Cerebellar Syndrome

Cerebellar Syndrome 2018-01-30T16:37:17+08:00

Relevant physical signs

  • Inspection
    • Scars to suggest previous decompression / tumour resection
  • Cranial nerves
    • Kayser-Fleisher rings (Wilson’s disease)
    • Multi-directional nystagmus
    • Internuclear ophthalmoplegia (demyelination)
    • Restriction of extra-ocular movements (Miller-Fisher syndrome)
    • Relative afferent pupillary defect (demyelination)
    • Ipsilateral loss of facial sensation (lateral medullary syndrome)
    • Ipsilateral Horner’s syndrome (lateral medullary syndrome)
    • Ipsilateral lower VII and VIII palsy (cerebellopontine angle)
    • Staccato, scanning speech
      • Say “baby hippopotamus” and “West Register Street”
  • Upper limbs
    • Holmes rebound phenomenon
    • Truncal ataxia (usually points to cerebellar vermis)
    • Intention tremor
    • Dysmetria
    • Dysdiadochokinesis
    • Parkinsonism (multiple system atrophy)
      • Lead-pipe rigidity
      • Cogwheeling
      • Resting tremor
    • Arreflexia (Miller-Fisher syndrome)
    • Power (if the patient has an ataxic hemiparesis)
    • Contralateral (to the cerebellar signs) spinothalamic sensory loss (lateral medullary syndrome)
  • Lower limbs
    • Toe clawing, pes cavus (Friedrich’s ataxia)
    • Dysmetria (toe to finger test)
    • Dyssynergia (heel-shin test)
    • Dysmetria (foot tapping test)
    • Pendular jerks
    • Ataxic gait, tends to veer towards the side of the lesion
  • Cardiovascular screening (stroke)
    • Pulse (atrial fibrillation)
    • Carotid bruits (carotid artery stenosis)
    • Diabetic dermopathy
  • Chronic alcoholism (bilateral cerebellar syndrome)
    • Stigmata of chronic liver disease
    • Dupuytren’s contracture
    • Parotid enlargement

Differential diagnosis

  • Unilateral
    • Ischaemic
      • Stroke within the cerebellar hemisphere
      • Stroke causing a lateral medullary syndrome
        • Ipsilateral Horner’s, facial sensory loss with contralateral spinothalamic loss
      • Lacunar infarct (ataxic hemiparesis)
    • Neoplastic
      • Cerebellopontine angle tumour
      • Primary brain tumour within the cerebellum
      • Cerebellar metastases
      • Neurofibromatosis type 2
  • Bilateral
    • Hereditary
      • Friedrich’s ataxia
        • Autosomal recessive
        • Trinucleotide (GAA) expansion repeat in the frataxin gene
        • Clinical features
          • Progressive cerebellar ataxia
          • Hyporeflexia / arreflexia
          • Distal weakness, initially involving the lower limbs
          • Dorsal column loss, initially distal
          • Dysphagia
          • Reduced visual acuity
          • Sensorineural hearing loss
          • Kyphoscoliosis
          • Pes cavus
          • Urinary incontinence
          • Cardiomyopathy
          • Diabetes mellitus
      • Spinocerebellar ataxia
        • Usually autosomal dominant
        • Some involve trinucleotide expansion CAG repeats
        • Clinical features
          • Usually presents in third or fourth decade
          • Progressive cerebellar ataxia
          • Dysarthria, bulbar palsy
          • May have slow saccades and saccadic pursuit
          • May have lid retraction
          • Increased tone
          • Hyperreflexia
          • Extensor plantar responses
          • May have peripheral neuropathy causing fasciculations and wasting
      • Wilson’s disease
        • Autosomal recessive
        • Clinical features
          • Tremor
          • Ataxia
          • Psychiatric disorders
          • Chronic liver disease
      • Ataxia telangiectasia
        • Autosomal recessive
        • Telangiectasia on face, ears, conjunctiva and skin creases
        • Associated IgA deficiency with recurrent chest infections
        • Associated lymphoreticular malignancy
      • Arnold-Chiari malformation
    • Inflammatory
      • Demyelination (multiple sclerosis)
      • Miller-Fisher syndrome
      • Degenerative
        • Multiple system atrophy
    • Drug-induced
      • Alcohol-induced cerebellar degeneration
      • Wernicke’s encephalopathy (ophthalmoplegia, ataxia and confusion)
      • Phenytoin
      • Lithium
      • Carbamezapine
      • Phenobarbitone
    • Neoplastic
      • Bilateral cerebellopontine angle tumours
      • von Hippel Lindau syndrome
      • Neurofibromatosis type 2
      • Paraneoplastic phenomenon (usually lung, breast or ovarian carcinoma)
    • Vascular
      • Bilateral cerebellar strokes

Investigations

  • Magnetic resonance imaging (posterior fossa is bony, hence computed tomography may be sub-optimal) of the brain to look for strokes, space-occupying lesions and cerebellar degeneration
  • In patients with a family history:
    • Genetic testing for trinucleotide expansion repeat in frataxin gene
    • Genetic testing for disabling mutations in the ATM gene (ataxia telangiectasia)
    • Genetic testing for SCA genes
    • Serum caeruplasmin (low) and 24h urinary copper excretion (high) for Wilson’s disease
  • Consider lumbar puncture (Miller-Fisher)
    • Normal cell differential count with elevated CSF protein (albuminocytologic dissociation)
  • Serology
    • Anti-GQ1b (ganglioside) antibodies in Miller-Fisher syndrome
    • Anti-Yo (Purkinje cell) antibodies in paraneoplastic syndrome (usually gynaecological and breast)
    • Anti-Hu (neuronal nuclear) antibodies in paraneoplastic syndrome (other malignancies)
  • Drug levels if drug-induced cerebellar syndrome suspected
  • If stroke, look for underlying cause
    • Trans-cranial Doppler
    • Doppler of the carotid arteries
    • Trans-thoracic echocardiogram to look for intracardiac thrombus
    • Holter monitoring to detect paroxysmal atrial fibrillation
    • Screening for cardiovascular risk factors (HbA1c, LPM)
  • Age-appropriate malignancy screening to look for underlying malignancy

Management

  • Multidisciplinary team approach
  • Physiotherapy, occupational therapy to maximize and preserve function
  • Consider genetic counselling for hereditary ataxias
  • Alcohol cessation and thiamine supplementation
  • Withdrawal of the offending drug
  • Consider referral to neurosurgery for excision of space-occupying lesion
  • Antiplatelet, statin and gastric protection for stroke
  • Consider Madopar for multiple system atrophy, but may not work
  • Treat underlying malignancy
  • Intravenous immunoglobulin or plasma exchange for Miller-Fisher syndrome

Cerebellar Syndrome

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