Cerebellar syndrome

Question 1

Cerebellar gait examination

Answer 1

Walk
Heel-to-toe
On tip toe
On heal
Romberg's

Question 2

Cerebellar examination with arms outstretched

Answer 2

Ataxia

Rebound

Question 3

Cerebellar signs

Answer 3

DANISH
Dysdiadochokinesia (hands and feet)
Ataxia
Nystagmus + rapid saccades that overshoot
Intention tremor + dysmetria
Slurred speech
Hypotonia

Question 4

Completion of cerebellar exam

Answer 4

Cranial nerves: brainstem stroke, MS, CPA lesion
Peripheral nervous system: MS
Signs of CLD
Drug chart: phenytoin (toxicity causes cerebellar damage)

Question 5

Causes of cerebellar syndrome

Answer 5

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

Question 6

Lateralisation of cerebellar signs

Answer 6

Ipsilateral to lesion

Bilateral cerebellar signs more likely to represent a global pathology e.g. alcohol, MS, phenytoin

Question 7

Presentation of cerebellar vermis lesion

Answer 7

Ataxic trunk and gait

Normal arms

Question 8

Differentiating cerebellar and vestibular nystagmus

Answer 8

Vestibular cause: fast phase away from lesion, maximal looking away from lesion
Cerebellar cause: opposite

Question 9

Specific history for cerebellar syndrome

Answer 9

MS: Paraesthesia, visual problems, muscle weakness
Alcohol consumption
Infarct: onset, stroke risk factors
Schwannoma: hearing loss, vertigo, tinnitus, raised ICP
FHx
DHx

Question 10

Investigations for cerebellar syndrome

Answer 10

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)

Question 11

General management of cerebellar syndrome

Answer 11

MDT
CV risk
Lower EtOH intake

Question 12

Concise treatment of MS

Answer 12

methylprednisolone

Question 13

Concise treatment of EtOH dependence

Answer 13

Pabrinex, tapering course of chlordiazepoxide

Question 14

Concise treatment of infarct (as cause of cerebellar syndrome)

Answer 14

Consider thrombolysis

Question 15

Concise treatment of Wilson’s

Answer 15

Penicillamine

Question 16

Concise treatment of Schwannoma

Answer 16

Gamma-knife, surgery

Question 17

Cause of Lateral Medullary Syndrome AKA Wallenberg’s

Answer 17

Occlusion of vertebral artery or PICA

Question 18

Features of Lateral Medullary Syndrome AKA Wallenberg’s

Answer 18

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

Question 19

Pathophysiology of vestibular Schwannoma

Answer 19

Benign, slow-growing tumour of superior vestibular nerve
SOL leads to cerebellopontine angle (80% of CPA tumours are Schwannomas)
Associated with NF2

Question 20

Presentation of vestibular Schwannoma

Answer 20

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

Question 21

Other name for vestibular Schwannoma

Answer 21

Acoustic neuroma

Question 22

Investigations for vestibular Schwannoma

Answer 22

MRI of CPA

Question 23

Treatment of vestibular Schwannoma

Answer 23

Gamma-knife

Surgery

Question 24

CPA tumour differential

Answer 24

Vestibular Schwannoma (AKA acoustic neuroma)
Meningioma
Cerebellar astrocytoma
Metastases

Question 25

Features of Von-Hippel Lindau syndrome

Answer 25

``` Renal cysts Bilateral RCC Haemangioblastoma (often in cerebellum: cerebellar signs) Phaeochromocytoma Islet cell tumours ```

Question 26

Pathophysiology of Friedrich's Ataxia

Answer 26

Autosomal recessive mitochondrial disorder Progressive degeneration of: dorsal column, spinocerebellar tracts and cerebellar cells Corticospinal tracts

Question 27

Associations of Friedrich's ataxia

Answer 27

HOCM and mild dementia

Question 28

Age of onset of Friedrich's ataxia

Answer 28

teenage years

Question 29

Features of Friedrich's ataxia

Answer 29

``` 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% ```

Question 30

Pathophysiology of Ataxia Telangiectasia

Answer 30

Autosomal recessive Defect in DNA repair Onset in childhood / early adulthood

Question 31

Features of Ataxia telangiectasia

Answer 31

Progressive ataxia Telangiectasia: conjunctivae, eyes, nose, skin creases Defective cell-mediated immunity and antibody production: infections Lymphoproliferative disease

Question 32

Cause of Wilson's disease

Answer 32

Autosomal recessive mutation of ATP7B gene

Question 33

Features of Wilson's disease

Answer 33

``` CLANK Cornea: Kayser-Fleischer rings Liver: CLD Arthritis Neuro: parkinsonism, ataxia, psychiatric problems Kidney: Fanconi's syndrome ```