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DystoniaMedical
ResearchFoundation
CONTRIBUTORS

The expertiseof the following individualscontributedsigrrifrcantly


to the developmentof this publication.Sincerethanksto:

A. Leland Albright, MD Blair Ford' MD


Children'sHospitalof Pittsburgh ColumbiaPresbyterian Medical
Departrnentof Neurosurgery Center,NeurologicalInstitute
Pittsburgh,Pennsylvania NewYork,NewYork

Jeffrey Arle, MD, PhD Mmiit Sangheta, PhD


LaheyClinic Baylor Schoolof Medicine
Deparnnentof Neurosurgery DepartmentofNeurology
Burlington, Massachusetts Houston,Texas

Joel Blumin, MD Beniamin L. ll/alter, fuID


PennsylvaniaHospital Emory UniversitySchoolof Medicine
Departmentof Otolaryngology Departrnentof Neurology
Philadelphia,Pennsylvania Atlanta,Georgia

Guy Bouvier, MD
Notre Dame HosPital
DeparEnentofSurgery
St. Larnbert,Quebec,Canada

This booklet was graciously reviewed


by Scientific Director Mahlon R. Delong, MD,
EmoryUniversitySchoolofMedicine
Departmentof NeurologY
Atlanta.Georgia

This bookletwasfundedin part


by a grant from the
Medtronic Foundation.

Writtenby JessicaFeeley,Editor/SpecialProjectsCoordinator
ADystonta Medical Research Foundation' Prinled 12/03 ' 500
Suncrcer- ron Dvsront.r
INrrnvENTloNs

TlsI,n oF CoNTENTS

I.Introduction.. ...2

I I . P e r i p h e r aSl u r g e r i e s . . . .....3
CervicalDystonia./SpasmodicTorticollis ....3
The BertrandProcedure:SelectivePeripheralDenervation
Rhizotomy
M icrovascularDecompression
SpasmodicDysphonia/Larytgeal Dystonia. . . . . . . . .1
SelectiveLaryngealDenervationand Reinnervation
Thyroplasty
Blepharospasm.... ........10
Myectomy Surgery
GeneralizedDystonia&Hemidystonia.. ....12
Intrathecal Baclofen

III. Brain Surgery: Lesioning Procedures &


D e e p B r a i nS t i m u l a t i o n .....15
Lesioning Procedures: Pallidotomy & Thalamotomy
Deep Brain Stimulation
Comparing Lesioning & DBS
Children & Brain Surgery

IV.Conclusion.. ........25

V.Appendix.... ........26
Dystonia
The Nervous System
Anatomy
Eyes
Neck
Larynx

VI. Sources .......31


Suncrcel hrrrrvrNttoNs ron DvsroNn

I.IxrnooucrloN
As researchaboutdystoniaprogresses, greatattentionis being paid to the
role of surgicalinterventionsfor alleviatingsymptoms.Surgicaltreatments
for dystoniamay be an option for casesthat do not respondto oral
medications or botulinumtoxin injections.Researchers areactivelv
refining currenttechniquesandcollectinginformationaboutwhich
patientsmay benefitthe most from surgicaltreatments.

Thereis no singlesurgicalprocedurethat can be appliedto all forms of


dystonia.Surgicalproceduresfor dystoniacanbe divided into two broad
categories:brain surgery andperipheral surgery. Peripheralsurgery
includesproceduresthat targetpartsofthe body otherthan the brain.

In both brain and peripheralprocedures,the goal of surgeryis to intemrpt


the faulty communicationbetweenthe brain and musclesthat causes
involuntarymusclemovements.Surgeryintendsto treat symptomsand
improvefunctionbut doesnot curethe underlyingcondition.

Becausedystoniais a chronicdisorder,the managementof symptomsis


an ongoing,lifelongprocess.Justasmedicationsandbotulinumtoxin
injectionsareoftennot singularsolutionsto an individual'sdystonia,
surgeryis one componentof the total management of dystonia.Surgery
doesnot necessarilyeliminatethe needfor additionalforms of treatment.
However,in many casessurgeryimprovcsquality of life and reducesthe
needfor medicationsor botulinumtoxin. Like all surgicalinterventions.
operationsto treatdystoniaare associatedwith the risk of certaincompli-
cations.

The patientselectionprocessfor determiningif an individual is a candi-


datefor surgeryis deliberateand precise.Only a neurologistor neurosur-
geonwho specializesin movementdisorderscan recommendsurgeryfor
dystonia.The cost of surgeryvariesby procedureand medicalcenter,
and coverageis often on a case-by-case basisfor Medicareand private
insurance.The successofany surgicalprocedurelies heavily in proper
diagnosis,the experienceof the clinical team,and the skill and artistryof
the surseon.
SuncrceLIltrnvEr-ttors rrx DystoNIe

II. Prnprnnar, SuRcnnv

The symptomsof dystoniaoccur when musclesof the body receivefaulty


informationfrom thebraincausingthemto contractinvoluntarily.These
originatemostcommonlyin a partof the braincalledthe
faulty rnessages
basalganglia.Thesemessages are conveyedover brainpathwaysto the
spinalcord and,from the spinalcord,extendinto the musclesvia nerves.

Peripheralsurgeriesoccur outsidethe brain and generallytargetthe


specific nervesand musclesaffectedby the incorrectmessagesfrom the
brain.Peripheralsurgeriesaregenerallyusedto treatfocal dystonia.An
exceptionis intrathecalbaclofen,which targetsthe spinalcord and is used
to treatgeneralizedor hemidystonia.However,for the purposeof this
publication,a discussionof intrathecalbaclofenis includedunderthe
categoryof peripheralsurgeries.

CERVICAL DYSTONIA/SPASMODIC TORTICOLLIS

The Bertrand Procedure: Selective Peripheral Denervation

Selectiveperipheral denertation surgery for cervical dystonia is


commonly referredto as the Bertrondprocedure.In the 1970s,Dr.
ClaudeBertrand,with the collaborationof Dr. PedroMolina-Negro,
developedthis procedureas a peripheralapproachto treat cervical
dystonia.The term selectiverefersto the caretaken to identify the
nrusclesof the neck affectedby dystonia,and the tenn denen'ation
refersto cuttingthe nervesthat supplythosemuscles.The purposeof the
Bertrandprocedureis to reduceabnormalcontractionsin the affected
musclesby severingthe nervesto thesemuscles.The goal of the proce-
dure is to leave intact the supply ofnerves to unaffectedor less-affected
muscles.

This procedureis tailoredto addressthe uniqueneedsand symptomsof


eachpatient.The initial approachis often to denervatethe muscles
causingthe mostprominentdystonicmovement,knowing that some
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lrroxs ron DvsroNtn

residualmovementsmay remainfrom lesser-affected muscles.If the


resultsdo not sufficientlyalleviatesymptoms.a secondproceduremay be
performed.In manycases,the initial surgeryis enoughto significantly
improvethe abnormalposture.More aggressive surgeries,
in which all
cervicalmusclesinvolvedin the dystoniaaredenervated in a single
operation,may result in temporaryweaknessin the neck.

An essentialparl of the procedureis the pre-operativeevaluationto


properlyidentifythe musclesinvolvedandro assess if theprocedurewill
benefitthe individual. Patientswho may be eligible for the surgeryare
observedclinically by the physicianandwith EMG equipmentto monitor
muscleactivity and pinpoint the musclesaffectedby the dystonia.

