Cerebellar Signs

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Cerebellar
Examination
Stem statement
 Giddiness, falls, unsteadiness
 Face, Speech, ULs and LLs
Unilateral
 Upper limbs
 Screen for pronator drift, ensure patient can see your finger!
 Cerebellar signs
 Dysmetria with intention tremor
 Dysdiadochokinesia
 Dyschronometria
 Power for ataxic hemiparesis
 Sensory
 Temperature/Pain loss in syringomyelia and LMS
 Tone for cogwheel and leadpipe rigidity
 Skin for neurofibromatosis
 Pulse for AF
 Face
 Gaze evoked nystagmus (in the direction of gaze), INO, RAPD
 Speech (Count 1 to 20; British Constitution; West Register Street)
 Cerebellar speech – jerky, explosive and loud; irregular syllables
 CNs
 CPA
 LMS
 III nerve palsy in Benedikt’s syndrome
 Xanthelesma
 Lower limbs
 Dysmetria and intention tremor for toe to finger test
 Dyssynergia for heel-shin test
 Dysdiadochokinesia for foot tapping test
 DM dermopathy
 Sit up with hands folded and tests for pendular jerks
 Gait
 Broad based gait with veering towards the side of the lesion
 Request to test visual fields for hemianopia
Bilateral
 Upper Limbs
 Cs of cerebellar (dysmetria, dysdiadochokinesia and dyschronometria)
 Sensory – loss of temperature/pain for syringomyelia
 Parkinsonism
 NF features
 Alcoholic features – dupytren’s contracture, stigmata of chronic liver disease
 Face
 CNs
 Bilateral CPA tumor
 Multiple sclerosis
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


Eyes
 Gaze evoked nystagmus
 KF rings
 INO, RAPD
 Mouth
 Gingivial hypertrophy
 Macroglossia
 Telengiectasia
 Parotidomegaly
 Goitre
 Speech
 Cerebellar speech
 Hoarseness of voice
Lower limbs
 Cerebellar signs
 Clawing of toes (Friederich’s ataxia)
Sit – truncal ataxia and pendular jerks
Gait – cerebellar gait
Presentation
Unilateral
Sir, this patient has a right sided unilateral cerebellar lesion as evidenced by presence
of a right dysmetria, dysdiadochokinesia and dyschronometria of the right upper limb.
The right lower limb also demonstrates presence of right dyssynergia on heel shin
test, with right dysmetria and intention tremor on toe-finger test and
dysdiadochokinesia. This is associated with a gazed evoked nystagmus on rightward
gaze with a broad based gait with veering towards the right. I did not detect any
cerebellar speech or any truncal ataxia.
There are no associated cranial neuropathies. In particular there was no evidence of
any cerebello-pontine angle lesion with CN V, VI, VII and VIII intact. (There are also
no signs of neurofibromatosis such as neurofibromas or café-au-lait spots.) There is
also no evidence of lateral medullary syndrome or III nerve palsy to suggest
Benedikt’s syndrome. There is also no pronator drift on the right to suggest a right
ataxic hemiparesis.
Patient is in sinus rhythm and not in atrial fibrillation with no xanthelesma or diabetic
dermopathy. There are also no bruises to suggest overanticoagulation.
There are no signs of Parkinsonism to suggest presence of multiple system atrophy.
There are also no associated features of multiple sclerosis such as RAPD or INO.
I would like to complete the examination by looking at
1. The patient’s temperature chart for fever (abscess in posterior fossa)
2. Visual fields for a left sided hemianopia, which can occur with a right posterior
circulation stroke
3. I would also like to do a fundoscopy for papilloedema for a SOL in the right
cerebello-pontine lesion as well as for optic atrophy from demyelinating disease.
In summary, this patient has got an isolated right cerebellar lesion. The differential
diagnoses include cerebral vascular infarction or haemorrhage or a space-occupying
lesions such as a mitotic lesion or an abscess.
Bilateral
Sir, this patient has bilateral cerebellar lesions as evidence of dysmetria with intention
tremor bilaterally associated with dysdiadochokinesia. Similar findings were also
present on examination of the lower limbs. There is also presence of multi-directional
gaze evoked nystagmus associated with a cerebellar speech, truncal ataxia and a broad
based gait.
There is no evidence of bilateral CPA lesion with no CN V, VI, VII and VIII
abnormalities. Patient is in sinus rhythm and not in AF with no xanthelesma or
diabetic dermopathy.
