Cerebellum

Anatomy
Midline vermis + 2 cerebellar
hemispheres
Anterior (I-V) and posterior (VI-IX) lobes
divided by primary fissure
Flocculonodular lobe (X), seperated by
posterolateral fissures

Cortex folded into folia

Anatomy

Deep Nuclei
Fastigial
stance and gait, controls muscles only in the
modes of sitting, standing, and walking

Globose + Emboliform
segmental reflexes, speeds the initiation of
movements triggered by somatosensory cues
that guide the response, stops unwanted and
promotes wanted oscillations, stabilizes holds

Dentate
Fine dexterity

Functional Anatomy
Archicerebellum
flocculonodular lobe
connections with the vestibular system,
inputs from areas of the brain concerned
with eye movements

Paleocerebellum
vermis of the anterior lobe, the pyramis, the
uvula, and the paraflocculus
Spinal cord inputs, controls muscle tone +
axial and limb movements

Functional Anatomy
Neocerebellum
middle portion of the vermis and most of
the cerebellar hemispheres
planning and initiation of movements,
regulation of fine limb movements

Functional Anatomy
Organized into Midline, Intermediate
and lateral zones
Midline project to fastigial nuclei
Intermmediate project to nucleus
interposed
Lateral project to dentate

Cerebeller Cortex
3 Layers:
Granular
Purkinje
Molecular

5 Cell types:
Basket, stellate, Purkinje, granule, and
Golgi

Physiology
Ascending Fibres:
Mossy
Diffuse projections through granule cells to
multiple Purkinje cells

Climbing
Terminal fibers of olivocerebellar tracts,
multiple synaptic contacts per Purkinje cell

Multilayered
Afferents from hypothalamus, raphe n, locus
ceruleus

Physiology
Descending Fibres:
Purkinje Fibres
Project from Purkinje cells in Purkinje
layer to deep cerebellar nuclei,
inhibitory

Deep nuclei then send excitory

signals to their efferent connections

Physiology
Stellate cells and basket cells cause
lateral inhibition of Purkinje cells
Modulation in spatial resolution

Golgi cells inhibit granular cells

Modulation in temporal resolution

Afferent and Efferents

Superior Cerebellar
Peduncle (brachium
conjunctivum)
Connects with midbrain

Middle Cerebellar
Peduncle (brachium
pontis)
Connects with pons

Inferior Cerebellar
Peduncle (restiform body)
Connects with medulla

Superior Cerebellar
Peduncle
Afferent:
Ventral spinocerebellar tract, transmits proprioceptive
and exteroceptive information from levels below the
midthoracic cord.
Tectocerebellar tract, arising in the superior and inferior
colliculi
ocarries auditory and visual information.
Trigeminocerebellar tract, proprioceptive fibers from the
mesencephalon and tactile information from the chief
sensory nucleus of the trigeminal nerve
The cerulocerebellar tract, fibers from the nucleus
ceruleus

Superior Cerebellar
Peduncle
Efferent: (most prominent)
Dentatorubral tract, output to the
contralateral red nucleus
Dentatothalamic tract, output to the
contralateral ventrolateral nucleus of the
thalamus
Uncinate bundle of Russell, output to the
vestibular nuclei and reticular formation

Middle Cerebellar Peduncle

Afferent fibers: (only)
Pontocerebellar (corticopontocerebellar) tract,
arises in contralateral pontine gray matter
oTransmits impulses from the cerebral cortex to
the intermediate and lateral zones of the
cerebellum

Inferior Cerebellar Peduncle

Afferent:
Dorsal spinocerebellar tract, originating in the
dorsal nucleus of Clarke (T1L2), carries
proprioceptive and exteroceptive information mostly
from the trunk and ipsilateral lower extremity
Cuneocerebellar tract, originating in the external
arcuate nucleus, which transmits proprioceptive
information from the upper extremity and neck.
Olivocerebellar tract, which carries somatosensory
information from the contralateral inferior olivary
nuclei.