One basic elementof the Bertrandprocedureis to cut rootletsof the


spinalaccessorynerve,which Figure I
supplystemocleidomastoid RotatoryTorticollis Laterocolis
musclesin the neck, and to
sparethe nervesto the
trapeziusmuscle.The spinal
accessory nerveis one of 12
cranialnervesthatoriginate
in the brainstem,which is the
junction of the brain and the Super ntecollis sup; Retrocottis
spinalcord.A second
elementof the Bertrand
procedureis cutting the
*'vL
(,tb3
rx
k^

posteriorrami (branch)of
one or more spinalnerves
along the cervical vertebrae. I n f e r i o r A n t e c o l l i s Inferior Retrocollis
(This elementof the proce- .)
dureis calledoosterior /"-
ramiseclonn'.) Spinalnerves
\/<4
i /-/.,\!,..
are arrangedin pairs along s1- \
the lengthof the spinalcord
and supplymusclesand
Y
organs.Some research The six elementalforms of spasmodictorticollis.
Sr nctc.qr-Ilrcnr' rr:rroNsron Dysrosl,r

the irnprovementin personswho


that the ramisectomyincreases
suggests
havebecomeresistantto botulinumtoxin tlierapy.

To dateover2.000ceruicaldystoniapatientshaveundergonethis proce-
dure.Somecentersrepofi significantimprovementin as manyas 887'oof
cases.Althoughthe proceduremay benefitindividualswith a rangeof
symptoms,the categoriesof patientswho may havethe bestresultsfrom
the Berlrandprocedureareindividualsin which:

. Symptomsmainly affectthe neck


. Symptomshavestabilizedfor 3 years
. The headturnsto oneside(rotationaltorticollis)
. The headis tilted(laterocolis)
. The headturnsandis pulledbackwards(rotationaltorticolliswith
superiorretrocollis)
. The headtumsandtilts forward(rotationaitorticoliiswith superior
antecollis)
. The headis pulledback(superiorretrocollis)

Dystoniain which the headturnsboth to the sideand eitherbackor


tbru'ardmay havethe bestoutcome.Indivrdualsr.r'lro
respondto botuli-
num toxin therapyaswell as non-responders rnaybe eligible.The
proceduremay alsobe an optionfor a smallnumberof patientswitli
generalizeddystoniawho havevery definedsymptomsin the neck.

Sideeff-ectsmay includenumbnessin the backof the head,tightrress at


thesurgerysite,sornerernainingmovements. difficulty swallolving,and
lack of beneflt.Patientsare often able to so homeafler two or three
nightsin thehospital.

Studieshavedemonstrated thatthe Bertrandprocedurecan significantly


improle the postureof the neckw'itha betterrangeof nrotion.Physical
therapyfbllowing the procedureis very importantto preserverangeof
motion.
poaDt'sroNre
Suncrclr-INtenvrNrroNs

Rhizotomy

Sincethe late 19thcentury,physicianshave attemptedto treatcervical


dystoniasurgicallyby cuttingthe spinalnervesthat supplythe contracting
muscles.While the ramisectomyelementof the Bertrandprocedure
involvesexcisingspeciticbranchesof the ceruicalspinalnervesnearthe
muscle(away from the spinalcanaland spinalcord),pioneeringsurgeons
initially attemptedto removethe nerveat the root (insidethe spinalcanal
near the spinalcord). The procedureofcutting a nerve at the root is
called a rhizotomy.

Each spinalnerve hastwo roots: a dorsal(posterior)sensoryroot and a


ventral (anterior)motor root. The sensolyroot conveyssensoryinforma-
joints andskin to the spinalcord,andthemotor
tion from the muscles,
root conveyssignalsfrom the spinalcord to the muscles.Cuttingthe
sensoryroots doesnot alter dystoniabut doeshelp spasticity.Cutting the
motor roots-which meanscutting 85-95%of the root innervatinga
dystonicmuscle-will effectivelydenervate the musclebut at the costof
inducingsignificantweakness.

Ventralrhizotornyfor dystoniawas usedwrdelybetweenthe 1930sand


the 1970sand was often combinedwith a denervationof the accessory
nerve.By destroyingthe nerveat the root, the effecton the muscleis
more generalizedand may causea greaterdegreeof weakness.The
resultsof theseprocedureswereoverwheluringly disappointingand
causeda high incidenceof complicationsin dystoniapatients.The ventral
rhizotornyas a treatmentfbr cervicaldystoniawas eventuallyreplacedin
the 1970sby the Berlrandprocedure.

However,selectmedicalcenterscontinueto incorporate rhizotomiesin


their surgicalapproaches dystonia,and researchers
to cer-vical continueto
explorethe effectsof severingthe nervesat differentlocationsalongthe
cervicalvertebraein orderto provideadditionaIsurgicaloptionsfor
dystoniapatients.Selectivedorsalrhizotomiesarecommonlydoneto treat
spasticstiffnessof the limbs(diplegia).
Suncrclr lNrpnvsNrror.ls
ron DvsroMA

Microv asc ular D ecompres sion

Microvascular decompressionsurgery for cervical dystonia is based on


the ideathat variousblood vesselscompressand irritate someof the
cranialnerves(particularlythe spinalaccessorynerve),resultingin
dystonicsymptoms.This surgicalprocedureinvolvesrelocatingthe blood
vesselswithout injuring the vessels,nerves,or muscles.The relocated
blood vesselsare held in placewith small implants.Sectioningof nerves
may or may not be includedin the procedure.There is very little pub-
lisheddataaboutthis procedure,and it has beenlargely abandonedfor
dystoniapatients.Adverseeffectsinclude lack of benefit,cerebrospinal
fluid leakage,and stroke.There is evidenceto suggestthat if a patient
undergoesdecompressionsurgeryand is not satisfiedwith the results,the
partial removal of the occipital bone and scarringthat results from
microvasculardecompression may make it difficult for a subsequent
surgeonto perform a safe and effective Bertrand procedure.

SPASMODTC DYSPHONIA/LARYNGEAL DYSTONIA

Selective Laryngeal Denervotion and Reinnemation

Selective laryngeal adduction denervation and reinnervation


(SLAD/R) is a surgicalprocedureto treat adductorspasmodicdysphonia/
laryngealdystoniaby cutting (denervating)selectedend branchesofthe
recurrentlaryngealnerye,which is a branchof the vaguscranial nerve.

The first attemptsto reducethe spasmsof spasmodicdysphoniaby


severingthe laryngealnervetook placein the 1970s.Cutting the laryngeal
nerveparalyzedthe musclescontrollingone sideof the larynx so that the
larynx could not contractexcessively.Early resultswere good,but
symptomsreappearedin manypatients.Subsequentpioneersin the field
soughtto improvethe procedureby varying the methodby which the
nerve was separatedfrom the muscle.Recurrenceof symptomsas well
as breathyvoice continuedto be a problemin many patients.
Sunocal hrBnvrxnoNs ponDvsroMA

The elementthat distinguishesSLAD/R from previousincarnationsof the


surgeryis that, after the recurrentlaryngealnerve is cut away from the
thyroarytenoidand lateralcricoarytenoidmuscles,the nervestumpsare
hookedup to anothernerve(reinnervated),one that is not associatedwith
the dystonia.Supplyingthe musclewith anothernervepreventsthe
problematicbranchof laryngealnervefrom growing back and reconnect-
ing to the muscle.Preventingthe laryngealnervefrom communicatingto
the musclepreventsthe spasmsfrom returningand helpsto changethe
closingforcesof the larynx. It is importantto notethat the procedureis
performedbilaterally,unlike previousnerveoperationsperformedfor
adductorSD. Becausethe disorderoriginatescentrallyin the brain,it
likely existsbilaterallyin the larynx. It is thereforelogicalto treatboth
sides.

