)
Lat6fa1
spinothalamic tract

Freenerve
endings W--
Neuron II Ventral while commissure
Figure 7-3. The hltcf:11 1f:!Ct. Imllulses comll!CtOO b)' this Imct rnt-Jiatc p;lin ;md thermal
sense. Numcrous collatcmls arc <!is\rihu{eu 10 the hrain stem reticul;lr (ormation. (Rcprimed with
(rom MH, Sutin J: f-IwntUl NCrlTO(lIlIl/0IllY. Williams & Wilkins, 1983. 1'. 274.)
42 Chapter 7
Large pyramidal
cells of Betz
Motor cortex

Medulllo
Spftlel eonl
Pons
Medulla
t:::=",. Longitudinal libelS in
basiler portion of pons
, __--Genu 01
Internal capsule
'-_--Anteflor limb 01
Internal capsule

;,-------Ventral corticospinal tract
(uncrossed Mons 01 neuron 1)
'-/---+----- Pyramldel decussaHon
... Ventral wtlite COtllilissure

CN III
CN VI ----.:!!
Lenticular nucleus
Laleral corticospinaltr8C\
(crossed !\)lons 01 neuron I)
""""-
,,'n
Figure 7-4. TIIC ItIlcrai :md WIllI'..1 (pyr:u1l1.bl) rrm::ts. TI,csc m:ljor mowl' r:llh.
nwdi:llC \olillom.1 mow! TI)(" cdl< I,f arc locall-J in the rremOIOI, the mOlor, :l1ld the ltI."n
.. cnrliCCS. CN = cr:m,;,1 nervc. (Rq,ril\l('d wilh fmm Cmpel1lcr MR, Sutin J: HllIntm Nell'
r(l{IIUl!Omy. Baltimorc, WiI]i;lIns & Wilkins, 1983,11.285.)
Traets of !he Spinal Cord 43
Ophthalmic artery

Middle ear
0.",,,,,,,,,,
sinus
/11'----_ eN v
Long V-3
ciliary nerve
Inlemal carotid artery
v-
To dilator
01_
Vessels of face
MuRe(s muscle
of eyelid
Sweat g,ands
lf
ollace .
Subclavlan artery
"' '.Y"" Superior
cervical ganglion
EXlemal
carotid artery
':::1--:o--S.:.plnal cord (T1)
ClliospInal center
(In lateral hom)
Sympathetic trunk
Figure 7-5. 1111,' oculosymp.-uhcl1C Hypollmt:llIllc iibcnol'roJecllo the ipslbteml dlio:>pin.'11 celller
of the intcrmediol:ncr..l cell culumn at T-I. The ciliospinal cemer prOjects preganglionic symp:llhClic iibers ro
lhc superior ccrviGlI g;mglion. "T1l<- supenor ceryiaol ganglion prOJ<."'Cts reriV;lSCUbr postJ;mglionic sympathetic
tihcT'$ through the lympantC (:;I\'il\', GI\cmou;; sinus. and $lll"erior orbit..1hssure to the dilalor muscle of lhe iris.
ImcrTllplion of lhis :my Ic\'c\ results in Homer's syndrome. eN"" cranial
c. The primary sensory cortex (Brodmann's areas 3. I. and 2)
d. Arm, face, and foot areas. The arm and face areas of the motor homunculus
are found on lhe lateral convexitY; the foot region of the mOlOr homunculus is
found in the paracentral lobule (sec Figure 232).
2. Termination. The lateral corticospin:ll (r.:Ict tcrmin:ltes contrabter.:llly, through
in[erneurons, un vetllr"l horn motor neurons.
D. Course elf Ihe Inleral corticospinal rmCI
1. Telencephalon. The breml corticospio:ll tr:tct runs in [he pDSt('rior Iimbofrhe in-
ternal capsule in the telencephalon.
2. Midbr.lin. The b[er.:ll corticospinal tract runs in the middle threl"-fifths of the crus
cerdlri in the miJbr.:lin.
3. Pons. TIle latcr.:I1 corticospin,,1 rr.:lCI nlllS in the base of the pons.
4. Medulla. TIle lateml corticospin:ll tmct runs in the mcdulbry pyramids. Between
85% and 90% of the corticospinal fibcrsdeeussate in [he pymmid:ll decuss... tion as
the l:ul"r.:Il corticospinallr.:lcl. TIle rctl\:lining 10% to 15% of Ihc fibers continue
as the :tnterior corticospinaltr:tC1.
5, Spinal cord. The I:ueml cortico:;pinaltr.lcl runs in Ihe dors...1quadram of the I:n
eral funiculus.
44 Chapter 7
E. Transection of the later-II corticospinal tract
1. Above the motor decussation. [F.msccrion resllhs in comralater.:ll spastic paresis
and B:lbinski's sign (llpgoing we).
2. In the spinal cord, transection rl'Sults in ipsilatcral spastic paresis anJ Babinski's
sign.
V. HYPOTHALAMOSPINAL TRACT ( F i g u ~ ).;)
A. Anatomic location. The hYPOlhalamospinal tmct projects withoot interruption from
the hypothalamus to the ciliospinal center of the intennediolareral cell column at T-
I to T2. h is found in rhe spinal CON at Tl or above in the dorsolateral quadrant of
the lateral funiculus. It is also foond in the lateral tegmentum of the medulla, pons,
and midbrain.
B, Clinical features. Interruption of this tract at any level results in Horner's syndrome
(i.e., miosis, ptosis, hemianhidrosis. and app.1rent enophthalmos). The signs are al-
ways ipsilmeral.
8
Lesions of the Spinal Cord
I. DISEASES OF THE MOTOR NEURONS AND CORTICOSPINAL TRACTS (F;g.
ures 8-] :mJ 8-2)
A. Upper motor neuron (UMN) lesions ;ITC cause..1 by Imn"t.'<:liun of the corticospinal
Imet or llcsrmclion of the cunical cdls of TIlty in sp.lstic p.lrcl1is wid,
l'yrnmiJal signs (Babinski's 5i1->1\).
B. Lower motor neuron (LMN) lesions arc caused hy J.nnaJ,.'(" to rhe mOhlf neurons.
They result in flaccid paralysis. ardlexia. mrophy. anJ fihrillations. Po-
or Wcrdnig-Hoffman disease (sec Figure S-2A) (rom J:nna,L't' lO rhe
mulor neurons.
Gracile fasciculus
Lateral
corticospinal tract
Lateral
spinothalamic Iract
Ventral while commissure
=
Ipsilaleralloss ollactile discrimination and
position and vibration sensation from leg
Ipsilateral loss ollaetile discrimination and
position and vibration sensation from arm
Ipsilateral spastic paresis
with pyramidal signs
Coolralaleralloss 01 pain and
1emperalure sensation one
segment below lesion
Ipsiialeraillaceid paralysis in affected myotomas
Bilateral loss or pain and temperature
sensation within dermatomes of invotved segments
Figure 8-1. Tr:ms\crsl.' ....>('!ion 01 the (cr\'[c;,l spin:,! cord. The dllllc.llI) 11l1lx.nam a-ecndllll,; and descend-
ing pmhw:,ys llr,' tlfl le!f. Clinic:ll ddidb thar Imlll thc mlerrupl K.Ill llllhc:>c an' shown
on 111( righf. C\."ilnlCfi\"c (ll the dn"",l rt>ulr III and Ill'dlexi,l. Ibln,clion o( duo
while commissuTC 11l1.'rrul''' rhc centrallr..msrn[,;,.lon of p.. in :md Ill1pl.lbc' h,l,ller..,lly IhfOU!!h lhe
brcml sl'lIlurhalamlc ImCI'.
4'
46 Chapter 8
Figure B-2. Cla:<.<ic ksi, "f Ihc spilwl c,)rd. (A) Puliumyclitis :lnd infantile muscu!:Jr ;]tmphy
(Wcrdnig-Ih,ffm;mn (/3) Mlllliplc .'iClewsis. (C) [xl[i;;11 column discasc (tabes dorsalis). (/)) Amr-
otT,'phic hlln:]1 :sckrrkib. (E) Ilcmbcct ion "I the spin;.l c,)rd (Brown-St::qu;lrd syndrome). (F) CtJmplclc vcmml
spin;,l ;lr!cry occlusion "f the -,pill;.l cord. (G) SulJacure comhincd dcgcncr:l\ion (vitamin BIZ Iwur"Iy'lIhy). (/-I)
S\,rln!=nmyt:"1i;].
C. CombinL-d UMN and LMN disease. An example of a combined UMN and LMN dis-
caS(' is amyotrophic lateral sderosis (ALS, or Lou Gehrig's disease) [S<.'C Figure 8-
lDI. ALS is by damage [0 the corricospinal,rncts. with pyrnmidlll signs. and by
damage [Q the LMNs. with LMN symptoms. Patients with ALS have no sensory
deficit:'.
Lesions of the Spinal Cord 47
II. SENSORY PATHWAY LESIONS. An example of a condition causN by these lesions
is dorsal column disease (tabes dorsalis) (see Figure 8-2e(. This disease isset'n in patients
wiTh neuro:syphilii. It is charneteri:ed by a loss of tactile discrimination and posirion and
\"Ibration sen&1tion. Irritatl\'c in\'olvellll'1l[ of the dors.'ll roots results in pain and pares-
thesias. Patiems have a Romberg sign. (Subject stands with his feet logether. When he
closes his eyes, he loses his halance. This is a sign of JOTS..'l1 column ataxia.)
III. COMBINEO MOTOR ANO SENSORY LESIONS
A. Spinal cord hemisection (Brown-Scquard syndrome) (see Figure 8-2E] is caused by
dnm:lge TO rhe following structures:
1. The dorsal columns [gracile (Ilog) and cuneate (arm) fasciculi]. Damage results
in ipsilatcrnlluss of taetile discrimination and position and vibrarion sensation.
2. The lateral corticospinal tract. D.lmaj,.'C results in ipsilateral spastic paresis with
pyramidal signs below rhe l{'sh..IIl.
3. The lateral spinothalamic tract. Damage results in cOnlralateralloss of pain and
temperature sensation one segment helow the lesion.
4. The hypothalamospinal tract at T-t and above. Damage results in ipsilateral
Horner's (i.e.. miosis. pwsis. hemianhidrosis. and apparent enoph-
thalmos).
5. The ventral (anterior) horn. Darn:lge results in ipsilar('ral t1accid paralysis of in-
ncrviltell muscles.
B. Ventral spinal artery occlusion (see Figure 8-2F) causes inf:lrction of the :lnter;or two-
rhirJs of rhe spinal cord, but spares the dorsal columns and horns. It results in damage
to rht, f,)lluwing structures:
1. The lateral corticospinal tracts. Damage results in bilateral spastic paresis with
pyramidal signs bEolow the lesion.
2. TIle lateral spinothalamic tracts. Damage results in bilateral loss of pain and rem-
perature sensation helaw the lesion.
3. TIle hypothalamospinal tract at T-2 and alxwe. Darna!;:e resulTS in bilateral
Homer's synJrome.
4. The ventral (anterior) horns. Dam:lgc results in bilateral flaccid paralysis of the
inm'rvarul muscl('s.
5. The corticospinal tr.lcts to the Solcr"l parasympathetic centers at S-l to S-4.
D,l1lwgc results in bibtcral d<l1llage imd loss of voluntary bladder and bowel
control.
C. Subacute combined degeneration (vitamin 8
11
neuropathy) (see Figure 82GI is
caused by pernicious (megaloblastic) anemia. It results from damage to the following
srructures:
1. The dorsal columns (gracile and cuneate fasciculi). Damage resulTS in bil:ueral
loss of tactile Jiscrimination and rosition and \'ibrarion ;;cnsmion.
2. The lateral corticospinal tracts. Damage results in bilateral spastic paresis with
pyramidal signs.
3. The spinocerebellar tracts. Damage results in bilateral .mn and leg dystaxia.
D. S)'ringomyelia (sce Figure 82H) is a central cavitation of rhe cen'ical cord of un-
known ('[iolngy. It res\lhs in d:lmage to the following structures:
48 Chapter 8
1. TIle ventral white commissure. Damage to decuS&"1ting lateral spinothalamic ax-
ons cauS\.'S bilateral loss of pain and temperature sensation.
2. The venlral horns. LM lesions result in flaccid pamlysis of the intrinsic muscles
of the hands.
E. Friedreich's ataxia has the 5."1me spinal corJ and symproms as sulxtcUle com-
bined degeneration.
F. Multiple sclerosis (see Fib'lJre 8-2B). Plolques primaril\' involw the while matter of lhe
cervical segmenlS of the spinal cord. The lesions are random and asymmeuic.
IV. PERIPHERAL NERVOUS SYSTEM (PNS) LESIONS. An example of a PNS lesion
is Guillain-Barrc syndrome (acme idiopathic JXllyneuritis, or postinfectious polyneuritis).
It affects the mowr filx-rs of the ventral rootS and peripheralnelTcs. and it pro
duces LMN symptoms (i.e., muscle weakness, ascending flaccid p,lfalysis, and areflexia.)
Guill<lin-Barrc syndrome has Ihe following features:
A. It is characteri:ed by demyelination .md cdem.!.
B. Upper cervical roor (C4) involvement and respirarory pamlysis arc common.
C. Caudal crani:ll nerve ill\'okemem with facinl diplegia is prcscm in 50% of cases.
D. Elcv:lted protein Icvcls may GlUS{' j);'lpilledema.
E. To a leSS\:r degree, sensor)' filx-rs arc afk-<:ted. resulring in paresthL'Sias.
F. The protein level in the cerebrospinallluiJ is ele\atecl. but without pk'OCytosis (albu-
minoc)'tologic dissociation).
V. INTERVERTEBRAL DISK HERNIATION is seen:lt the L-4 to L) or L) to 51 inler-
space in 90%ofcases. It appears at thcC-)IOC-6orC-6IOC-7 interspace in 10%ofGtscs,
A. Intervertebral disk herniation consists of prolapse. or herniation. of the nucleus pul-
posus through the defective anulus fibrosus and into the vertebral canal.
B. TI\e nucleus pulposus impinges on the spinal roots, resulting in spinal root symptoms
(i.e.. p:lrcsthcsias, p.1in. scnsoryloss. hyporellexi<l. and muscle weakness).
VI. CAUDA EQUINA SYNDROME (SPINAL ROOTS L3 TO CO) results usually from
a nerve root rumor. an ependymoma, a dermoid tumor. or frOill a lijXlma of the lerminal
cord. Is characteri:ed by:
A. Severe radicular unilateral pain
B. Sensory distribution in unilmeral saddle-shaped area
C. Unilaterall1l11scle rltrophy and absl'nt quadriceps (LJ) and ;InkIe jerks (51)
D. Incontinence al,d sexual functions are nOt marked
E. Onset gr.ldual and unilateral
VII. CONUS MEDULLARIS SYNDROME (CORD SEGMENTS 53-CO) usually resulrs
from an intramedullary tumor, e,g, ependymoma, Is characterized by:
A. Pain usually bilateral and nO{ sc\'erc
B. Sensory distriburion in bilateral saddle-shaped ar("rI
C. Muscl(" changes nOt marked: quadriceps and ankle reflexes nomlal
D. Inconrinence and scxual functions sc\'erly impaired
E. Onset sudden and bilaTeral
9
Brain Stem
I. OVERVIEW. Tllt' brain stem inciuJes the medulla, pons. and midbrnin. If extends from
tht pyramidal dccu$S.lfion [0 rhe poslcrior commi.ssure. The "min s[eln recein'S its blo..-,J
supply from Ihl' \"cnchrobasilar system. h contains cralli,,1 nernos (eN) III to XII (except
the srin0l11Y,ln of eN XI). Figures 9-1 and 9-2 sho\\' ilssunacc ,lllillOm)'_
II. CROSS-SECTION THROUGH THE MEDULLA (F;.u'C 9)
A. Medial structures
1. TIl(' hypoglossal nucleus of eN XII
2. TIle nwdiallemniscus, which l"lllllains ClOSS("J fihcrs from rhe gracile and cunc:ue
nuclei
3. TIle pyramid Cconico:;:pinal trans)
Third ventricle
Inlerlor cerebellar peduncle
Striae medullares
Cuneale tubercle
Gracile tubercle
Cuneale fasciculus
Gracile lasciculus
"
,
f
"'".-' Lateral geniculate body
Medial geniculate body
Crus carebri
cerebellar peduncle
. . Middle cerebellar peduncle
Pineal body
Superior
colticulus
Hypoglossal trigone
Vagal tOgene
Inferior coIliculus
Facial colliculus :>.:'Z;;;;:ttl
Vestibular area
Figure g..1. The- oofl'al of Ihe hruin SICIll. Thc three CCf(;hdbr pcJundC's h.-cn rl'l1Ion-J 10 e,,-
rose Ihe rhlllnhcml (0I'S.'1. TIle rrnchlcar ncrve is Ihe only nervc 1<, l.'''U Ihl.' hr.lin from II\\.' dorsal surface.
TI1C filoCi,ll surmpun.,; the b'Cnu of lhe f;lei,,1 nerve .111.1 Ihe ahducem nudeu). eN '"
49
so Chapter 9
CN VUI
CN XII
00ve,-,""-,,
Py<an;d
cervical nerve rft
Olfactory bulb (CN I)
Optic chiasm
Olfactory tract
Infundibulum
Tuber cioereum
\ /"'" ./Mammary body
Optic""'"
CN III
;-=It:= CN IV
CN V(motor root)
V (seo5OfY root)
Middle cerebeRar CN VI
eN VII
peduncle eN VII rlfllermEKiate)
CN VIII
CNIX
eNX
CNXI
CN II
OlfactOl'y trigone
Anterior perforated
","'Ian<e --"PC
Inteq:leduncular lossa
Crus cerebri
(cerebfal
Figure g..2. TIll: ,'cmrJI surf.-ee of rhe hr:.in stem anJ the all:-ehcJ crnnial IlCn:es (eN).
Hypoglossal nucleus of eN xu
Inlerlor olivary nucleus
Solilary tract and nucleus
Dorsal motor nucleus 01 CN X
Inferior cerebellar peduncle, ---\-
--W'@
Nucleus ambiguus (CN X)
Vestibular nuclei
Hypolhalamospinal tract
Spinal trigeminal nucleus
Spinal trigeminal lract
Spinal lemniscus
CN XII
Pyramid
Medial lemniSC1JS
Figure g..3. Tr.lllSvefSC K'Clioll of the mcJulla al the miJoli"ary level. Thc ,'agal nen:e !crnnial nerve (CN)
XI, hytJ'Ol,(loss.:llner...e (CN XII), :mJ vL"S[ibubr ner...e (eN VIII) :nc prominem in this seclion. TIle nucleus am
bigulls giv/:;S risc [0 sp:ial "isccr:.l cffcrtom iibclS to CN IX, X, anJ Xl.
Brain Stem 51
B. lateral structures
1. The nucleus ambiguus (C IX, X, and XI)
2. The \'estibular nuclei (CN VlII)
3. TIle inferior cerebellar peduncle, which contains the JotS.."l1 spinocerehdlar. cu-
neocerebellar, and oli\"(x:erebellar tracts
4. The lateral spinothalamic tract (spinal lemniscus)
5. The spinal trigeminal nucleus and tract of eN v
Ill. CROSS-SECTION THROUGH THE PONS (Figure 94). The pons has ;l durs,"ll
tegmentum and a \'entral base.
A. Medial structures
1. Medial longitudinal fasciculus
2. AbduceOl nucleus ofCN VI (underlies f,Kial colliculus)
3. Genu (iOlcmal) ofCN VII (underlies facial nerw) Ifacml colliculusl
4. AbJucent fibers ofCN VI
5. Medial lemniscus
6. CorricospinClI tract (in the base of the pons)
B. Lateral structures
1. FClcial nudeus (CN Vll)
2. Facial (imraClxiClI) nen'e fibers
3. Spinal trigeminal nucleus and tract (CN V)
4. L"lteral spinothalamic n<lcr (spinal lemniscus)
5. Vestibular nuclei ofCN VIII
6. Cochlear nuclei ofCN VIII
Abductml nucleus (CN VI)
Vestibular nerve (CN VIIl)
Facial nucleus (of CN VII)
Spinal lemniscUS
Foorth
ventricle
Vestibular nuclei (01 CN VIII)
~ Inferior cerebellar peduncle
\S........ \J--J- Spinal trigeminal
tract and nucleus
CNVII
Middle cerebeYar pedunde
Medial lemniscus
CNVI
Trapezoid body
Figure 9-4. Tmns\'el'$C section of the pons at the l ~ v e 1 of the rtbdueent nucleus of cmnlal nerve (eN) VI anu
[he facial nucleus of eN VII. MLF "" medial longirudln'll fasciculus.
52 Chapter 9
MLF
Spinal lemniscus
Cerebral aqueduct
Superior collic1Jlus
Periaqueductal gray
_ , ~ - cerebral pedoode (crus carobri)
ConicospinaJ tract
Red nucleus
Cortioobulbar tract
1'c1-----.f4c"t---\i-OcuIomotor nucleus
CN III
Substantia nigra
Medial lemniscus
Oentatolhalamic tract
Medial geniculate bod
y
",(/::::;""),
Figure 9-5. Tmnsvc('S(" se<:lion of (hc midbrain.n the level of (he supcriOf eoUiculus. oculomoror nucleus of
eromi.ll nerve (CN) Ill. .1lK! red nuclcus.l\'ILF = mooiallongiludio..",l fasciculus.
IV. CROSS-SECTION THROUGH THE ROSTRAL MIDBRAIN (Figure 9-5). The mid-
brain has a dorsal tectum. an intemlooiate tegmentum, and a base. The aqueduct lies be
tween the rectum and rhe tegmentum.
A. Dorsal structures include the superior colliculi.
B. Tt.-gmentum
1. Oculomotor nucleus (eN III)
2. Medial longitudinal fasciculus
3. Red nucleus
4. Substrlntia nigm
S. Dcntrltorh;llamic tract (crossed)
6. Mcdial1cmniscus
7. LaremI spinothalamic tmct (in the spinallemnisclls)
C. Crus ccrebri (basis pedunculi cerebri, or cerebral peduncle). The corticospinal tr.lct
lies in rht, middle three-fifths of the crllS cercbri.
V. CORTICOBULBAR FIBERS (see also Figure 13-4) project bilatcmlly to all motor era-
ninl nerve nuclei excepl the facifll nucleus. The division of the (acial nerve nucleus thflt
innervales lhe upper f<lcc ([he orbiculflris oculi muscle and above) receives bilateral cor-
ticobulbar input. The division o( the (acial nerve nucleus th:1.t innerv:l.tcs [he lower face
receives only contralateral corticobulbar input.
1.0
Trigeminal System
I. OVERVIEW. The trigeminal system provides sensory innervation (0 the face. oral cav-
ity. and supratentorial dura through general somatic affcrcnl (GSA) fibers. It also inner-
vales the muscles of mastication through special visceral cfferenl (SVE) fibers.
II. THE TRIGEMINAL GANGLION (semilunar or gasserian) conrains pseudounipolar gan-
glion cells. It has three di\jsions:
A. The ophthalmic nerve [cranial nerve (eN) v- I] 1il's in the wall of the c:l\'cmous si-
nus. It cmel'S the orbit through the superior orbital fissure and innervates the forehead,
dorsum of the nose, upper eyelid, orbit (cornea and conjunctiva), and cranial dura.
The ophthalmic nen'c mediates the afferent limb of the corneal reflex.
B. The maxillary nerve (eN V.2) lies in the wall of the cavernous sinus and innervates
the upper lip and cheek, lower eyelid. anterior portion of the remple. oral mucosa of
the upper mouth, nose, pharynx, gums, teNh and palate of the upper jaw. and cranial
dura, h exits the skull through the foramen rorundum.
C. The mandibular nerve (eN V-3) exits the skull through the foramen O\'ale. Its sen-
sory (GSA) component innervates the lower lip and chin. posterior portion of the
temple, external3uditory meatus, and tympanic membrane, external ear. teeth of the
lower jaw, oral mucosa of the cheeks and floor of the mouth, anrerior two-thirds of the
tOl)gue, tempJromandibular joint, and cranial dura.
D. The motor (SVE) component of eN v accompanies the mandibular nerve (eN
V-)) through the foramen 0\'3Ie. It innervates the muscles of mastic:nion, 111ylohyoid,
anterior belly of the digastric, and rensores tymp.mi .md veli palatini, It inncn':Hes the
muscles that move the jaw, the lateral and medial prerygoids (Figure 10-1).
III, TRIGEMINOTHALAMIC PATHWAYS (Fig"," 10-2)
A. The ventral trigeminothalamic tract mediates pain and temper:lture sensation from
the face and oral cavity.
1. First-order neursms are located in the trigeminal (gasserian) g:mglion. They give
rise to axons that descend in the spinal trigeminal tracr and synapse with second-
order neurons in the spinal trigeminal nucleus.
2. Second-order neurons are locatecl in the spinal trigemin... l nucleus. The) give rise
to decuss..'lring axons that terminate in the contralateral ventral posterollll-dial
(VPM) nucleus of rhe thalamus.
3. Third-order neurons are locatecl in the VPM nucleus of the thalamus. They pro-
53
54 Chapter 10
Motor cortex -,.:.>
UMN
Superior cerebellar peduncle
Chief sensory nucleus CN V
7
'--L
Motor nucleus eN v
Medial lemniscus
Corticospinal tract
lMN
Condyloid PflXesS
4th ventlicle
CNV molor
-\-Poo,
Lateral pterygoid muscle

,
Figure 10-1. Function and innerv:nion 0( {hc latcral prCtH,'oid musclcs (LPMs). TI1C LPM r('Ccivcs irs in-
nervation from rhe motor nucleus of the rril,,'cmin,,l nerve found in thc rosTr;11 JXlns. Bilareral innerv,lIion of rhe
LPMs results in prorrusion of rhe rip of thc mandible in the midline. TIle LPMs also open the j;III'. Dcn('"rvarion
of one LPM results in dC"i:ltion of thc mandiblc 0 the ipsihllcnll or wcak side. The trigemin"llllofOr nuclellS
receives bilarer,ll corticobulb:lr input. eN = cmnial nerve; LMN = lower motor neuron: UMN = upper motor
neurOll.
jcct through the posterior limb of thc internal capsule to the face area of the s0.-
matosensory cortex. (Brexlmann's areas 3, I, and 2).
B. The dorsal trigeminothalamic tract mediates tactile Jiscrimination and pressure sen-
s.."1tion from the fuce and oral cadty. It receives input from Meissner's and Pacini's cor-
puscles.
1. First-oroer neurons arc located in the trigeminal (gasserian) ganglion. They
synapse in the principal sensory nucleus of CN V.
2. Second-order neurons are located in the principal sensory nucleus ofCN V.
They project to the ipsilateral VPM nucleus of the thalamus.
Trigeminal System 55
Internal capsule
(posterior limb)
- - - Dorsal IrigeminothaIamic tract
nucleus