Inferior Cerebellar Peduncle

Vestibulocerebellar tract, transmits information
from vestibular receptors on both sides of the
body.
Reticulocerebellar tract, arises in the lateral
reticular and paramedian nuclei of the medulla.
Arcuatocerebellar tract, arises from the arcuate
nuclei of the medulla oblongata.
Trigeminocerebellar tract, arises from the spinal
and main sensory nuclei of the trigeminal nerve
Efferent:
fastigiobulbar tract -(through Juxtarestifrom body),
cerebellovistibular
cerebelloreticular pathways

Vascular Supply
PICA
From intracranial vertebral artery,
supplies the lateral medullary
tegmentum, inferior cerebellar peduncle,
the ipsilateral portion of the inferior
vermis, and the inferior surface of the
cerebellar hemisphere
Medial branch supplies dorsolateral
medulla and medial cerebellum, lateral
branch supplies inferiopostalateral

Vascular Supply
AICA
above the origin of the basilar artery, supplies the
anterior petrosal surface of the cerebellar
hemisphere, flocculus, lower portion of the middle
cerebellar peduncle, and lateral pontomedullary
tegmentum

SCA
distal segment of the basilar artery just below the
terminal bifurcation into the paired PCAs, and
supplies the upper surface of the cerebellar
hemisphere, ipsilateral portion of the superior
vermis, most of the dentate nucleus, upper portion
of the middle cerebellar peduncle, superior
cerebellar peduncle, and lateral pontine tegmentum

Stroke Syndromes
PICA (40%)
Proximal (usually in vertebral arteries)
Wallenberg syndrome
Distal:
Medial branch occlusion will cause acute
vertigo and truncal ataxia
Lateral branch occlusion :unsteadiness, limb
ataxia, and dysmetria without dysarthria

Stroke Syndromes
AICA (5%)
Prominent vertigo, nausea, vomiting, and
nystagmus (vestibular nuclei)
Ipsilateral facial hypalgesia and
thermoanesthesia, and corneal hypesthesia
(trigeminal spinal nucleus and tract)
Ipsilateral Horner syndrome
Contralateral trunk and extremity hypalgesia and
thermoanesthesia (lateral spinothalamic tract).
Ipsilateral ataxia and asynergia (middle cerebellar
peduncle and cerebellum).
Ipsilateral deafness and facial paralysis (lateral
pontomedullary tegmentum).

Stroke Syndromes
SCA (35%)
Vertigo and vomiting (vestibular nuclei and
connections)
Nystagmus (MLF and cerebellar pathways)
Ipsilateral Horner syndrome
Ipsilateral ataxia and asynergia (superior cerebellar
peduncle and cerebellum)
Ipsilateral intention tremor (dentate nucleus and
superior cerebellar peduncle)
Contralateral trunk and extremity hypalgesia and
thermoanesthesia (lateral spinothalamic)
Contralateral hearing impairment (crossed fibers of
the lateral lemniscus)
Contralateral fourth nerve palsy (pontine tectum)

Stroke Syndromes
Watershed (20%)
Etiologies: focal hypoperfusion
secondary to occlusive disease in
vertibobasilar vessels, emboli,
intercranial atheroma, global
hypoperfusion
Physical findings variable

Stroke Syndromes
PICA vs SCA
SCA has less frequent vertigo and H/A,
both with gait disturbance
SCA typically more benign clinically

Always need to be aware of possible

herniation (tonsillar or transtentorial)
with cerebellar stroke

Clinical Features
Ataxia
Dysmetria, dysdiadokinesis, rebound,
truncal, gait, localization dependent

Hypotonia, pendular reflexes

neocerebellum

Intention Tremor
Dentate nucleus, rubro-olivo-cerebellar
circuit

Clinical Features
Dysarthria
Scanning speech, agrammatism, involovemnet of
tongue representation in cerebellum (L
paravermal)

Nystagmus
PAN, Rebound, Gaze evoked, downbeat (post.
midline)

Abnormal Saccades
Square-wave jerks, macro square-wave jerks
Occular dysmetria

Difficult to localize eye movement

abnormalities between cerebellum and
brainstem

Acute Ataxia
Idiopathic (acute cerebellar
ataxia)
Metabolic
Hypoglycemia
Hyponatremia
Hyperammonemia
Biotinidase deficiency
Wernicke's encephalopathy
Infections
Bacterial meningitis
Viral meningitis
Brainstem encephalitis
Toxins
Trauma (battered child)
Hydrocephalus
Cerebellar lesions
Neoplasm (primary or
metastatic)
Infarct
Hemorrhage
Neuroblastoma
Polyradiculoneuropathy
GBS
Fisher variant of GBS
Tick paralysis
Labyrinthitis
Brainstem tumors
Multiple sclerosis