The procedureis accomplishedby makingan incisionin the neckand


then creatinga small window into the laryngealcartilageto exposethe
underlyingnervesand muscles.An operatingmicroscopeis often usedto
aid in identificationand suturingofthe tiny nervebranches.The proce-
dure takesthreeor four hours to complete.Greatcare is takento pre-
servethe back part of the cartilagethat protectsthe nerve branchesto
the breathingmuscles.

SLAD/R is bestsuitedfor individualswith spasmodicdysphoniawithout a


tremor.It may be an option for personswho are not satisfiedwith
botulinumtoxin treatments.More than two hundredpersonswith spas-
modic dysphoniahaveundergoneSLAD/R over the courseof about l0
years.During the initial recoveryperiod,all patientsexperiencetempo-
rary voice breathinessand someexperienceswallowingdifficulty. These
issuesresolveover a few monthsand the patientis left with a near-
normalvoice, free of spasm.Studieshave indicatedthat as many as 85-
90Yoof patientsare very satisfiedwith the resultsof surgery and the
results.so far. havebeenlife lons.
Sunctcel INrsnwNloNs ron DYsroNn

Thyroplasty

'fiwroplas\,
surgeriesincludea group of surgicaltechniquesto modif-v
'fhese
thecartilagesurroundingthe larynx. adjustableandreversible
procedures involvemanipulating thecartilageby implantingwedeesor
to hold the tissuein place.A numberof variationsof this procedure
:-;hims
are currentlyusedand are effectivefor restorationof the voice after
paralysisor in changingthe pitchof the voice.

'lype
I thyroplastyhasbeenusedfor the abductorvarietyof spasmodic
dysphonia.In this procedure,the vocalcordsare broughtclosertogether
in hopesof decreasingthe effectof the abductorspasms.Resultsare
mixed,with somepatientsgettinggoodreliefandothershavingminimal
effect.

-lype
lI thyroplastyis a procedurefor adductorspasmodicdysphoniathat
involvesspreadingthe vocalcordsapartby insertinga shimthatprevents
them from contactingeachother during the spasmsthat occur with this
disorder.Although somepatientshavereportedgood relief of vocal strain,
othersfeel the trade offto a breathvand weak voice is excessive.

Researchers in the US and abroadcontinueto investigatethyroplasty


procedures.The advantageofthese proceduresis that they are largely
non-destructiveand do not alter the musclesor nerve supplyof the
larynx. They work throughadjustmentof biomechanicsaloneand are
theoreticallyreversible,althoughin practicethe reversibilitymay be
limited by scarring.
Sunctcal lttrnvENTtoNs FoRDvsro:l'te

BLEPHAROSPASM

Myectomy

Surgicalrem.val'f the eyelidand brow-squeezingmusclesis ref'errecl


to
as a mvecromvprocedureand is usedto treatblepharospasm. Ml,ectomv
preventsthe musclessurroundingthe eyesfrom beingstimulatedby
removingthemuscle.

tseforetheavailabilityof'botulinumtoxin,mvectomvwasessentiall;-
'[-he the
only treatmentoptionfor blepharospasm. introductionot'botulinunr
toxin injectionsin r989benefitedmanypersonswith brepharospasm
tf erebychangingthepopulationof individualseligiblefo. myeciorn,r-..
candidatesfbr mv'ectomv becamethosefor whom botulinumtorin rsnot
sufficient.

Justas the patientselectionchanged,trreprocedureitselfevorved.


lnitially,theprocedureinvolvedremovingall eyelid-squeezingmuscres in
both upperand lower lids as well as the brow areaat one time.
At the
presenttime,the procedureis tailoredto the needsof the patients.
It is
mostcommonfor the surgeonto removethe muscrein the upper
eyerids
and brow (full upper mvectomy)and then re_evaluate the neld fo. a
lower myecromyat a later date. patientshearfasterwhen the procedure
is done in stages,and someindividuarsdo not requirethe rower
myec-
tomy.

The full uppermyectomymay be doneentirelythroughan eyebrow


incision.The incisionliesimmediatelyadjacentto thebrowhairand
allowsaccessto the upperlid orbicularismuscle,andpartof the rower
lid
orbicularismuscleas well as the procerusand muscresin the
"o^rguto.
brow area.Most of the orbicularismuscleis removed during the eyelid
surgery.A strip of densemuscleis left at the margin of the upper
eyelid
to help maintainsomevoluntaryclosureand to protectthe eyelash
roots.

l0
Srrncrclr-INrenvrNrroNsponDvstotue

A limited upper myectomyis a partial upper myectomy. It is available for


thoseindividualswho arebenefitingfrom botulinumtoxin but need
somethingextra to restorefunction of the eyes.lt may be helpful for
thosepatientswho haveapraxia(difficulty openingthe eyes)or for those
who in additionto blepharospasm haveptosis(droopinglids).Partial
removalof the orbicularismay subsequentlydecreasethe needfor
botulinumtoxin in thesepatients.A limited myectomyis donethroughan
uppereyelidcreaseincisionand involvesremovalof the orbicularis
musclewithin the upper lid areaonly. Becausethere is lesstissuere-
moval than the full uppermyectomy,patientsrecoverin lesstime. A
limited myectomyalsogivesmorepredictablecosmeticimprovement
becauselesstissueis removed.lt is not designedto replacea full upper
myectomy.Most patientswill still requirebotulinumtoxin injections
following the limited myectomyprocedure.

Personswho havestoppedrespondingto botulinum toxin as well asthose


rare individualswho fail to respondat all may be eligible for myectomy.
Individual surgicalcentershavetreatedhundredsofblepharospasm
patientswith myectomy.Techniquesusedfor cosmeticsurgery suchas
sculpting the fat beneaththe brow and manipulating the placementof the
brow, may be implementedto provide a beneficialaestheticas well as
functionalresult.

Myectomy surgerycan be done under local or generalanesthesia. The


healingprocessfollowing a myectomymay take up to a year.ln most
cases,the patientsare able to keeptheir eyesopen immediatelyfollowing
the operation.However,considerableswelling,hematomas(blood accu-
mulationin lid), lymphedema(tissuefluid), and bruisingmay be present
early in the post-operativeperiodand preventcompleteeyelid opening.
Cool compressesin the first four to five daysfollowed by warm com-
pressesarevery helpful at settlingthe lid swellingand bruising.