Ventral posteromedial'
nucleus of thalamus,
Yeotraltrigeminothalamic tract - - - -
Face area of
postcentral gyrus -.. -r:'!!i"",,i:!
MotOf branch of CN Y3
5enSOl'y branch of CN Y-3
,
,
Spinal trigeminaltraet
5eoSOl'y branch of CN Y2
'0-4>7
, Principal senSOl'y nucleus of CN Y
/ / 5eosory bfanch of CN V-'
,
,
'" Mesencephalic nucleus of CN Y
,
,
,
,
,
/
,
Spinal trigeminal nucleus /
Pons
Midbrain
,
Motor nucleus of CN Y/
Spinal cord
Figure 1()'2. TI1C ventral (p;lin lltld temper-,lfurc) and dOl'$.11 (discrimin:uive lOuch) plllh-
wuys. eN '" cr.lllial nerve.
3. Third-order neurons are loc,ned in the VPM nucleus of the thalamus. They
project through the posterior limb of the internal capsule ro the bee area
of the somatosensory eonex. (Brodmann's areas 3, I, and 2).
IV. TRIGEMINAL REFLEXES
A. Introduction (Table 10-1)
1. The corneal reflex is a consensual disynaptk reflt'x.
56 Chapter 10
Table 10-1.
The Trigeminal Reflexes
Reflex
Comeal reflex
Jaw jer1l.
Tearing (lacrimal) reflex
OCulocardiac reflex
Afferent Umb
Ophthalmic nerve (CN Vol)
Mandibular nerve (CN V-3)*
Ophthalmic nerve (CN V-l
Ophthalmic nerve (CN Vol)
Efferent Umb
Facial nerve (CN VII)
Mandibular nerve (CN V-3)
Facial nerve (CN VII)
Vagal nerve (CN X)
The cell bodies are found in the meseocephalic nucleus of CN V.
CN ... cranial nerve.
2. The jaw jerk reflex is a monosynOlptic myotatic reflex (Figure 10.3).
3. The tearing (lacrimal) reflex
4. The oculocardiac reflex occurs when pressure on the globe results in bradycardia.
B. Clinic.ll corrchuion. Trigeminal neuralgia (tic douloureux) is chataclerizeJ by recur-
n::nt of sharp, Slabbing pain in one or more hranches of the trigeminal nl'nl'
on one siJe of Ihe face. II usually occurs in people older than 50 years uf age, and it is
mote common in wumen Ihan in men. Carbama:epine is the drug of choice for idio-
pathic trib'Cminal neuralgia.
MOIOr nucleus CN V
with secondary neuron
Mesencephalic nucleus
with primary neuron
V-3
Muscle spindle
from masseter muscle
Masseter muscle
L-----Motor division CN V
Principal sensory nucleus of CN V
Spinallrigeminal nocleus
Agure 1Q..3. Th(" ,a"" lerk (m.:lSSClCr) reltex. 11,e .. ffercnt limb is V3, and the efferem limb is the IllO(Ot rt)(l(
lll:1( accofllp<lllies V3. SCI'lSOf't' neurons arc Ioc:lll-J in the mesencephalic nucleus. The jaw jerk reflex,
like all muscle stretch rt:flcxe.., I.S <Illlonosptaptic rcilex. HnJCITctlexia indicates an upper mor:or nctl-
rtln lesion. eN = cr:mial nene.
Cavernous
sinus
Pituitary gland
(hypophysis)
Infundibulum
Trigeminal System 57
Optic chiasm
Intemal carotid ar1ery
Anlerior
process
CN III

'.),1,..-- CN V-I
tii--=--l!,--_ CN VI and
postganglionic
sympalhelics
CN V-2
Agure 10-4. TIle CContents of thl' c:,,cmous sinus. TIle wall of the contains the phlh:tlmic
cmni:llncr...c (CN) V-I and maxil1,lry (CN V2) di\'isions of the trib'Cminal nl'l"\l' (CN V) and the
(eN IV) and oculomotor (eN HI) The siphon of tile imern,ll carotid artery :mJ thl" :11>.. lu'nt ner...e (CN
VI), along with fibers, lil-s within the C""'emotIS ShIll',
v. THE CAVERNOUS SINUS (Figure 10-4) contains th<.- following :>!TlIClureS:
A_ Internal C:lrotid :lrlery (siphon)
B. eN III, IV, V_I, V-2, ,md VI
C. Postganglionic sympathetic fibers en route to th: orhit
58
1.1.
Auditory System
I. OVERVI EW. The 3udiwI)' system is an exteroceptive special somatic ,,((crem system that
can derecr sound frequencies from 20 H: to 20,CXX) H:. h is dcri\"ed from the otic veside,
which is a derinlth'c of the otic placode. a thickening of the surface ectoderm.
II. THE AUDITORY PATHWAY (Figure 11-1) consists of the following structures.
A. The hair cells of the org.m of Corti are innervated by the peripheral processes ofbipo-
Jar cells of the spiral ganglion. TIley are stimulated by vibrations of the basilar membrane.
1. Inner hair cells arc the chief sensory elements; they synapse with dendrites of
rn}'elimued neurons whose axons comprise 90% of the cochlear nerve.
2. Outer hair cells synapse with dendrites of unmyelinated neurons whose axons
comprise 10%of the cochlear nerve. The OHCs reduce the threshold of the IHCs.
B. The bipolar cells of the spiral {cochlear} g.1nglion projecr peripherally [Q the hair cells
of the 0'l."'n ofConi.1l1ey projCCI centrally as [he cochlear nerve ro [he cochlear nuclei.
C. The cochlear nerve [cranial nerve (eN) VIII] extends from the spiral ganglion to
the c("rebelloponrine angle. where it emers the brain Slem.
D. The cochlear nuclei recei\'e input from the cochlear nerve. They projccr comralater-
ally to the superior olivary nucleus and larerallemniscus.
E. The superior olivary nucleus, which plays a role in sound localization, receives input
from the cochlear nuclei. It projects to the lateral lemniscus.
F, The trapezoid body is located in the pons. It contains dccussaling fibers from the ven-
nnl cochlear nuclei.
G. The laternllemniscus rece yes input from the contralateral cochlear nuclei and supe-
rior olivary nuclei.
H. The nucleus of inferior colliculus receives input from the lateral lemniscus. It pro-
JCCtS through the brachium of the inferior colliculus to the medial geniculate lxxIy.
I. The medial geniculate body receives inpul from the nucleus of inferior colliculus. h
projects through the internal capsule as the auditory radiation ro (he primary auditot')
conex. rhe trnns\'crse temporal gyri of Hesch!.
J. The transverse temporal gyri of Heschl contain the primat')' auditory cortex (Brod-
mann's areas 41 and 42). The gyri are located in the depths of the lateral sulcus.
III. HEARING DEFECTS
A. Conduction deafness is caused by internlption of the passage of sound waves through
the external or middle car. It mOlY be caused by obstruction (e.g., wax), otosclerosis,
or otitis media.
Auditory System 59
Nucleus of inferior coIiculus
Intemal capsule
Caudate nucleus Thalamus
M""'" --k--
Putamen:J
LentifOfTTl nucleus
pallidu
'i. Transverse gyrus
of temporal lobe
f..--- AuditOfY radiations in sublenticular
part of internal capsule
Brachium 01 ----Medial genic\lIate body
inferior coIliculus Commissure 01
inferior coIIiculus
<l:P------LaterallenvVscus
Nucleus and commissure
ollateraJ IerTlliscus
Tectorial membrane
Hair cells
Spiral ganglion
Cochlear nerve (CN VIII)
Superior
olivary nucleus
Dorsal and ven""':'-"--I-Ci,-tj-,
cochlear nuclei
Trapezoid body
Pyramidal tract
Base of pons
Figure 11-1. Periphcwlllnd centrol connections of the auditory system. TIl is syStem nriS(.s from t he h:lir cells
of Ihe organ of Coni :md tcrminates in the transverse Icmpoml gyri of I-lcschl of the superior lempor.IIl;)'nlS. It
is chamctcrited by the bi1:lter.l1ity of projections nnd the lOnOlopic locnlimtioll of pilch ,If ;llllc\c\s. For exam-
ple. high pitch (20,000 I-h) is loc,llilcd ,It the b,ISC of the cochlca and in the pnstcromedi,]l p,lrt of the tmnsvcrse
tcmporol gyri. eN "" emni,]1 nerve.
B. Nerve deafness (sensorineural, or perceptive. deafness) is causc<1 by disease of the
cochlea. cochlear nerve (acoustic neuroma), or cenrral <luditory conncClions. It is usu-
ally caused by presbycusis that results from degenerative disease of the organ of Corti
in the first few millimeters of the bas.11 coil of the cochlea (high-frC<luency loss of
4tJ00.-8000 H,).
IV. AUDITORY TESTS
A. Tuning fork tests (Table 11-1)
60 Chapter 11
Table 1.1.-1.
Tuning Fork Test Results
otologic Anding
Conduction deafness (left ear)
Conduction deafness (right ear)
Nerve deafness (left ear)
Nerve deafness (right ear)
Normal ears
Weber Test
Lateralizes to left ear
Lateralizes to right ear
Lateralizes to right ear
Lateralizes to left ear
No laterlization
Rinne Test
BC > AC on left
AC > BC on right
BC > AC on right
AC > BC on left
AC > BC. both ears
AC > BC. both ears
AC > BC. both ears
Cooduction deafness'"' middle ear deafness (e.g. otosclerosis. otitis medial: nerve deafness'"' sensorineural deaf-
ness (e.g., presbycusis: AC '"' air conduction: BC = bone conduction.
1. Weber's test is perf,)rmcd by placing:l vibrating tuning fork on the vertex of the
skull. Normally, a patient hears ('qunlly on lx)[h sides.
a. A pntiellt who h;lS lInihlleral conduction deafness hears the vihration more
loudly in thc affectloJ car.
b. A patient who has unilateral partial nerve deafness hears the vibration more
loudl) in Ihe normal car.
2. The Rinne test compares air and bone conduction. It is pcrformcd by placing a vi
brating lUning fork on the mastoid process ulltilthe \'ibration is no longer heard;
Ihen the fork is hdd in front of the car. Nonnally. a patient hears the vibration in
Ihe air aftcr bonc conduction is gone.
a. A patient who has unilateral conduction deafness docs nOI hear Ihe \'ibra-
tion in the air after bone conduclion i:. gone.
b. A pat ient who has unilateral partial nerve deafness hears the vibmtion in Ihe
air aftcr hone conduction is &'One.
B. Brain stem auditory c\'oked potcntials (BAEPs)
1. Testing method. Clicks arc prescmcd to one car. then to the other. Scalp ek'(:
trodes and :l computet genet::ltl' a scties of sc\'en The waves arc associalOO
wilh specific areas of the :ludiwly p:uhw:l)'.
2. Diagnoslic value. This method b valuable for diagnosing brain S!<.'1ll lesions
tiple sclerosis) and ,x.mcrior fossa wmms {acoustic nellromas).lt is also useful for
assessing henring in infants. Approximatcly 50% of pnticms with multiple sclcro
sis have ;lbnorl11:11 BAEPs.
:1.2
Vestibular System
I. OVERVIEW. Like the <luditory system, the vestibular system isdcrivcd from the otic
cleo The otic vesicle is a derivative of the otic placode, which is a thickening of the sur,
face ectoderm. nlis system maintains posture and equilibrium and coordinates head and
eye movements.
II. THE LABYRINTH
A. Kinetic labyrinth
1. Three semicircular ducts lie within the three semicircular canals (Le.. superior,
latcml, and posterior).
2. These ducts respond to angular acceleration and dei:e1eration of the head.
a. They contain hair cells in rhe crista ampullaris. The hair cells respond to en-
dolymph flow.
Enoolymphalic
I duet
.Ampulla
/ / and crista
..- Utricle
","" I and maCtJla
,
, ,
\ Cochlear duet
,
Saccule and macula
5emicircular ducts

\'
" ' ,,
,,
, ,
, ,
,
,
, ,
L.. Vestibular nerve and
ganglion in intemal
auditory meatus
- Carebello-
pontine angle
,
'Pyramid Medial Iermiscus I
Inferior
olivary
nucleus- ---
MLF.
Vestibular
oodei __ "S--': - I FJoccuIus
,
Inferior \ - - -Juxtarestilonn
cerebellar 00 body
peduncle .--
Figure 12-1. Peripheral connections of [he \-est:ibolar The h.'1ir cells of [he crumc ampullares and
the 1113CUbe of [he utriclc and So"lCCule projen, through [he \'f,'sribolar nerve, to the vC5tibular nuclei of (he
medulla and pan;; and [he tlocculonodular lobe of [he cerebellum (\'Wibulocerebellum), MLF '" mediallol\gi-
[udinal fasciculus.
61
62 Chapter 12
b. EnJolymph flow W\\';lrd the :'llllpulla (ampullopcrnl) or utricle (\ltriculopcrol)
is a smmb'Cr stimulus th:'ln is endolymph flow in rhe op(X)Sirc direction.
B. Sialic labyrinth
1. The utricle anJ s"lccule rcspunJ to the position of the head with respeet 10 linear
accelcr.nion and the pull of gr:wity.
2. The utricle and sacculc contain hair cells whosc cilia arc embc...IJl'{l in the
molirhic 111l."mhmne. Whcn hair cclls ;"Ire bent tuward the longest cilium (kinneil.
illlll), the fr....quency of sensory Jischarge incr....ascs.
III. THE VESTIBULAR PATHWAYS (Figllre's 12-l and 12-2) consisrof the followingstruc-
HIres.
A. Hair cells of Ih.... scmicircular dUCIS, s.1ccule. and utricle are inncrv"ul,J by periph-
eml proceSSl-'S of bipohlr cells of the "cstibubr ganglion.
Vestibular area of
cerebral COl1ex
Thalamus
Ventral posterior >;
inftlrior nucleus
Veslibulothalamic tracts
M<bam ----------<{
,
.-
"
Abducent nudeus
of eN Viol pons
MLF
--0;--'=\;';-- Nodulus of cerebellum
MLF
I. .-,;;;:'::.<'::=---- Vestibular ganglion

JuxtareSliform body -_/
Vestibular nuclei
<jf--- lateral vestibulospinal (Deiters') tract
Figure 122. TI1C" major ccncral conneclions o( lhc syslem. VC'Slihular nuclei projen. chrtlu!:h che
a,ocl'nJing llll'l.lialiongillidinal (:HICiellll (MI-F), til (he ocubr lIl,nor nuclei anJ \"eslibulo-ocubr rdlcxes,
ihul:.r Iluc!e'i al.... ' pr,'jccl. chrou!:h Ihe descendlllg MLF ;Ind ];ueml \'cstiblllo>pinal tmclS, to Ihe ,"emr,11 h, ,m
Ill< 'I, 'r IlCl,r"n.< ,,( Ihe ,pin,,1 CNd ;,nd 111('-li;lIe tX.l$tur.,1 eN = cwni;ll nerve.
Vestibular System 63
B. The veslibular ganglion is iocared in the fundus of the infernal au<litory meatus.
1. Bipobr neurons project through their peripheral processes to the hair cells.
2. Bipolar neurons project their central processes as thc \'cstibubr ncr\c !cranial
ncrve (CN) Villi to the \,c:>tibular nuclei and to the l1occuloncxlubr lobe of the
cerebellum.
C. Vestibular nuclei
1. These nuclei receive input from:
a. The semicircular ducts. s.."1ccule, and utricle
b. The l1occulonooular lobe of the cerebellum
2. The nuclei project fibers to:
a. The l1occulonooular lobe of the cerebellum
b. CN III, IV, and VI through the medial longitudinal fasciculus (MLF)
c. The spinal cord through the laternl \'estibulospinal tract
d. The \entral posteroinferior and posterolat'cral nuclei of the thalamus, both of
which project to the postcentral g)TUS
IV. VEST1BUlG-OCULAR REFLEXES arc mediated b)' the \cstibular nuclei, MLF, ocular
mOtor nuclei, and CN III, IV. and VI.
A. Vestibular (hori:ontal) nystagmus
1. The fast phase of nystagmus is in the direction of rotation.
2. The slow phase of nystagmus is in the opposite direction.
B. Postrotatory (hori:ontal) nystagmus
1. The fast phase of nystagmus is in the opposite direction of rotation.
2. TIlC slow phase of nystagmus is in the direction of rotation.
3. Thc patient past-points and falls in thc direction of previous rot<ltion.
C. Caloric nystagmus (stimulation of hori:ontal ducts) in normal subjects
1. Cold water irrigation of the external audito!)' meatus results in nystagmus to the
opposite side.
2. Warm water irrigation of the external auditory meatus rcsuhs in nystagmus to the
same side"
Normal conscious subject Bralnstem intact MlF (bilateral) lesion Low brainslem lesion
Figure 123. Cold C:lloric responses in the unconscious patien!. When the br:.lin inmcl, the eycs de
viate the irrigated with bilateral transection of the mcJiallongilUdin;11 fasciculi ('\'ILF), the eye Jc.
viau:$ 10 Ihe abducted DesInICtion of thecaudal brain stem rcsuhs in no deviation of the e)"cs. l)(mbkhetided
arJ"O\.l'S indicate nysugmus; single-headed arJ"O\.l'S indicale deviation of the e)"cs to one Side.
64 Chapter 12
3. Remember the mnemonic COWS: Cold Opposite, Warm Same.
D. Test resulrs in unconscious subjects (Figure 12.3)
1. No ll\,smg-mus is secn.
2. When rhe brain stem is intact, there is deviation of the eyes to the side of the cold
irrigation.
3. With bilateral MLF transection, there is deviation of th" alxl.ucting eye to the side
of the cold irrigation.
4. With lower brain stem damage to the vcstibular nuc1('i, there is no deviation of the
"res.
1.3
Cranial Nerves
I. THE OLFACTORY NERVE, the first cranial nerve (eN I) [Figure 13-11, mediates ol-
faction {smell).lt is the only sensory system th:\[ has no prcconical reby in the ,hal;lllllls.
The olfactory nerve is a special visceral afferenr (SVA) nerve. It consists of unmyelinated
axons of bipolar neurons that aTC located in the nasrll mucosa, the olf,ICtory epithelium. It
enters the skull through the cribriform plme of the ethmoid bone (sec appendix).
A. OlfacTory pathway
1. Olfactory replOt cells are firsl-ordcr neurons that project to the mitral cells of
the olfactory bulb.
2. Mitral cells are the principal cells of the olfactory bulb. They are excitawry and
glurnminergic. They project through the olfactory tract and lateral olfactory stria
to the primary olfactory cortex and amygdala.
OIfaetOf)' td) (CN I)
CN II Optic chasm
---''-OIfactOf)' tract
Olfactory trigone Infundibulum
Anterior uber cinereum
substance - Mamiflary body
Interpeduncular fossa Optic tract
Crus cerebri CN til
(cerebral peduncle) eN IV
CN V lmotor root)

V sensory root)
CNVI
Middle cerebellar CN VII
peduncle CN VI1 (inlellTlediale)
eN VIII
CNIX
CNX
CNXI
Agure 13-:1. The base of the brain "'jrh anac.hed cranial nerves (eN). (Reprinted "i[h renmsslOl'l from
Tmex Re, Kellner CEo Detailed Adas of HeM ond Neck. New York, Oxford University Press. 1958, p. 34.)
.5
66 Chapter 13
3. TI,e primary olfactory cortex (Brodmann's area 34) consists of the piriform cor-
tex dKI[ O\'erlies the uncus.
B. Lesions of the olfactory pathway result from trauma {e.g.. skull frncture) and. often,
from olfactory groove meningiomas. ThC5t' lesions cause ipsilateral anosmia
ing value). Lesions that invoke the (XlrahippocalllJXlI uncus may cause olfuctory hal-
lucinations luncinate fits (sei:ures) with deja \ul.
C. Foster Kennedy syndrome (FKS) consists of ipsilateral anosmia, ipsilateral optic atro-
phy, and contrahneml p<!pilledema. It is usually cause<1 by an anterior fossa meningioma.
II. THE OPTIC NERVE (CN II) is a special somatic afferent (SSA) nerve that subscrves vi-
sion and pupillary lighr reflexes (afferent limb) (see Olaprer 191. It is nor a true peripheral
nerve, but is a tract of rhe diencephalon. A tmnsccro... -d optic nerve canoot rq;,>enerare.
III. THE OCULOMOTOR NERVE (CN III) isa general somatic efferent (GSE). b>eneral vis-
ceral efferent (GVE) nen:e.
A. General characteristics. The oculomOlor nelYC moves the eye, constricts the pupil,
accommodates, and converges. It exits rhe bmin stem from the interpt.-duncular foss.."1
of rhe midbrain, paSSt.-"'S through the ca\"cmous sinus. and enters the orbit through the
superior orbital fissure.
1. 111e GSE component arises from the oculomo{Qt nuclell3 of the rostral miJbmin.
It innervates four extraocular muscles and the levator palpebrae muscle. (Re-
member the mnemonic SIN: superior muscles are intoners of the globe.)
a. TI,e medial rectus muS(;le adducts thc eye. With irs opJXlSire parmer, it con-
verges the eyes..
b. 11,e superior rectuS muS(;le elel'ates. intorts, anJ adducts the eye.
c. 11,e inferior rectus muS(;le Jepresses, cxrorts, and adducts the eye.
d. 11,e inferior oblique muscle elevates. extorts, and alxlucrs the eye.
e. TI,e levator palpebrae muscle e1e\'ates the upper eyelid.
2. The GVE component consists of preganglionic p:lfasympathetic fibers.
a. TIle Edinger-Westphal nucleus projects preganglionic parasympathNic fibers
to the ciliary ganglion of the orbit through CN III.
b. The ciliary ganglion projects postgangliollic p:lrasrmpathetic fibers to the
sphincter muscle of the iris (miosis) and the ciliarr muscle (accommodation).
B. Clini..:al correlation
1. Oculomotor paralysis (palsy) is seen with transtentorial herniarion (e.g., rumor,
subdural or epidural hemawma).
a. Denervation of the levator palpebrae muscle causes ptosis (i.e... drooping of
the upper eyelid).
b. Dcnervation of the extraocular muscles causes the affect\d eye to look "down
and om" as a result of the unopposed action of the lateral recrus alld superior
oblique muscles. TIle superior oblique and lateral rcctus muscles are inner-
vated by CN IV and CN VI, respecrivcly. Oculomotor palsy results in diplopia
(double \'ision) when dle patiem looks in the direction of rhe parNic muscle.
c. Interruption of parasympathetic innervation (internal ophthalmoplegia) re-
sults in a dilated. fixed pupil and p<"1ralrsis of accommodation (cycloplegia).
2. Other conditions associated with eN III imp."1irment
a. Transtentorial (uncal) herniation. Increased supratelltorial pressure (e.g.,
from a tumor) forces the hippocaJ1\p<!luncus through the temorial notch and
compresses or stretches the oculomotot nene..
Cranial Nerves 67
(1) Pupilloconstrictor fibers are affected first, resulting in dilatt.-d, fixed pupiL
(2) Somatic efferent fibers are affected later, resulting in external strabis
mus (exotropia).
b. Aneurysms of the carotid and posterior communic:uing aneries of[('n com-
press CN III within the cavernous sinus or imerpedllncul:u cistern. They usu-
ally affect the peripheral pupilloconstricror fibers first (e.g., uncal herniation).
c. Diabetes mellitus (diabetic oculomotor palsy) often affects rhe oculolllotor
nerve. h damages the cemrnl fibers aocl sjXlrcs rhe pupil1oconstricwr fibers.
IV. THE TROCHLEAR NERVE (CN IV) is a GSE ncrve.
A. General characteristics. The trochlear nerve is a pure mOtor nl'rvt' that innt'rvates the
sllperioroblique muscle. This muscle depresses, intons, and alxluclS the eye. (Sce Rg-
ure 1740.)
1. It arises from the comral:neml trochlear nucleus of the caudal midbrnin.
2. It decussates beneath the superior velum of tht' midbmin and exits the
brain stem on irs dorsal surface, caudal to the inferior colliculus.
3. It encircles the midbrain within the subarachnoid space, passes through the cal"
emous sinus, and enters the orbit through the superior orbital fissure.
B. Clinical correlation. CN IV paralysis resulrs in thl' following conditions:
1. Extorsion of the e)'c and weakness of downward ga:e
2. Vertical diplopia, which increases when looking down
3. Head tilting 10 compensate for extorsion (may be misdiagnosed as iJiopalhic tor-
ticollis)
4. Head trauma. Because of irs course around the midbmin, the trochlear ncrye is
panicularl)' vulnerable to head trauma. The trochlear dl'CUS$..'l1 ion underlies the su-
perior medullary velum. Trauma at this site often results in bilateral founh-nen'c
palsies. Pressure against the free border of the tentorium (herniation) may injure
the nerve.
V. THE TRIGEMINAL NERVE (CN V) isa spedal visceral efferem (SVE), general somatic
(GSA) nerve (sec Chapter 10).
A. General characteristics. The nerve is the nerve of pharyngeal (brachifll)
arch 1 It has three divisions: ophthnlmic (CN V-I), maxillary (CN
V.I), mflndibular (CN V-3) Isee Chapter 101. ./
1. The SVE component arises from the motor trigeminal nucleus rhat is found in the
Iater:11 midpontine tegmentum. Ir innervates the muscles of mastic.ltion (i.e.
tempomlis, lateral, and prerygoids), rhe tensores tympani and veli
palatini, the m)'elohyoid muscle, and rhe anterior belly of the digastric muscle.
2. 111e GSA component provides sensory innervation to the face, IllUCOUS membranes
of the nasal and oral cavities and frontal sinus, hard .. te, and dccp stnlctures of
rhe head (proprioception from muscles and the temporomandibular joint). It inner-
vares rhc dum of the amerior and middle cranial fOSSo'le (suprntentorial dura).
B. Clinical correlation. Lesions result in the following neurologic deficits:
1. Loss of general sensation (hemianesthesia) from the face and mUCO\b membranes
of the oral and nasal cavities
2. Loss of the corneal reflex (afferent limb, CN V.I) (Figure 1321
68 Chapter 13
Primary neuron
Principal sensory
nucleus (eN V)

