Cerebellar Syndrome

Routine if asked to examine upper limbs:

 

Inspect (walking aids etc)

Pronator drift

Rebound test

Tone

Power

Reflexes

Coordination (dysdiadochokinesis, finger-nose testing). When notice problem with coordination proceed to:

Eye movements (nystagmus, INO), pupils (RAPD), Visual fields, observe for Horner’s syndrome

CNs V + VII (and look at gums for hypertrophy and face for telangiectasia) + VIII

Speech (eg. “How did you get here today?”)

Sensation

Hands and arms (clubbing, tar staining, dupuytrens contracture, diabetic fingerprick marks telangiectasias, signs of chronic liver disease)

Foot tapping test if sitting and observe for pes cavus

 

If time allows proceed to:

Gait, Heel to toe walking, Rhombergs test

Lower limbs (heel shin testing, tone, power, reflexes, look for pes cavus)

 

 

 

Routine if told patient is “unsteady, proceed as appropriate”:

 

Inspect (walking aids etc)

Start with gait, heel to toe walking and Rhomberg’s test

Pronator drift and rebound test

Dysdiadochokinesis, finger-nose testing

Eye movements (nystagmus, INO), pupils (RAPD), visual fields, observe for Horner’s syndrome

CNs V+VII (and look at gums for hypertrophy and face for telangiectasia) +VIII

Speech (eg. “How did you get here today?”)

Foot tapping whilst sitting and observe for pes cavus

Upper limbs Tone, Power, Reflexes, Sensation

Hands and arms (clubbing, tar staining, diabetic fingerprick marks, dupuytrens contracture, telangiectasias, signs of chronic liver disease)

If time allows: examine lower limbs (heel shin testing, tone, power, reflexes, look for pes cavus)

 

 

 

Present to the examiner:

 

This patient has a bilateral cerebellar syndrome as evidenced by:

 

Ataxic wide based gait

Inability to walk heel to toe

Rhombergs test was negative but there was increased stability with the feet together

Dysdiadochokinesis, past pointing, intention tremor, rebound phenomenon

Heel shin ataxia

Nystagmus

Ataxic dysarthria

 

Comment on tone (reduced), power, reflexes (pendular) and sensation. NB: increased tone and reflexes and pyramidal weakness suggests corticospinal tract involvement

 

In terms of aetiology:

 

There was (no) internuclear opthalmoplegia (INO)/ RAPD/ UMN weakness/sensory disturbance to suggest demyelinating disease

There was (no) cachexia/clubbing/tar staining to suggest paraneoplastic cause

There was (no) weakness or visual field defect to suggest a stroke/space occupying lesion as the cause

There was (no) CN V, VII, VIII to suggest cerebellar pontine angle lesion

There was (no) parkinsonism to suggest multi systems atrophy

There was (no) gum hypertrophy to suggest phenytoin use

There was (no) dupuytrens contracture/stigmata of chronic liver disease to suggest alcohol as the cause

There was (no) opthalmoplegia to suggest Miller Fischer as the cause

There was (no) pes cavus to suggest Friedreich’s Ataxia as the cause

 

 

Alternatively……

 

This patient has a right/left sided unilateral cerebellar lesion as evidenced by:

 

Ataxic wide based gait with veering towards to right on heel toe walking

Rhombergs test was negative but there was increased instability with the feet together

Dysdiadochokinesis, past pointing, intention tremor, rebound phenomenon

Right heel shin ataxia

Gaze-evoked nystagmus on rightward gaze

Ataxic dysarthria

 

Comment on tone (reduced), power, reflexes (pendular) and sensation. NB: increased tone and reflexes and pyramidal weakness suggests corticospinal tract involvement

 

In terms of aetiology:

 

There was (no) internuclear opthalmoplegia (INO)/ RAPD/ UMN weakness/sensory disturbance to suggest demyelinating disease

There was (no) weakness or visual field defect to suggest posterior circulation stroke/space occupying lesion as the cause

There was (no) CN V, VII, VIII to suggest cerebellar pontine angle lesion

There was (no) Horner’s syndrome to suggest lateral medullary syndrome

 

To complete my examination I would like to perform fundoscopy (Optic atrophy in demyelinating disease, papilloedema in space-occupying lesion), a full lower and upper limb motor and sensory examination, full cranial nerve examination

 

 

Most likely causes of bilateral cerebellar syndrome are:

 

Demyelination (Multiple Sclerosis)

Paraneoplastic conditions

Bilateral posterior circulation strokes/space-occupying lesions

 

Other causes of bilateral cerebellar syndrome include:

 

Multiple System Atrophy

Drugs: phenytoin, carbamazepine, lithium

Toxins: Alcohol

Metabolic: hypothyroid, wilson’s disease, coeliac, B12 deficiency

Infective: HIV, enteroviruses, syphilis, CJD, toxoplasmosis, lyme disease

Inflammatory: Miller Fischer variant of GBS

Hereditary: Friedreich’s Ataxia, ataxic telangiectasia, spinocerebellar ataxias, von hippel landau

Bilateral cerebellar pontine angle lesion eg. neurofibromatosis

 

 

Most likely causes of unilateral cerebellar syndrome are:

 

Demyelination (Multiple Sclerosis)

Posterior circulation infarction/haemorrhagic stroke

Space occupying lesion in the posterior fossa eg. tumour, abscess

 

Other causes of unilateral cerebellar syndrome:

 

Cerebellar pontine angle lesion eg. neurofibromatosis

Lateral medullary syndrome

Ataxic hemiparesis following lacunar stroke

Multiple System Atrophy

 

 

Causes of spastic paraparesis and cerebellar signs:

 

  1. Demyelination (Multiple sclerosis)
  2. Friedreich’s ataxia
  3. Spinocerebellar ataxia
  4. Arnold Chiari Malformation
  5. Syringomyelia

 

 

 

Investigations according to most likely cause:

 

MRI brain and spinal cord

Bloods: TFT, copper studies, paraneoplastic screen, coeliac, screen for infection and inflammation, FBC, LFT, B12, drug levels (phenytoin, carbamazepine, lithium)

Lumbar puncture to examine CSF (eg. for oligoclonal bands)

EMG and nerve conduction studies

Genetic testing

 

Manage according to cause

 

 

 

Multiple Sclerosis

 

This is a progressive demyelinating disease of the central nervous system (brain and spinal cord)

CNS inflammation is disseminated in time and place

Involvement of spinal cord, cerebellum, brainstem, optic nerve

*does not affect peripheral nerves*

Visual evoked potentials are reduced (slow conduction in optic nerve)

CSF: increased protein, increased lymphocytes, unmatched oligoclonal bands

MRI: active inflammation, hyperintense on T2 (periventricular white matter lesions, corpus callosum, brainstem, cerebellar, spinal cord, optic nerve)

Management: MDT approach, high dose iv steroid acutely, symptom management eg. antispasmodics, disease modifying therapies

 

 

Written by Dr Sarah Kennedy

 

Resources used to write this document are listed in the references section of this webpage.