There are numerouspotentialside effectsassociatedwith myectomy


surgerythat are predictableand,to somedegree,occur in most patients.
Numbnessof the foreheadregionoften occursand is usuallytemporary

ll
Sunctcel In-rrrveMnoNsron DysroMA

but may last a year or more. Loss of tissuevolume in the eyelid areamay
occurwith the muscleremoval,but the improvedbrow, lid position,and
decreased eyelid wrinkling generallygivesan improvedcosmeticappear-
ance.Decreasedeyelid closureoccursas a result of eyelid muscle
removaland may requirethe needfor additionalartificial tearsand
lubricatingointment.As the eyelidswellingresolves,the eyelidclosure
improvesand the dry eye symptomsgenerallyimprove.Chronic lid
swellingwhich may last six monthsor longerin somepatientscan be a
chronicandtroublesomecomplication.Chroniclid swellingis much less
severeand persistentin the modernmyectomypracticesin which upper
and lower lid myectomiesare performedseparately.lnfection,hematoma,
brow hair loss,and abnormalpositioningof the lower lid canoccasionally
occurbut are uncommon.

Patientscontinue to improve in function as well as in appearancefor


about six months to a year after myectomy surgery.Reports have shown
thatvisualdisability is improvedin approximately90Yoof patients.Some
patientshave more improvementthan others.Touch-up proceduresare
requiredin somecases,and someindividualscontinueto requirebotuli-
num toxin injections.

GENERALALED DYSTONIA & HEMIDYSTONIA

Intrathecal Baclofen: The Baclofen Pump

Baclofen(Lioresal@)is a medicationintroducedin the late 1960sas a


treatmentfor spasticity.I'he medicationis alsocommonlyusedto treat
selectcasesof dystonia.Baclofenin the spinalfluid aroundthe brain and
spinalcord supplementsthe body'ssupplyof a chemicalneurotransmitter
calledGABA, which relaxesmusclemovement.The drug may be given
orally,but very high dosesmust often be usedto ensurethat the drug
safuratesthe blood streamand reachesthe spinalfluid. High dosesof
baclofenmay causeintolerableside effectssuchas muscleweakness
andfatigue.A surgicallyimplantedbaclofenpumpdeliversbaclofen
directlyto the spinalfluid, and only very small dosesare needed.(The
term intrathecal meansin the spinal fluid.)
SI R(,IC\I I\I FR\I NIIU\\ I ' ( ) RD \ S I O N I \

Intrathecalbaclofentherapyis Figure 2
a non-destructive,adjustable,
and reversibletreatment.
l
Severalhundred dystonia a
*
patientshavebeentreated 7
,{r
u ith intrathecal
baclofenover rt
the courseofabout l0 years. :\{t
It hasbeenusedfor children
lfi

*s
andadultswith generalized
dystonia(bothprimaryand ;a
secondary t andhemidystonia
u,horespondto baclofen.
Many personstreatedwith
intrathecalbaclofenhavea
combinationof dystoniaand
cerebralpalsy.lntrathecal
baclofenmay be usedto treat
dystoniaaffectingthe upper
andlowerlimbs.

In orderto determineif an
f'
individualis eligiblefor intrath-
I tt tt'q tlt ec'qI bcrc ktf'ert
ecalbaclofen,he/shewill undergoa
hutdvare in body.
screeningtestto obseruethe body's
responseto baclofen.A responseto the oral drug rnaynecessitate
a screeningtestto observethe body'sresponse to a small doseinjected
directlyinto thespinalfluid. Thernedicationis injectedusinga standard
lumbarpunctureor spinaltap.The screeningtestprocedureinvolves
injectionof the medicationfollowedby severalhor.rrs of observation.
Relaxationof themusclesindicatesthatan irnplantedbaclofenpurnpwill
likely be eff-ective.The effectsof the screeningtest are telnporaryand
may lastseveralhoursafterthe injection.If a patientdoesnot responda1
all to the screeningtest,a secondtestusingthe sameproceduremay be
tried the next day or at a later date.

IJ
Suncrcer-INrenveunoNsror. DysroNn

Somephysiciansusea continuousintrathecalinfusionof baclofenasa


screening method,sincemorepatientsrespondto continuousinfusionthan
to singleinjectionscreening doses.In theinfusiontechnique,a small
catheteris insertedinto the spinalfluid andis connected
to an external
pumpthat infusesbaclofenin increasingdosesover two to threeda_vs.

Starting intrathecal bacl of'cntherapyi nvolvessurgically'implantinga


'[-he
pro-{ramrnable pump in thc body. deviceis usuallyplacedeitherto
the rightor left of the bellv button,beneaththe skin and fat of the abdo-
'['he
rnen. purnpis connectedto a thin tLrbethat is tunneledaroundthe
sideof thebodyto the back.A srnallneedle introduces thetubeto the
spinalcanal. Oncethesurgicalincisions areclosed.thepumpis adjusted
by a remotecomputerized deviceto deliverthe amountof medication
appropriatefor the individual.The proceduretakesone to two hours.and
the hospitalstay may rangefrom four to sevendays.Modest improve-
mentof symptomsmay be noticeablebeforethe individualis discharged
from the hospital,and it may takesix monthsor moreto achievethe full
extentof benefit.

Regularmaintenance is a key componentof intrathecalbaclofentherapy.


Regularexamsand physicaltherapymay be a componentof postopera-
tive care.Pumpsmustbe refilledeveryoneto four monthsin the
physician'sofltce as a straightforwardoutpatientprocedure.The pump is
refilledby insertinga thin needlethroughthe skin,into thepump.The
frequencyof refillingthe pumpdependson the doserequired.If neces-
sary,the doctor may adjustthe delivery rateof the pump at the time of
the refill by remotecontrol.The pump batterylastsapproximatelyseven
years,dependingon how much medicineis programmedto be delivered
eachday.Before the batteryruns out, the pump will needto be replaced
with a new pump througha surgicalprocedure.The cathetercan usually
stay in placeand be reconnectedto the new pump.

Baclofenin the spinalfluid relaxesmusclesthroughoutthe body,and


appearsespeciallyeffectivefor targetingdystoniain both the upperand
lower half of the body.ITB may be more effectivefor treatingsecondary
dystoniathanfor primarydystonia.

t4
Suncrcer-Ilrrnmmons nonDvsroNIA

Studieshave shown that intrathecalbaclofen can dramatically improve


symptomsand quality of life. Somecentershavereportedsignificant
irnprovementin as much as 85Yoof patients.However,like any surgery
the procedureis not without risks.Hardwarecomplicationsmay also
arise including infection and catheterbreakageand disconnection.In a
small percentageof cases,symptomsmay resumeor worsenwithin the
first year.The most common side effectsare constipation,decreased
musclecontrol,and drowsiness.

III. BncN Suncnny:LnsroxnvcPnocnouRns


& DnBpBnenvSrnrur,l^nox

The goals of brain surgery for personswith dystonia are to decrease


musclespasms,increasemobility andfunction,and improvepain.

There are currently two categoriesof brain surgery for dystonia:


lesioning procedures, which involve selective destruction of targeted,
abnormal brain tissue, and deep brain stimulation (DBS), which mimics
the effectsof lesioningby manipulatingselectivebrain areaswith non-
destructiveelectricalpulses.

Although risks exist,casestudieshaveshownthat both lesioningproce-


duresand DBS can result in markedimprovementof dystoniawith
minimal complications.Somepatientsare able to decreaseor altogether
stopdrugtherapyfollowing surgery.