V-3
V3 (motor)
CNVII

.
<P' --

- -- .
Secondary neuron
Spinallrigeminal nucleus
$pinal trigeminal lracl
Trigeminolhalamic
pain liber
Facial nudeus
Genu eN VII-;,_.::.
Decussating
corneal renex fiber
Figure 13-2. 11K: (nnw:.l rdlc:< pmhw;'\y ....ing the Ihlce neurons :md Jeeuss.:.nion. 111ls rcllCl< is Ct)llSC'Il-
'tl,ll. IIh rhe pUllill,lry [,gill reflex. Second-order p;-ain neurons f'lI.m,1 in rhe c:m..!:J1 divblon of till,' .'l'in:,1
tril;"IIL1nal So.:.octJ'l<.I-'JI"OCr CUnIcal reticle nCU!\)lb ;Irt: ("und;1( more r""lmllc\'ds.
3. Flaccid paralysis of the muscles of masticmion
4. Dc\,jation of the jaw to the weak side as a fesult of the UIlOppOSCJ action of lhe
"Pl't)sitc later.11 pterygoid muscle
5. Paralysis of the tensor tympani muscle. which leads to hypoacusis (pani"t dc'lf
ness to low-pitched sounds)
6. Trigeminal neuralgia (tic douloureux), which is characterized by recurrent parox-
ysms of sharp. stabbing pain in one or more branches (If the navc (sec Chaptcr (0)
./
General characteristics. The abducent nerve is a purc GSE nerve that innervates the
blt::r;ll r('ClllS muscle. which alxlucts the eye.
1. It ariS{'s from the alxlucent nucleus that is found in lhe dorsomC(lial tegmentum of
thl' caudal pons.
2. Exiting intraaxial fibers pass through lile corticospinal tracr. A lesion results in
ternating abducent hemiparesis.
VI. THE ABDUCENT NERVE (CN VI)
A.
3. It passes lhrough ,he pontine cistern and c:",ernous sinus :lIld emers the orbit
through the superior orbiTal fissure.
B. CliniC'I! correlation. eN VI paralysis is the mOSt common isolated lhat resuhs
from I he long course of ,he nerve. It is seen in patientS with meningitis. Sl.lb
arachnoid hemorrhab't'. late-stage syphilis. and tT:luma. Abducent nerve paralysis reo
suits in the following defects:
Cranial Nerves 69
1. Convergent (mt-odiall strabismus (esotropia) with inabiliry to alxlucr rhe eye
2. Hori:ontal diplopia with maximullI scpararion of the Jouble images when look-
ing toward d,e paretic lateral rectus muscle
VII. THE FACIAL NERVE (CN VII)
A. General characteristics. The facial nerve is a GSA, general visceral afferent (GVA),
SVA, GVE, and SVE nelTC (Fib'llreS 13-3 and 134). It mediates facial movements,
taste, s.,livation, lacrimation, and general sensation from the external car. It is the
nerve of the pharyngeal (brachial) arch 2 (hyoid). It includes th(' facial nerve proper
(motor division), which contains the SVE fibers that inner""te the muscles of facial
(mimetic) expression. C 1 V)I includes the imennediate nen:c. which contains GSA.
SVA. and GVE fibers. All first-order sensory neurons are founJ in the geniculare gan-
glion within the temfXJral bone.
1. Anatomy. The facial nerve exirs the bmin stem in th(' cerebellopontine angle. It
enters the imernal auditory mearus and the facial canal. h then exits the facial
canal and skull through the stylomastoid foramen.
2. TIle GSA component has ce\l1xxlies locared in the geniculate ganglion. It inner-
vates the posterior surface of the external ear through lh(' posterior auricular
branch ofC VII. It projecrs cemrnll) to lhe spinal rrigcminaltract and nucleus.
3. TIle GVA component has no clinical significance. The cdl hodies arc located in
the geniculate ganglion. Fibers innervate the soft palare and the adjacent pharyn-
geal wall.
4. TI,e SVA component (taste) has cell bodies locared in the geniculate ganglion. II
......... Motor root nerve 01 CN VII
In stylomasloklloramen
CN 11
lacrimal gland
Pterygopalatine ganglion
Nasal and
palatine glands
Tongue
(taste. anterior
two-thirds)
Ungual nerve ---j;
Submandibular ganglion
Sublingual gland
CN V-2
CN V3
CN V-1
Stapedial
nerve
Trigeminal ganglion
Major petrosal nerve
Superior salivatory
nucleus (GVE)
I > I ~ I - Motor nucleus of CN VII (SVE)
Nucleus 01 solitary tract
Solitary lract (SVA)
Agure 13-3. TIle functional components of the f'lei:.1 nerve Icrani;l1 ncl'\'l,' (eN) VIII.
70 Chapter 13
UMN lesion 01
COIticobulbar tract --<iel>
(e.g. stroke of
internal capsule)
Facial nudeus of pons
Upper lace division
lower face division
LMN lesion 01 eN VII
(e.g. 8elrs palsy)
Orbicularis oris ------1,<
Muscles oIladal expression: r- ...... "
Frontalis -----+f----,,- \
"""",.ris I
Buccinator

Platysma -------1
\ J
l
Figure 13-4. Cmicobulbar innervation of the f.lcial nerve [crnnial nerve (eN) VIII nucleus. An upper mo-
tor Ilcur,m (UMN) lesion (c.g.. stroke involving the imernal c;lpsule) results in COt1lr;llilleml ,,e;lkne.s:; oi the
luwer iacc, with sparing o( the uPlx'r face. A lower motor neuron (LMN) lesion (e.g., rcsuhs in raral-
\'sis ur' thc fnci:d muscles ill both the upper and lower (iICC.
projects centrally to the solirary tr:lCt and nucleus. It innervntes the taste buds from
the :It\terior two-thirds of the tongue through:
a. The intcrmediale nerve
b. The chorda tympani, which is loc:ued in the tympanic cavity medial to the
tympanic membrane and malleus. It comains the SVA and aVE (parasympa-
thetic) fibers.
c. The lingual nerve (a branch ofCN V.3)
d. TIle central gustatory pathway (sec Figure 13-3). T."lSte fibers from CN VII.
eN IX. and eN X project through rhe solimry tmet to the solit:lry nucleus.
TIle solitary nucleus projCCts through the cenrraltegmental traci 10 Ihe ven-
tml posteromedial nucleus (VPM) of thc thalamus. TIle VPM projects 10 the
gllsralOry cortex of (he parierallobc (paricral operculum).
5. TI,e eVE component is a parasympatheric companelll that innervalcs the
Cranial Nerves 71.
lacrimal. submandibular. and sublingual glands. It contains parasym
pmhetic neurons that are located in the superior sali\'alol)' nuclell5 of Ihe caudal
pons.
a. Lacrimal pathway (see Figure 1)-]). The superior sali"<ltory nucleus projects
through the inteffilediate and greater petros.."1l nerves to the pterygopalatine
(sphenopalatine) ganglion. TIle pterygopalatine ganglion projCCts to the
lacrimal gland of the orbit.
b. Submandibular pathway (see Figure 1)3). The superior salivatory nucleus
projects through the intermediate nerve and chorda rympani to the suh
mandibular ganglion. The submandibular ganglion projecrs to and innervates
the submandibular and sublingual glands.
6. TIle SVE component arises from the facial nucleus, loops around the abJucent nu
c1eus of the caudal pons, and exits the brain stem in the cercbelloponrinc angle. It
enters the internal auditory meatus, tra\'erses the facial canal. sends a branch to
the stapedius muscle of the middle car. and exits the skull through the st}lomas.
toid foramen. It innervares the muscles of facial expression. the stylohyoid muscle.
the posterior belly of the digastric muscle, and rhe stapedius muscle.
B. Clinical correlation. Lesions (see Figure 142) cause the following conditions:
1. Aaccid paralysis of the muscles of facial expression (upper and lower face)
2. Loss of the corneal reflex (efferent limb), which may lead to corneal ulceration
3. Loss of laste (ageusi:l = gust:ltory :lnesthesia) from the anterior two-thirds of lhe
tongue, which Ill"y result from damage to rhe chorda tympani
4. Hyperacusis (increased acuity to sounds) as a resull of st:lpedius
5. Bell's palsy (peripheral facial paralysis). which is caused by trauma or inf<.'Ction
and involves d,e upper and lo.....er face
6. Crocodile tears syndrome (Iacrimmion during caring). which is a resuh o( "ber.
rant regeneration after trauma
7. Supranuclear (central) facial p."11sy, which resulrs in contralmeral we"kncss o( the
lower face, with sparing of the upper ("ce (sec Figure 134)
8. Bilateral (acial nerve palsies, which occur in Guillain-B;lffc syndrome (sec Chap.
tcr 14)
9. Mobius' syndrome, which consists o( congcnit:ll f:lci;']1 diplegia (CN VII) and con-
vergem srrabislllus (CN VI)
VIII. THE VESTIBULOCOCHLEAR NERVE (CN VIII) is an SSA nerve. It h"s 111'0 (unc-
tional divisions: the vestibular nervc, which maintains equilibrium and b"lance, and the
cochlc"r nerve, which mediates hearing (see Chapters II and I2). II exits th.:- brain srem
m the cercbellopomine angle and emers rhe imemal auditory meatus. It is confined 10 the
temporal bone.
A. Vestibular nerve (sec Figure 12-1)
1. General characteristics
a. h is associated funetion"lIy with the cerebellum (f1occulonodular lobe) anJ
ocular mOtor nuclei.
b. It regulares compensawry eyc lllovemelllS.
c. Its firstorder sensory bipolar neurons "rc located in rhe \'estibular ganglion in
the (undus o( the internal auditory meatus.
72 Chapter 13
d. It projectS its peripheral processes to the hair cells of the cristae of the semi-
circular ducts and [he hair cells of the utricle ami saccule.
e. It projects irs central processes to til(' four \'cslibul:u nuclei of Ihe brain stem
and the Ilocculonodular lobe of the cerebellum.
f. It conducls eff('rem fibers to dk' hair cells from the brain stem.
2. Clinical correlation. Lesions result in disequilibrium, vertigo, anJ ny.stagmus,
B. II-I)
1. General characteristics
a. Its first-order sensory hipolar :Irc lucateJ in the spiral (nx:hlear) gan-
glion of thl." modiolus of [he cochlea, within ,11(' temporal bone.
b. It projCCtS its peripheral 10 the hair cells of til(" Ofl,.",n of Coni.
C. It projccts irs central proce:;scs to thc JorsoiIl anJ velUml cochlear nuclei of [he
brain stem.
d. It conducts efferent fibers to the hair cells from ,he brain stem.
2. Clinical correlation. o...--structi\"(.' lesions cause hearing loss (sensorineural deaf-
ness). Irri"'th'e lesions can calise tinnitus (ear ringing). An acoustic neuroma
(schw:mnoma) is a Schwann ccllrumor of rhe cochlear nef\'e lhar causes Jeafness
{sec Chapter 14}.
IX. THE GLOSSOPHARYNGEAL NERVE (eN IX) ;,,, GSA, GVA, SVA, SVE, ,,00
eVE nCf\'c (Figure U-S).
A. General char.lcteristics. The glossopharynb'Cal nerve is primatily a sensory nef\('.
Along with C X, CN XI, and CN XII, it mediates t,lste, salivation. and swallowing.
It mediates input from the carotid sinus, which cunwins harorecepwrs th:1( monitor
arterial bloo.:l prCSSllfl'. It al50 mediates input from the carotid body. which conwins
chemorecerwrs lhal m(mitor the COl and O! concentr:uion of the blood.
1. Anatomy_ CN IX is the nerve of ph:H)'nge:l1 (branchial) arch J. It exits the br:lin
stem (medulla) from the postolivary sulcus with eN Xand CN XI. It exits the skull
through the jugular foramen with eN X and CN Xl.
2. The GSA component innervates part of the externall'ar and the eXlernal ;Iudi-
tot)' meatuS through the auricular branch of the \'agus nerve. It has celt bodies in
the supNior ganglion. It pmjens its central pr,x;es.ses tl) the spillal ttigeminal tract
nnd nucleus.
3. The eVA component innervates structures thaI arc deriveJ frolll rhe endoderm
(e.g., pharynx). It innerv:ltes the mucous memlmUles of the posrcrillr one-third
of the tongue. tonsil. upper pharynx. tympanic cnvity, and ilu,liwry tube. It also in-
nerv:IlCS the carotid sinus (bnroreccprors) ilnd carotid body (chemoreceptor,,)
through the sinus nerve. It has cell hodies in the inferior (l'cITosal) ganglion. It is
the "ffcrent limb of the b"'g reflex and the carotid simlS rellex.
4. The SVA component innerv:ltes the taste huds of thc posterior one-third of the
tongue. It has cell bodies in the inferior (petro:;al) ganglion. It projecls its central
processes to the solitary tract and nucleus (for a Jiscussion of the central pathway.
see VII A 4 J).
5. The SVE component innerva'cs only the styl<lph:lf)'ngcus muscle. It arises from
the nuclcus ambiguus of the Iateml medulla.
6. TIl(' eVE component is a parasympathetic component that innerv:ltl.'S lhe jXlrotid
glanJ. Preg:lIlglionic pamsymparhctic ncurons arc located in the inferior s:.llivatol'y
nucleus of the medulla. They project lhrough the tympanic and h,'sset peITos:11
Motor cortex k,.\---T.-UMN
<\;:>--+- Corticobubar tract
Decussation
Cranial Nerves 73
,--UMN
Medial lemniscus
Pyramid
A
amiguus
eN x (vagal nerve)
LMN
Levator veli palatini
and palatal arches
UMN lesion
P---+-LMN
B
Figure 135. Inncrvation of the palmal \lrches :md uvula. Sensory inncrvmion is 1l1L"ilimcd by thc glO$<
ncrvc [cranial nervc (eN) IXI. Motor inncrvation of the palatal arches and u\'ula IS mcdiarcd hy
Ihe VlIl.'lIS nerve (CN X). (A) A normal JXI!:nc and uvu1:l 1n:1 person who is sa)'ing (B) A JXllielll wilh:m
upper momr ncuron (UMN) lesion (leM :md a lowcr mowr neuron (L\iN) lesion (righl). When !Ius p:llieOl sa)'S
M Ihe palatal arches sag. The uvula deVlalCS lhc intacl (Iefl) side.
nerves 10 lhe olic ganglion. Poslganglionic fibers from {he mic ganglion projecl to
the pnrotid gland through the auriculolemponll nerve (eN V-3).
B. Clinical correlation. Lesions calise the follow;r,g condirions:
1. Loss of the gag (pharyngeal) reflex (intemlprion of the afferenl limb)
2. Hypersensitive carotid sinus reflex (syncope)
3. Loss of general sensation in [he pharynx. tonsils, fauces. and back of [he tongue
74 Chapter 13
4. Loss of tllsle from Ihe posterior one-thinl of Ihe tongue
5. Glossopharyngeal neuralgia. which is char.lCteri:ed by sc\"cre stabbing pain in the
rout of the wng\IC
X. THE VAGAL NERVE (eN X) is a GSA. GVA. SVA, SVE. and GVE ner\"e (,)\."'C fig-
ure 13-5).
A. General characteristics. The \'aboal nerve mediates phonation, swallowing (wilh CN
IX. eN XI. and C XII). c1e\';ltion of Ihe pabte, taste, and cUlancous scns.-"i...lO from
the ear. II inner\'ates the \'iscera of th<.' neck, thorax, and abdomen.
1. Anatomy, Th.. vagal nen'e is Ihe n('rv(' of pharyngeal (bmchial) arches 4 and 6,
Pharyng<.'nl nrch 5 is cilher absent or nk.limentary. It exits Ihe brain slem (medulla)
from the !XlSI,.,lhfll) sulcus. It exits the skull dlfough the jugular foralllen with CN
IX eN Xl.
2. The GSA component innervates the infr;atclllorial dum, external car. ext('rnal au-
diTOry lIle;l!US, :lnd tymp:mic membrane, J t has cclllxx1ies in till' superior (jugular)
ganglion. and it projects its ccntml processes to the spinal trigeminal tmcl nu-
cleus.
3. eVA component innen'aH's thl' mucous membmnes of Ihe phnrynx. larynx.
l'sophagus. tmche:l. anJ thomcic and abJominal \'iscem (0 the left colic flexure).
It has ccll bo.xlies in Ihe inferior (mxlosc) ganglion, II projects its celllr.ll processes
to th.. sOlitaf)' trOlo.;t :lnd nucleus.
4. The SVA component innervates the (aSle buds in the epiglottic region. It has cell
in til\.' inft'rior (noJosc) ganglion. It projects its ccnlmll'roc..SSC's ro til\' s<11i-
fal)' 1r,ICf and nudeus. For a discussion of the ccntml pathway. S("C VII A 4J.
5. TIn' SVE component inner\'ates rhe phaf)'ngcal (brachial) arch tnllsck-softhe lar-
ynx anJ phal)'nx, [he stri:ucJ lllll:>cle of the upper esophab'US. the muscle of the
U\'ula, anJ Ih" It.'vawr \'eli palmini anJ palatoglossus muscles. It f<."'Cci\"es SVE in-
put from rhe cmnia! di\'ision oflhe spinal acccssof)' ner....e (CN XI).lt arises fmm
Ihe nuclt'us amhib'\llIS in rhe Iateml medulla. TIle SVE component provides the ef-
ferent limh of the gag renex.
6. TIll' eVE componcnl inncn'mes the viscer:l of the neck and the thomcic (heart)
:IllJ ahJuminal cadties;ls f:lr as lhe lef, colic flexure. pamS)'lllpa-
fhetic neurons Ihal :Ire located in the dorsal1l10IQr nucleus of the medulla project
to 111<: rennin:ll (inrr.lIlurml) ganglia of the \<isc('ral organs (sec Figult' 18-2 and
T:rble 18-1).
B. Clinic:11 correlation. Lesions and reflexes C(lllse the following conditions:
1. Ipsilatcral pimllysis of the soft palnte, phnrynx, anJ laf)nx dmr leaJs to dyspho-
nia (hU:lrscness). dyspnea. dysarthria. and dysphagi:l
2. Loss of the g:lg (p.1latal) reflex (efferent limb)
3. Anesllu'sia of Ihe pharynx and larynx that leads to unilateml loss of the cough
rdlex
4. Aortic aneurysms and tumors of the neck and thomx Ihat frequentl)' compress
the \"agal ner...e
5. Completc laryngt.":I1 paralysis. which can be mpidly fatal if it is bilateml (asphyxia)
6. Parasymp.1thctic (wgelati\,e) disluroonces, including bradycanlia (irrirmin' le-
sion). rachycanlia (destructi\'e lesion). and dilation of the stomach
Cranial NeNes 75
7. The oculocardiac reflex, in which pressure on the eye slows the heart rare (affer-
ent limb of CN VI and efferent limb ofCN X)
8. The carotid sinus reflex, in which pressure on the carotid sinus slows the heart
rmc (bradycardia) lefferent limb ofCN XI
XI. THE ACCESSORY NERVE (CN XI), or spinal accessory nerve, is an SVE nerve (Fig-
urc 13-6).
A. General characteristics. The accessory nen'e mediates head and shoulder movement
and innervates the laryngeal muscles. It has the following divisions:
1. The cranial division (accessory portion), which arises from the nucleus ambiguus
of the medulla. It exits the medulla from the posrolivary sulcus and joins the va-
gal nerve (CN X). It exits the skull through rhe jugular foramen wirh CN IX and
CN X. It innervates the intrinsic muscles of the larynx through the inferior (re-
current) laryngeal nerve, with the exception of the cricothyroid muscle.
2. The spinal division (spinal portion), which arises from the ventral hom of cervi-
cal segmenrs Cl through C6. The spinal roots exit the spinal cord laterally be-
tween r1w vemml and dorsal spinal rOO[5, ascend rhrough the foramen magnum,
and exit thc skull through the jugul::l.r foramen. It innervates the
mastoid muscle with the cervical plexus (C-2) and the trapezius muscle with rhe
cervical plexus (Co3 and C-4).
B. Clinical correlation. Lesions cause the following conditions:
}-------!f-- Facial nucleus WI pons
CN
Ambiguus nucleus
in medulla ---++1
Jugular foramen
CNIX--
CNX--t--O
II
ep-CNXI
t--t---Accessory nucleus
in spinal cord (C1-C5)
Figure 1J..6. The crnnial and spinal di"isioru; of acces:sory nerve (cranial nerve (eN) lX). The cranial
di\'ision hitchhikes a ride wim the accessory nerve. men joins the vagal nerve to become me weriDt (recurrent)
hlf)'I\l,.>C;11 ner....e. The recurrem laf)'fll,.ocal nef"e innef''ates the intrinsic muscles of me larynx, except for the
cricothyroid muscle. The spin:11 dh'ision innef''ates the trapezoid and stemocleidomasroid muscles. Three nerves
pltSS through the jugular foramen (glomus jugularc tumor).
76 Chapter 13
1. Paralysis of the sternocleidomastoid muscle that results in difficulty in turning
the head ro the contralateral side
2. Par.llysis of the trapezius muscle that results in shoulder droop and inability ro
shrug the shoulder
3. Paralysis of the larynx if the cranial root is invoked
-'--UMN
o '
o --UMN
Corticobulbar tract --cb
ct>-- Corticobular tract
Decussation
\/
\
LMN
LMN lesion
(flaccid paralysis)
UMN lesion
(spastic paralysis)
Decussation --",
LMN
Hypoglossal nerve
c
o
Pyramid
Medial lemniscus
A B
Figure 13-7. Mowr inner,,;uion of Ihe 101lj,'lIC. Conicobulhar lil>cn; proje<1 preJorn;n:mtly 10 the comr;:lbl-
er.,1 hYJXll;lossal n u d c u . ~ . An upper lllotor neuron (U/I.IN) lesion causes JC\'iat;on 0( the pr(l(nxkoJ 10nl,'UC to Ihe
we;'1,; (comrahlternl) Side. A lower motor neuron (L\1N) lesion c:m>C5 deviation of tile 1'r(l(n1dcd 101'b'Ue 10 Ihe
lI'e;,1,; (IJblhueflll) side. (A) Nomlal tOIlI.'lIC. (B) TOIll,'UC wilh UMN and LMN lesions.
Cranial Nerves 77
XII. THE HYPOGLOSSAL NERVE (eN XII) is a GSE nerve (Figure 13-7).
A. General characteristics. The hypoglossal nen'(' mediates tongue movement. It arises
from the hypoglossal nucleus of rhe medulla and exirs the medulla in the preoli\'ary
sulcus, It exirs du," skull through rhe hypoglossal canal, and it innervates the intrinsic
and extrinsic muscles of the tongue. Extrinsic muscles arc the genioglossus. str-
loglossus, and hroglossus.
B. Clinical correlation
1. Transection resuhs in hemiparal)'sis of the tongue.
2. Prorrusion causes the lOngue to point 1O\I:aru the weak side bc<:ausc of the unop-
posed acrion of the opposite genioglossus muscle.
1.4
Lesions of the Brain Stem
I. LESIONS OF THE MEDULLA 14-1)
A. Medial medullary syndrome (anterior spinal artery syndrome). Affected structures
and rcsulmm dellciLS include:
1. The corticospinal tract (medullary pyramid). Lesions result in commbtcral spas-
tic hemiJXlresis.
2. The medial lemniscus. Lesions rcsuh in contralatcmlloss of wetile and vibration
scnso1tion from the trunk and extremities.
3. The hypoglossal nucleus or intraaxial rOOI fibers rcr.mial nerve (eN) XII]. Le-
sions h.-sult in ipsiialCrnl flaccid hcmiparalysis of the longue. When promlded. the
longue points 0 the sid' of the k'Sion (i.e., the weak side). Sec Figure IJ. 7.
Vestibular nuclei
Homer's lrad
Inferior cerebellar peduncle
Spinal lrigemlnal Iract and

Nucleus ambiguus
Lateral spinothalamic tract
CNXII
Pyramid
Medial lemniscus
NudeIJS of solitary tract
Dorsal molor nucletJs 01 eN x
Hypoglossal nucleus

A
Figure 14-1. Vasculllr lesions of the cauu.11 pons at the level of the hyJlOt:los.s...1nuclcus of cranial nerve (CN)
XII :IIlU ,hc dorsallJ'lO(of nucleus of CN X. (A) McJi:11 m... -Jullliry (arlcrial spmal :uu::ry). (8) L'ucral
lllC\lulbry lrosu:rior inferior cerehcllar ;Inel)' (PICA)I syndrome.
78
lesions of the Brain Stem 79
B. Lateral medullary syndrome Lposterior inferior cerebellar artery (PICA) syndrome]
is characterized by dissociated scn;;ory loss (see I B6---7). Affe<:too stnlClll!\.'S anJ re
sultant deficiu include:
1. The vestibular nuclei. Lesions rcsuh in nausea. vomiting. and \'enib'O'
2. TIle inferior cerebellar peduncle. Lesions result in ip$ilatcral cerehcllar signs le.g..
dysmcnia (pas( poiming), drsdi3dochokincsb].
3. TIle nucleus ambiguus of CN IX, eN X, and eN XI. Lesions result in ipsil:neml
laryngeal. pharyngeal, and palatal hemipamlysis li.e.. loss of the l;:ag reflex (effer-
ent limb). dys.'mluia. dysphagia. and dysphonia
4. The glossopharyngeal nerve roots. Lesions result in loss of the b':lg reflex (,Ifferelll
limb),
5. The vagal nerve roots. Lesions result in the sallie deficit.s as seen in lesions in-
volving the nucleus ambigllus (sec I B).
6. The spinothalamic tracts (spinal lemniscus). Lesions result in conrralarcralloS$
of pain and tcmpenll'ure sensation frOllllhe trunk and exrremities.
7. The spin'll trigeminill nucleus and tract. Lesions result in ipsilateral loss of r:lin
and temperalllrc sensation from the face (faci:ll hemianesthesia).
8. TIle descending sympathetic trdCI. Lesions result in ipsibter:ll Horner's syndroml'
(i.e.. ptosis. miosis. hemianhidrosis. and apparelll enophthalmos),
II. LESIONS OF THE PONS (Figure 14-1A)
A. Medial inferior pontine syndrome results from occlusion of the paramedian branches
of the basilar artery. Affe<:ted structures and resultant deficits include:
1. The corticospinal tract. Lesions result in comrnlateral $p<,stic hemiJY.lresis.
2. The medial lemniscus. Lesions result in contrnlaternllossof tactile sensation from
the trunk and cxtrcmitit.'S.

