What the main clinical features of PSP?
- mean age of onset
- clinical features
- pathological findings
- supporting investigations
- Mx
- 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
MSA -
- what are the main subtypes and how do they present?
- pathological hallmarks
- supporting Ix
- treatment
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
Corticobasal degeneration:
- what are the clinical features?
- pathology
- treatment
- 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
What are the acute causes of cerebellar ataxia?
Acute = mins -hours
1) vascular; haemorrhage or stroke
2) toxins: alcohol, drugs (cytarabine, phenytoin)
3) infections = meningoencephalitis, post-infectious cerebellitis
What are the subacute causes of cerebellar ataxia?
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
What are the chronic causes of cerebellar ataxia?
Genetic -AD: spinocerebrllar ataxia AR: freidrichc’ ataxia, telangiectasia Neurodegenerative: - MSA-C, PSP, small vessel ataxia