Dystoniamost often originatesin a part of the brain called the basal


ganglia which are involved in the coordinationand control of muscle
movements.The basalgangliaare a group of structuresthat includethe
globus pallidus (also called the pallidum), the thalamas, and the subtha-
lamic nucleus.Lesioningproceduresfor dystoniausually targetthe
globuspallidusor the thalamus;deepbrain stimulationusuallytargetsthe
globuspallidusor subthalamicnucleus.The globuspallidusis responsible
for the outputof messagesfrom the basalganglia.The recipientof this
output is the thalamus.The subthalamicnucleusis a tiny structurelocated
directly beneaththe thalamusand is connectedto the globuspallidus.
ponDysroNn
Suncrcel lNrsnvsNTroNs

Different partsof the brain work togetherto help the body accomplisha
specifictask,suchas tappingthe foot. 1-hepartsof the brain communi-
catevia pathwaysof individualbraincellsthattransmitchemicalmes-
sagesfrom one to the other.In an indrvidualwith dystonia,the pathways
that facilitatethe movementof the foot are disruptedby abnormal
activity.The goal of brain surgeryis to freeup the pathwaysso that the
brain andbody may accomplishthe intendedfunction-in this case,
movingthe foot.

Brain surgerymay be performedunilaterally(on one sideof the brain) or


bilaterally(on both sides).The effectsofsurgery occur on the side ofthe
body oppositeto the surgicalsite.

To date,most personswho haveundergonebrain surgeryfor dystonia


were treatedfor generalizeddystonia.However,individualswho may be
eligible for brain surgeryincludepersonswith focal, segmental,or gener-
alizeddystoniawith significant,disablingsymptomsthat do not respond
satisfactorilyto othertherapies.Adults aswell as childrenwith primary
and secondarydystoniamay be eligible.

Basedon the limited availabledata,different categoriesof patientsmay


responddifferently to brain surgery.Although casesof both secondary
dystonias(includingtardivedystonia)andfocal dystoniasmay be eligible,
it appearsas thoughpersonswith DYT-l generalizeddystoniaare the
bestcandidatesfor brain surgery---e ither lesioningor DBS. Studieshave
shownas much as 60-90%o improvementin DYT-l patientstreatedwith
lesioningor DBS. Patientswith secondaryhemidystoniamay b9 eligible
for brain surgery,though they may not benefit as much as thosewith
DYT-l dystonia.Researchers are examiningthe possibilitythat persons
with secondarydystonia may get greaterbenefit from lesioning or DBS to
the thalamusrather than the globuspallidus.

There is limited data about the long-term effects of each approach.Brain


surgeryfor dystonia is an evolving science,and investigatorsare continu-
ally collectinginformation.

l6
Suncrcel hrsnvENTroNS
FoRDysroNrA

Lesioning Procedures: Pollidotomy & Tholamotomy

'l'he
practiceof lesioningpartsof the brain in dystoniapatientswasverv
comnlonin the 1950sand 1960s,sinceat thattime it wasessentially the
only availabletreatmentfor severecases.Theseprocedures. as practiced
50 'vearsago,had mixed results.In sonrecasesthc inrprovement u'as
spectacular;in othercasescomplications developed;and in still other
casesrepeatedprocedureswere necessary.By the 1980s,brain surgery
for dystoniahad fallen out of favor and was not widely practiced. How-
ever,the increased understanding of the basisof movementdisorders
suchas Parkinson'sdiseaseand the successin treatingit with surgical
approaches, plus the developmentof brain imagingtechnology,led to a re-
evaluationof surgeryas an option for patientswith dystonia.

The procedurethat involvescreatinga destructivelesionin the globus


pallidus is called pallidotomy,and the procedurethat involvescreatinga
lesion in the thalamusis calledthalsmotorny.A permanentlesion is made
in the brain tissueby heatingthe tip of an electrodeand coagulatingthe
intendedtissue.

When lesioningsurgeryis chosen,pallidotomyis now preferredover


thalamotomyand providesa reasonablealternativeto pallidal DBS for
patientswho are averseto the cosmeticappearance of the implanted
pulse generatoror do not want to be burdenedby repeatedbattery
replacements. Bilateral pallidotomyhasshownan averageof 67-80%o
improvementin the Burke-Fahn-Marsden dystoniarating scalein patients
with generalizeddystonia.Primarygeneralizedpatientsrespondbetter
than focal or secondarydystonias.ln Parkinson'spatients,bilateral
pallidotomiesareavoidedbecausethey causehypophonia,a quietingof
speech.This hasnot beenobservedin dystoniapatients,and many
dystoniapatientshavehad bilateral pallidotomieswithout significant
worseningof speech.

Although thalamotomywas once the most common brain surgeryper-


formed for dystonia,it is now usedalmostexclusivelyin casesof stable
hemidystonia,and a very specificsite in the thalamusis targeted.The
procedureis performedunilaterally.Bilateralbrain surgeriesincreasethe
risk of complications,and bilateralthalamotomiesin particularareknown
to often causespeechimpairment.

The primary factor that distinguishesmodern lesioning proceduresfrom


thoseof 50 yearsago is that surgeonsare able to locatethe lesioning
targetmore accurately.The following factorsmake it much easierfor the
surgicalteamto locatethe targetwithin the brain,which is crucialto
reducingthe risk of complications:

. Stereotaclrcs-Surgeons are able to target the precise area of the


brain with a computerized,3-dimensionalscaleusingMRI and CAI
scans.

. Microelectrode recording and brain mapping-The surgical team


hasthe ability to listento the soundsof brain cells firing messages to
one another.Cells in different parts of the brain fire at very specific
ratesand in characteristicpatterns,and by listening to thesecells the
surgeonknows exactly wherethe electrodeis within the brain.
Severalrecordingtractsmay be necessaryto identiff the precise
target.

Once a physician has recommendedbrain surgery and pre-operative


screeningtestsand preparationsare complete,the basicplan of operation
for pallidotomyandthalamotomyarethe same.The individual is fitted
with a headframe under generalor local anesthesia. The brain is mapped
with imagingtechnologyto createa blueprintfor planningand measuring
the placementof the electrode.Under local anesthesia, the electrodeis
insertedthrougha smallhole in the skull into the brain.The brain itself
doesnot feel pain, and the patientis awakeduring most of the procedure.
The surgical team interactswith the patient throughoutthe procedure,and
the patientprovidesfeedbackaboutsymptomsand how he/shefeels.
Microelectroderecordingis usedto confirm the target.The mapping
procedurealone may take up to severalhours. Once the target is defined,
the surgeoninsertsthe thermal electrodeand createsa lesion. The
thermal electrodeis removedand the procedureis complete.A bilateral

l8
proceduremay be done in a single surgeryor in two separatesurgeries.
lf a secondtarget is to be lesioned,the mappingprocedureis repeatedfor
that specifictarget.Most patientsare in the hospitalfor two or three
days.IVledications may be temporarilyresumed,and after a short time the
patientreturnsto the neurologistfor a follow-up exam.
'l'here
is a smallbut realrisk of complications
associatedwith lesioning.
'l'he
mostseriousrisk is a 1-2Yoincidenceof strokeor hemorrhage during
the mappingphaseof the surgery.Also, the targetof the pallidotomy,the
internalsegmentof the globuspallidus,is locatedright abovethe optic
tract which may be damagedif the electrodeis not targetedprecisely.
-l'here
alsoexiststherisk thatthe pallidotomywill not improvethe symp-
toms.Howeveqthe procedurehasbeenshownto dramaticallyimprove
dystoniain somepatients.