Medial lemniscus
Corticospinal traCl
Vestibolar (MJClei
Abducent nucleus
Lateral sPinothalamiC traCl
CN VIII (vestibular nerve)
CN VII
Nucleus CN VII
Spinal trigeminal nucleus and tract
Agure 14--2. Vascular lesions 'If the cltl"bl pon":11 the level of the abducent nucieUli of cmnlal ncr\"C (CN)
VI lInJ the facial nucleUli of CN VII. (Al mfcrior ponllne SynJrOfnC. (8) L'ller,ll inferior pontine syn-
drome 1:IIlICrior inferior cerebellar :lrlCI)' (AlCA) synJromel. (C) Mcdiallongitudmal fa:.ciculw. (MLF)
dn:lInc.
80 Chapter 14
3. The abducent nerve roors. lesioll5 resuh in ipsilmerallareral reclUs paral}'sis.
B. Lateral inferior pontine syndrome lamerior inferior cerebellar artery (AICA) syn-
drome) ..ure 14-28). Affccled ,.tNc!Ures and resultant deficits include:
1. The facial nucleus and intraaxial ncr\'{' fibers. lesiuns resulr in:
a. Ipsibrernl filcial nervc p:unlrsis
b. Ipsibtemlloss of mste from the illllcrior lwo-lhirds of rhe TOngue
c. Ipsibtemlloss of Iflctil1l:tt iOll lind reduced salivlll ion
d. nf cornc;ll ;lnJ stapedial reflexes (efferent limbs)
2. TIle cochlear nuclei and intraaxial nerve fibers. result in unilaternl cen-
nal Jcafnl'ss,
3. TIl{' veslibular nuclei and intraaxial nerve fibers. lesions result in nySlagmus.
nausea. vomiting. and \,('(tigo.
4. The spinal trigeminal nucleus and tract. lesions result in of rain
and tempcrarurc S('ns.'lrion from Ihe face (facial hemianesthesia).
5. The middle and inferior cerebellar peduncles. lesions result in ipsilatemllimb
and gait dysraxia,
6. The spinothalamic tracts (spinal lemniscus). lesions rcsuh in conrmlrHemlloss
of pain and temperarure semalion from Ihe Trunk and extremilies.
7. The descending sympathetic trl.lct. lesions result in ipsilareml Horner's syndrome.
C. Medial longitudinal fasciculus (MLF) syndrome (internuclear ophlhalmoplegia)
[sec Figure 14-2CI interrupts fibers from rhe conrr.lbtcral abducent nucleus Ihal pro-
jecl, lhrough rhe MLF. to rhe ipsilareral medi:ll rectus subnucleus ofCN Ill. It causes
medial rectus pals}' on aucmproo lateral conjugate ga:e and nystagmus in the ab-
ducring c)'e. Convergence remains inmcr. This syndromC' is ofren seen in pmicms wilh
multiple sclerosis.
O. Facial colliculus syndrome usually resulrs from a pontine or a \'ascular acci-
denr. The internal g.:-nu ofC VII and the nucleus ofCN VI underlie the facial col-
liculus.
1. Lesions of the internal genu of the facial nerve cause:
a. Ipsilateml f.Jcial paralysis
b. Ipsilaremlloss of the corneal reflex
2. Lesions of the abducent nucleus cause:
a. Lateral rcctus paralysis
b. Medial (wnvergent) strabiSlllus
c. Hori:ontal diplopia
III. LESIONS OF THE MIDBRAIN (F;g"" 143)
A. Dorsal midbrain (Parinaud's) syndrome (Sl.'C 14jA) is often lhe r('Suh of:1
pinealoma or germinoma of rhe pineal region. AffL'CtN structures and resulmnt
Jeficils include:
1. The superior colliculus and prelt.'Ctal area. Lesions cause paralysis of upwarJ anJ
downward ga:e. pupillary disturbances, and absence of com'ergence,
2. TIle cerebral aqueduct. Compression causes noncommunicating hydrocephalus.
B. Paramedian midbrain (Benedikt> s}'ndrome (sec Figure 14313). Affecred strucltlres
nnd resultant deficirs include:
Lesions of Ihe Brain Stem 81
Red nucleus
c
Medial lemniscus
IY:rt--+- Dentatothalamic
tract
A
Posterior commissure and center lor verticaJ conjugate gaze
St4lerior colliaJlus
Nucleus of CN III
Spinothalamic tract

Corticobulbar tract
Substantia nigra -1--+
Conicospinallract
Medial geniC1Jlate body
CN III
Figure 14-3. Lesions of the rostral midbrain at the level of the superior collieulus and oculomotO nucleus
ofemnial ne....c (CN) Ill. (A) Dorsal midbmin (Parinaud's) syndromc. (B) rammedian midbrain (Benedikt) syn-
drome. (C) Mcdial midbrain (Weber) syndrome.
1. The oculomotor nerve roots (intraaxial fibers). Lesions cause complete ipsilateral
oculomotor pflrfllysis. Eye abduction and depression is caused by the intact lateral
rectus (eN VI) and superior oblique (eN IV) muscles. Ptosis (paralysis of the le-
vator palpebra muscle) and fixation and dilation of the ipsilateral pupil (complete
internal ophthalmoplegia) also occur.
2. The dentatothalamie fibers. Lesions cause contralateral cerebellar dystaxia with
intenrion nemor.
3. The medial lemniscus. Lesions result in contralateral loss of tactile sensation from
the trunk and extremities.
C. Medial midbrain (Weber) syndrome (see Figure 14-3C). Affected structures and re-
sultant deficits include:
1. The oculomotor nerve roots (intraaxial fibers). Lesions cause complete ipsilateral
oculomotor paralysis. Eye alxluction and depression is caused by intact lateral rec-
tus (CN VI) and supetior oblique (CN IV) muscles. Ptosis and fixation and dila-
tion of the ipsilateral pupil also occur.
2. The corticospinal tracts. Lesions result in contralateral spastic hemiparesis.
3. The eorticobulbar fibers. Lesions cause contralateral weakness of the lower face
(eN VII), tongue (CN XII), and palate (CN X). The upper face division of the
facial nucleus receives bilateral conicobulbar input. The uvula and pharyngeal
wall are pulled toward the normal side (CN X), and the protruded tongue points
to the weak side..
IV. ACOUSTIC NEUROMA (SCHWANNOMA) [Figure 14-4J is a benign rumor of
Schwann cells that affects the vestibulocochlear nerve (CN VlIl). It accounts for 8% of
all intracranial tumors. It is a posterior fossa tumor of rhe internal auditory meatus and
cerebellopontine angle. The neuroma often compresses the facial nerve (CN VII), which
accompanies eN VIII in the cerebellopontine angle and internal auditory meatus. It may
impinge on the pons and affect the spinal trigeminal tract (CN V). Schwannomas occur
twice as often in fcmalcs as in males. Affccted structures and resultant deficits include:
82 Chapter 14
Figure 14-4. rC>.Oll:mce image of an acousrlc neuroma. This cororml "hows dilalion of lhe
\cnmck.". The "c"lIhulocochll:;lr ncr\'(' is "biblc in the Icft imemHl audlf{)('}' lI\('atus. Thc Ilunor indent:> Ihc hlr
o:rall"'lh. Cmnl,,1 ner\'e (CN) Imbics induJe CN V, VII. anJ VIII. Syml'lOllb includ... ullilmer..11 ,Ie"focss. f,I(I,,1
,me:.lhe:.I.1 ,Illd "...,llme.. and an ;lhscnt coronal rcllcx. Tllb is ,I Tl-weighrcd llll.l/:e.
A. The cochlear nerve of CN VIII. Damag... resulrs in tinnitus and unilal'cral ncrvcdeaf-
ness.
B. The vcstibuhlr nerve of CN VIII. Damage rl'sults in "crt igo. nystagmus, nausea, ,'om-
iting, and unsteadiness of gait.
C. The facial ncrvc (eN VII). Dmn<lge resulrs in f<lcialweakness <lntl loss ofthecorne:ll
reflex (efferent limb).
D. The spinal Iril;cminal tract (CN V). Da1ll<lge results in p:lresthesin. <lnest hesia of the
ipsilateral face. and loss of the corneal reflex (afferent limb).
E. Neurofihromatosis type 2 often occurs with bibleral acousric neurumas.
V. JUGULAR FORAMEN SYNDROME usu<ll1y results frum a posterior fossa tumor (e.g.,
l:lomus jUl:ul:lrc Itlmor, rhe most common inner e;lT tumor) thm compresses eN IX. X,
;mtl Xl. Affecled snucturcs and resultant deficits include:
A. The glossopharyngeal nerve (eN IX). D;l111age results in:
1. lpsilarernlloss of rhe gag reflex
2. lpsilaternlloss of pain, tempcrnture. and taste in the tonl,'Ue
B. The ,ragal nenrc (eN X). Damage results in:
1. Irsilmeml pamlysis of the soh and larynx
2. lpsil:ucmlloss of the l,':Ig reflex
Lesions of the Brain Stem 83
C. TIl(' accessor)' nerve (eN XI). Damage result's in:
1. Paralysis of the sternocleidomastoid muscle. which results in rhe inabiliry ro rum
the head (0 the opposite side
2. Paralysis of the tr.lpc:ius muscle. which cauS(.'S shoulder droop and inability to
shrug the shoulder
VI. "LOCKED-IN" SYNDROME is a lesion of the base of [he pons as [he result of infarc
[ion, trauma. tulllor. or demyelination. TIle corticospinal and conicobuloor tracts arc af
Leh SCLA

VA
ASA
BCT

Leh SCLA
VA
BCT
'"

MeA MCA ACOM
ICA
e-
SCA
AICAJ,EA
ECA
PICAjof
ECA
I
ASA
Aorta Aorta
A II B II
Figure 14-5. An,uomy of the suhcf:I\'ian s\'nJrome. Thrumbosis of Ihe proxill'l.'ll pan o( the subcb\'ian
,mery (kfl) rcsulrs in retrograde hlooJ no\\' dnough d'lC ipsi!mcml \'enebr.11 artery and imo the Ic(t M1hcb\'ian
,mery. Blood can be shumoo (rom Ihe righl vertebral anery ,md down the left \'enebral 'lrll:ry (A), Blood m,ly
.. Iso reach the le(t \'cnehr.11 anery through thc cafO(id circuhnion (8). ACA = ,u\lcriorccrebell.. r anc!)'; ACOM
=alllcrtor communic,lling ,InC!)'; AICA =anterior infcrior cerebellar .me!)'; ASA = amcrior spinal :Inc!)'; BA
.,. basi1;1r ,InC!)'; BCT = br.loChioc:cphalic lrunk: CCA = comlllon c.lfO(iJ anery; ECA = CXlen'l.,1 cafO(id ancry;
ICA = imemal carOlid :mcry; MCA "" middle cercbr.11 :mcl)'; PeA ,.. JlOSlcrior cerebral tlnc!)'; PCOM = por
Icrior communic:uing ancry; PICA = po:.terior infcriorccrchclhlr :U1cry; SCA = superior communicating :mc!)';
SCtA = subchwi:m anery; VA = vcn('br.ll artcry,
84 Chapter 1.4
feered bilaterally. The oculomotor and trochlear nerves are not injured. Patients arc con-
scious and may communicate through vertical eye movements.
VII. CENTRAL PONTINE MYELINOLYSIS is a lesion of Ihe base of the pons that affccls Ihe
corticospinal and corticobulbar tracts. More than 75%ofcases are associated with alcoholism
Of rapid cOlTCCtion of hnXlfIatremia. Symptoms include spastic Quadriparcsis, pseudobulbar
palsy, and mental changes. This condition may become the locked. in syndrome.
VIII. "TOP OF THE BASILAR" SYNDROME results from embolic occlusion of the rostral
basilar artery. Neurologic signs include optic ataxia and psychic paralysis of fixation ofgaze
(Balint's syndrome), ectopic pupils, somnolence, and conical blindness, with or without
visual anosognosia (Anton's syndrome).
IX. SUBCLAVIAN STEAL SYNDROME (Figure 14-5) results from thrombosis of the left
subclavian artery proximal to the vertebral artery. Blood is shunted rerrograde duwn (he
left vertebral nrrery :md into the left subclavian artery. Clinical signs include transient
wenkncss nnc! claudication of the left arm on exercise and verrcbrobnsilar insufficiency
(i.e., verrigo, dizziness).
X. THE CEREBELLOPONTINE ANGLE is rhe junction of the medulla, pons, and cere-
bellum. eN VII and Vll1 arc found there. Five brain tumors, including a cyst, are often lo-
Cated in thecerebcllopontine angle cistern. Remember the acronym SAME: schw:mnoma
(75%), arachnoid cySt (I %), meningioma (10%), ependymoma (I %), and epidermoid
(5%). TIle percentages refer to cerebcllopomine angle tumors.
1.5
Cerebellum
I. FUNCTION. The cerebellum has three primary functions:
A. Maintenance of posture and balance
B. Mainlcnancc of muscle tone
C. Coordination of voluntary mOlor acth'ity
II. ANATOMY
A. Cerebellar peduncles
1. TIle superior cerebellar peduncle contains the major output (rom the cerebellum,
rhe dentatotha1:unic mer. 111is rrae! !Cnninnles in the \'cntmll:ucml nucleus of
the thalamus. It has one major affeTenr pat hway, the ventral spinocerebellar tmcr.
2. 111e middle cerebellar peduncle receives pontocerebellar fibers. which project to
the neocerebellum (ponroccrcbcllum).
3. The inferior cerebellar peduncle has three major afferent tracts: the dorsal spi-
nocerebellar tract, the cuneocerebellar tract, and fhe olivoccrcbcllar [mCl (rom the
conrrabrcml inferior olivary nucleus.
B. Cerebellar cortex, neurons, and fibers
1. The cerebellar cortex has three layers.
a. The molecular layer is the outer layer underlying the pia. It contains stellate
cells, basket cells, and the dendritic arbor of the Purkinje cells.
b. The Purkinje cell layer lies between the molecular nnd rhe gramlle ccillayers.
c. The granule layer is rhe inner layer overlying the whire maner. It contains
granule cells, Golgi cells, and cercbell:1r glomeruli. A cerebeiLu glomerulus
consists of a mossy fiber rosette, granule cell dendrites, and n Goigi cell axon.
2. Neurons and fibers of the cerebellum
a. Purkinje cells convey the only output from the cerebellar cortex. They pro-
jeer inhibitory output [Le., "Y-mllinoblltyric acid (GABA)] to the cerebellar
and nuclei. These cells arc exciTed by parallel and climbing fibers
and inhibited by GABA-ergic basket and stellate cells.
b. Granule cells excite (by way of gluramatc) Purkinje, basket, stellare. and
Golgi cells through pMallel fibers. They ;Ire inhibited by Golgi cells and ex-
cited by mossy fibers.
c. Par-illel fibers are the axons of granule cells. These fibers extend into the mo-
lecular layer.
d. Mossy fibers are the afferent excitatory fibers of the spinocerebellar, pontO-
85
86 Chapter 15
cerebellar, and wSlibuloccrcbellar tracu. They Icrminale as moss)' flber
roscues on granule cell dendrites, The)' excilc granule cells to discharge
Ihrough their parallel flbers.
e. Climbing fibers are Ihe afferent excirawT)' (b)' \\'3)' of aspartalc) fibers of Ihe
oli\'ocerebellar trael. These fibers arise from the contralateral inferior olh":lty
nucleus. They terminate on neurons of the cerebellar nuclei and dendrites of
Purkinje cells.
III. THE MAJOR CEREBELLAR PATHWAY (Figure 15-1) consisls of Ihe following
structures.
A. The Purkinje cells of the cerebellar cortex projeci to the cerebellar nuclei (e,g., den-
nne, cmooliform, globose, and fasligial nuclei),
B, The dentate nucleus is the major eff('clOr nucleus of the cerebellum. It gi\"Cs rise t(1
the dentalOl halamic traCI, which projects I hrough the superior cerebellar peduncle to
the conlrnlnleral venl'rall:n\'rnl nucleus of the rhalamus. The decussation of the su-
perior cerebellar peduncle is in the cnudal midbrnin tCgllll'ntUIll.
COfIicopontine neuron
Corticospinal neuroo
Ventral lateral
nucleus 01 thalamus
Internal capsule
(posterior limb)
Lentiform
""""'"
,.,..!>"""r- Red nucleus
Emboliform Superior cerebellar
peduncle
Dentate nucleus -f7''''-..",''
Pyramidal decussatioo
Middle cereoenar peduncle
Rubrospinal tract
Corticospinal tract
Agure 15-1, The priocip.... l ccrchdbr conne<:tions. l1lc major c((crcm is Ihe OCIU:lI01h:,brllO(:or
r1CallT:lCI. TI1C ccrehcllunl rl"Cl."i\'('! inpul (rom Ihe ccrl."bml COrlCX Ihrough the COrlicopomocl."rcbclbr ImCI.
Cerebellum 87
C. The ventral lateral nucleus of the thalamus receives the dentalOthalamic tract. It
projects to the primary mOlOr cortex of the precentral gyrus (Brodmann's area 4).
D. The motor cortex (motor strip, or Brodmann's area 4) receives input from the ven
trallareral nucleus of the thalamus. It projects as the corticopontine nac[ to the pon
tine nuclei.
E. The pontine nuclei receive input from the motor cortex. Axons project as the ponto-
cerebellar rmcr [0 the contralateral cerebellar cortex, where they terminate as mossy
fibel1i, thus completing the circuit.
IV. CEREBELLAR DYSFUNCTION includes the following triad:
A. Hypotonia is loss of the resistance normally offered by muscles to palpation or passive
manipul:niol\. It results in a floppy, loose-jointed, rag-doll appearance with pendular
reflexes. The patient appears inebriated.
B. Dysequilibrium is loss of balance characterized by gait and trunk dystaxia.
C. Dyssynergia is loss of coordinated muscle activity. It includes dysmetria, intention
tremor, failure 10 check movements, nystagmus, dysdiadochokinesia, and dys-
rhythmokinesia. Cerebellar nystagmus is coarse. It is more pronounced when the
patient looks toward the side of the lesion.
V. CEREBELLAR SYNDRDMES AND TUMDRS
A. Anterior vermis syndrome involves the leg region of the anterior lobe. It results from
atrophy of the rostral vermis, mOSt commonly c:lused. by :llcohol :loose. It causes gait,
trunk, and leg dystaxia.
B. Posterior vermis syndrome involves the flocculonodular lobe. It is usually the result
of brain tumors in children and is most commonly caused by medulloblastomas Ot
ependymomas. It causes truncal dystaxia.
C. Hemispheric syndrome usually involves one cerebellar hemisphere. It is often the re-
sult of a brain tumor (astrocytoma) or an abscess (secondary to otitis media Ot mas-
toiditis). It causes arm, leg, and gait dystaxia and ipsilateral cerebellat signs.
D. Cerebellar tumors. In children, 70% of brain tumors are found in the posterior fossa.
In adults, 70% of brain tumors are found in the supratentorial compartment.
1. Astrocytom<ls constitute 30% of all brain tumors in children. They are most often
found in the cerebellar hemisphere. After surgical removal, it is common for the
child to survive for many years.
2. Medulloblastomas are malignant and constitute 20% of all brain tumors in chil-
dren. They are believed to originate from the superficial granule layer of the cere-
bellar cortex. n,ey usually obstruct the passage of cerebrospinal fluid (CSF). As a
result, hydrocephalus occurs.
3. Ependymomas constitute 15% of all brain tumors in children. They occur most
frequently in the fourth ventricle. They usually obstruct the passage of CSF and
cause h)drocephalus.
..
1.6
Thalamus
I. INTRODUCTION. The thalamus is the largest division of Ihe diencephalon. II plays an
impon;l.m role in the integration of the .sensory and mawr systelllS.
II. MAJOR THALAMIC NUCLEI AND THEIR CONNECTIONS (Figu", 16.1)
A. The anterior nudcus rccein's hypothalamic input from the ,"amillary nucleus
rhrough rhe mamillothalamic tract. It projectS lO [h(' cingulOllc .l,,'ynls and is part of rhe
Pape: circuit of emotion of rhe limbic system.
B. TIle mediodorsal (dorsomooial) nucleus is reciprocally connected to the prefrontal
cortex. II has abumlam connections wid, inrmlaminnr nuclei. It rccci,cs input from
the :JmYl.tdala, .subsmntia nigra. and temporal neocortex. When it is (k'Stroyed, mem-
ory loss occurs (Wcrnickc-Kors.1koffsyndrome). The mwiooors::ll nucleus plays a role
in rhe expression of affect, emotion, and beh:wior (limbic (unction).
C. The centromeclian nucleus is the largest intmkunin:u nucleus. It is redprocally con-
nected to the motor cortex (Broomann's arca 4). TIle centromooian nucleus receives
inpur from the globus pallidus. It projects to the strinrum (caudate nucleus and puta-
men) and projects diffusely to the emire neocortex.
D. The pulvin:lr is the largest thalamic nucleus. It has reciprocal connections with the
association cortex o( the occipital, parietal, and JX>Stcrior tcmpor:lllobes. It receives
input from the lateral and medial geniculate Ixxlies and the superior col1iculus. It plays
a role in the integriltion of visual, auditory, i1nd somcsthctic input. Destruction of
the domin:lnt rulvinar may result in sensory dysphasia.
E. Ventral tier nuclei
1. The ventr,ll anterior nucleus receives inpur from the globus pallidus and sub-
st:mti:l nigra. It projects diffusely to the prefrontal cortex, orbital cortex, :md pre-
morar cortex (Brodmann's area 6).
2. The ventrill [ateral nucleus receives input from the cerebellum (dentate nucleus),
globus pallidus, and substanria nigra. It projects ro the motnr cortex (Broomann's
arm 4) and the supplementary mntor cortex (Brodmann's area 6).
3. The ventTOl1 posterior nucleus (ventrob:ls:ll complex) is the nucleus of termin:l-
tion ofgeneral somatic afferent (fotlCh, pain, and temper:llure) and spedal visceral
afferent (taste) fibers. It has twO subnuclei.
a. The ventTOl1 posterolateral nucleus receives the spinOlhalamic tmcts and the
Hll'di:l[ lemniscus. It projecrs to the somesthctic (sensory) cortex (Brodmann's
areasJ.l,and 2).
b. The \'entral posteromedial (VPM) nucleus receives the rrigeminothalamic
tracts and projects to the somesrhctic (sensory) carrel( (Brodmann's are:lS J, I,
and 2). TIle gustatory {tasre) pathway originates in the solitary nucleus and
Thalamus 89

Anteriof'
nuclear group ntemal medulary lamina ____.1
VPL
8
Mediodorsal
nucleus
Pulvinar
lateral geniculate geniculate body
Me<ial1emnisaJs }
Spinothalamic tracts
(
Amygdaloid """""
Tempol'al neocortex
Substantia nigra
Prefrontal cortex
.. {AfeaS18and19
Inferior parietal lobule

{Inferiof ooiliculus Lateral lemniscus


Areas 41 and 42
Optic tract
Area 17
Cingulate gyrus
Area 4
Dentate nUCleUS)
Globus paliidus
Slbstantia nigra
Area 6 )
DlHuse frontal cortex

Mamitlothalarnic tract}
Fornix
Globus pallldus }
Substantia nigra
Areas 3, 1, 2
Trigeminothalamic
tracts and Iaste palhways
Figure 16-1. M:ljOf th:lbmi( nudci :md their (Olllll..'(lions. (A) Don;ohuer.11 aspt."CI :lnd Ill"jor nuclei, (8)
Major afferem and efferent (onncnions. VA = vcnlr.ll allicrior nuclcus; VL = VClllr.lll:llCr.11 nuclcus; VPL =
vcntml poo;tcrior l:lIeral nuclcus; VI'M = \'cmml poslcrior meJial nucleus.
projects via the cemraltegmelllallTact to VPM, and thence to the gustatory
cortex o( Ihe postcentral gyrus (Brodmann's area 3b), o( the (ramal opercululll
and insular cortex. The taSle pathway is ipsilateral.
F. Metathalamus
1. TIle lateral geniculate body is a visual rela)' nucleus. It receivcs retinal input through
the optic tmcl and projccrs to the primary visual cortex (Brodmann's area 17).
2. The medial geniculate body is an auditory relay nucleus. It reh'cs auditory in-
pur through the brachium o( the inferior colliculus and projts to the primary au-
ditory (Quex (Brodmann's areas 41 and 42).
G. TI,C reticular nudcus of thalamus surrounds (he (halamus as a Ihin lorer of 'Y-aminobu-
lyric acid (GABA)-ergic neurons. II li<:s betwccn the external tm.. -dullary lamina and the
imernal capsule. It receives cxcit<lIol)' collateral input from corricothalamic and thala-
mucortical fibers. It projects inhibitory fibers to thalamic nudei fr01l1 which it n:{:eivcs
input. It is thought tn pIny ;l role in nQrmal clcctrocncephaolgrarn rendings.
90 Chapter 16
III. BLOOD SUPPLY. The thalamus is irrigmed by three arteries (see Figure J.).
A. The posterior communicating artery
B. The posterior cerebral artery
C. The anterior choroidal arter)' (Iatcral body)
IV. THE INTERAL CAPSULE (Figure 16l) is a layer of white matter (myclinau.-d axons) that
separates the caudate nucleus and the thalamus medially from the lentiform nucleus klTerally.
A. The anterior limb is located between the cllud:1te nucleus :md the lentiform nucleus
(globus pallidus and putamen).
B. The genu contains the corticobulbar fibers.
C. The posterior limb is located between the thalamus and the lentiform nucleus. It con
tains conicospinal (pyramid) 6bers as well as sensory (pain. temperature. and touch).
visual. and auditory ooia[ions.
D. Blood supply
1. The anterior limb is irrigated by [he medial srri:ne branches of [he anterior cerebral
artery and rhe lateral striate (lenticulostriate) branches of [he midJle cerebral artery.
2. The genu is perfused either by dircct branches from the internal carotid anel)' or
b\' p:lllidal branches of the anterior choroidal artery.
3. TIle posterior limb is supplied by branches of the anterior choroidal anery nnJ
lenticulostriate branches of the middle cercbml aneries.
caudate nucleus --/-'-
Corticobul>a.r fibers --r"-14-fJ
Posterior limb --+--+t-
Thalamus --1--
Sensory radiations from
VP nucleus to areas 3. 1. 2
Lateral geniculate body
(vision)
/'Anterior
Globus pallidus
--<-_Putamen
fibers
I Auditory radiation to transverse
tempol"al gyri of Heschl
(areas 41 and 42)
VISual radiation 10
striate COl1ex of oocipitallobe (area 17)
Agure 16-2. l-Iori:omal.scClion o( Ihe righ, imernal capsule showing rhc m:ljor iihcr f'rojc<:tions. Cll1lical1\'
important lraclS lie in the i,ocnu and IXJ<iilerior timbo Lesions o( Ihe internal capsule caus.:: COntrablleml hemi
p;lresis ;)nd cOlltml:ueral hemianopia. VI' = \"enrr.ll posterior nucleus.
1.7
Visual System
I. INTRODUCTION. The visual )'s[em is served b)' the OPlic: neryc, which is a special so-
marie afferent nervc.
II. THE VISUAL PATHWAY (Figure 17-1) includes the following
A. Ganglion cells of the rNina foml the optic nerye [cranialncrvc (eN) II). pro-
jecr from the nas;11 hcmiretina to the comralateral lateral gcniculrnc body and from
the temporal hcmiretina to the ipsil::ncrallrltcral gcnicul:uc body.
Visual corteIC area 17
Visual radiation to lingual gyrus
Visual radiation 10 cuneus
Retina
+i-Jlf---Lateral geniculate
body
Optic tracl
,
'fIJ>-Optic nerve
7
')
, ,
3
5
'0
(),()

()ca

G'c.
(){t
Agure 17-1. The visual p:nhway (rom the retina to the visu'll conc:o; showiny ViSU;11 iicld defecls. (I) lpsilm.
cr.11 hllndness. (2) Hinas.'l.l hcmianopi:l. (3) Bilcmpoml hemi:mopia. (4) Higlll hcmi:ltlopia. (5) Right upper quad-
r..lnt:1ll0pl:I. (6) Rightlowerquadr:lnltlnopia. (7) Right hemianopia wIth 111.1Cularsparing. (8) Le(t cOflsuicuxl6c1J
a5 a result of cl'ld-Sl<lb'C gbUCOln:I. Bil:ller.ll comuicled fields may be sccn H1 (9) Le(t cemml scOl:omtl a5
,)Cen in optic (relrobulbar) neuritis in mulliple sclerosis. (10) Upper altllUdinal hCllllanopia AS a rcsoll oi'bil:lrcral
dt'SIRJClion of Ihe lingu.11 b'yri. (I J) Lower ahirudin.11 hemianopia a:> a rcsoh o(hil:ller..ll of the cunei.
91
92 Chapter 17
B. The optic nerve projectS from the lamina cribrosa of [he scleral canal. [hrough the op-
[ie canal, to Ihe op[ic chiasm.
1. Transection causes ipsilmeral blindness, wi[h no direc[ pupillary light renex.
2. The section of the optie ner"e at the optic chiasm rransecTS all fibers from [he ip-
silateml retina as well as fibers from the contralateral inferior nas..'ll quadram that
loop into the optic nerve. TIl is lesion cauS\."'S ipsilateral blindness and a comralat-
eraJ upper temporal quadrant defect (junction scotoma).
C. The optic chiasm contains decuss..'lting fibers from [he twO nasal hemirClinas. It con-
mins noncrossing fibers (rolll [he twO Icmpoml hemiretinas and projects fibers to Ihc
supmchiasmatie nucleus of the hYP()Ihalamus.
1. Mids.1gittal transection or pressure (o(ten from a pituitary tumor) causes bitem
poral hemianopia.
2, Bilatcrallatcral compression causes binasal hemianopia (calcified internaI carotid
arteries),
D. The optic tract contilins fibcrs (rom the ipsilateral temporal hcmirelinil ilnd dH.'
contralateral nasal hemiretina. It projecls to the ipsilaterallarcral geniculate body,
prelccral nuclei. and superior colliculus. Transection C<IUseS cOlllralareral herni-
'lIlopia.
E. TIle lateral geniculate body is a six-layered nucleus. Layers 1,4, and 6 receh'e crosseJ
fibers; layers 2, J, and 5 receive uncrossed fibers. The klleml geniculare bod)' rccci,'es
input from I:lyer VI of the striarc correx (Brodmann's area 17), h also receives fibers
from the ipsilateml temporal hemirc[ina and ,he commlatcml nas..11 hemire[ina, II
projects thrO\lgh the geniculocakarine tr,lct to la)'er IV of the primary ,'isual coru'x
(Brodmann's area 17),
F. Thc gcniculocalcarine tmct (visual radiation) projects [hrough twO divisions to the
visual cortex.
1. The upper division (Figure 172) projects (Q rhe upper bank o( the calcarine sul-
cus, the cuneus. It conrains inplll from the superior retinal quadranls, which rep-
resem the inferior visuallie1d quadmnts,
a. Transection causes a contmlmcmllo\\'er quadrantanopia.
b. Lesions that im'olve bOlh cunei cause a lower alrirudinal hemianopia (alii.
rudinopia),
2. The lower division (see Figurc 172) loops from ,he lateral geniculate body anle
riorly (Meyer's loop), rhcn POStcriorly, [0 terminare in the lower bnnk of the cnl
carine sulcus, the lingual gyrus. It contains input from the inferior retinal quad.
mnts, which represent the superior visuallield quadrants.
a. Transcction causes a contmlatcral upper quadrantanopia ("pic in the sky"),
b. Transection o( both lingual gyri causes nil upper altitudinal hemianopia (ai-
titudinopial,
G, The ,'isual cortex (Brodmann's area 17) is locared on [he banks of Ihe calcarine lis
sure, The cuneus is rhe upper bank. The lingual b')'rus is the lower bank. Lesions cause
contmlateral hemianopia wirh macular sparing. The ,'isual conex has a rctinotopic
organi:ation:
1. The posterior area recehes macular input (cemral vision).
2. The interml'diate area receives parmnacular input (peripheral inpur),
3. The anterior area recch'es monocular input.
Visual System 93
FIeld detects
Lower r. homonymous quadrantanopia
Lesion A 01 visual radiations to
sup. bank of calcarine sulcus
I
I
I