Episodic/Recurr
ent Ataxia
Channelopathies
Episodic ataxia Type 1
Episodic ataxia Type 2
Paroxysmal
choreoathetosis with
episodic ataxia
Periodic
vestibulocerebellar
ataxia
Familial hemiplegic
migraine
Basilar artery migraine
Benign paroxysmal
vertigo of childhood
Epilepsy (post-ictal
state)
Toxins
Metabolic
Hypoglycemia
Hyperammonemia
Organic acid
disorders
Hartnup disease
Hyperpyruvic
acidemias
Pyruvate
decarboxylate
deficiency
Refsum disease
Porphyria
Leigh syndrome
Maple syrup urine
disease

Causes of Chronic Ataxia

Fixed deficit
Cerebral palsy
Malformations (i.e.,
Dandy-Walker, Chiari,
rhomboencephalosynapsi
s, cerebellar agenesis,
hypoplasia of the
cerebellar vermis, familial
agenesis of the cerebellar
vermis, pontocerebellar
hypoplasia, Joubert's
syndrome, cerebellar
cortical malformations,
macrocerebellum, etc.) glutamyl-cysteine
synthetase deficiency
Triosephosphate
isomerase deficiency
Chediak-Higashi disease
Autosomal dominant
SCA1
SCA2
SCA3 (MJD)
SCA4
SCA5
SCA6
DRPLA
SCA7 (Ataxia/retinal
degeneration)
SCA8
SCA 9 (not assigned)
SCA 10
SCA 11
SCA 12
SCA 13
SCA 14

SCA 15 (genetic locus not

yet identified)
SCA 16
SCA 17
SCA 1821 (not yet
published, but registered)
SCA 22
SCA 25
Autosomal recessive
Friedreich's ataxia
Early onset cerebellar
ataxia with retained
muscle stretch reflexes
Ataxia with vitamin E
deficiency
Ataxia with oculomotor
apraxia
With hypogonadism
With myoclonus
(Ramsay Hunt syndrome)
Infantile onset
spinocerebellar ataxia
With pigmentary
retinopathy
With optic atrophy
mental retardation
(including Behr syndrome)
With cataract and
mental retardation
(Marinesco-Sjgren
syndrome)
With childhood deafness
With extrapyramidal
features
Autosomal recessive
late onset cerebellar
ataxia

Autosomal recessive
spastic ataxia of
Charleroix-Saguenay
Ataxia telangiectasia
Wilson's disease
Refsum's disease
Bassen-Kornzweig
disease
(abetalipoproteinemia)
Spinocerebellar ataxia
with axonal neuropathy
Inborn errors of
metabolism
Cerebrotendinous
xanthomatosis
Other inherited disorders
Sphingolipidoses
Ceroid-lipofuscinosis
X-Linked spinocerebellar
ataxia
Acquired diseases
Hypothyroidism
Drugs or toxins
Multiple sclerosis
Neoplasms
Hamartomatous
malformations (i.e.,
dysplastic gangliocystoma
or Lhermitte-Duclos
disease)
Erdheim-Chester
disease
Paraneoplastic
cerebellar degeneration
(anti-Yo, anti-Hu, anti-Ri,
anti-Tr, anti-CV2,
antibody-negative)

Video Cases
SCA6 is a relatively pure cerebellar ataxia that usually begins in midlife. Many
cases
The are
presentation
MJD/SCA3
varies greatly
butfamily
always
includes
sporadic, ie,ofoccur
in the absence
of a known
history.
In
ataxia.
affected
mother
and daughter
both suffer
from
mild,
addition
to This
describing
so well
her disease
onset, progression,
and
current
progressive
gait ataxia
accompanied
mildinscanning
symptoms,
this patient
relates
how it becameby
clear,
retrospect,dysarthria,
that her
mild incoordination,
and,
in the mothers
case,
mothers
gait problems and
cerebellar
disease were
notophthalmoparesis
due to chemotherapy,
on lateral
gaze.
represent
an unusual
pair
of cases
asnoted
had been
assumed.
The They
examination
illustrates
common
features
of SCA6,
because
their disease
repeat
lengths
were classified
by the
gene than
including
scanning
dysarthria,
end-gaze
nystagmus,
and gait ataxia
greater
testing service
appendicular
ataxia.as intermediate size alleles of indeterminate
significance. Their repeats (54 and 52, respectively) were slightly
smaller than the accepted repeat range of >56; in contrast, normal
repeats are 42 or smaller for this gene. The ataxic presentation in
this mother-daughter pair illustrates that intermediate repeat
lengths can in fact cause disease. When interpreting the
significance of repeat lengths in dynamic repeat diseases, the
clinician should use clinical judgment to help determine whether
those uncommon repeat lengths falling within a gray area are
actually the cause of disease.