Deep Brain Stimulation

Deepbrainstimulation(DBS) involvesimplantingstimulatingelectrodes
into selectedtargetsin the brain in orderto mimic the effectsof lesioning.
SurgeonsbeganusingDBS in placeof lesioningfor Parkinson's disease
patientsin the mid-1990s.DBS alsohasapplications to tremorand pain.
WhereasDBS has beenusedto treat thousandsof personswith
Parkinson's disease,the procedurebeganbeingappliedto dystoniaonly in
the late 1990s.The resultsof more than 200 dystoniapatientshave been
publishedascasestudiesin medicaljournals.

BilateralpallidalDBS producessignificantbenefitin dystoniawith


averageimprovementsof about 50-60%in the Burke-Fahn-Marsden
dystoniaratingscale.Someprimarygeneralized patientshavebeen
repoftedto haveup to 90% improvement.DBS has also beenperformed
on personswith secondarydystonias,cervicaldystonia,segmental
dystonia,andmyoclonicdystoniawith encouragingresults.

l9
The complete Figure 3
DBS apparatus
includesthe
DBS electrode.
a connecting
wire, and a
pulsegenerator
(a.k.a."brain
pacemaker"or
stimulator)that
contains
a battery.The
initialprocedure
to implantDBS
is identicalto that Deep brain stimulation horchrore in body.

of the pallidotomyandthalamotomy. Oncethe braintargetis rnappedand


identified,insteadof creatinga lesion,the surgeonplacesthe DBS
electrodeinto the target.The wire and pulsegeneratormay be irlplanted
at the sametime as the electrodeor at a laterdate.The generatoris
implantedunderthe collarboneor in the abdomen.The ri ire is then
tunneledup tlie neck,behindthe ear.and to the siteof the electrode(the
patientis undelgeneralanesthesia fol this part of the procedure). The
wire is connectedto the electrode.and the incisionsareclosed.Most
DBS procedures involvethe irnplantation of two generators anc'laredone
in two surgeries. It is possible
to implantbothgenerators in a single
surgery,but this is a very dernanding approachfor the patient.lrnmedi-
atelyaftertheoperation, thepatientmay terrporarilyresumeuredications.
The patientmay be discharged the next day.

Oncethe generatoris implanted,the patientmustwait a week or two


beforethe batteriesare activated.This waiting periodis necessary to
allow the swellingthatnormallyoccurswith thesurgeryto diminish.The
DBS electrodeconveyselectricalpulsesinto the brain usingpower
producedby the batteryin the generator.A seriesof visitsto the hospital
arerequiredto adjustthe voltagesettingsto the needsof the individual.It
mav take severalweeks or monthsto achievethe correct settines.The
patientcan checkthe statusof the generatorusing a handhelddevicethat
resemblesa TV remotecontrol. Using this device,the patientcan
determineif the generatoris on or off, and can turn it back on in the
eventthat it shutsdown unexpectedly.(Certainphenomenonsuchas
magneticfields causedby securitydevicesmay causethe batteryto
temporarilystopworking.)

The expectedlife spanof a batteryat a typical voltage is about3-5 years.


At a very high voltage,the batterymay needto be replacedafter a year,
at a very low voltage,perhapsup to sevenyears.Replacinga batterycan
be done undergeneralor local anesthesiaas an outpatientprocedure.

Dystoniadoesnot respondto DBS in the samemanneras other move-


ment disordersdo. For example,personstreatedfor tremor will generally
improvewithin secondsof turning the generatoron. In patientswith
dystonia,improvementmay be delayedfor days,and weeksor months
may passbeforethe full extentof the benefit is reached.DBS doesnot
necessarilyeliminatethepossibilityof subsequent drug or botulinumtoxin
treatments.

Sideeffectsare minimal, but no procedureis without risks.The main risk


in DBS is a fatal hemorrhage.However 99-99.5%of patientsdo not have
sigrificant bleeding.Despitevigorouseffortsto avoid it, infectionis a risk
in approximately 2%oof patients.lnfection can be seriousand warrant the
removalof the hardware.If this happens,it may be possibleto re-implant
the hardwareonce the infection is treated.Hardware failure is also a
concern,thoughthis is rare and precautionsare in place in the eventof
situationssuchas a batteryfailing. lt is estimatedthat in 5% of DBS
proceduresfor dystoniasomecomplicationmay arise,most of which can
be addressedwithout removingthe hardware.

Although no longerconsidered"investigational"for dystoniaby the Food


& Drug Administration,DBS is in its infancyas a treatmentfor this
disorder.The preliminaryresultsare quite positive,and the procedureis
expectedto evolve over time as more patientsare treatedand more data
is collected.
Comparing Lesioning & DBS

Studieshaveshownthat both lesioningand DBS can dramaticallyim-


provedystonia.Both approachesare associatedwith a small,but real,
-fhere
risk of complications. hasnot beena clinicalstudyto comparethe
resultsof lesioningproceduresand DBS. andthe advantages anddisad-
vantagesof eachremain an open issue.

Lesioningprocedures
andDBS havemanyelementsin commonincluding:

. Patientselectioncriteria
. Area of brain targeted
. Basicsurgicalprocedure
. Potentialfor profoundbenefitto eligiblepatients
. Risk ofcomplicationsincludinghemorrhageduringsurgeryhemiple-
gia or hemiparesis,sensoryimpairments,speech/language
impairment

In both cases,the chanceof benefitmust be weighedagainstthe risk of


complications.No two casesof dystoniaare alike, and determiningthe
specificapproachto treatment-in this caselesioningor DBS-must be
decidedafter carefuldiscussionsamongthe patient,family members,
neurologist and neurosurgeon.

Of the dystoniapatientswho areeligible for brain surgery more individu-


als are currentlybeingrecommendedfor DBS than pallidotomy.The
pallidotomy,howeveqis by no meansan obsoleteprocedure.Unlessa
patientis againsthavinghardwareinstalledin his/herbody,the tendency
is to try DBS beforeproceedingto the pallidotomybecauseDBS is
adjustableandreversible.

Financialand geographicalissuescannotbe overlooked.Personswho


haveDBS must visit the doctorregularlyfor maintenancecheck-ups.
Peoplewho live in remoteareasor areasnot in proximity to a major
movementdisordercentermay be at a disadvantage. Travelto and from
the center-and the expenseof this travel-is a part of the ongoing
managementrequiredof DBS patients.

2.
Llecause lesioningcreatesa permanentchangein the brain tissue,thereis
a slightl;-higherrisk of permanent complications duringthe surgerysuch
asswallowingdifficult1,, speechdifflrcultv.andcerebralhemorrhage.
BecauseDllS involvesthe implantationof hardware,complications
':ssociated * ith theapparatusarepossible. includinginfection. erosion
'l-he
the skin.hardwarebreakage.
ilrrr-rugh and stimulatorfailure. risk of
irardu'arecomplications existsfor as longas the hardwareis implanted.