Loop 01 Meyer ..- / Upper r. homonymous quadrantanopia
/
Lesioo B of visual radiations 10
info bank 01 calcarine sulcus
lat. geniculate body,
Figure 17-2. Rekltions of the left tipper ,md left lower divisiorl5 of the geniculocalcarine tract to the lateral
ventricle and calcarine sulcus. TnmsectiOIl of the upper division (A) results in right lower homonymous quad-
rantanopi;l. Tmnscctioll of the lower division (8) results in right upper homonymous quadmntanopia.
(Reprinted with permission from Fix JD: BRS NeurOOlltllomy. Baltimore, Williams & Wilkins, 1997, P 261.)
III. THE PUPILLARY LIGHT REFLEX PATHWAY (Figure 17-3) has an afferent limb
(eN II) and an efferem limb (CN III). It includes the following S[[ucrures:
A. Ganglion cells of the retina, which project bilaterally to the pretectal nuclei
B. The pretectal nucleus of the midbrain, which projects (through the posterior com-
missure) crossed and uncrossed fibers to the Edinger-Westphal nucleus
C, The Edinger.Westphal nucleus of eN III, which gives rise to preganglionic parasym
pathetic fibers. These fibers exit the midbrain with CN 111 and synapse with postgan-
glionic parasympathetic neurons of the ciliary ganglion.
D. The ciliary ganglion, which gives rise to postganglionic parasympathetic fibers. These
fibers innervate the sphincter muscle of the iris.
IV. THE PUPILLARY DILATION PATHWAY (Figure 17-4) is mediated by the sympa-
thetic division of the autonomic nervous system. Interruption of this pathway at any level
causes ipsilateral Horner's syndrome. It includes the following structures:
A. The hypothalamus. Hypothalamic neurons of the paraventricular nucleus project di-
rectly to the ciliospinal center (TI-T2) of the intennediolateral cell column of the
spinal cord.
B. The ciliospinal center of Budge (Tl-T2) projects preganglionic sympathetic fibers
through the sympathetic trunk to (he superior cervical ganglion.
C. The superior cervical ganglion projects postganglionic sympathetic fibers through the
perivascular plexus of the carotid system to the dilator muscle of the iris. Postgan-
glionic sympathetic fibers pass through the tympanic cavity and cavernous sinus and
enter the orbit through the superior orbital fissure.
94 Chapter 17
Posterior commissure
Prelectal nucleus
Brachium of
superior oollic1Jlus-7<
Eamger-Weslpl'\al )><fl'\-
nucleus of CN III
Red nucleus
cerebral aqueduct
Thalamus
genic1Jlate
nucleus
lateral geniculate
"""""
Crus cerebri
,....,,,-CN II
Retinal ganglionic cell
;?4
L
Sphincter muscle of Iris
Figure 17-3. TIle pUllilbry light Lighl shinl-d illlOone eye C;UlSCS bOlh pupils 10 cOlulric!. The re-
Spol'ISC in the stimubtcd ere is called dw din-oct pupilbry liglll retlex. TIle rc.>pol"ISC in the opposite eye is c:III..,.;I
the consensual pupilla'1' light reflex. eN - cmnial ner'..e.
V. THE NEAR REflEX AND ACCOMMODATION PATHWAY
A. The cortical visual pathway projects from the primM)' visual conex (Brodmann's :Irea
17) to the visual association cortex (Brodm:mn's area 19).
B. The visual associ;ltion cortex (Broomann's area 19) projecrs through the conico-
rectal tr:lCl to the superior colliculus and pretectal nucleus.
C. The superior col1iculus and pretectal nucleus projcct to the oculomotor complex of
the midbrain. TIlis complex includes the following smlclures:
1. TI,e rostral Edinger,\\'estphal nucleus. which mediates pupillary constriction
through the ciliary I,':tnglion
2. TIl(' caudal Edinger.Westphal nucleus, which mediates contrnction of the ciliary
muscle. This contraction increases the refractive power of the lens.
3. TIle mediall"(.octus subnucleus of eN 111, ",hich mediates convergence
VI. CORTICAL AND SUBCORTICAL CENTERS FOR OCULAR MOTILITY
A, The frontal eye field is locauxl in the posterior pan of lhe middle fromal gyrus (Brod
mann's area 8). It regulates voillmary (saccadic) eye movementS.
Visual System 95
A

~ i&5 ~
iOO:) . ~
~
e
Flashlight swung from right eye to left eye
e
Looking straight ahead
C
0
cit
. ~
aD ~ ~ ~ aD aen
Looking righl Looking left Eyes converged LooIOng straight ahead
E F G
~ aD ~
zr=]$
~ illi Efu Cii
Looking right Looking up Eyes converged Looking left and down
H I J
fi%. illi Efu ~
. ~
ib a
t r ! j ~
No reaction to light Eyes converged Eyes of a comatose patienl Looking straight ahead
Agure 17-4. OcUIHr mOlOf p:lbics and pupillal')' s)"IldmmL"S. (A) Rebrive ll(("relll (M:lrcUS CunnI pupil. left
eye. (0) Homer's syndrome. le(t e)c. (C) huemUc!CHr opluhalrnuplcgi'l, right cye. (0) TIlirdner....e lJHls)', left
eye. (E) Sixthnerve pals\', right eye. (F) P:lrnl)'sis o( upw;.lfd gaz" and COIl\"erl,'encc (Parinaoo's syndrome). (G)
Founhncrve pollsy, right eyc. (H) AIb'Yll RobertsOn pupil. (/) DcSlfucti\"e lesion o( the righl (ronrHl C)'C tick!, U)
Tllird-!lL'f\'e 1l111sy with ptosis, righl eyL'.
1, Stimulation (e,g" from an irrimrive lesion) causes contralateral deviation of the
eyes (i.e" away from the lesion).
2. Destruction causes transient ipsilatcml conjul,;ate deviation of the eyes (i.e. to-
ward the lesion).
B. Occipital eye fields arc located in Brodmann's areas 18 and 19 of the occipirallobes.
These fields nrc corticnl centers for involuntary (smooth) pursuit and tr:leking move
ments. Stimulation causes contralateral conjllg:ue deviation of the eyes.
C. The subcortical center for lateral conjugate gaze is locared in the abducent nucleus
ofrhe pons (Figure 17-5). Some authorit ics place the "center" in the pammedian pon-
tine reticul:lr formation.
1. It receives inpu(from the contr.:llateral frontal eye field,
2. It projects to the ipsilater..lliateml recrus muscle :lnd, through the mediallongitu.
dinal (:lsciculus (MLF). ro the comraknemllllCl.lial rectus subnucleus of the ocu
lomotOr complex,
D. The subcortical center (or vertical conjugate ga:e is loc3[l.-'C! in Ihe midbmin at the
Ic\'c1 of the posterior commissure. II is called Ihe rostral interst itial nucleus of the MLF
and is associated with Parinaud's syndrome (sec Figurl.-'S 14-}A and 174F),
96 Chapter 17
Bilateral MLF syndrome
-
Lelt
A


. ,
-+-
Right
-
B


. .
.......
Convergence
C


Patient with MLF syndrome camot
adduct the eye on attempted lateral
conjugate gaze, and has nystagmus
in abducting eye. The nystagmus is
in the direction ollhe large arrow-
head. Convergence remains inlaet.
Right MlF LeIt MLF
Medial rectus
subnucleus
01 CN III
I Lateral rectus musde

!I\J\ '--/
Medial
rectus
muscle
Midbrain
Figure 17-5. GJnnt'clions of lhe pOlllino: cemer for hlteml conjugtUc gll:C. Lesions of the medi:lllnngiludi-
1l;t1 (;lsciculus (MLF) between the ;llxlucem :lnd oculomotor nuclei result in l1lcJiall"t-"(:IUS pals\' {HI aucrnplcJ
hueT:II conjug;I1C gaze and horizonlal n\'stagmus in the ;llxlucfing eye. Convergence remains intaCl (inwr). A un i-
hueral MLF lesion would afTec! only the ipsiblcmlmcJial recrus. eN "" CT:lllhll nerve.
VII. CLINICAL CORRELATION
A, In MLF syndrome, or internuclear ophthalmoplegia (sec Figure 174), [here is dam-
age (dcl1\)'din:uion) [0 the MLF between the Ollxluccnt and oculomoror nuclei. It
causes medial rectus palsy on attempted lateral conjugate ga:e and monocular hori-
:omal nystagmus in d\e alxluc[ing eye. (Convergence is normal.) TI\is syndrome is
most commonly seen in multiple sclerosis,
B. One-and-a-half syndrome consists of bilaternl lesions of the MLF and a unilatemlle-
sion of the abducent nucleus. On aucmptcd latcml conju&"lte ga:e, the only muscle
thm functions is the intaCtlmeral rectus.
C. Argyll Robertson pupil (pupillary light-ncar dissociation) is the absence of a miOtic
reaction to light. both direct and consensual. with the preservation of:1 miOlic rcac-
tion to n(Oar stimulus (accommodation-eon\'ergcnce). It occurs in s\,philis and
diabet(Os.
Visual System 97
" " , .
D. Horner's syndrome is caused by mmscction of the oculosympathetic pathway at any
level (see IV). This syndrome consists of miosis. ptosis. apparent enophthalmos, and
hemianhidrosis.
E. Relative afferent (Marcus Gunn) pupil results from a lesion of the optic nerve, the
afferent limb of the pupillary light reflex (e.g.. retrobulbar neuritis seen in multiple
sclerosis). The diagnosis can be made with the swinging flashlight test (see Figure 17-
4A).
F. Transtentorial (uncal) hemiation occurs as a result of increased supratentorial
sure, which is commonly caused by a brain tumor or hematoma (subdural orepidural).
1. The pressure cone forces the parahippocampal uncus through the tentorial in
cisure.
2. The imp.1.cted uncus forces the contralateral crus ccrebri the tentorial edge
(Kemohan's norch) and putS pressure on the ipsilaternl eN III and posterior cere
bral anery. As a result, the following neurologic dek'Cts occur.
a. Ipsilateral hemiparesis occurs as a result of pressure on the corticospinal tract.
which is locared in the contralateral crus cercbri.
b. A 6.."ed and dilated pupil, ptosis, and a "down-and-out,,, eye are caused by
pressure on the ipsilateral oculomotor
c. Contralateral homonymous hemianopia is caused by compression of the pos-
terior cerebral artery, which irrigates the visual cortex.
G. Papilledema (choked disk) is noninflammatory congestion of the optic disk as a re-
suh of increased intracranial pressure. It is most commonly caused by brain rumors.
subdural hematoma, or hydrocephalus. It usuall)' does not alter visual acuity, but it
may cause bilateral enlarged blind spots. It is often asymmetric and is gre:uer on the
side of the supratentorial lesion.
H. Adic's pupil is a large tonic pupil that reacts slowly to light but docs react to ncar (Iight-
ncar dissociation). Frequently sccn in females with absem knee or or ankle jerks.
9.
1.8
Autonomic Nervous System
I. INTRODUCTION. The autonomic nervous system CANS) is a general visceral efferent
mQlOr system that controls and regulates smooth muscle, cardiac muscle, and glands.
A. The ANS consists of (Wo types of projection neurons:
1. Preganglionic neurons
2. Postganglionic neurons. Sympathetic ganglia have imcmcurons.
B. Autonomic output i5 conrrolled by the hypothalamus.
C. TI,c ANS has three divisions:
1. Sympathetic. Figure 18-1 shows the symp:uhctlc innenr(uion of the ANS.
2. Parasympathetic. Figure 18-l shows the parasympathetic innen"ariono(the ANS.
Table 18-\ compares the effects of symp:llhClic and p.1r3symp:Hhcric aClivilY on
organ sySl'cms.
3. Enteric. TIle emeric division includes the intramural ganglia of the g:lSlrointesrj.
nal trner, submucosal plexus. and myenteric plexus.
II. CRANIAL NERVES (CN) WITH PARASYMPATHETIC COMPONENTS ;ndud,
dlC following:
A. CN III (ciliary ganglion)
B. CN VII (ptcrygopalatine and submandibular ganglia)
C. CN IX (olic ganglion)
D. CN X [rerminal (mural) ganglial
III. COMMUNICATING RAMI of the ANS include:
A. White communicating rami. which are found berwcen T J and L3, arc myelinatcd,
B. Gray communicating rami, which are found at all spinallcvc1s, arc unmyelinated.
IV. NEUROTRANSMITTERS of [he ANS includc:
A. Acetylcholine, which is rhe neurotransmitter of the preganglionic neurons
B. Norepinephrine. which is rhe neurmrnnsminer of the posq.'<lnglionic neurons. with
the exception of sweat glands and some blood vessels that receive cholinergic sympa-
thetic inner\'ation
C. Dopamine. which is the neurotransminer of rhe small intensely fluorescent (SIF)
cells, which are interneurons of rhe sympathetic ganglia
Autonomic Nervous System 99
,p Tarsal muscle
..__------1.;.<J
..
, , lacrimal gland
"Eye: dUator ol pupil
St.tJmandibular and
sublingual glands
Parotid gland
.....
T+,

1!
~
"
Bronchial tree
~
1! ~ l a c plexus

..

1l
,
"-rio<
.""""'"
E
'<.
" rnesentelic plexus
~

Small intestine

l
Adrenal medulla'

>
i
Large intestine
;;;;
J ~
Inferior
L3
mesenteric plexus
~
Ductus deferens
,
, ,
, ,
, ,
, ,
, ,
, Sympathetic trunk'
Figure 1.8-1. Thc sympmhcric (thoracolumoor) inncl'o'ation of the alilonomic ncl'o'OUS SYSlcm. TIle emire
symp:tthelic innerv:llion of the head is through rhe superior cCI'o'ical g;lIll;lion, Gnly cOnlmunicatinl: nlmi ,ue
found :1( :lll spinal cor.:llevcls. While communic:lting nlllli arc found only in spinal segments TI throol;h LJ.
100 Chapter 18
Medulla
Dorsal motor " ,
nucleus 01 "
vagal nerve,'
Ciliary gangion
Pterygopalatne gangion
Eye: constrictor
of pupil and
diary body
~ " ' "
~ ~ " " "
5 __
and sublingual
glands
Parotid gland
Hear1
Bronctial tree
Stomactl
SmaB intestine
""-
large intestine
t
J
t
5-2
5-3
V
5-4
Urinary bladder
Pelvic
A
splanctnc
"""'"
tW
GeoitaI erectile tissue
FIgure 18-2. The pamsymp:lIhclic (craniosacral) innervaliOll 0{ lhe aUlooomic nervous system. Sacral OUI-
/low includes 5q,'lllenu S-2 dUOUb"\ 5-4. Cranial ourflow is mediated through cranial nerves (CN) III, VII, IX,
and X.
Autonomic Nervous System 101.
Table 18-1
Sympathetic and Parasympathetic Activity on Organ Systems
Structure
E",
Radial muscle of iris
Circular muscle of iris
Ciliary muscle of ciliary body
Lacrimal gland
Salivary glands
Sweat glands
Thermoregulatory
Apocrine (stress)
Heart
Sinoatrial node
Atrioventricular node
Contractility
Vascular smooth muscle
Skin. splanchnic vessels
Skeletal muscle vessels
Bronchiolar smooth muscle
Gastrointestinal tract
Smooth muscle
Walls
Sphincters
secretion and motility
Genitourinary tract
Smooth muscle
Bladder wall
Sphincter
Penis. seminal vesicles
Adrenal medulla
Metabolic functions
Liver
Fat cells
Kidney
Sympathetic Function
Dilation of pupil (mydriasis)
Viscous secretion
Increase
Increase
Acceleration
Increase in conduction velocity
Increase
Contraction
Relaxation
Relaxation
Relaxation
Contraction
Decrease
Little or no effect
Contraction
Ejaculation-
secretion of epinephrine and
norepinephrine
Gluconeogenesis and
glycogenolysis
Lipolysis
Renin release
Parasympathetic Function
Constriction of pupil (miosis)
Contraction for near vision
Stimulation of secretion
Watery secretion
Deceleration (vagal arrest)
Decrease in conduction velocity
Decrease (atria)
Contraction
Contraction
Relaxation
Increase
Contraction
Relaxation
Erection-
Note erection versus eJaculation: Remember point and shoot: p - parasympathetic, s - sympathetic.
Reprinted with permission from Fix J: BRS Neuroanatomy. Media. PA, Williams & Wilkins, 1991.
O. Vasoactive intestinal polypeptide (VIP), a v;lsodi!awr that iscolocalized with acetyl.
choline in some postganglionic parasympathetic fibers
E. Nitric oxide (NO). a newly discovered neurotransminer that is responsible for the reo
laxation o( smooth muscle. h is also responsible (or penile erection (sec Chapler 22).
V. CLINICAL CORRELATION
A. Megacolon (Hirschsprung's disease, or congenital aganglionic megacolon) is char-
acteti:ed by extreme dilation and hypertrophy of the colon, with fecal retention. and
by the absence of ganglion cells in the myenteric plexus. Ir occurs when neural crest
cells do not mif,'fOne into The colon.
B. Familial dysautonomia (Riley.Day syndrome) predominantly affects Jewish chilo
dren. h is an autosomal recessh'e trail that is characterized by abnonnal swearing. un
102 Chapler 18
smble blood pressure (e.g., orthosmtic hypotension), difficulty in feeding (as a rcsuh
of inadequate muscle (one in the gastrointestinal tract), and progressive sensory loss.
h results in the loss of neurons in the au(onomic and sensory 1p.nglia.
C. Raynaud's disease is a painful disord("r of the terminal arteries of the extremities. It is
characteri:ro by idioparhic paroxysmal bilareral cyanosis of the digirs (as:l resuh of ar-
rerial and :lrteriolar consrriClion because ofcold or emorion). It may be uemro by pre-
grmglionic sympathectomy.
D. Peptic ulcer disease results from excessivc production of hydrochloric acid occause of
increased parflsympflthetic {tone) stimulation.
E. Horner's syndrome (see Chapter 17) is oculosympathetjc paralysis.
F. Shy-Drager syndrome involves preganglionic symparheric neurons from rhe inter-
mroiolareml cell column. It is charocteri:ro by orthostatic hypotension, :lnhidrosis,
impotence, and bladder atonicity.
G. Botulism. The toxin ofClostridilfln oondimtffi blocks the release of acerylcholine and
results in paralysis of aU striated muscles. Autonomic efreer;;; include dry eyes. dry
mouth. and gastrointestinal ileus (oowcl obstruction).
H. Lambert-Eaton myasthenic syndrome (St.'e Chapter 22)
1.9
Hypothalamus
I. INTRODUCTION
A. General structure and function. The hYPOlhalamus is a division of Ihe diencephalon
that subscn'CS three systems: the autonomic nervous s)'srcm. enJoerine system. and
limbic system. The hypmhalamus helps to maintain homOOSlasis.
B. Major hypothalamic nuclei and their functions
1. TIle medial preoptic nucleus (Figure 19-1) rC!.'lllarcs th" release of !,'Onadotropic
hormones from rhe adenohypophysis. It contains the scsuall)' dimorphic nucleus,
the de'l'e1opment of which depends on tcsrosu.'ronc
2. The suprnchiasmaric nucleus receives direct input (rom the retina. h pbys a role
in the regulation of circadian rhythms.
VentromedIal nucleus
satiety center
destruction results in obesity
and savage behavior
Mamlltary body
receives input from
hippocampal formation
via lornix
projects to anterior nucleus
ot thalamus
contains hemorrhagic tesions
in Wernicl<e's encephalopathy
Dorsomedial nucleus
stimulation results in obesity and savage behavior
Posterior nucleus
thermal regulation (conservation 01 heal)
destruction results in inabilily to thermoregulate
stimulates the sympathelic NS
Lateral nucleus
stimulation induces eating
destruction results in slarvation
Arcuate nucleus
produces hypolhalamic releasing lactors
contains DOPA-ergic neurons thaI inhibil prolactin release
Paraventricular and supraoptIc nuclei
regulate water balance
produce ADH and oxytocin
destruction causes diabetes insipidus
pamvenlricular nucleus projects to
autonomic nuclei of bminstem and
spinal cord
Anterior nucleus
thermal regulation
(dissipation ot heat)
stimulates parasympathetic NS
destruction results in hyperthermia
Preoptic area
contains sexual dimorphic nucleus
regUlates release of gonadotropic
hormones
Suprachlasmatlc nucleus
receives inputlrom retina
controls circadian rhythms
Agure 19-1. Major hypOlh:Lhllnic nuclei and their fUllcriolls. ADH =:uuidiurclic honnonc; eN =cranial
nerve; DOPA = Oop;.llll1ne; NS = nervous system,
1.3
104 Chapter 19
3. TIlC anterior nucleus plays a rolc in remperature regulation. It stimulates the
parasympmheric nervous system. Destruction results in hyperthemlia.
4. The paraventricular nucleus (Figure 19-1) synthesizes hormone
(ADH). oxytocin, and corticotropin-releasing hormone. It gives rise to rhe
suprnopricohypophyseal tract. which projectS to the neurohypophysis. It regulates
water balance (conservation) and projects directly to the autonomic nuclei of the
brain stcm and all levels of thc spinal cord. Desrruclion results in diabetes in-
sipidus.
S. The supraoplic nucleus synthesizes ADH and oxytocin (similar [0 the par-lVen-
tricular nucleus).
6. The dorsomedial nucleus. In animals. So'wage behavior resultS when this nucleus
is stimulated.
7. The ventromedial nucleus is considered a satiety center. When stimulated, it in-
hibits the ut},'e to eat. Bilateral destruction results in hyperphagia. obesity. and sav
age bcha\ior.
Paraventricular nudeus
ThO'"
ventricle
Atcuate (tubefal) nucleus
Tuberohypophyseallract
Posterior lobe (neurohypophysis)
trael
Sinusoids of infundibular stem
_
Hypophyseal vein
Inlerior hypophyseal artery
Supraoptic nucleus
Optic
chiasm
Hypophyseal portal veins

