Neurology

Question 1

What the main clinical features of PSP?

• mean age of onset
• clinical features
• pathological findings
• supporting investigations
• Mx

Answer 1

• Age: 63
• falls early on, symmetrical Parkinsonism, cognitive decline, dysarthria, dysphagia (pseudo bulbar palsy), supra nuclear gaze palsy, astonished facial expression
• pathological findings: neuronal loss, gliosis, aggregates of Tau and Neurofibrillary tangles
• Ix; MRI hummingbird sign - atrophy of the midbrain
• amantadine may help, little response to Levodopa

Question 2

MSA -

• what are the main subtypes and how do they present?
• pathological hallmarks
• supporting Ix
• treatment

Answer 2

MSA - Parkinsonism predominant
- asymmetrical Parkinsonism, poorly responsive to L-dopa, prominent autonomic dysfunction: postural hypotension, loss of sweating, urinary incontinence/sweating, early erectile dysfunction

MSA - cerebellar predominant
- cerebellar ataxia predominates. Can also get orofacial dystonia, myoclonus, abnormal respiratory patterns. Emotional lability

• pathology: glial cytoplasmic inclusions that contain alpha synuclein in the basal ganglia/cerebellum/pons/motor cortex
• MRI may show hot cross bun sign: degeneration of the middle cerebellar peduncles and pons

Treatment: symptomatic, trial of amantadine and L-dopa

Question 3

Corticobasal degeneration:

• what are the clinical features?
• pathology
• treatment

Answer 3

• unilateral involvement with rigidity and dystonia in an arm. No tremor
• cognitive impairment, visuospatial neglect, limb apraxia and myoclonus of the affected arm
• ‘alien limb’ phenomenon
• eventually both sides affected

Pathology: tau inclusions found post mortem

Tx: supportive, l-dopa little or no effect

Question 4

What are the acute causes of cerebellar ataxia?

Answer 4

Acute = mins -hours

1) vascular; haemorrhage or stroke
2) toxins: alcohol, drugs (cytarabine, phenytoin)
3) infections = meningoencephalitis, post-infectious cerebellitis

Question 5

What are the subacute causes of cerebellar ataxia?

Answer 5

Subacute: hours-days

1) atypical infections e.g. pml, cjd
2) metabolic: alcohol, b1, b12 deficiency, vit E, hypothyroidism
3) autoimmune:
- paraneoplastic or non-paraneoplastic (Miller fisher, MS, anti-GAD ataxia)
4) structural: tumour/met, hydrocephalus

Question 6

What are the chronic causes of cerebellar ataxia?

Answer 6

Genetic 
-AD: spinocerebrllar ataxia
AR: freidrichc’ ataxia, telangiectasia
Neurodegenerative:
- MSA-C, PSP, small vessel ataxia