It rernainslo be seenwhetherthe pallidotomyor DBS is moreeffective


thanthe other.l-he experienceof the surgeonand rnedicalteamare the
'l'he
inostimportantdetenninants of successand risk. lowestincidenceof
corrrplicationsoccursin majormedicalcentersthat performtheseproce-
Jrrresofierr.Patientsshouldchoosea centerwith a long-standing exper-
'iisein rnovenrentdisordersanda clinicalteamdevotedto surgeryfbr
dl'stoniaand movementdisorders. A movementdisorderneurologistanda
surgeonshouldbe speciallytrainedin functionalsurgeryandan electro-
physiologistshouldbe on stafffor brainmapping.An experienced nursing
staffis alsoimportant.

of brain surgerymay benefitfrom physical


Patientsof both categories
therapyand supportivetherapyfollowingthe procedure.

Lesioning

Controlleddestruction Non-destructive

Few post-oprestrictions Common-sense restrictions


regarding activity; must avoid

No cosmetic
issues Hardwaremay be slightly
visible beneathskin in
some people

B
Children & Brain Surgery

Childrenoverthe ageof sevenareeligiblefor lesioningandDBS, al-


thoughthe longeronewaits.the lessbrainand skin growthwill occur
afterdreoperation.l]orvever.thereis linle dataavailableaboutlong-term
efl'ectsof DIIS and how a child's developmentmay affectthe hardware.
Stepscan bc takenduringsurgeryto ensurcthat the apparatus can
accomnrodate the chrld'sgrowth.Childrenand adolescents may be at a
slightlyhigherrisk of complications from DBS because generalrather
thanlocalanesthesia 1susedduringimplantationandpost-operatively
childrenaremorelikely to engagein roughplay that may affectthe
hardware.

Surgerydoesnot necessarilyhaveto be consideredonly as a last resort.


Certainly,if an individualis satisfiedwith how symptomsrespondto a less
invasivetreatmentsuchasbotulinumtoxin or medications, thereis no
needto considerbrainsurgery.However,especiallyin children,early
intervention maysignificantlyimprovequalityof life.The benefitsof brain
surgeryincludemorethanimprovedmobility-a child's ability to function
comfortablyat school(bothacademicallyand socially),to makefriends,
and to be activeare importantfactorsto consider.In both childrenand
adults,brain surgerycan drasticallyimprovepain,which is often a major
componentto a person'squality of life.

z+
IV. Coxcr,usroN
Having surgeryis a very significantstepfor an individual to take in the
treatrnentofdystonia. Ifyou are consideringsurgeryor ifsurgery has
beenrecommendedto you by a movementdisorderspecialist,the follow-
ing questionsmay helpyou initiatediscussionswith your doctors:

. What is the nameof the operationand what doesthe namemean?


. Why is this specificsurgeryappropriatefor my case?
. What are the advantagesof having surgery?
. What benefitsmight I expect?
. What are the risks?
. What happensif I don't havethe surgery?Are their alternative
treatments?
. Where can I get a secondopinion?
. What is the experienceof the medicalcenterand surgeonwith this
procedure?
. Doesthe medicalteam publishthe resultsof surgicalcasestudies?
. Wherewill the surgerybe done?
. What kind of anestheticwill be used(generalor local)?
. How long is the recoveryand what rehabilitationis necessary?
. How much will the surgerycostand who will pay for it?

Surgicalprocedures may improvefunction and betterthe lives of patients


who do not receiveadequaterelief from medicationsand/orbotulinum
toxin injections.A patientwho is consideringsurgerymust weigh the
opportunityfor benefitandthe risk of complications.Carefuldiscussions
with movementdisorderspecialistsand being as knowledgeableas
possibleaboutdystoniaand surgerymay aide in the considerationpro-
cess.The DystoniaMedical ResearchFoundationcan provide namesand
contactinformationof dystoniaspecialists.

Until a cure for dystoniais achieved,researchers


are working diligently
toward developingtreatmentoptionsto improvethe lives of affected
individuals.Surgeryis an areaof researchin which vastprogressis being
madeand in which the prospectof developingmore effectivetreatments
is tremendously promising.
V.Appnuox

Dystonia

Dystoniais a neurologicalmovementdisorderthat causesmusclesin the


body to contractor spasminvoluntarily.The involuntarymusclecontrac-
tions causetwisting, repetitive,and patternedmovementsas well as
abnormalpostures.

Dystoniais not a singlediseasebut a syndrome-a set of symptomsthat


cannotbe attributedto a singlecausebut sharecommonelements.Some
forms of dystoniamay affect a specific body area,suchas the neck,
face,jaw, eyes,limbs, or vocal cords.When dystoniaaffectsa single
body area,it is called/ocal dystonia.Focal dystoniasincludecervical
dystonia,blepharospasm, oromandibulardystonia,writer's cramp,and
laryngealdystonia (spasmodic dysphonia).Segmentaldystoniaaffects
two or more adjacentbody areas.lf two or more non-adjacentbody
areasare affected, the dystonia is termed multifocal. Generalized
dystonia refers to dystonia that may affect the limbs, trunk, and other
major body areassimultaneously. When dystoniaonly affectsmuscleson
one side of the body, it is calledhemidystonia.Although the outward
appearancesof the various forms of dystonia may appearvery different,
they all sharethe elementof repetitive,patterned,and often twisting
involuntary musclemovements.

Dystoniaaffectsmen.women,and childrenof all agesand backgrounds.


Dystoniamay be geneticor causedby factorssuchas physicaltrauma,
exposureto certainmedications,or otherneurologicalconditions.

Dystoniais the third mostcommonmovementdisorderafter Parkinson's


diseaseand tremor,affectingan estimated250,000personsin North
America.Nonetheless,dystoniais often misunderstoodby the public and
misdiagnosedby medicaldoctors.Dystoniais neithera psychological
disorder,nor doesit affect intellect.Dystoniais not fatal, but it is a
chronicdisorderthat causesvarying degreesof disability andpain, from
mild to severe.

25
Tlrc Nervous System & Brain
Figure 4

The nervoussystemis
dividedinto two parts:the CentralNen'ous Systenl
centralnervoussystem Brain and Spinal Cord

(CNS) and the peripheral


PeripheralNenous
nervoussystem(PNS).
The CNS consistsof the
brain and the spinalcord. Cen'ical
The PNS consistsof the region

nervesextendingfrom the
Thoracic
spinalcord. region

Thesetwo systemsare Lumbar


responsible for all bodily reqion

rangingfrom
activities, Sacral
heartrateand muscle region

rnovement to emotionsand
leaming.

The brainis the most


complexandintricate
organin the humanbody.
The r.vrinkledgraymass
thatmakesup 80% of the
brainis calledthecerebrql
corler. This part is respon-
siblefor activitiessuclras
A na|om.t' of the rtet'vottr .i l s/er?
thinking,perceiving, and
producingand understandinglanguage.The cerebralcortex is divided into
trvo sidesor "hemispheres"-the right and the left. Although both henri-
spheresappearidentical,they differ in purposeand firnction. Language.
r e a s o n i n ga, n d l o g i c c a p a b i l i t i e os r i g i n a t ei n t h e l e f t s i d eo f t h e b r a i n
whereasappreciationof shapesand texturesand artistic talentsoriginate in
the right side.

The cerebral cortex is further divided into four sections or "lobes"-frontal,


parietal,temporal,and occipital.The frontal lobe is involved in movement
Figure 5

Basal ganglia
sncl related stnt(tttres of
ilrc brain--pt'ofi I e vi ew.