Superior hypophyseal artery
Anterior lobe (adenohypophysis)
Figure 19-2. The hypophyscal porrol] s)'stem. The paraventricular and supmoplic nuclei produce antidiuretic
hormone (ADH) (Ind OXytOCill and transpon: them through the supraopticoOypopnyseal Inlel 10 the capillary
bed of the ncuroll)J'Ophysis. The arcuate nocleus of the infundibulum transport$ hypothalamic-stimulating hor-
moncs through the wbcroh)'pophyseal nact to the sinusoidsof the infundibular srem. 1bese sinusoids Ihen drain
into Ihe sccondary capillary plexus in the adenoh)l)ophysis.
Hypothalamus :1.05
8. The arcuate (infundibular) nucleus contains neurons that produce facwrs thm
stimulate or inhibit the action of the hypothalamus. This nucleus gives rise to
the tulx-rohypophyseal tmct, which tenninates in the hypophyseal ponal system
(sec Figure 19-2) of the infundibulum (medium eminence). It comains neurons
that produce dopamine (i.e., prolactin-inhibiting factor).
9. The mamillary nucleus receives input from the hippocamJXlI formarion rhrough
the po:5tcommissural fornix. It projecrs [0 the anterior nucleus of rhe thalamus
through the mamillorhalamic tract (pan of the Pape: circuit). Patients with
Wernicke's encephalopathy. which is a thiamine (\'itamin B
I
) deficiency. ha\'e
lesions in the mamillary nucleus. Lesions are also associatt.-d with alcoholism.
10. The posterior hypothalamic nucleus plays a role in thermal I\.""gularion (Le.,
consct\'ation and increased production of hear). Lesions result in poikilother-
mia (i.e. inabili[)' [0 thermoregulate}.
11. The lateral hypothalamic nucleus induces eating when stimulated. Lesions
cause anorexia and starvation.
C. Major fiber systems of the hypothalamus
1. The fornix is the largest projection to the hypothalamus. It proj(>Cts from the hip-
pocampal formation ro the mamillary nucleus. anterior nucleus of the thalamus.
and sepral area. The fornix then projects from the sepral area to Ihe hippocampal
fomlation.
2. The medial forebrain bundle trnvcrscs the entire Iaternl hypothalamic area. It in-
terconnecrs the orhitofromal conex. septal area. hypothalamus, and midbrnin.
3. The mamillothalamic tract projects from the mamillary nuclei to the anterior nu-
cleus of the thalamus (pan of the Pape: circuit).
4. The stria terminalis is the major p<1thway from the amy/,tdala. It inrerconnect5 the
septal area, hypothalamus. and amygdala.
5. The supr"dopticohypophysial tract conducts fibers from rhe suprnoptic and par-
avenlricular nuclei to the neurohypophysis. which is the release sitc for ADH and
oxytocin.
6. The tuberohypophysial (tuberoinfundibular) tract conducts fibers from the ar-
CUflre nucleus to the hYlXlphyscal portal system (see Figure 19-2).
7. The hypothalamospinal tract contains direct descending autO lOmic fibers. These
fibers influence the preganglionic sympathetic neurons of th,: inrcrmediolateml
cell column and prcgflnglionic neurons of the sacral pamsymp.lthet ic nucleus. [n-
terruption above the first thol<lcic segment (T.\) Cflllses Honwr's syndrome.
II. FUNCTIONS
A. Autonomic function
.1. The anterior hyPothalamus has an excitatoT)' effect on the parasympathetic ncr-
\'OUS system.
2. The posterior hypothalamus has an excitatory effect on the s)'mpathetic nervous
system.
B. Temperature regulation
.1. The anterior hypothalamus re/,'lllates and maintains body tcmpernture. OcstnJc-
tion causes hyperthermia.
106 Chapter 19
2. The posterior hypothalamus helps w produce and conserve heat. Destruction
causes d l ( ~ inability [0 thermoreguhue.
C. Water balance regulalion. The paraventricular nucleus synthesizes ADH, which
comrols water excretion by the kidneys.
D. Food intake regulation. Two hypothalamic nuclei playa role in the control of ap-
petitc.
1. When stimulated, the ventromedial nucleus inhibits the urge TO cat. Bilateral de-
struction results in hyperphagia, obesity, and s."lVage behavior.
2. When stimulated, the lateral hypothalamic nucleus induces the urge to cal. De
struction causes starvation and emaciation.
III. CLINICAL CORRELATION
A. Diabetes insipidus. which is characterized by polyuria and polydipsia, is the Ix-st
known hypothalamic syndrome. It results from lesions of the ADH p.nhways to the
posterior lobe of the pituimry gland.
B. The syndrome of inappropriate ADH secretion is usually caused by lung tumors or
drug therap}' (e.g. carbam3zepine, chlorpromazine).
C. Crnniopharyngioma is a congenital tumor thar originates from remnants of Rathke's
pouch (see Chapter 4). This tumor is usually calcified. It is the most common supra-
tentorial tumor in children and the most common cause of hypopituitarism in chil-
dren.
1. Pressure on the chiasma results in bitcmporal hemianopia.
2. Pressure on the hypothalamus causes hypothalamic syndrome (i.e., adiposity, di
abetes insipidus, disturbance of temperature regulation, and somnolence).
D. Pituitary adenomas account for 15% of clinical symptomatic intracranial tumors.
They arc rarely seen in children. When pituitary adenomas arc endocrine-active, they
C:lUSC endocrine ahnonnalities (e.g., amenorrhea :Ind galactorrhea from a prolactin-
secrcring adenoma, rhe most common typc).
1. Pressure on the chiasma results in bitemporal hemianopia.
2. Pressure on the hypothalamus may cause hypothallllnus syndrome.
20
Limbic System
I. INTRODUCTION. The limbic system is considered the anatomic substrate Ihm under-
lies bchaviomJ .. ndemotional expression. It isexprcsS<.'(llhrough the hypothalamus by way
of rhe "Ulonamic nervous system.
II. MAJOR COMPONENTS AND CONNECTIONS
A. 111e orbilofronlal cortex mediates the conscious perception of smell. II has recipro-
cal connections wilh rhe mediodorS;ll nucleus of the L1mlamus. It b inlcrcQnne<:ted
lIuough the medial forebrain bundle wid, the septal area anJ hypOIhalamic nuclei.
B. The mediodorsal nucleus of the thalamus has reciprocal connections wilh the or-
bitofronlal;md prefronml cortices as well as the hypothalamus. It rccci\'t"S input (rom
the am)'gJab and plays a role in affective behavior anJ memory.
C. The anterior nucleus of the thalamus receives iopur from the mamillary nucleus
through rhe mamillorhalamic tract and fornix. It projects to the cingulare gyrus and
is a major link in ,he Pape: circuiL
D. The sepral area isa telencephalic structure. It has reciprocal connections with the hip-
Ix>campal formation through the fornix and with the hypothalamus through the me-
dial forebrain bundle. It projects through the stria Tll<.-duUaris (thalami) to the habe-
nular nucleus.
E. The limbic lobe includes the subcallosal atea, paraterminal gyrus, cingulflte gyrus and
isthmus, <lnd parahippocampal gyms, which includes dl(' uncus. It cont"ins, buried in
the parahiPIx>campal gyms, the hippoc:unpal formatiOl' and amygdaloid nucle"r com
plex.
F. The hippocampal formation is a sheet of archicorrcx that is jelly-rolled into the
parahippocrllllpal gyrus. It functions in leflrning, mcmory, and recognition of novelty.
It receives major input through the entorhinal cortex and projects Ilmjor output
through the fornix. Its major structures include the following:
1. The dentate gyrus, which has a three-layered archicorrex. It conrainsgnmule cells
thai receive hipPQ:amJXII input and project Output to the pyramidal cells of the
hippocampus and subiculum.
2. The hippocampus (cornu Ammonisl, which has a three-la)'ered archicortex. It
contains pyramidal cells ,hat project through the fornix ro the sepral [Irea and hy.
porhalaTllus.
3. The subiculum, "'hich receives input rhrough rhe hippocampal pyramidal cells. It
projects through the fornix to the mamillary nuclei and rhe anrerior nucleus of Ihe
thalamus.
107
108 Chapter 20
G. The amygdaloid complex (amygdala) IFigurc ZO.I; see also Figure 21.11 is a b..'lsal gan-
glion that underlies the parahippocampal uncus. In humans, stimukuion causes fear
and signs of s}'mpmheric overnctivity. In othcr animals, stimulation resulrs in cess.....
tion of activity and heightened attenriveness. Lesions cause placidity and hypersexual
behavior.
1. Input is from the sensory associ:nion cortices, olfactory bulb and cortex, hypo.
thalamus and sepral area, and hippocampal fonnarion.
2. Output is through the stria tcrminalis [0 the hypothalamus and scpml arca. TIlerc
is :llso output to rhe mediodors:ll nuclcus of the thalamus.
H. TIle hypothalamus h:ls reciproc:ll connections with the :lllly!.'<bla.
I. TIle limbic midbrnin nuclei and associ.ned neurotransmitters include rhe ventral
tegmental arca (dopamine), ",phe nuclei (sctownin). and locus ceruleus (norepi
nephrine).
III. THE PAPEZ CIRCUIT (Figure 202) includes rhe following limbic struCtures:
A. The hippoc:nnp<ll formation, which projects lhrough the fornix 10 the mnmillary nu
c1eus ,lIld septal area
B. TIle mamillnry nucleus
Hippocampal
lonnation
7
,-Striatenninalis
_
. .
5eptaI area Hypothalamus
VAFP/VAPP,
-.
of Broca
,
.
--
.
Olfactory bulb and
:
Autonomic centers
olfactory cortel(
Amygdaloid nllCleus
or brain stem
,
,
VAFP/VAPP
Sensory association
and cortices
Fom
Diagonal band
Agure 20-1. Millor connections of the nucleus. This nucleus receives input from three major
sources: Ihe olfaclOry SCfUOry a$SOCiarion and limbic cortices, and hyporhalamlls. Majorompm is through
twO channe1s: [he stria teml;n:.lis projects to Ihe and rhe .seplrJl llTC3. and rhe \'entr,l! amyg-
dalofugal p.1rhway (VAH') projects 10 the hyporhalamus. brain stem, :md spin:ll cord. Asm;lller effel'\'nt bundle.
rhe band of Broca, projc.'Cu to the .septal :ll'\'a. Affel'\'nt fibers {rom the hypothalamus .md brain SH:m en-
ler Ihe amygd.1loid nucleus lhrough the \'entral (VAPP).
Limbic System 109
Figure 20-2. Major and efferent limbic
connections of the hippocllmp,11 (ormation. TIlis forma
tion hllS three components: the hippocampus (cornu
Amn"lOllis), subiculum, and dentale b"lnIS. TIle hip-
poc:nnpus proje<:ts to lhe scpml area, IIlC subiculum pro-
jeers to lI,C mamillary nuclei, and lhe dencue b"l"tUSdoes
llO( project beyond the hif'POCUmp;l1 (orm."ldon. Thecir-
cuit of rape: (0110""5 [his route: hippocampal form."ltion
[Q mamillary nuclcu:s to anterior thalamic nuclCU'l [Q
cingulmc b'YfUS 1Oen[jxhinal C(ltex 1O hippocmnpal (or-
mation.
5eptaJ ""'.
Mamillary body
- - -- - _. Mammothalamic
trnct
Anterior nudeus

- -- --- - -Anterior 6mb of
internal capsule
Ciogulate gyrus
--
-
- _ - Cingulum
--
Entorhinal cortex
t-- -- ----- -Perforant
palhway

formation
C. The anterior thalamic nucleus
D. The cingulatc gyrus (Brodmann's areas 23 and 24)
E, The entorhinal area (Brodmann's area 28)
IV. CLINICAL CORRELATION
A. KluverBucy syndrome results from bilateral ablation of rhe amerior temporal lobes.
including the amygdaloid nuclei. It causes psychic blindness (visual agnosia). hyper-
phagia, docility (placidiry), and hypersexuality.
B. Amnestic (confabulatory) syndrome results from bilateral infarction of the hip
pocampal formarion (i.e., hippocampal branches of rhe posterior cerebral arteries and
anterior choroidal arteries of [he inrernal carotid arlcries). It causes anterograde am
nesia (j.e., inabiliry (() learn and remin new information). Memory loss suggests hip-
pocampal pa[hology.
C. Foster Kennedy sy';!drome resul[S from meningioma of the olfactory groove. The
meningioma compresses rhe olfacrof)' traC[ and optic nerw. Ipsilateral anosmia and
opric arrophy and conrralareral papilledema occur as a result of increased imracranial
pressure.
D. The hippocampus is the mosr epileprogcnic parr of rhe cerebnnn. Lesions cause
psychomotor arracks. Sommer's secror is very sensitive to ischemb.
E. Bilateral transection of the fornix may cause rhe aClllC amnesric synJrome (I.e., in
abili[y to consolidate shorr-rem memory inro long-rerm memory).
110 Chapter 20
Agure 20-3. Midsagirml SC(;tiOll rhrou,;h the
brain stcm and dicnccphalon showing the dimibu-
rion of leiians in Wcmicke's encephalOfXlrhy. (A)
nuclcusofthe r1l<I!;IIII11S. (8) Massa in-
rcn",,--dia. (C) Pcrh'enrricubr arC-d. (D) Mamillary
nuclei. () Midbrain and pontine ll-'b'tllCntum. (F)
In{crial- colliculus. Lesions in rhe m."I11lillary nuclei
an:: :1.iSOCimc.:1 widl Wemickcsenccph:llopi.lthy and
lhi:unine (vimmin B.) ddiciency.

A
.
.
.

B
.:.
C
F
0
E
F. Wernickc's encephalopathy results from a thiamine (vitamin B
1
) deficiency. The clin-
ical triaJ includes ocular dismrbances and nystagmus, gait ataxia, and mental dys-
function. Pathologic features include mamillary nuclei. MD nuclei of the thalamus.
and pcriaqueJucml gray and pontine tegmentum (Figure ZO-3).
G. Strachan's syndrome results from high-dose thiamine (vitamin 8
1
) thempy. The clin-
ical triad includes spinal ataxia, optic atrophy, and nerve deafness.
H. Bilateral destruction or removal of the cingulate gyri causes loss of initiative and in-
hibition as well as dulling of the emotions. Memory is unaffected. Lesions of the :m-
rerior cingulate gyri cause placidity. Cingulectomy is used to trear severe anxiety and
depression.
21.
Basal Ganglia and Striatal Motor System
I. BASAL GANGLIA (Figure Zl-l)
A. Components
1. CaudaIe nucleus
Globus Lentiform
panidus nucleus
ippocampus
Fomil(
Internal
capsule
Claustrum
Third q""'\;'" z..:::::l'C Subthalamic
ventricle nucleus
00"' 1m"
Substantia nigra Maminary
I>ody
caudate


FIgure 211. CoronalliCClloll Ihroul;!llhe miJth:.ll:nnlu:u the level of the m,'llllillary N.d.($. 1be rosa11o'<ln-
glia are :.11 prominent lit this and include the striatum lind lentiform nucleus. 11lC :lUbthalamlc nudell) and
sl.lhsr.mria nib'T3 are imrorrnm components of rhe )lrialal IllOlOr Sy)!CIll. eM = nu<:lcu); VA =
veruml anrerior nudell:>; VL = \"cmmll:lrcml nucleus.
1.1.1
112 Chapter 21
2. Putamen
3. Globus
B. Grouping of the basal ganglia
1. The strialUm consisrs of the caudate nucleus and putfl.men.
2. The lentiform nucleus consists of the globus pallidus and putamen.
3. The corpus striatum consists of the lentiform nucleus and caudate nucleus.
4. The claustrum lies between the lentiform nucleus and the insular cortex. It h;l$
reciprocal conncctions between the scnsory cortices (i.e., visual cortex).
II. THE STRIATAL (EXTRAPYRAMIDAL) MOTOR SYSTEM (.,., F;gme 211) pby,
a role in the initiation and execulion of somatic mOlor activity, especially willed move-
ment. It is also imolved in automatic slcreorypt.-.J poslural and reflex motor activity (e.g..
normal suhjccts swing their arms when they walk).
A. SlrUClUre. TIle suiaral motor system includes the following srruclUres:
1. Neocortex
2. Striatum (caudatoput'dmen. or neostriatum)
3. Glohus pallidus
Neocortex
<)
0

0
"
VA, VL eM S1rlatum

,
,
Thalamus
f


i}
::
0
Subthalamic Globus


nucleus pallidus
S
"-
I
if
Substantia
a
nigra
I
Bl11in stem and
spinal cord
Figure 21-2. Major afferent :md efferent conneclions of lhe Strialal syStem. 1ne strialum receives major in
put (rom Ihrcc sources: Ihe thalamus. neocortex. and subsmmia nigra. 1ne striatum projccl5 10 the globus p;ll.
lidus and substantia nigra. 111e globus p.. llidus is Ihe e((<.'Clor nucleus of the s}'stem; it projects 10 thc thal-
amus and $ubd".hlmic llucleus. 111C substanlia nigr:1 also projects to dlC Ihabmus. 111e striatal motor systcm is
c:-;prcsscd throul:h the conicobulb:lr and cOrlic05Pin;11 tmCts. eM = ccntrorncdian nucleus: GAOA "" ..,.
aminobmyric lldd: VA = \'entr:ll anterior nucleus; Vl :so \'cnlr:lllatemi nucleus.
Basal Ganglia and Striatal Motor System U3
4. Subthalamic nucleus
5. Substantia nigra (i.e., pars compacta and pars rei icularis)
6. Thalamus (ventral anterior, ventral lateral, and ccntromcdbn nuclei)
B. Figure 21-2 shows the major afferent and efferent connections of the slrillml system.
C. Neurotransmitters (Figure 21-3)
III. CLINICAL CORRELATION
A. Parkin,;on's disease is a degenerative disease that affl"Cu the substantia nigra and its
projections to the striatum.
1. Results. Parkinson's disease causes a depletion of dopamine in the substantia ni-
gra and striatum as well as a loss of me1anincontaining dopaminergic neurons in
the substantia nigra.
2. Clinical signs are bradykinesia. Stooped posture. shuming b':lil. cOb'whecl rigidity.
pill-rolling tremor, and masked facies. Lewy bodies arc found in {he mclanin-
conmining neurons of {he substantia nigra. Progrl'Ssive supranuclear palsy is
associ3led with Parkinson disease.
3. Treatment has been successful with L-Dop.1. Surgical intervention includes palli-
dotomy (rigidity) and ventral thalomotomy (tremor).
on's disease)
struction results in
atkinson's disease)
GLU
GLU
Neocortex
Blain stem and
spinal cord
"
, (Destruction results in Huntingt ~
~
.
.
.
.
GLU Dopamine
S. nigra:
Thalamus
--"m
Compacta

(De
ACh-

Relicularis
-p
~
~
,
GABAISP
I"
,
,
I
~
,
~ ~
~
,
,
~ ~
~
,
,
,
,
GLU
,
Subthalamic Globus

- - - ~ (Lesions found here
nucleus pallidus
in Wilson's disease)

GABA
,
, ,
,
, ,
, ,
(Destruction results
in hemlballJsm)
(Lesions found here
in kernicterus)
Agure 21-3. M,ljor neurotr:.Illsminers of the slri"tal mOlOr SYSlem. Wllhin lIlI.' sHilltum,!:lohm rallidus. :mJ
pMS relicularis of the SUhsl,lll(ia nigr:1 (S. nigra). y-'lminoh.Jtyric :lCid (GABA) is the prl-dorninalll IlCurouans-
mitler. GABA nlay COCXiSI in the same neuron with enh'ph:llin (ENK) or suhsronce P (SP). Dopamine-
conminin!: neurons :Ire founJ in t h ~ ' p;.lrs comp;.lcm of lI,e substmuia nigr.l. Acelylchollne (ACh) is found in the
loc;II circuli ncurolu of the slri:lIum. 11\e subthalmllic nucleus projecLS excimrOf)' glmmllinell:ic fibers lO the
globos 1:l.11Iidus. GI.U "" jl:lulalliate.
114 ChaPter 21
B. Methylphenyhetrahydropyridine (MPTPHnduced parkinsonism. MPTP is an analog
of meperidine (DemelOl). It destroys dopaminergic neurons in the substantia nif,,1fa.
C. Huntington's disease (chorca) is an inherited autosomal dominant movement dis,
order thm is traced ro a single gene defecr on chromosome 4.
1. It is associated with degenenltion of the cholinergic and y.aminoburyric acid
(GABA),ergic neurons of the striatum. It is accompanied by gyml atrophy in rhe
ftontal and temporal lobes.
2. Glutamate excitocoxiciry. GLU is released in the striatum and binds to its receprors
on striatal neurons resulting in an action potental. GLU is removed from the extra
cellular space by astrocytes. In Huntington's disease GLU is bound to the N-methyl
D-aspanate (NMDA) receptor resu\[ing in an influx of calcium ions and subsequent
cell death. This cascade of events with neuronal death most likely occurs in cere
brovascul:lr accidents (e.g., stroke).
3. Clinical signs include choreiform movements, hYJXItonia, ,md progressive de
mentia.
D. Other choreiform dyskinesias
1. Sydenham's chorea (St. Virus' dance) is the most common cause of chorea over-
all. It occurs primarily in girls, typically after a bout of rheumatic fever.
2. Chorea gravidarum usuallyoccurs during the second trimester of pregnancy. Many
pmiems have a history of Sydenham's chorea.
E. Hemiballism is a movement disorder that usually results from a vascular lesion of ,he
subthalamic nucleus. Clinical signs include violcm contralateral flinging (ballistic)
movements of one or both e"tremities.
F. Hepatolenticular degeneration (Wilson's dise,lse) is;m autosomal recessive disorder
that is caused by a defect in the mct,lbolism of copper. The gene locus is on chromo-
.some: 13.
1. Clinical signs include choreiform or atherotic movements, rigidity, and w i n g ~
beating tremor. Tremor is the most common neurologic sign.
2. Lesions are found in rhe lentiform nucleus. Coppcr deposirion in the limbus of
the cornea gives rise to the corneal Kayser.F1eischer ring, which is a pathogno-
monic sign. Deposition of copper in the Ih'er leads to muhilob.-.r cirrhosis.
3. Psychiatric symptoms include psychosis, personality disorders, and dementia.
4. The diagnosis is based on low serum ceruloplasmin, elevated urinary excretion of
copper, and increOlsed copper concentration in a liver biopsy specimen.
5. Treatment includes penicillamine, a chelator.
G. Tardive dyskinesia is a syndrome of repetitive choreic movement that affect the face
and trunk. h results from treatmem with phenothiazines, bmyrophenones, or mew
c1oprnmide.
22
Neurotransmitters
I. IMPORTANT TRANSMITIERS AND THEIR PATHWAYS
A. Acetylcholine is the major transmiuer of the pcriphernl ncn'Oll:. sy:m'm, neuromus-
cular junction, parasympathetic nervous system, preganglionic symp:nhcric fibers. and
p:>:sq;anglionic sympathetic fibers that inner....:ne SWe:lt glands and some hlOlXl ,'essels
in the skeletal muscles (Fil:.'ure 22-1). ACelylcholine is found in rhe neurons of the so-
malic and visceral mowr nuclei in the brnin Sh'm and spinal cord. It is also found in
the basal nucleus of Meynert. which degencl"3tcs in AI:heimer's disease.
B. Catccholamines. Figure 222 shows the biosynrhcric pathway {orc:uccholamincs. Epi-
nephrine. although a catecholamine, plays an insigninC:lIlt role as a central n('n:QUs
system neurotransmitter. In the body, epinephrine is found primarily in rhe adrenal
medulla. In thc ccntral neryous sysrcm. ir is rcsrricte<! to small ncuronal c1ustcrs in rhe
brain stem (medulla).
1. Dopamine (Figure 22-3) is deplered in parients wirh Parkinson's disease and in-
creased in patients with schi:ophrcnia. Dop..1mine is found in the arcuafe nucleus
of the hypothalamus. It is the prolactin-inhibiting factor. Irs tWO major receptors
are D
l
and D
l
.
Acetylcholine (ACh)


Nucleus basilis 0' Meyner1 in 'orebraink-- -'-- .'
(Alzheimer's disease)
Cranial nerve. molor neurons. and
preganglionic parasympalhetiC neurons
Spinal molor neurons
AUlooomic preganglionic neurons
Local cireuil neurons
in strialum (caudalopulamen)
Hippocampallormalion
Agure 22-1. DistribUlion of acelylcholine-comaining neurons :Illd their axonal projccllons. Tl'IC nu
deus of Meynert rrojcclS 10 Ihe emire COrtcx. Tllis nucleus dcb'CncTmcs in pmicllls with Al:heimcr's dbeas<:'.
5uiaml accl)'lcholinc Ioc:ll circuiT neurons deb'Cnerare in p;lliems wirh d,...ca;;c.
1.15
1.16 Chapter 22
Tyrosine
Tyrosine hydroK)ifase
L-Dopa
Oops d9C8tboxy1ase
Dopamine
Norepinephrine
I'henyMthanoIatnine N-methyt transferase
Epinephrine I
Agure 22-2. Symhesis of catecholamines (rom
phenylalanine. Epinephrine:. which isderived (rolll nor-
epinephrine. is found primarily in the adrenal medulla.
a. 0l receplors are postsynaptic. They activate adenylate cyclase and are exci-
tatory.
b. O
2
receptors arc both postsynaptic and presynaptic. TI,ey inhibit ::ldcnylatc
CyciOlSC and arc inhibitOry. Antipsychotic drugs block O! receptOrs.
2. Norepinephrine (Figure 22-4) is the transmitter o( mOst postganglionic sympa-
thetic neurons. Antidepressant drugs enhance its transmission.
a_ Norepinephrine plays a role ill anxiety slates. Panic attacks are believed to re-
sult (rom paroxysmal discharges from rhe locus ceruleus. where norepineph-
rinergic neurons arc found in Ihe highesl concenlTmion. Most poslsynaptic re-
ceplOTS of Ihe locus ccruleus palhway are 13
1
or 13
1
receptoTS thar activate
a<!('n},late cyclase and ar(' excitatory.
Dopamine
k'''----Cerebellum
""'"
MedoRa
Nigrostriatal tl8Cl

Ventral tegmental
area 01 midbrain
-"'
Substantia nigra of rnidbfain
Mesolimblc Iract (mesocoltlcal tract)

callosum Umbic coltex (cingulate gyrus Striatum (caudate


nUCleus and putamen)
\ SplnoJ ""'"
Figure 22-3. DisrriOOlion of dopamine-conmininG neurons and Iheir projcclioru. Two mlljor ascending
dopamine arise in the midhmin: the nigrostri:mll tmct from the subst;Ulti,1 niGra and the mesolimbic
tmct from the ;lre'l. In patients with Pilrkin;;on's disease, loss of dopaminergic neurons occurs
in the subSlllntia nigra and the ventral teGmental area. Doplll1linergic neurons from the arcuate nucleus of the
hl'l>Olhalallllls projecl to the portilll'esseis of the infundibulum. DopaminerGic neurons inhibit prolncl in.
Neurotransmitters ll7
Norepinephrine (NE)

-
locus ceruleus of pons and midbrain
Thalamus
Cerebellar corteI':
Flgure 22-4. of nnrer;nephrint'-eOflfaininJ.; neur,IOS ,u'K.Ifhcir rnlJCCll0m. The l"cII. ccrult'us
in the pons :md rnidbr,.in) is fhe chief 50tJrcc of Ilor.tdl'<'neq.:ic 6hcf". TIle locus I'roJt'cu h) 1111
f"LnS of the cemml nervous S}S'!("lll.
b, The cCltecholamine hypothesis of mood disorders sTall,'S th:.t r\..JucN norepi-
nephrine activity is related to depression. and that incrca:.cd nllrcpincphrinc
activity is relared to mania.
C. Serotonin [S-hydroxytryptamine is an ind,olaminc (FiguTl.' 22-). Scrol:onin-
containing neurons are found only in the r-dphe nuclei of fhe br..;n stem.
1. The permissive serotonin hypothesis states fhar when )-I-IT activity is rcJuced,
decreased levels of catecholamines cause depression and insomnia. In addition,
when )-HT acrh'iry is increased, elevated levels of c,lIccholamincs cause mania.
Dysfunction of 5-HT may underlie obsessive-compulsivc disorJer.
2. CCTlain antidepressants increase )-HT availability by reducing its rellplake. )-HT
agonists that bind )-HT
1A
and those that block )-HT! have antiJepreSS<l1ll prop-
cnies. Fluoxetine is a SClcclive serotonin reupmke inhibitOr (SSRI).
D. Opioid pcptides (endogenous opiates) induce responses similar to rhose ofl\t'roin and
morphine.
1. Endorphins include f3-endorphin, which is the major endurphin (ounJ in the
brain. It is one of the most powerful analgesics known (48 times 1I10re potent than
morphine). Endorphins arc found exclusively in the hYlxllhabmus.
2. Enkephalins are the most widely distributed and abundant opiate Ix:ptides. They
arc found in [he highest concentration in the glubus pallidus. Enkepha1ins coex-
ist with dop..'1mine, "'f-aminoburyric acid (GABA), norepinephrine. ;lnd
choline. They arc in GABAcrgic pallidal neurons. ;lnd th\.y playa role
in pain suppression.
3. Dynorphins folloll' the distribution map for enkephalins.
E. Nonopioid neuropcptides
1. Substance P plays a rotc in pain transmission. It is most highly cunccnrratt-d in
the substantia nigra. h is also found in the dor.;.,l TOOl ganglion cells :md sub:itan-
tia gelatinQS.1. h is colocali:ed with GABA in the srri:uonigrnl tract and plays a
role in movement disorders. Substance P le\'cls arc reducL-d in paticlUs with
..ton's disease.
us Chapter 22
Serotonin (S-HT)
Raphe nuclei in
midbrain. pons. and medulla
Spinal cord
Hippocampallormation
cerebellar cortex
TI)'plophan
Tryptophan-S-hydroxylaS6
SoHydroxylryptcphan
Aromatic L-amino acid d6catboJl)'faS6
serotonin (SoHydroxylryptamine)
Figure 22-5. Dbuibulion of 5.hydroxyll)'pl:lmine (serolOnin).conl,llning neurons :lnd their projcclions.
Serolonin.conl:lining neurons are (ound in the nuclei o( the mphI,'. TIley proj"'C1 widch' to Ihe (ord)min, cere
bellum. and spin.,1 cord. TIle inset shows the symhetic of .serof(>niJ>
2. Somatostatin inhibiting f;;.c!ur). Somatostatinergic neu
rons from the anterior hypothalamus project their ::rxons to the median eminence,
where som:uost:uin enters the hypoph}'seal portal system and regulates the release
of growth hormone and hormone. The concentration of s0-
matostatin in the neocortex and hippocampus is significantly reduced in patients
with AI:heimer's disease. Srri:ltal somatostatin 11,'\'1,'15 are increased in patients
with Huntington's disease.
F. Amino acid transmitters
1. Inhibitory amino acid transmitters
a. GABA (Figure 22-6) is the major inhibirory neurotransmitter of the brain. Pur-
kinjc, stell<lte, basket, <lnd Goigi cells of the cerebellar cortex arc GABAergic.
(1) GABA-ergic striatal neurons project to the globulus pallidus ::mel sub-
st:llltb nigm.
(2) pallidal neurons project to t.he thalamus.
(3) GABA-crgic nigral neurons project to the thalamus.
(4) GABA receptors (GABAA and GABAB) are intimately associated
with bemodi<lzepinebinding sites. Bcmodia:epines enhance GABA ac
th'ity.
(a) GABA-A receptors open chloride channels.
(b) GABA-B receptors arc found on the terminals of neurons [h;lt use
:mmhcr transmitter (i.e. norepinephrine, dopamine, serotonin).
Activarion ofGABA-B recepwrs decreases the release of the other
mmsmitter.
b. Glycine is rhe major inhibitory neurotrnnsmitter of the spinal cord. It is used
by I he Renshaw cells of the spinal cord.
Neurotransmitters 119
y-Aminobutyric acid (GABA)
Pur1dnje cells of
cerebellar cortex
local circuit GABA neuron
Hypothalamus
Substantia nigra (pars reticularis
Lateral vestibular nucleus
-


