Cerebellar Disorders

Objectives

• Neurological examination
To understand how to localize lesions within the
cerebellum

• Disorders of Cerebellum
 Presentation
• Ataxia - syndrome of imbalance and
incoordination involving gait and limbs

• Cerebellar vs Sensory ataxia vs motor vs

spinal cord disease vs basal ganglia vs
vestibular disease vs Cortical ataxia
• Anatomy of cerebellum
 Cerebellar Syndromes
• In general, precise clinical localization is difficult in the
cerebellum
• Some syndromes can be classified anatomically:

– Rostral vermis syndrome (anterior lobe)

– Caudal vermis syndrome (flocculonodular, posterior

lobe)

– Hemispheric syndrome (posterior lobe, variably

anterior too)

– Pancerebellar syndrome
 Cerebellar Syndromes- rostral vermis

– Wide-based stance and gait

– Ataxia of gait, proportionally little ataxia on heel-shin
with pt lying
down
– Normal or slightly impaired arm cooordination
– Infrequent hypotonia, nystagmus, dysarthria

• alcoholics (restricted form of cerebellar cortical

degeneration)
 Cerebellar Syndromes- caudal vermis
– Axial dysequilibrium, staggering gait
– Little or no limb ataxia
– Sometimes spontaneous nystagmus
– Rotated postures of head

• Seen in diseases that damage the flocculonodular

lobe (esp medulloblastoma in children) — as tumor
grows, a hemispheric cerebellar syndrome may be
superimposed

• Need to also consider other signs of  ICP

(obstruction of CSF)
 Cerebellar Syndromes - hemispheric
– Incoordination of ipsilateral limb movements

– More noticeable with fine motor skills

– Incoordination affects most noticeably muscles

involved in
speech and finger movements

• Etiologies include infarcts, neoplasms, abscesses

 Cerebellar Syndromes- pancerebellar
• Combination of all the other syndromes
• Bilateral signs of cerebellar dysfunction involving trunk,
limbs, cranial
musculature

• Etiologies usually infectious/parainfectious processes,

hypoglycemia,
paraneoplastic disorders, toxic-metabolic
disorders,hereditary
 Localisation in cerebellum
• Lateralized cerebellar lesions - ipsilateral
symptoms and signs
• Generalized cerebellar lesions - symmetrical
symptomatology.
• Vestibulocerebellar lesions cause disequilibrium
and an ataxic gait.
• Vermis, ―spinocerebellar‖ organ, truncal and gait
ataxia with relative sparing of the limbs.
 Time period

• Acute - severe abnormalities early, but

recovery with time.
• Chronic - progressive diseases gradually
declining balance with longer lasting effects.
 Symptoms
Gait Disturbances
• Insecurity while walking, especially -
turning or balancing on a narrow ledge.
• Specialized skills such as skiing,
bicycling, or climbing.
• Report the sense of imbalance as
dizziness.
• Increase imbalance when visual cues
removed = sensory
 Symptoms

• LimbAtaxia - clumsiness and tremor,slow

movements to be more accurate. symptoms one-
sided with lateralized lesions.
• Truncal Ataxia - head tremor and truncal
instability
 Symptoms
• Dysarthria and Bulbar Symptoms - slurred
speech and abnormalities of pitch and volume
control (scanning speech).

• Dysphagia - incoordination of swallowing

muscles. Ineffectiveness of cough may also be
a symptom
 Symptoms
• Visual Symptoms - blurriness or a sense of
environmental movements as a result of
cerebellar ocular oscillations
 Symptoms in Sensory Ataxia