B a s a lG a n g l i a

G l o b u sP a l l i d u s
Thalamus

S u b s t a n t i aN i g r a

Cerebellum

and decision-makin-s skillsl the parietallobe interpretstouch, pain. and


temperatllre:the temporallobe is involved in hearingand memory; and the
occipital lobe containsthe vision center.

Beneaththe cerebralcortex lie structuresthat help us move, sleep,wake,


breathe,smell. hear,see,taste.and eat. Dystonia affectsan area in this deep
part of the brain Lrelieved to re_er"rlate lrovement calledthe basalean_slia. An
irnbalance o t ' b r a i nc h e m i s t r .cya u s e st h e b a s a lg a n g l i at o s e r r di r r a p p r o p r i a t e
messagesto the muscles.causingthem to contractand spasm iuvoluntarily.

Figure 6
Artatomy Orbicularis
oculi
>\
Eyes L o 1 1 1 1 u1 1 . 1 1 ,'

Theorbicularisoculimuscle Pr,,..-rur-;P
encirclesthe openingof theeye
socketand actsto closethe Y..i
,j*:{'
eyelids.The corrugatorr.nuscle
draw'sthe eyebrollstogetherand
wrinklesthe brorv.The procerus J
.t
muscleis a facialrnusclebetrveen
the eyebrowsand down the nose. &
Ey'entttsclesin pro/ile
28
Neck Figure 7
The stemocleidomastoid and
trapeziusmusclesare major
musclesin the neck.The two -ilt*r...-
/
stenrocleidomastoid muscles -: ,,/
are thick muscleson eachside S t e r n o cl e i d o mas t o i d
ofthe neckthat act to bend,
rotate,flex, and extendthe
head.The trapeziusmuscle
movesthe shoulderblades Trapezius

upward in a shrug.

Anatomy of the neck.

Larynx

The larynx or "voice box" is an organ in the neck that plays a crucial role
in speakingand breathing.The framework of the larynx is madeup of the
thyroid cartilage.The front portion of the thyroid cartilageis visible in
somepeopleas the "Adam's apple." The vocal cords are locatedin the
centerof the larynx. The thyroarytenoidmuscleis responsiblefor closing
the vocal cords,and the posteriorcricothyroidmuscleis responsiblefor
openingthe vocal cords.
Posterior
Figure8 cr i co a r y t e n o d
i

Lateral
Thyroarytenoid c r ic o a r y t e n o d
i
muscle muscle

Inside larynr, viev'-from above

D
Larynx (continued)

Figure 9

Outer cartilage of larynx,angled viewfrom front

Figure l0

Cartilage , Thvroid Stemocleidomastoid


of I .. cartilage Trapezius
larynx \-
Cricoidcartilage Supraclavicular
.. fossa
t"i-'o:'

;;&,
Surfaceofthe neck.
VI. Souncps:
''Surgery
Panel" presentations
at 7d Worldwide DystoniaPatientSymposiurn& 2"d
I-'amilySymposium. November8-10.2002.

"tJpdateon SpasmodicDysphonia"& "Surgical Options for Dystonia" Presentations


at
M id-Atlantic RegionalSymposium,May 12, 2003.

Activa@ TherapyFact Sheet,Medtronic (2003) <http'//medtronic.com./


downloadablefiles/dystoniaHDE-ActivaTherapyFactSheet.pdt>

Be lnformed: Questionsto Ask Your Doctor BeforeYou Have Surgery,AHCPR Publica-


tion #95-0027.January1995,Agency for HealthcarePolicy and Research,Rockwell, lVlD
< www.ahrq.gov/consumeri surgery.htm>

Hyman-NewmanInstitutefor Neurology & NeurosurgeryBeth IsraelMedical Center,


New York, NY <http://nyneurosurgery.org>

I fB@ TherapyFact Sheet,Medtronic (2001) <http'/lmedtronic.conlidownloadablefiles/


ITBTherapyFS.pdD

I- Albright, MJ Barry,DH Shafton,SF Ferson,Intrathecalbaclofenfor generalized


dystonia,DevelopmentalMed Child Neurol 2001; 43:652-657

L Albright, lntrathecalBaclofenfor SevereSpasticity,Part I & 2. reprintedffom Excep-


tional Parent.November 1996<www.medtronic.com>

GS Berke,KE Blackwell,BR Genatt,AVerneil, KS Jackson,JA Sercarz,Selective


larl'ngealdenervation-reinnervation:
A new surgicaltreatmentfor adductorspasmodic
dysphoni4 Ann Otol Minol Laryngol 1999;108:227-231

Berke GS and Blumin JH: SpasmodicDysphonia:TherapeuticOptions.Curr Opvt


Otolaryngol.2000;8(6):509-5
I3

P Coubes,A Roubertie,N Vayssiere,S Hemm, S Tuffery B Echenne.P Frerebeau.Earlv-


onsetgeneralizeddystonia:Neurosurgicaltreatmentby continuousbilateralstimulationof
the interbalglobus pallidus in l5 patients,Neurology,2000 (suppl.3),332.002(Abstract)

E-Move reportsflom 7s IntemationalCongressof Parkinson'sdiseaseand Movement


Disorders,November10-14,2002 (MovementDisorders2002: 17 (suppl. 5) P95I ; 5289-
290

B Ford, Surgeryfor Parkinson'sDisease:AGuide for Patients,Families.& Caregivers,


Parkinson'sDiseaseFoundation(2003)

3l
SM Kitgore,H Bronte-Stewart,Outcomeof palladialsurgeryin primary vs, secondary
dystoni4Neurology,2002(suppl7); 58, P05.l5l; ,4.395

A Lang, SurgicalTreatmentof Dystoni4 Dystonia3: Advancesin Neurology,Vol 78,


1998.

A Muenchau,J Plamer,D DressleqN Quinn,A Lees,KP Bhati4 Selectiveperipheral


denrvation with posterior primary ramisectomy is useful for botulinum toxin resistant
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MY Oh, AAbosch, SH kim, AE Lang, AM Lozano, Longterm hardware-relatedcompli-


cations of deep brain stimulation, Neurosurgery,2002;50 1268-1276(Abstract)

T Taira, T Kobayashi, K Takahashi,T Hori, A new denervationfor idiopathic cervical


dystoni4 J Neurosurg2002, Sep;97(2suppl):201-6(Abstract)

RH Walker, DM Sope, FO Danisi, IM German, RR Goodman, MF Brin, Intrathecal


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JL Vitek. M Evatt, IY Zhang, V Chockan, MR Delong, S Triche, RAE Bakay,


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Images

Figure l-l'arsy, D., Vitek, J., Lozano, A., Surgical Treatmentof Parkinsonb Diseaseand
Other MovementDisorders,Totow4 NJ: HumanaPress,2003.
Figure 2-Image provided courtesyof Medtronic.
Figure 3-Image provided courtesyof Medtronic.
Figure 4-Dystonia Medical ResearchFoundation,8-18 Guidebook,1994.
Figure S-Henkel,J, "Parkinson'sDisease:New TreatmentsSlow Onslaughtof Symp-
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Bartleby.com,2000. www.bartleby.com/ I 07l. | | I 1212003.
Figure 74ray,H., Anatomltof theHyman Bod,, Philadelphia'.Lea&Febiger,l9l8;
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Figure9-Gray.H.,AnatomltoftheHumanBody,Philadelphia: Lea&Febigeq l9l8;
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