Striatum local circuit GABA neurons
Globus pallidus
HypothaIamocort tract Nigrothalamic net
StrIatonigraltract \._-+- Cerebellar nuclei
cortex
Figure 22-6. Distribution of '1-aminobut)'ric acid (GAI3A)containing neurons and their pwjl'ctions.
GABA-ergic neurons ,lfC the m;ljor inhibitory cells of the central n('ryous sySt('m. GABA local circuit neurons
;lfC found in the neocortex, hippocamp;11 fonmltion, ,mel cer{"helhlr coftex (Purkinje cells). Suimal GABA-ergic
n('Uf(ms project to Ihe Ih;lbmus ;md suhlll;lhunic nuc1('us (nOt sho"'n).
2. Excitatory amino acid tmnsmitters
a. Glutamate (Figure 22-7) is the major excitatory tmnsminer of the bmin.
Neocortical glutamatergic neurons project to the 5'fr;atum, .5Ubthal:ltllic nu-
cleus, and thalamus.
(1) Glutamate is the tr.ln5'mittcr of rhc cerebellar I,rranule cells.
(2) Glutamate is also the transmitter of nonnocicepthc, large, primaf)' af-
ferent fibers that enter the spinal cord and brain stem.
(3) Glutamate is the transmincr of the corticobulbar and corticospinal
tracts.
Glutamate
Pyramidal neurons 01 neocortex
_, ./ Striatum
Corticoslriatal fibers --;/-----\)fl-J
Fomix
---'
Septal nuclei
CorticoOOlbar and corticosPioaJ tr,... \
G-.,---\-- Pyramidal cell 01
hlppocampallormation
Granule cells of
cerebellar cortex
! .--,<--Proprioceptive fibers in dorsal roots
Agure 22-7. DbrributiQIl of gluramatC'-i;:omaining Ill'Ul'OIlS :uld lheir projCCtlollS. GluramalC' is IhC' Ilktje,r
excilatory transm;tterof Iht cemml llC'rvOUSS)'lllem. Oxtkal glulamatergic neurons projCCl1OIh(' striatum. Hip-
poclmp:11 :md ;>ubicu!:lr glut1umltcrgic n('urons project through t!\(' (ornlX 10 the S('pt"J.1 area anJ hnx)lh.. lamus.
The gmnul(' cdls of the cerebellum :lre glut;tmarergic,
120 Chapter 22
b, Aspartate, a majorexciratory transmitter of the brain, is the transmitter of the
climbing fibers of the cerebellum. Neurons of climbing fibers arc found in the
inferior olivary nucleus.
c, Beha,'ioral correlation. Glutamate, through its N-methyl-D-aspartate
(NMDA) reptors, plays a role in long-term pmenti:uion (a memOf)'
process) of hippocampal neurons. Glutam:ue plays a tole in kindling and sub-
sequent seizure activity. Under certain conditions, glummat(' and its analogs
arc neurotoxic.
d. Glutamate excitotoxicity. GLU is released in the striatum and hineL. to its re-
ceptors on striatal neutons resulting in an action potential. GLU is removed
from the extracellular space by astrocytcs. In Huntington's d i s c a ~ GLU is
bound (0 the N-methyl-D-aspartatc (NMDA) receptor resulting in an influx
of calcium ions and subsequent cell death. This cascade of evems wirh ncu
ronal death most likely occurs in cercbrovascular accidems (stroke).
3. Nitric oxide is a recently discovered gaseous neurotransmitter thm is produced
when llitric oxide-synthase converts arginine to citrulline.
a. It is locared in rhe olfacwry system, striatum, neocortex, hippoc:unpal forlll:l'
tion, supraoptic nucleus of rhe hypothal:llllus, and cerebellum.
b. Nitric oxide is responsible for smooth muscle rebxation of the corpus cavcr-
nosum and thus penile erection. It is also believed to playa role in memory
formaTion becausc of its long-term potentiation in the hippocampal forma-
tion. In addition, nirric oxide functions as a nitrovasodilator in the cardio-
vascubr system.
II. FUNCTIONAL ANO CLINICAL CONSIDERATIONS
A. Parkinson's disease results from degenetation of the dop:.lminergic neurons that are
found in the pars comp'1Cm of the substantia nigra. It causes a reduction of dopamine
in the striatum and substantia nigra (.sec Chapter 21 III A).
B. Huntington's disease (chorea) results from a loss of acetylcholine- and GABA-
containing neurons in the striatum (caudaloputamen). The effect is a loss ofGABA
in the stri:lrulll and substantia nigra (.sec Chapter 21 III C).
C. AI:heimer's disease results from the degeneration ofcorrical neurons and cholinergic
neurons in th(' basal nucleus of Meynert. It is associated with a 60% to 90% loss of
choline acetylrr;msferasc in the cerebral cortex. Histologically, Alzheimer's dise;lse is
characteri:('d by the presence of neurofibrillary tangles, senile (neuritic) plaques,
:\myloid substance, gr.mulovacuolar degeneration, and Hirano bodies_
0, Myasthenia gravis results from aU[Qantibodies against the nicotinic acctylcholine re-
cepror on skeletal muscle. These antibodies block the postganglionic acctylcholine
binding site. Thymic cells augment B-cell production of autoantibodies. Thc cardinal
manifestation is fntigable weakness of the skeletal muscle. The extraocular muscles,
including the levator palpebrae, are usually involved. Edrophonium or nl"OStigmine in-
je<:tion is used for diagnosis.
E. lambert-Email myasthenic syndrome is caust.'d by a presrnaptic clefL'Ct of acetylcholine
release. It l"aU5C.-S weakness in the limb muscles. bur not in the bulb.1t muscles. Fifry per-
cenr of cases arc associared with neoplasms (i.e., lung. breast, prostatd. In thL"SC
p.1tienrs, muscle strength imprOl;cs with u ~ . (In myasthenia gravis, musch.' use rcsults
in muscle fini/,'11e.) Autonomic d)osfunction includes cll')' mouth, constipation. impo-
tence. :md urinary incontinence.
23
Cerebral Cortex
I. INTRODUCTION. The ccrcbrnl conex. the lhin. gray cO\wing of both hemispheres of
the brnin, has twO rypes: the neocortex (90%) and the allocortex (10%). Motor cortex is
the thickest (4.5 mm): visual cortex is the rhinest (1.5 mill).
II. THE SIX-LAYERED NEOCORTEX. L.1)'CrS II and IV of the neocortex arc mainl)' af
ferent (j.e., receiving). L1.)'crs V and VI are mainly efferem (i.e" scnding).
A. Layer I is the molecular layer.
B. Layer II is the external granular layer.
C. Layer III is the external pyramidal layer. It gives rise [Q association and commissum!
fibers and is the major source of carricQConical fibers.
D. Layer IV is the inlernal granular layer. It receives th:llamocortic:ll IibcN from the
thalamic nuclei of the "cnrral tier (i.e.. \'cmral po5tcrohueral and \'cntral posterome-
dial). In the visual correx (Brodmann's area 17), layer IV reeeh'cs input fl"Ofll the lat-
eral geniculme body.
E, Layer V is the internal pyramidal layer. It gh'cs rise 10 corricobulbm, corricospin:ll.
and corricostriaral fiben:. II conlains Ihe giam pyramidal cells of Bet:. which are found
only in the mOlor conex {Brodmann's area 4}.
F, Layer VI is the muhifonn layer. It is the major source of corticOlhalamic fibers. It
gi\'cs rise (0 projection, commissural, and association fiben:.
III. FUNCTIONAL AREAS (Figure 23-1)
A. Frontal lobe
1. The motor corlex (Brodm:mn's area 4) and premOlor cortex (BroJmann's area 6)
are somatotopically organitecl (Figure 232). Destruction of these areas of the
frontal lobe causes contralateral spastic paresis. Contralmeral promllor drift is as-
sociated with frontal lobe lesions of the corticospinal tracl.
2. Frontal eye field (Brodmann's area 8). Destruction causes deviation of thc eycs 10
the ipsilatcral side.
3, Broca's speech area (Brodmann's areas 44 and 45) is located in the posterior part
of Ihe inferior fronml gyrus in the dominam hemisphere (Fibrurc 23-3). Destruc-
tion resuhs in elCpressi\e, nonfluem aphasia (Broca's aphasia). The patient under-
srands both wrincn and spoken language, but cannot aniculme speech or wrile
nonnally. Broca's aphasia is usually associated with contralateral mcial and arm
weakness because of the itwol\'e1l1Cnl of the motor strip.
4, Prefrontill corlex (Brodmann's nreas 9-12 and 46-47). DcstTucrion of Ihe amI.'-
.1.22 Chapter 23

-Primary visual cortex (17)


_ -JVisual association
1 cortex (39, 19, 18)
\
Auditory associalion cortex (Wernicke's
speech area of lett hemisphere) (22)
4
6
8
,
,
9
PrimaIy motor cortex (4)
\ ,Primary somatosensory cortex (3, 1, 2)
...Vestibular cortex (2)
,
Somatosensory associalion
cortex (5, 7, 40)
Frontal eye field (8) \
Prefrontal cortex
(9,10, 11, ... ..t!:-... r
10 45
Broca's speech area of
lett hemisphere (44, 45) I
Secondary somatosensory ...
and gustatory cortex
,
Primary auditory cortex (41, 42)'
B
Premotor cortex (6) \
Primal)' motor cortex (4h
,
__-r'T--......,I./ Primal)' somatosertSOfY cortex (3, " 2)
Visual association
cortex (19, 18)
-limbic: lobe
,Somatosensory association
, cortex (5, 7)
4
6
,
8
r ......-",,\\ate COrtex
24
9
.r
,f)-
Limbic lobe .... ,
'-...:.'!-' ",,:,'"
,
Septal area"""" 38 \' 37-,19"
Primary visual cortex (17)
Limbic lobe I I \ \ Uncus (28)
, ,
, ,
Primary olfactory cortex (34) 'Parahippocampal gyrus
Prefrontal cortex
(9,10,11,12)-..-..
Figure 23-1_ Some mow.. and sensory areas of the cerebral cortex. CA) Lateral co,wex surface of the hemi-
sphere. (8) MeJial surface of ,he hemisphere. The numbel'5 rcter to ,he Brodmann brain map (Brodmann's areas).
Cerebral Cortex 1.23
B Motor homl,lnCl,lll,lS
Figure 23-2. The :>CI\5OI)' and mOlor homunculi. (A) Scn5Or)' reprcscnmlion in the pom:cnual gyrus. (B)
MOlor reprcscnr:ltion in lhe pr\.'Cemml gyrus. (Reprimed wilh permission from Penfield W, RasmUS$(':n T: T ~
u'Tebrul Corle.1; of Mall. New York, H:lfncr, 1968, pp. 44, 57.)
rior two-thirds of the frontallobc convexity results in deficits in concentr:ltion,
orientation, abstrncting ability, judgment, :md problem-solving ability. Other
fronwl lobe deficits include loss of initiative, inappropriate behavior, release of
sucking and grasping reflexes, &oait apraxia, and sphincteric incontinence. De-
suuction of the orbital (frontal) lobe results in inappropriate social behavior (e.g.,
usc of obscene language, urinating in public). Perseveration is associated with
fronrallobe lesions.
B. Parietal lobe
1. The sensory cortex (Brodmann's areas 3, I, and 2) is somaroropically organized
(see Figure 23-1). Destruction results in contralateral hemihypcs[hesia and aster-
eognosis.
2. The superior pilfietallobule (Brodmann's areas 5 and 7). Desuuction results in
eontmlrllcral flstereognosis and sensory neglecL
3. The inferior parietal lobule of the dominant hemisphere. Damage results in
Gerstmrmn's syndrome, which includes the following deflcits:
a. Right and left confusion
b. Finger a\;nosia
c. Dysgraphia and dyslexia
d. Dyscalculia
e. Contralateral hemianopia or lower quadrantanopia
4. TIle inferior parietal lobule of the nondominant hemisphere. Destruction results
in the following deficits:
a. Topographic memory Joss
124 Chapter 23

(superior longitudinal) fasciculus


(
cooctuction ",nh"",,,)
Motor cortex vocatization (6, 4) "t"' ........

Broca's speech area (44, 45)
(Broca's aphasla)
VISUal association
cortex (18, 19)
Primary visual cortex (17)
speech area (22)
(Wernicke's aphasia)
Figure 23-3. Corti"ll af\'as of Ihe dominant hemisphere that play an important role in l:mguage production.
The visual image of a word is projccu:d from the visU:ll Cortcx (Broom:mn's area 17) 10 the visual associ:Jfion
conices (Broom,mns areas 18 and 19) and then to the angular gyrus (Broomann's area 39). Further procwing
occurs in Wemickc's spo..-ech ,lrell (Brodm:ll\n's area 22), where Ihe auditory form of Ihe word is recalled. Through
the arcuate fasciculus, this inform:llion reaches Broca's speech area (Brodmann's areas 44 and 45), ..,here motor
)pecch programs control the \'ocali:ation mechanisnlS of the precentral gyrus. Lesions of Broca's spcc<:h area.
Wcmicke's speech are:l, or the :lrcuale fasciculus in dysphasia.
b. Anosognosia
c. Construction apraxia (Figure 234)
d. Dressing apra.xia
e. Contralateral sensory neglect
f. Contralateral hemianopia or lower quadrantanopia
C. Temporal lobe
A

..
t(

10
12. II
+-
B c
Agure 23-4. for constRIction apraxia. (A) Thc patielll was asked to copy the f:ICe of a dock. (8)
Th<:- p;!l'lelll was asked to hisect a hori:onralline. (C) The patient w:u asked to copy a cross. 11ldC drawings
)how cOlllralaleral nq:k"Cl. TIle rcsponsihle lesion is found in the l\OI"loiominant (right) parietal lobe. A left
hemi:lll<'Pla. by itself, does not rt"SUh in conrralatetal neglect.
Cerebral Cortex 125