Acute Ataxia
Idiopathic (acute cerebellar
ataxia)
Metabolic
Hypoglycemia
Hyponatremia
Hyperammonemia
Biotinidase deficiency
Wernicke's encephalopathy
Infections
Bacterial meningitis
Viral meningitis
Brainstem encephalitis
Toxins
Trauma (battered child)
Hydrocephalus
Cerebellar lesions
Neoplasm (primary or
metastatic)
Infarct
Hemorrhage
Neuroblastoma
Polyradiculoneuropathy
GBS
Fisher variant of GBS
Tick paralysis
Labyrinthitis
Brainstem tumors
Multiple sclerosis

Episodic/Recurr
ent Ataxia
Channelopathies
Episodic ataxia Type 1
Episodic ataxia Type 2
Paroxysmal
choreoathetosis with
episodic ataxia
Periodic
vestibulocerebellar
ataxia
Familial hemiplegic
migraine
Basilar artery migraine
Benign paroxysmal
vertigo of childhood
Epilepsy (post-ictal
state)
Toxins
Metabolic
Hypoglycemia
Hyperammonemia
Organic acid
disorders
Hartnup disease
Hyperpyruvic
acidemias
Pyruvate
decarboxylate
deficiency
Refsum disease
Porphyria
Leigh syndrome
Maple syrup urine
disease

Causes of Chronic Ataxia

Fixed deficit
Cerebral palsy
Malformations (i.e.,
Dandy-Walker, Chiari,
rhomboencephalosynapsi
s, cerebellar agenesis,
hypoplasia of the
cerebellar vermis, familial
agenesis of the cerebellar
vermis, pontocerebellar
hypoplasia, Joubert's
syndrome, cerebellar
cortical malformations,
macrocerebellum, etc.) glutamyl-cysteine
synthetase deficiency
Triosephosphate
isomerase deficiency
Chediak-Higashi disease
Autosomal dominant
SCA1
SCA2
SCA3 (MJD)
SCA4
SCA5
SCA6
DRPLA
SCA7 (Ataxia/retinal
degeneration)
SCA8
SCA 9 (not assigned)
SCA 10
SCA 11
SCA 12
SCA 13
SCA 14

SCA 15 (genetic locus not

yet identified)
SCA 16
SCA 17
SCA 1821 (not yet
published, but registered)
SCA 22
SCA 25
Autosomal recessive
Friedreich's ataxia
Early onset cerebellar
ataxia with retained
muscle stretch reflexes
Ataxia with vitamin E
deficiency
Ataxia with oculomotor
apraxia
With hypogonadism
With myoclonus
(Ramsay Hunt syndrome)
Infantile onset
spinocerebellar ataxia
With pigmentary
retinopathy
With optic atrophy
mental retardation
(including Behr syndrome)
With cataract and
mental retardation
(Marinesco-Sjgren
syndrome)
With childhood deafness
With extrapyramidal
features
Autosomal recessive
late onset cerebellar
ataxia

Summary Slide

Autosomal recessive
spastic ataxia of
Charleroix-Saguenay
Ataxia
telangiectasiaWilson's
disease
Refsum's disease
Bassen-Kornzweig
disease
(abetalipoproteinemia)
Spinocerebellar ataxia
with axonal neuropathy
Inborn errors of
metabolism
Cerebrotendinous
xanthomatosis
Other inherited disorders
Sphingolipidoses
Ceroid-lipofuscinosis
X-Linked spinocerebellar
ataxia
Acquired diseases
Hypothyroidism
Drugs or toxins
Multiple sclerosis
Neoplasms
Hamartomatous
malformations (i.e.,
dysplastic gangliocystoma
or Lhermitte-Duclos
disease)
Erdheim-Chester
disease
Paraneoplastic
cerebellar degeneration
(anti-Yo, anti-Hu, anti-Ri,
anti-Tr, anti-CV2,

References
Blumenfeld
Brazis

Thanks