• Patients with a sensory basis for ataxia

usually do not experience dysarthria orvisual
symptoms.
• Symptoms of sensory pathway diseasesuch
as parasthesias and numbness.
 Signs in Cerebellar Ataxia
• Deficits involving gait and stance, limb
incoordination, muscle tone, speech, and the
oculomotor system.
• Subtle cognitive deficits.
 Stance and Gait
• Increase in body sway,feet are placed
together. Stance more than 12cm.
• Early - tandem or stand on one foot;30Sec
• Romberg test - prominent in proprioceptive
or vestibular lesions.
 Stance and Gait
• Straight path-widened base and an irregular
staggering appearance -alcoholic.
• Speed not impaired.
• Steps are irregular and the patient may lurch in
unpredictable ways.
• Rhythmic oscillations trunk and head - titubation.
• Truncal ataxia - inability to sit upright without back
support.
Romberg’s asking the patient to stand unaided - eyes closed.
If the patient sways or loses balance this test is positive.
 Dysrhythmia
• Inability to tap and keep a rhythm. It can be
tested by tapping the table with a hand (or
the floor with a foot) and asking the patient to
repeat the maneuver.
 a b c
Cerebellar
Ataxia

Ataxic gait and

position:
d Left cerebellar tumor

a. Sways to the right in

standing position
b. Steady on the
right leg
c. Unsteady on the
left leg
d. ataxic gait
 Cerebellar examination
• Rebound
• examined by allowing the patient to flexthe
elbow against the examiner's hand and then
abruptly removing the resistance and
assessing the patient's ability to arrest the
sudden flexion movement.
 Finger-to-nose test
• Touch the tip of his nose withhis index finger
then the tip ofyour finger.
• As fast as possible while you slowly move your
finger.
• Toe to finger
• Dysmetria,intention tremor
 Heel-to-shin test
• Patient lying down get him to run the heel of
one foot down the shin of the other leg and
then to bring the heel back up to the knee and
start again. Repeat the test withthe other leg
 Dysmetria
• Inaccuracy of movement so that the desired target
is either under-reached (hypometria) or over-
reached (hypermetria).
• Dysmetria is evident in the finger-chase and toe-to-
finger tests.
• Disturbance of the rate, range, andforce of
movement.
• Increased by adding a mass to the hand
 Kinetic (Intention) Tremor

• Oscillations during a voluntary movement intended

to reach a target increasing in amplitude as the
target is reached.
• Result from instability at the proximal, rather than
the distal, portions of the limb typically
perpendicular to axis ofmotion.
• Finger-to-nose and the heel-to-shinmaneuvers
detect the kinetic tremor.
• Kinetic tremor is better evaluated when mass is
added to thehand
 Other Tremors

• Cerebellar lesions can give rise to a postural tremor initiated

by keeping the arms outstretched or pointing the fingers
steadily at each other. In the legs, maintaining one heel on the
opposite knee can bring out suchtremor.

• Axial tremor involving thehead and shoulders. Also, a severe

tremor in the upper limbs that has both an intention and a
postural component can appear in cerebellar outflow tract
disease. “rubral” or “wing-beating”tremor.

• ETIOLOGY- multiple sclerosis, Wilson's disease, and midbrain

strokes.
 Dysdiadochokinesia

• Irregularity of the rhythm and amplitudeof

rapid alternating movements.
• Simple tapping tasks such as the index finger
on the thumb crease or the feet on the floor
can also detect the disturbance in rhythm
(dysrhythmokinesis).
 Dysdiadochokinesia
• Rapid alternating movements - supinateand
pronate the forearm in the unsupported
position.
OR
• clap one hand on the palm of the other
(stationary) hand alternately with the palm
and dorsum of the clappinghand
 dysdiadochokinesis
 Show the patient to clap by alternating the
palmar and dorsal surfaces of the on hand.
Ask to do this as fast as possible and repeat
the test withthe other hand.
 Hypotonia
• Decrease in the normal resistance offeredby muscles
to passivemanipulation.
• When an affected limb is shaken,flapping
movements of the hands appear of widerexcursion
than normal.
• Also, a forced flexion of the arm at the elbow may
obliterate the space between the volar aspect of the
wrist and the deltoid.
• Not consistent sign
 Oculomotor Disturbances
• Fixation abnormalities are examined by askingthe
patient to maintain sustained gaze at the
examiner's finger held about 2 feet infront.
• Follow finger as it is moved slowly in all directions
of gaze (pursuit).
• Eccentric gaze is maintained (at about30 degrees
deviation) to check fornystagmus.
• Saccades are examined by having the patient shift
gaze quickly between an eccentrically held finger
and the examiner's nosein the middle.
 Oculomotor dysfunction
• Nystagmus frequently seen in cerebellar disorders
• Gaze-evoked nystagmus, upbeat nystagmus, rebound nystagmus,
opticokinetic nystagmus may all be seen in midline cerebellar
lesions