c
( (
9O"11aIa18ra1 Sj)&Slic paresis In leg area
"
Contralalerallowef
quadranlanopia
Figure 23-5. Focal dcslnlcrivc hemispheric lesions and the resulting symptoms. (A) Lmeral convcx surface
of the dominant left hemisphere. (B) Larcml convex surf..KC of the nondorninant right hemisphere. (C) Medial
surface of the Ilondomimlnt hcmispherc.
126 Chapter 23
........................................................................................................................................................._ .
Field at vision
l R
..~ ~ . : . . . . ...
OlfKtk.n-......
l
,,,,,,
Writini
Simple language
comprehension../
l ~ eM
R
visual
'-'--field
Major I'lemisphere
Minor I'lemisphen!
Tnnsected corpus callosum
Figure 23-6. Funclions of the split bnlin a(ter [nmsc<:tion of th!! corpus calloswn. TaClile and visual perc!!P-
tion is projcclN! to the conrr:l!::tter:ll hemisphere. olfaction is perceived on the same sid!!. and audition is per-
ceil'ed predomin:llltlr in the opposite hemisphere. The left (l) hemisphere is dominant for language. The right
(R) hemisphere is dominant for spatial construction and nonverbal idemion. (Reprinted with pcnnission from
Nob"ck CR. Dcm;lrcst RJ: The HUllum Nen'Qus System. Malvern, PA. Lea & Fcbiger, 1991, p. 416.)
1. The primary audimry cortex (Brodmann's areas 41 and 42). Unilateral destruc-
tion resuhs in slight loss of hearing, Bilateral loss results in COrlical deafness.
2. Wernicke's speech area in rhe dominant hemisphere is found in rhe posterior pan
of the superior temporal gyrus (Brodmann's area 22). Destruction results in recep-
ti\"(', fluent aphasia (Wernicke's aphasia), in which the patient cannot understand
any form of language. Spee<h is spontaneous, fluent, and rapid, bur makes little
sense.
3. Meyer's loop (see Chapter 17 11 F 2) consists of the visual radiations that project
to the inferior bank of the calcarine sulcus. Interruption causes conrralatemlup-
per quadrant anopia ("pie in the sky").
Cerebral Cortex 1.27
4. Olfactory bulb, tract, and primary cortex (Brodmann's arca 34). Dcstmetion re-
sults in ipsilateral anosmia. An irritative lesion (psychomotor epilepsy) of the un-
cus results in olfactory and gustatory hallucinations.
a. Olfactory groove meningiomas compress the ollnctory tl<lCt and bulb result-
ing in anosmia. See Foster Kennedy syndrome Chapter 13 I C.
b. Esthesioneuroblastomas (olfactory neuroblastOmas) arise from bilx>lar sen-
sory cells of the olfactory mucosa; they can extend through the cribrifonn plate
imo the amerior cranial fossa. Preseming symptOms arc similar to Foster
Kennedy syndrome.
5. Hippocampal cortex (arehicortex). Bilaterallcsions result in thc inability to eon-
solidme short-term memory imo long-tern) memory. Earlier memories are retrievable.
6. The anterior temporal lobe, including the amygdaloid nucleus. Bilateral damage
results in Kluver-Buey syndrome, which consists of psychic blindness (visual ag-
nosia), hyperphagia, dociliry, and hypersexuality.
7. Inferomedial occipitotemporal cortex. Bilaternllesions result in the inability to
recognize once-familiar faces (prosopagnosia).
D. Occipital lobe. Bilateral lesions cause cortical blindness. Unilateral lesions cause con-
tmlmernl hemianopia or quadramanopia.
IV. FOCAL OESTRUCTIVE HEMISPHERIC LESIONS ANO SYMPTOMS. F i g u ~ 23
SA shows the symptoms of lesions in the dominam hemisphere. Figure ZJ-5B shows the
symptoms of lesions in the nondominant hemisphere.
V. CEREBRAL DOMINANCE is determined by the Wada test. Sodium mnoharbital
(Amyml) is injected into the carotid anery. If the patient becomes aphasic, the anesthetic
was administeu.-d to the dominant hemisphere.
A. The dominant hemisphere is usually the left hemisphere. It is responsible for propo-
sitionallanguage (grammar, symax, and semantics), SlXcch, and calculation.
B. The nondominant hemisphere is usually the right hemisphere. It is responsible for
threc-dimensional, or spatial, perception and nonverbal ideation. II also allows supe-
rior recognition of faces.
Agure 23-7. Chimeric (hybrid) figure of a face
used to examine the hemispheric funC[ion of com-
missurotomized patients. The patiem is instructed to
fixate on the dot and is asked to describe whar he sees.
If he says that he sees the face of a man, chen the left
hemisphere predominates in vocal tasks. Ifhe is asked
to point to the face and he points to the woman, then
the right hemisphere predominates in poiming tasks.
/
128 Chapter 23
VI. SPLlTBRAIN SYNDROME (Figure 2}6) is a discoooeClion syndrome th:u results
from tr:msL'<:tion of the corpus callosum.
A. The dominant hemisphere is bener at "ocal naming.
B. The nondominant. mute hemisphere is benerat pointing (Q a stimulus. A person can-
nOI name objects thm are presemed to the nondominam visual COrtex. A blindfolded
person cannot name objects that are presemed to the nondomin:m[ sensory cortex
duough touch.
C. Tesl (Figure 23-7). A subject ,iews a composite picture of tWO half-faces (i.e., a
chimeric, or hybrid. fib'llre). The right side shows a man: the left side shows a .....oman.
TlH' picture is removed. and the subject is asked to describe what he 53W. He may re-
slxmd that he saw a man. bm when asked to point to what he saw, he points to the
woman,
D. In a p,'uiem who has alexia in the left visual field. the \'erool symbols seen in the right
visual cortex ha\'e no access to the language cemers of the left hemisphere.
VII. OTHER LESIONS OF THE CORPUS CALLOSUM
A. Anterior corpus callosum lesion may result in akinetic mutism or tactile anomia.
B. Posterior corpus callosum (splenium) lesion may result in alexia without agraphia.
C. Callosotomy has been successfully used to treat Mdrop attacks" (colloid cyst of third
,'entriclc).
VIII. BRAIN AND SPINAL CORD TUMORS (.e< Ch,p,,, 5)
24
Apraxia, Aphasia, and Dysprosody
I. APRAXIA is the inability to perform mowr activities in the presence ofinrxt motor and
sensory systems and noml"l comprehension.
A. Ideomotor apraxia is rhe inability, in response (0 [1 vernal command. (0 perform mo-
Wf that can be pcrfomloo with ease sponr:alloously (e.g., slicking our the
tollbruC). This condition is associated with a lesion in the dominant hemisphere.
B. Ideational apraxia is the inability to penonn a multistep activity Of demonsume the
use of a real objt.'Cl (e.g.. (001). This condition is associated with a lesion in rhe dom-
inant hemisphere.
C. Construction apraxia is the inability w clrnwor consrrucr a geometric figure (e.g., the
(ace of a clock), If the patient draws only the right half of the clock. this condition is
called hemincglecr. and the lesion is loc:ned in the riglu inferior parietal lobule (see
Figure 23-4).
D. Gait apr.l.xia is the innbility to use the lower limbs properly. The patient has difficulty
in li(ting his (eet (rom the floor, a frontal lobe sign seen with normal pressure hydro-
cephalus (gait apraxia, dementia, incontinance).
II. APHASIA is impaired or absent by speech, writing, or signs (i.e., loss
o( the capacity (or spoken language). The lesions arc located in the dominant hemi-
sphere. Associate the (allowing symptoms and lesion sites with the :lppropriate aphasia
(Figure 241).
A. Broca's (motor) aphasia
1. Lesion in frontal lobe, in the inferior frontal gyrlls (Brodmann 44, 45)
2. Good comprehension
3. Hfortful speech
4. speech
5. Telegraphic speech
6. Nonfluent spe<.><:I\'
7. Poor repetition
8. Contralnterallower f3cial and upper limb weakness
B. Wernicke's (sensory) aphasia
1. Lesion in posterior temporal lobe, in the superior temporal gyrus (Brodmann 12)
2. Poor comprehension
129
130 Chapter 24
Fluent
,-
Nonnuent
,-
Good
comprehension
Conduction
aphasia
Broca's
aphasia
Transcortical
mol'"
aphasia
Poo'
comprehension
Wemicke's
aphasia
Transcortical
sensory
aphasia
Mixed
transcortical
aphasia
Figure 24-1. TIle Mllphllsia sqUllre
M
makes it easy [0 differentiate the six most common Mnational board"
:tphasias. concluclion, :tnd Wernicke's llphasias are all ch:uoctcrized by poor repetition, (Adapted w(th
pcmlission (rom Miller j, Foumain N: Neur%gJ Recall, B."llrimorc, Willi:tnu & Wilkins, 1997, p. 35.)
3. Flucnr speech
4. Poor repetition
5. Quadrantanopia
6. Paraphasic errors
a. Non sequiturs (L. docs not follow) are statements irrelevant to the quesrion
asked.
b. Neologisms MC words with no meaning.
c. Driveling speech
C. Conduction aphasia
1. Transection of the arcuate fasciculus; the arcuate fasciculus inrerconnects Broo-
m,l1ln's speech area with Wernicke's speech area.
2. Poor repetition
3. Good comprehension
4. Fluent speech
D. Transcortical motor aphasia
1. Poor comprehension
2. Good repetition
Apraxia, Aphasia. and Dysprosody 131
3. Nonfluent speech
E. Transcortical mixed aphasia
1. Poor comprehension
2. GoOO repetition
3. NonAuent speech
F. Transcortical sensory aphasia
1. Poor comprehension
2. Good repetition
3. Fluent speech
G. Global aphasia n."sults from a lesion of the pcrisylvi:m area, which conrains Br0c3's:and
Wernicke's :arcas. Global :aphasia combines :all of the symplOffiS of Broca's and Wer-
nicke's aphasias.
H. Thalamic aphasia is a dominant th:alamic syndrome. It closely rt.'SCmbles a thought
disordcr of patients with schi:ophrenia and chronic drug-induced psychosis. Symp-
roms includc fluent paraphasic speech with nonnal comprehcnsion:and repetition.
I. Basal ganglia. Diseases of the basal ganglia may cause :aphasia. lesions of the anterior
basal ganglia result in nonfluent aphasia. Lesions of the JXlStcrior basal g:mgli3 rC$Ult
in nuent aphasia.
J. Watcrshed infarcts are areas of infarction in the b:>und:lry mnes of the anterior, mid-
dle. and postcrior cerebral artcries. These areas arc vulnerable ro hypoperfusion :and
thus may sep.1ratC Broca's and Wernickc's speech arcas from the surrounding corrcx.
TIlese infarcts cause the motor, mixed, and sensory transcorrical:aphasias.
III. DYSPROSODY is a nondominant hemispheric language deficit that serves propositional
lanb'Uab7C. Emotionality, inflection, melody, emphasis, :lOd gesturing are affccted.
A. Exprcssivc dysprosody results from a lesion that corresponds to Broca's arca, but is 10-
catcJ in the nondominam hemisphere. Patients cannOt express emotion or inflection
in their speech.
B. Reccptivc dysprosody results from a lesion that corresponds to Wernickc's area, but is
locnted in the nondominant hemisphere. P3tients cannot comprehend the emotion-
ality or inflection in the speech they hear.
Appendix: Table of Cranial Nerves
Cranial Nerve T,pe Origin Function Course
I--Olfaetory SVA Bipolar olfactory Smell (olfaction) Central axons
neurons (in project to the
olfactory epithe- olfactory bulb via
lium in roof of the cribriform plate
nasal cavity) of the ethmoid bone.
ll-optic SSA Retinal ganglion cells Vision Central axons con-
verge at the optic
disk and form the
optic nerve, which
enters the skull via
the optic canal. Optic
nerve axons terminate
in the lateral
geniculate bodies.
lIl-ocutomotor
Parasympathetic GVE Edinger-Westphal Sphincter muscle Axoos eltit the
nucleus (rostral of iris; ciliary midbrain in the
midbrain) muscle interpeduncular
Motor GS, Oculomotor nucleus Superior, inferior,
fossa, traverse the
(rostral midbrain) and medial recti
cavernous sinus,
and enter the orbit
muscles; inferior
via the superior
oblique muscle;
levator palpebrae
orbital fissure.
muscle
IV-Trochlear GS, Trochlear nucleus Superior oblique Axons decussate in
(caudal midbrain) muscle superior medullary
velum, exit dorsally
inferior to the
inferior colliculi,
encircle the mid-
brain, traverse the
cavernous sinus,
and enter the orbit
via the superior
orbital fissure.
V-Trigeminal
Motor SVE Motor nucleus CN V Muscles of masti- Ophthalmic nerve
(mid pons) cation and tensor exits via the
tympani muscle superior orbital
Sensory GSA Trigeminal ganglion Tactile, pain, and
fissure; maxillary
and mesencephalic thermal sensation
nerve exits via the
nucleus CN V from the face; the
foramen rotundum;
(rostral pons and oral and nasal
mandibular nerve
exits via the fora-
midbrain) cavities; and the
men ovale; ophthal
supratentorial dura
mlc and maxillary
nerves traverse the
cavemous sinus;
GSA fibers enter the
spinal trigeminal
tract of CN V.
VI-Abclucent GS, Abducent nucleus lateral rectus Axons eltit the pons
(caudal pons) muscle from the inferiOr pon-
tine sulcus, traverse
the cavernous sinus,
and enter the orbit
via the superior
orbital fissure.
(appendix cont.)
133
134 Appendix
Cranial Nerve
VII-Facial
Parasympathetic
Motor
sensory
Sensory
VIII-Vestibull
cochlear
Vestibular nerve
Cochlear nerve
IX-GlosSI
pharyngeal
Parasympathetic
Motor
Sensory
sensory
sensory
Type
Gil!
SVE
GSA
SVA
SSA
GVE
SVE
GSA
GVA
SVA
Origin
Superior salivatory
nucleus (caudal
000"
Facial nucleus
(caudal pons)
Geniculate ganglion
(temporal bone)
Geniculate ganglion
Vestibular ganglion
(intemal auditory
meatus)
Spiral ganglion
(modiolus of
temporal bone)
Inferior salivatory
nucleus (rostral
medulla)
Nucleus ambiguus
(rostral medulla)
Superior ganglion
Uugular foramen)
Inferior (petrosal)
ganglion (in jugular
foramen)
Inferior (petIosal)
ganglion (in jugular
foramen)
Function
Lacrimal gland
(via spheno-
palatine ganglion);
submandibular
and sublingual
glands (via suo.
mandibular
ganglion)
Muscles of facial
eltpression;
stapedius muscle
Tactile sensation to
skin of ear
Taste sensation
from the anterior
two-thirds of tongue
(via chorda tympani)
Equilibrium
(innervates hair
cells of semi-
circular ducts,
saccule, and
utricle)
Hearing (innervates
hair cells of the
organ of Corti)
Parotid gland (via
the otic ganglion)
Stylopharyngeus
muscle
Tactile sensation
to eltternal ear
Tactile sensation
to posterior third
of tongue, pharynx,
middle ear, and
auditory tube; input
from carotid sinus
and carotid body
Taste 'rom posterior
third of the tongue
Course
A.xons exit the pons
in the cerebellar
pontine angle and
enter the internal
auditory meatus;
motor fibers
lJaverse the facial
canal of the
temporal bone and
eltit via the stylo-
mastoid foramen;
taste fibers tr&
verse the chorda
tympani and lingual
nerve; GSA fibers
enter the spinal
trigeminal tract of
CN V; SVA fibers
enter the solitary
tract.
Vestibular and
cochlear nerves join
in the internal audi-
tory meatus and
enter the brain stem
in the cerebellopon-
tine angle; vestibular
nerve projects to the
vestibular nuclei and
the nOCC\llooodular
lobe of the cerebel-
lum; COChlear nerve
projects to the coch-
lear nuclei
A.xons eltit (motor)
and enter (sensory)
medulla from the
postolivary sulcus:
altons eltit and
enter the skuli via
jugular foramen;
GSA fibers enter
the spinal trigeminal
tract of CN V; GVA
and SVA fibers enter
the solitary tract.
(appendix cont.)
Appendix 135
............................................................................................................ ............................................................................................
Cranial Nerve T,pe Origin Function C......
X-Vagal Axoos exit (motor)
Parasympathetic GV, Dorsal nucleus of Viscera of the and enter (sensory)
CN X (medulla) thoracic and medulla from the
abdominal CaY- postolivary sulcus;
ities to the left alIOOS exit and
colic flexure enter the skull via
(via terminal the jugular fora-
(mural) ganglia) men: GSA fibers
enter the spinal
MOlOr SV, Nucleus ambiguus Muscles of the trigeminal Ifact
(midmedulla) larynx and of CN V; GVA and
pharynx SVA fibers enter
Sensory GSA Superior ganglion TactHe sensation
the solitary tract.
(jugular foramen) to the external ear
Sensory GVA Inferior (nodose) Mucous membranes
ganglion (in of the pharynx,
Jugular foramen) larynx, esophagus.
trachea. and
thoracic and
abdominal viscera
to the left colic
nexure
Sensory SVA Inferior (nodose) Taste from the
ganglion (in epiglottis
jugular foramen)
XI-Accessory SVE Axons from the cranial
Motor (cranial) Nucleus ambiguus Intrinsic muscles division exit the
(medulla) of the larynx medulla from the
(except the cOCo- postolivary sulcus
thyroid muscle) and join the vagal
via recurrent nerve: axons from
laryngeal nerve spinal division exit
Motor (spinal) Ventral hom Sternocleidomastoid
the spinal cord.
neurons Cl-e6 and trapezius
ascend through the
muscles
foramen magnum,
and exit the skUll
yia the jugular
foramen.
XII-Hypoglossal GS, Hypoglossal nucleus Intrinsic and extrinsic Axons exit from the
(medulla) muscles of the preolivary sulcus
tongue (except the of the medulla
palatoglossus and exit the
muscle) skull via the hypo.
glossal canal.
SVA - special visceral afferent: SSA - special somatic afferent: GVE - general visceral efferent; GSE general s0-
matic efferent: SVE '" special visceral efferent: GSA '" general somatic afferent: GVA .., general visceral afferent: CN
- cranial nerve.
Index
lullic numbers dcsign:l.lc figures: numbers followed by desii,'l1..11C mblcs; (stt also) refers to related
IOpies Of more detailed IOpic brCllkdowllS. Topics h3\'iog more than one subcntl1' 3rc listed under lhe noun le.g..
Body (bodies): c:;Il"(l(idl.
AbJocenl ncrve (CN VI) (see llnder Cranial nerves)
Accessc>r)' ncr"... (CN XI) (see 1I!1deT Cranial nen'es)
Accommod.1tion. ocular, 66-67
Acetylcholine. 98. 113, 115
Acoustic neuroma ($Chwannoma), 33. 34, 60,
81--82
Al,'flO5ia
I ZJ
\,jsual (d)'slc:da), 125
Alar (sensory) pl:ue, 14. 24, 25
AI:hcimer's disease. 32, 115. J15. 118
,-Aminobulyric acid (GABA), 85, 89, 113, 114,
117.118,1/9
Amyorrophic l:ncral sclerosis (ALS. Lou Gehrig's
disease),46
Ancnc('ph:II)' (mcro;lllcnccphaly). 26, 27
Aneurysm, 67. 74
Angiogmphy
C:lrorid, 18, 19, la, 11
ccrcbml blood supply, 18-19, 10, 21, 22
digiml subtraction, 19.1J-lZ
\cnebral. /9. 21
Anosmi:l,66, 125
Anosognosia.l24
I 29-IJ 1, 130
bus:!1 ganglionic, IJ I
121. 125, 129
conduction, 125. 130
gloh,d. lJ I
Ihalamic,131
transcortical mb:ed. 130
Imnscortiealmotor, 130-131
t r;lllscortiC:l1 sensory, I JI
Wernicke's, 125. 125, 129
Apmxin,129
conmuction, 124, 124. 129
dressing, 124
ideation;ll, 129
idcomotor. 129
Aqucducml stenosis, 28, 29
Amchnoid gmnu[;lfion. 9
Argyll.RobertSOn pupil. 95. 96
ArnoldCfli;lri m:Mormation. 28. 29
Astereognosis. 125
Astrocytes, 31-32
Astrocytoma, 3-4, 87
cerebdlar,34
Ataxia, Fricdrekh's, 48
AudilOl)' system, 58--60
auditOl)' 58.59
hearing defects. 58-59
Autonomic nen'GUS Syslem, 98-102, 99, 100.
101 t (su also Hypor.h.... lamus; Limbic
system)
Axonal transport. 3\
Basal "..anglia, 111-112. 131
Bas:ll (mQ(or) plate. 14. 25
Bet: pyramid..-II cells, 42
Bloodbrain b.1rrier, 32
BloodCSF barrier, 32
Blood supply, 15-23 (see also Anciography and
sptcijic 'ascis)
of internal capsule, 18,90.90
intemalcarolidsystem,15,16,16-17
middle meninl,tCal :Irlery, 19, n, Z3
spinal cord :tnd lower brain stem. 15. 15
thalamic, 90
veins. 18
venous dural sinuses, 18
vertebrohasibr system, 15, 17-18
Body (bodies) (scc also Nucleus InucleiD
carotid, n
Cowdry type A inclusion. 32
geniculate
Inteml, 49, 89, 89, 90, 91 , 93 (sec also Cranial
nerves; II (optic) 91)
medi:ll, 49, 89,89,90
Himno,32
juxtarestifoml, 61,62
Lewy, 32, 113
rruunill:Jry, 1,2,6,7,50,103,105,109
mcdiall,'Cniculare, 52,58,59,81
Negri,32
pineal. I, 49
tmpc:oid, 51 , 58,59
Botulism, 102
Brain lUmors, 28,33-34,82,84,87
137
138 Index
Br.lin
llCOUSlic nO::lIrom:t (schw;mnoma), 3J.--.-34, 81--82
aSUOq'lOma, 34, 87
"blillerl1)' gliOlml" (see Glioblastollla)
bmin abscess. 34
choroid plexus J)<lpillomas, 33
colloid cyst oflhird n'lluide, 33
cmniorharyngiomas, 28,34
ependymoma, J3-J4, 87
1.'Cnninoma of pineal region, 34, SO
gliobl:tslom:l muhifonllC. 33, 34
gliom:l. romine. oplk. 7.34
heln:lllgiobl:btoma.34
llwdul1obbSlOma,3J-J4
meningioma, 33-34
oli1.'Olkndrq;liOfn:I. J4
;!dcrlOlll;l. J4
pcobclinom;l, J4
Broca's aphasia, Ill, 1Z9
Brodrmmnsan.-as.lll
C:.IUda equina, 37
C:.1\"emol.lssinus.57
Centr.ll pollline 1ll)e1inolysis. 1M
Cerebellum. connt"Clions, 85-86
s)ndromc:s. 87
lUmors.87
Ccrebr:ll cono::x fllllclion. 121-1 Zi
front;11 k>he. Ill-I ZJ
limhk. 107-110
1l:lrielllllobc.I23-I24
rO::lI1poT:lllohe. 124
ocdl'il:lliobc. 1Z7
Spill-hmin sydrornc. 127-128
Cerebrospinal tluid (CSF). II, Ill, 32
OlUTea
gr:l\'id;\fUIll, 114
Huntington's, 113, 114,117.118
Sydenh:lm's (Sc Vitus' dance), 114
Choroid pb:us, 34
Omc:\:
allocoftcl(, 121
archicortcl(, 127
cntnrhinnl. 109
no::ocortex, 121
piriform, 66
Corti. orglln of, 58, 59
Cr:1I1inlnerves, 65, 133-lJ5
llhdllCerlt (VI), 65, 67, 68-69,133
Hccessory (XI), 65, 68, 69, 135
HCOl.lSlic (VllI), 58-60, 65.134
cochlear (VllI), 58, 60, 65.134
fad;!1 (VIl), 65. 69-71, 133
glossophnryn1.'\':11 (IX), M, 72-74. 133
hypogloS$al (XII), 65. 76-77, iJ5
illl'cnnedilllO:: (VII), 65, 69, 70
oculomOlOf (Ill), 65, 66. 67. 133
otf."lClOry (1).65.66. 1J3
oplic (II), 65--66. 91, 97, 13J
spinal accessory (XI). 65, 75, 135
Trigeminal (V), 53, 55-57, 67. 68, 133
uochlear (IV). 65, 67, 133
\'agal (X), 65, 7+-75, 135
\'estiblilar (VIII), 61. 6+-65, 71.72, IH
veslibulocochle3r (VIlI), 58, 6+-65, 71, 131
Craniopharyngoma, 27, 28. 31
Crest. neural, 14. 25-26
CriSia amplilbris, 6/
Dandy-W3lker malformation. 19, 19
Deafness. 80
conduction. 58
nerve (sensorineural, perceptive). 59. 68
Diabetes insipidus. 101, 106
Dura mater, 8, 9.21.13
helIlOTThage. 11
O)'norphins. 117
Dyscalculia, 113
Dysdiadochokinesia, 87
Dysequilibrium.87
Dysgraphia, 113
Dyskinesia, tardh'e, 114
Dyslexia, 113, /25
Dysmenia.87
Dysphasi3.88
O)'Sprosodia (dysprasody), /15, 13 I
Dysrhythmokinesia,87
Dyssynergia, 87
DySiaxia, BO, 87
Endorphins, 117
Ependymoma, 33. 87
Eye fields
fromal, 9+-95, III
occipital, 95
Facial nerve (CN VII) (see 14nder Cmnial nerves)
Fewl alcohol syndrome, 29
Filum 9
Finger agnosill, 123
Foster Kennedy syndrome, 66, 109
Fricdfcich's atal(ia, 18
Gait apraxia (dystaxia), 80
'Y-aminobUlyric acid (GABA), 85, 89.113,114,
!17, 118, 1/9
Genninoma, 34, 80
GI:lnd(s)
pineal, 1,2,5
pituitary (hypophysis), 1. 3,27,17,18,57
GlioblaslOma mliltiforme, 33. 34
Glomus. calcified, /4
Glossopharyngeal nerve (CN IX) (seo: under CT3niat
nerves)
Glummate. 119, / /9.120
Glycine. 118
Head tilting, 67
Hearing defeclS, 5&-59
Hematoma
epidur.:il,8, 13, 14,12
subduml,8, 13, 23
Hemianesthesia, 67. 73-74, 80
Hcmianhydl"(l5is, 97
Hemianopia
altitudinal,91
bilemporal, 91,106
with macul:lr sparing, 18,91
HemilxJl1ism, 113, 1Ii
Hemihypesthc,;kl, J25
Hemiparesis (hemiplegia), 125
altem.uing abducem, 68
ipsilaterial,97
spastic, 82
Hepatolcllticular degeneratioll (Wilson's disc'ISC),
113,114
Hernilnion
cerebml,II-14
hippocamp."ll gyrus, 12
illlerverlebral disk, 18
subfa1cial,12
tonsillar (uansfomminal),12
u"dnstemorial (uncal), J2, 66,97
Hippocampalform:uion, 107, 108, 109, 115, /Ji,
JIB
Hippoc;unpus (cornu Ammonis), 4,6, 107
Sommer'S5e(:torof,l09
Hirschsprung'sdisease (mqrdCOlon), 101
Holoprosencephaly, 29
Hydr.lllencephaly, 29
Hydrocephalus, 10, 29
Hypcracusis.71
HYpe5lhc,;ia, 125
Hyperphagia, 109, 127
Hyperreflexia, 56
Hyperscxu;llity, 109, 127
HYPOf;lossal nerve (eN XII) (sec llIW'I" emnial
nerves)
Hypophysis (pituitary gland), 3, Z7. 27, 2B, 104
anterior lobe, 104
lXlsterior lobe, 104
HY1Xlpituilarism, 106
Hypothalamus, 43, 93, 98, /03, 10]-106, /04, log,
IIi, 1/8
in cerebellar disease, 87
Internal capsule, 90
anterior limb of, 90
of, 90
poslerior limb of , 90
JUj,'\llar fommen syndrome, 82-8]
Kayscr-Acischer ring, 114
Kernicterus (nuclear jaundice), 1/3
139
Kemoh."ln's nOlch, /2, 97
L"Imina lermin."llis, 1
Lentiform nucleus, 112
Limbic SYSlem, 107-110, 100, 109
Lipofuscin l,'T'dnulcs, 32
LMN (lower mOlor neuron) lesions. i5, 45-46,
,.
Locus cemleus, 116, 1/7
Medial forcbr.:lin bmin bundle, 105
Megacolon (Hirsch.>prung'sJiscasc), 101
Meninl,'CS,8-IO
Meningitis, 8-10
Ir.lCteri'll, 9-10
viml, 10
Mcnin",'ornyclocdc,28
MClhylphcnyltcrtahydropyridinc (MPTP) induco..'d
patkinsonism, 113
Meyer's loop, 9/, 92,125
Microgli,l (HOrleg,1 cells), 26, 32
Multiplc sclerosis, 48, 80
Myastheni:1 gm\'is, 120
Myelin;ltion, 26
Myotactic reflex, 37
NI.."Ul1Il CfCSt, 24, 25-26
Neurop;lthy
vilamin 81. 105, 110, 110
vilamin B
I
!. 47
Nroropore
anlerior, 26
poslerior, 26, 26
NeUfOlr.:lllsminers, 115--120 (see also specific sub-
_fi)
acetylcholinc.115
'lSp."lrr,Lle, 120
OOp;lmine, 116
Jynorphins, 117
endorphins, 117
enkcphalins, 117
GABA,118
glutam:llc, 119
glycine, 118
nitricoxidc (NO), 101, 120
norepincphrine, 117
scrownin, 117
sotll,ltos(;ltin, liB
sltbilanceP,117,118
\'asoacli\'c intestin;il polypt:plKle (VIP), 101
Nis.;;l substance, 31
Nucleus (nuclei)
abducem,ofCNVI,51,79
:tmbil,'\lus, 50, 51, 73. is, i8, 79
amY1,'<1aloidcus, J. 6, lOS, 108, 127
arCIt."lle (infundibular), 103, 105
arcuale (ruber:tl) hypothalamic. 104, 1/6
basal of Mc)'ncrl, 115, 115
140 Index
Nucleus (nucicil--COll!imd
caudate, 3. 'I, 5. Ii. 41, 55. 90. Ill. "I, 116
cr.mial nen;e nuclC'i
ofCN III
Edinger-Westphal. 94
n'lOl:or. 52.66
ofCN IV, 50
ofCN V, 51. 54
chief sensor)'. 54
mesencephalic. 55-56
mowr.54
spill"!. 55
dCN VI, 5\
of eN V[I, 51. 68-70
ofCN VIII
cochlc"r, 59
veslibular, 50-51, 61--62
of eN IX
;\mbiguus.75
ofCN X
ambigulls. 50. 73
ofCN XI
ambiguus. 75
spinal accessor)'. 75
ofCN XII
hypoglass.'ll. 76. 78
cuneatc, of medulla. 39
denmte. 86. 89
globuspaltidus.III-112
gracile. of medulla. 39
of inferior colliculus, I
hYPolhalamic. 1/6
Icmifonn. III
mamilla!"'i.6. lOS (SCt aUo Body: lnamilJary)
niger. III
oli\'ary
inferior, 50, 61
superior. 58. 59
paran'mricubr of hypOlh"ltunus. 104
phrenic, 37
preoptic, 103
prctccral of midbrain. 94
putalllcn, III
raphe, 116, 117
red, 6, 51, 8/, 86,
rubel', 6. 52, 81, 86
scptal. 109, 116-118
so!itarius (of SQlimr)' [racl),
sublhal;unic. 4, Ii. 113. 113
suprachiasmiatic.103
supraoptic, UN
dl.'1.lamic, 88--89,107,109
-melh)'I-D-asran:ue receplors, 120
)".Slagmus. n. 80
caloric, 63, 63
cerebelbr, 87
IJOSlrol3tory (hori:omal). 63
in unconsciousnc:s.s. 64
\'estibular (hori:omal), 63
Oculomotor nCIYC (CN III) (J' IlI'Idrr Cmni,'ll IlCn"el)
Olfaclory ncn'c (CN I) (J' Imlkr Cmnial
OlfaclOl')' sySlem, 65, 66
Oligodcndrocytcs. 32
Optic nen'e (CN II) (see undtrCranial nen'cs)
Otic placode. 61
Otitis media. 58
Otosclerosis, 58
Oxytocin, 104, 104
113
P"petcircuit, 108--109, 109
Papilledelll;\ (choked disk), 97
Parkinson's disease, 113,\15, 1/6
Pe!"SCver:ltion, 123
Pia-amchnoid, 8
Polyomyetilis, 45--46
Polyneuritis, acutc idiopathic (Gui1tain H.1ITC syn-
drome).48
Posrinfeetious polyneuritis (Guil\ain-B.'lrre
drom<"), 48
Presbycusis, 59
Prolactin.inihihiting factor. 115
Pronator drift, 121
Prasopai,'llosia, /15
Pscudolumor cerehri. 3D
Psychic blindness. 109, \27
Ptosis, 66, 82, 95. 97
Pupil
Argyll-Robertson, 95. 96
fixed dilated. 97
relath'e afferent (Marcus Gunn), 95, 97
Purkinje cell, 85-86
Pyramidal rra<:t, 42--44
Quadranmnopia ("pie in the 91, lB. 124.
115,130
homonymous, 93
Ramus (rami)
communicating, of :lUWllOlllic ncrvous systcm, 98
gray and white communiC;lting, 36, 36
Raphe nuclei, 118
Rathke's pouch, n,17
Raynaud's disease, 102
Rcllex(es)
carO(id sinus. 73, 75
corneal,55. 55l.67,68. 71.80
gag (faucial), 73
jaw jerk (masseler). 56, 56,561
myO(aclic. 36,37
pupillary light (see Cr.mial nen'cs; II (oplic)
91)
Relina,91
Rinne ICSt. 60
Romberg. 47
Index 141
.....................................................................................................................................................................................................-
S,.ccule, 6/,62
Schw,mn cells, 32
Schw,mnoma (:ICOllstic neumm,I), J3, 34, 60
ScOtoma, junclion, 91
Serotonin (5hydroxylryptamine, 5.HT). 117. /18
Signs
&lbinski's,44
Kcrnig's,9
Sinuses, of the dur::l,lO, 57
Spina bitid;l, 26-28
Spin'll cord. 1, 8, 36-48, /16, 1/7
blood supply, 15, 15
components, 36-37
Ie,;ions. 45-48 (St.. Iso specific rnriries'
of mOCot neurons and conicospinalrr.lcu. 45.
45-46,46
mucor/sensory combined. 47-48
pcripher:11 nervous S)'Slem (PNS), 47
sensol')' path....":I). 46,47
positional in development, 27
tracts of, 36-44 (sa Iso TractlsU
SIr:lhismw, 69. 80
Sui.te medullares, 49
Srri;lrcnninalis, 105, 108
Suimum, 112-113, 115-116, 118-120
SulxJrur:11 hClllatOl1m, 8, 13, 23
Substance?, 1/3,117
Suh.tantia nigra, 4. 6, 7, 17, 52, 81,89, J 12,113,
/13,1/6,1/9
Sydenham's chorea (St. Virus' dance), 114
Syndromes
amnCSric (confabulal')'j, 109
,mterior spin31 artery, 46-47
,1Ilterior vermis, 87
Amon's, 84
ArnoldChi;lri,28
Argyll-RobertsOn pupil. 95. 96
Balint's. 84
Bcnedikl's,80-81
Brown !Xqu,ml (spinal cord hemisccrion), 46---47
Central JXlllline myelinolysis, 84
crocodile le,lrs, 71
Dandy-W;llker, 29
dorsal midbmin (P,lrinilud's), 80, 95, 95
(acial collic\llus, 49, 80
(etill alcohol, 29
Foster Kenn'd\', 66, 109
(rontililobc, ) 25
Gersllm1l\I1'S, 125
Guillain 48, 71
Horner's, 47, 79, 80, 93, 95, 97, 102, 105
of inapprollriMe ADH secreTion (SIADH), 106
intcrnucle:lr ophthalmoplegia, 80, 96
jugubr for:lInen, 82-83
Kluver.BlIey, 109, 127
l:lIubertEmon my:lsrhenic, 102
hueml inferior pontine, 80
hlleml medullary (PICA). 78. 79
"Iockoo-in," 8J-84
medi;ll inferior romine, 79, 79-80
mediallongirudinal fasciculus (MLF, internuclear
opluhalrnoplegia), 80, 96, 96
medial medullary, 78
medial midhl3in (Weber's), 82
MObius, 71
m)'cslhenia gl3vis, 120
of tlondolninant inferior p:.IIietallobule, 125
one-and-a-half,96
paramooian midbl3in (Benedikt's), 80-81
PICA. 78
poliom)'elitis,45--46
posterior vermis, 87
pupillaI')' light, 95
Riley-D.lY (familial dysautonomia), 101-102
102
Srrnchan's. 110
subcla\'ian Sle'oIl, 83, 81
of the basilar," 81
Weber's, 81
Werdnig-Hoffmann,46
WernickeKOI$llkoff, 88
Wernicke's encC'ph:llopalhy, 110
S)"ringomelia, 47-48
Tabes dOlS3lis, 46
T..1n)'Cyles, 32
Tardive d)'Skinesia, 114
Thalamus, I. 2, 4, 5, 13, 17,39,41,42,49,62,
88-90,112,113, 113, 1/7, 1/9
blood supply, 90
internal capsule, 90, 90 (sa ol.so Capsule: internal)
major nuclei and connections, 89
Thiamine (vimmin 13
1
) de6denC)', 105, 110, 110
TIc douloureux (trigeminal neul3lgia), 56, 68
Tmcts (see aho P:lthway[sll
conicobulbar, 52, 73, 76, 81,1/9
corticospin:ll, 12, 38, 42, 45, 45-46, 46, 47, 51.
51,54,78,79,82,86,1/9
dcnfOthahlmic,8/
descending sympathetic, 79
dorsal colllmn-meJiallemni:;cus pathway, 38-40, 39
gcniclllocillc;lrinc (ue also Cmnial nerves: II (01'-
tic)91)
Horner's, 78
mamillothalmnic,B9.105
olfactory, 50, 65,127
optic, 4, 6, 7,17, B9, 91 (see ahoCranial nerves:
II (optic))
solitary, 50, 69
nucleus of, 78
spinocerebellar, 00rs.1l, 38
spinorh:llarllic, 38, 47, 51. 79,80, BI , 89
ltltcl3l,78
50, 51, 5/,68,79,79,80
supraopticohypophyseal, 104, 105
trigemi1lQ(halamic, 53-55, 55, B9
::1.42 lnoex
TrilCI.r--Cmuimd
ruberohypophyseal. 104
veslibulospinal, laleml, 38
Tmll5pon, axonal, 31
Trigemin.'ll nerve (CN V) (su undeTCranial nerves)
Tril,.ocminal neuralgia, 56
Tremor, intention (cerebellar), 87
stalic, rdling (Parkinson), 113
Trochlear nerYe (CN V) (su under Cranial nerves)
Tuning fork 1t':S15, 59. 60t
Ulcer, peptic, 102-
Uncal (lranSlclllOial) hernialion, 12, 66, 97
Uncinate ri15, 115
Uncus, 6, 7
UPMN (upper mOlOr neuron) lesions, 45, 45-46,46
Urinary bbdder, 100
Utricle, 61, 62
Uvula,73
VH!.'l.IS nerve (CN X) (see lIluler Cmnial nerves)
V"SQ;l(rive intesrinal peptide (VIP), 101
Ventricles, 8, 9
Ventrolateral thalamotomy, 113
Vertigo, n. 80
Vest:ibular system, 61-64
Vesc:ibuloeoehlear nervt'; (CN VIII) (sa Ul'IdtT
Cranial nerves)
Visual pathway, 91-92
Visual system, 91, 91-97,93-96
Vitamin B
I2
neuropathy, 47
Vi[;lmin B
1
(thiamine) deficiency, 105, 110,
110
Wallerian degeneration, 31
Walel'$hd infarcts, 131
Weber's test, 60
Wernicke's encephalopathy, 103, 105
Wernicke's (fluent) aphasia, 125
Wernicke's speeeh area, 125
Wemig-Hoffman disease, 46
White and gray communicating rami, 36, 36
Wilson's disease (hepatolenticular degeneration),
113,114

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