There is no evidence of KF rings to suggest presence of Wilson’s disease. There is
also no RAPD or INO to suggest multiple sclerosis. There is also no gingival
hypertrophy to suggest chronic phenytoin use. Patient has no goitre or features of
hypothyroidism such as a cream and peaches complexion, no hoarseness of voice or
macroglossia. There are also no features of chronic ethanol ingestion such as
Parotidomegaly, dupytren’s contracture or stigmata of chronic liver disease. There is
no associated Parkinsonism signs to suggest multiple system atrophy such as presence
of cog-wheeling or leadpipe rigidity. There are also no neurofibromas present to
suggest presence of NF type 2. Patient is also not cachexic looking and there is no
clubbing to suggest underlying malignancy.
I did not detect any telengiectasia to suggest presence of Ataxia telengiectasia and
there is pes cavus to suggest Friederich’s ataxia. (Think of Wilson’s, MS, Phenytoin,
Hypothyroid, Alcohol, Parkinsonism, NF, paraneoplastic, telangiectasia and FA)
I would like to complete the examination by
1. Looking at the temperature chart for fever
2. Performing a neurological examination of the lower limb to look for spastic
paraparesis
3. I would also like to do a fundoscopy for Optic atrophy, which may suggest
demyelinating disease.
In summary, this patient has bilateral cerebellar syndrome. Possible causes include
drugs such as phenytoin, metabolic conditions such as hypothyroidism, chronic
ethanol ingestion, paraneoplastic conditions and infection such as enteroviruses and
bilateral cerebellar strokes.
Questions
What are the differential diagnoses for a unilateral cerebellar syndrome?
 Isolated
 Cerebrovascular accident – infarction or haemorrhage
 SOL in posterior fossa – abscess or mitotic (primary vs secondary)
 Associated
 CN
 CPA and/or neurofibromatosis
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 Lateral medullary syndrome
 Jugular foramen (Arnold-Chiari or Dandy-Walker)
 Benedikt’s syndrome
Ataxic hemiparesis (lacunar stroke)
Parkinsonism in Multiple system atrophy
Demyelinating such as Multiple sclerosis
What are causes of bilateral cerebellar syndrome?
 Acquired
 Infection
 Viral – HIV, Enteroviruses
 Spirocheatal – Lymes and Tabes dorsalis
 Others – Toxoplasmosis and CJD
 Metabolic
 Wilson’s disease
 Hypothyroidism
 Drugs
 Phenytoin and Carbamazepine
 Lithium
 Alcohol
 Causes bilateral cerebellar signs
 Causes isolated lower limb cerebellar signs
 Affects the anterior vermis
 Due to thiamine deficiency
 Multiple system atrophy
 Neurofibromatosis type 2 with bilateral CPA tumor
 Bilateral Strokes
 Paraneoplastic – Ca lung or ovary
 Hereditary
 Ataxia telangiectasia
 Autosomal recessive
 Childhood with death by 20s or 30s
 Ataxia, choreathetosis and telengiectasia on the face, eras and conjunctiva
and skin crease
 Low IgA with recurrent chest infections and lymphoreticular malignancy
 Friederich’s ataxia
 Scoliosis, pes cavus
 Spastic paraparesis, dorsal column loss, absent ankle jerks
What are the signs of a midline lesion (cerebellar vermis) and what are the causes?
 Signs : truncal ataxia, abnormal heel-toe walk test, cerebellar speech
 Causes : Midline tumor, paraneoplastic
What are the causes of cerebellar signs with spastic paraparesis?
 Friederich’s ataxia
 Spinocerebellar ataxia
 Arnold-Chiari Malformation
 Lesion at the craniospinal junction eg meningioma
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Syringomyelia
Multiple sclerosis
Syphilitic meningomyelitis
How are cerebellar signs located?
 Limb ataxia = cerebellar lobes
 Gait ataxia = anterior vermis
 Truncal ataxia = posterior vermis
What are the differences between cerebellar and sensory ataxia?
Cerebellar
Sensory
Site
Cerebellar
Posterior column, nerves
Tone
Reduced
Normal
Reflexes
Normal or pendular
Reduced
Sensory
Normal
Reduced
Sphincter disturbance
Nil
Affected when posterior
column involved; overflow
incontinence
How would you investigate?
 Imaging – MRI brain
 Blood tests according to the causes
How would you manage?
 Depends on underlying cause
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