• Other ocular lesions seen include opsoclonus, skew deviation,

ocular bobbing
• Most of the disorders giving rise to these affect brainstem structures,
too: cerebellar role in their onset not well-defined

• Overall, most ―cerebellar‖ eye signs cannot be localized to specific

areas of the cerebellum
 Nystagmus
• Gaze-evoked - eccentric gaze is maintained at30
degrees . Eyes repetitively drift toward midline
followed by saccades to eccentric position. Fast
phase of the nystagmus is always to the side of
gaze.
• Gaze-evoked nystagmus fatigues and reverses
direction after a few seconds, reboundnystagmus
• Downbeat nystagmus characterized by rapid phase
in down direction- downgaze or gaze to the side-
craniovertebral junction
• Upbeat primary position nystagmus - anterior
vermis.
 Speech and Bulbar Function
• Listening to spoken words - speak standard phrases.
• Slowness, slurring of the words, and a general
inability to control the process of articulation,
leading to unnecessary hesitations and stops,
omissions of pauses when needed, and an
accentuation of syllables when not needed.
• Variability in volume and pitch of words and
inappropriate control of the breathing needed for
speech- scanning dysarthria.
• Milddysphagia is not uncommon
 Sensory Ataxia

• Defective proprioception.
• Impaired position and vibration sense - deep
tendon reflexes are often lost - afferent fiber
pathology.
• Romberg test ispositive
• Degenerative ataxic syndromescombine features of
cerebellar and proprioceptive deficits in variable
proportion.
 Ataxic sensory gait
a)brisk leg movements;
b) legs placed far apart to correctinstability;
c) steps of variable length;
d)need for carefully watching the ground.
e)Incoordination enhanced when deprived of
visual information.
 Causes of Ataxia
Congenital: “Ataxic” cerebral palsy, other early insults

Vascular: Ischemic stroke, hemorrhagic stroke, AV

malformations

Infectious/transmittable: Acute cerebellitis, postinfectious

encephalomyelitis, cerebellar abscess, Whipple's disease, HIV,
CJD

Toxic: Alcohol, anticonvulsants, mercury, 5-FU, cytosine

arabinoside, lithium

Neoplastic/compressive: Gliomas, ependymomas,

meningiomas, basal meningeal carcinomatosis, craniovertebral
junction abnormalities

Immune: Multiple sclerosis, paraneoplastic syndromes, anti-

GAD, gluten ataxia

Deficiency: Hypothyroidism, vitamin B1 and B12, vitamin E

 GENETIC CAUSESOF ATAXIA

Autosomal recessive: FA, AT,AVED, AOA 1, AOA 2,

other inborn errors ofmetabolism

Autosomal dominant: SCA types 1 through 28,

episodic ataxias (types 1, 2,others)

Mitochondrial: NARP, MELAS, MERRF, others

including POLG mutations, Kearns-Sayresyndrome
 Causes of ataxia
• Investigation in patients with ataxia
IMAGING
 Summary of signs
• V-vertigo
• A – Ataxia
• R–R ebound
• N - Nystagmus
• I -Intention tremor (tremor that increases on activity)
• S–Speech (staccato or scanning speech)
• H – Hypotonia
• D -Dysmetria (overshooting or undershooting)
• D -Dysdiadochokinesis (difficulty with rapidalternating
movements)
• T–tremor,titubation
THEEND