Cerebellar syndrome Flashcards
Cerebellar gait examination
Walk Heel-to-toe On tip toe On heal Romberg's
Cerebellar examination with arms outstretched
Ataxia
Rebound
Cerebellar signs
DANISH Dysdiadochokinesia (hands and feet) Ataxia Nystagmus + rapid saccades that overshoot Intention tremor + dysmetria Slurred speech Hypotonia
Completion of cerebellar exam
Cranial nerves: brainstem stroke, MS, CPA lesion
Peripheral nervous system: MS
Signs of CLD
Drug chart: phenytoin (toxicity causes cerebellar damage)
Causes of cerebellar syndrome
DAISIES Demyelination Alcohol Infarct (brainstem) SOL e.g. schwannoma + other CPA tumours Inherited e.g. Wilson's, Friedrich's, Ataxia Telangiectasia, VHL Epilepsy medications: Phenytoin System atrophy, multiple
Lateralisation of cerebellar signs
Ipsilateral to lesion
Bilateral cerebellar signs more likely to represent a global pathology e.g. alcohol, MS, phenytoin
Presentation of cerebellar vermis lesion
Ataxic trunk and gait
Normal arms
Differentiating cerebellar and vestibular nystagmus
Vestibular cause: fast phase away from lesion, maximal looking away from lesion
Cerebellar cause: opposite
Specific history for cerebellar syndrome
MS: Paraesthesia, visual problems, muscle weakness
Alcohol consumption
Infarct: onset, stroke risk factors
Schwannoma: hearing loss, vertigo, tinnitus, raised ICP
FHx
DHx
Investigations for cerebellar syndrome
ECG: arrhythmia (DDx brainstem stroke)
Bloods: EtOH (FBC, U+E, LFTs), thrombophilia (clotting), Wilson’s (low caeruloplasmin)
CSF: oligoclonal bands
Imaging: MRI is best to visualise the posterior cranial fossa
Audiometry: CPA lesion (pure tone audiometry)
General management of cerebellar syndrome
MDT
CV risk
Lower EtOH intake
Concise treatment of MS
methylprednisolone
Concise treatment of EtOH dependence
Pabrinex, tapering course of chlordiazepoxide
Concise treatment of infarct (as cause of cerebellar syndrome)
Consider thrombolysis
Concise treatment of Wilson’s
Penicillamine
Concise treatment of Schwannoma
Gamma-knife, surgery
Cause of Lateral Medullary Syndrome AKA Wallenberg’s
Occlusion of vertebral artery or PICA
Features of Lateral Medullary Syndrome AKA Wallenberg’s
All ipsilateral except pain anaesthesia
DANVAH
Nucleus ambiguus(motor to CN9/10): Dysphagia
Inferior cerebellar peduncle: Ataxia, Nystagmus
Vestibular (CN8) nucleus: Vertigo
[Anaesthesia:]
Spinothalamic tract: reduced pain sensation contralaterally
Spinal trigeminal nucelus: reduced pain sensation ipsilaterally
Sympathetic fibres: Horner’s
Pathophysiology of vestibular Schwannoma
Benign, slow-growing tumour of superior vestibular nerve
SOL leads to cerebellopontine angle (80% of CPA tumours are Schwannomas)
Associated with NF2
Presentation of vestibular Schwannoma
Unilateral SNHL, tinnitus, vertigo Raises ICP: headache Ipsilateral CN5,6,7,8 palsies and cerebellar signs: Facial anaesthesia + absent corneal reflex Lateral rectus (abducens) palsy LMN facial nerve palsy SNHL DANISH
Other name for vestibular Schwannoma
Acoustic neuroma
Investigations for vestibular Schwannoma
MRI of CPA
Treatment of vestibular Schwannoma
Gamma-knife
Surgery
CPA tumour differential
Vestibular Schwannoma (AKA acoustic neuroma)
Meningioma
Cerebellar astrocytoma
Metastases
Features of Von-Hippel Lindau syndrome
Renal cysts Bilateral RCC Haemangioblastoma (often in cerebellum: cerebellar signs) Phaeochromocytoma Islet cell tumours
Pathophysiology of Friedrich’s Ataxia
Autosomal recessive mitochondrial disorder
Progressive degeneration of: dorsal column, spinocerebellar tracts and cerebellar cells
Corticospinal tracts
Associations of Friedrich’s ataxia
HOCM and mild dementia
Age of onset of Friedrich’s ataxia
teenage years
Features of Friedrich’s ataxia
Pes cavus Bilateral cerebellar ataxia Leg wasting + areflexia but extensor plantars Loss of vibration and proprioception High-arched palate Optic atrophy and retinitis pigments HOCM DM in 10%
Pathophysiology of Ataxia Telangiectasia
Autosomal recessive
Defect in DNA repair
Onset in childhood / early adulthood
Features of Ataxia telangiectasia
Progressive ataxia
Telangiectasia: conjunctivae, eyes, nose, skin creases
Defective cell-mediated immunity and antibody production: infections
Lymphoproliferative disease
Cause of Wilson’s disease
Autosomal recessive mutation of ATP7B gene
Features of Wilson’s disease
CLANK Cornea: Kayser-Fleischer rings Liver: CLD Arthritis Neuro: parkinsonism, ataxia, psychiatric problems Kidney: Fanconi's syndrome