Related Subjects:Multiple System Atrophy (MSA)
|Parkinson Plus syndromes
|Parkinsonism
|Idiopathic Parkinson disease
|Progressive Supranuclear Palsy
|Drug Induced Parkinson disease
About
- Survival about 6 to 9 years on average. Seen in those aged 50+
- Faster progression than Idiopathic Parkinson's Disease
Incidence
- 6/100 000 per year, reaching 3/100 000 per year in those over 50
Aetiology
- Alpha-synuclein positive cytoplasmic inclusions in glial cells
- This is a misfolded, hyperphosphorylated, fibrillar alpha-synuclein
- Neuronal loss: basal ganglia, cerebellum, pons, inferior olivary nuclei, and spinal cord
- Autonomic failure in combination with parkinsonism or cerebellar ataxia
Clinical
- Pyramidal signs - extensor plantar, hyper-reflexia
- Others: Dysarthria, frequent falls, urinary symptoms, stridor, Pathological laughter or crying, Jerky, myoclonic postural or action tremor
- MSA-C : Olivopontocerebellar atrophy - cerebellar ataxia
- MSA-P: Striatonigral degeneration - parkinsonism predominates but tremor less common all poorly responsive to levodopa. Rigidity, Postural instability, Postural tremor and resting tremor, Freezing of gait
- Shy-Drager: autonomic involvement with falls, orthostatic hypotension
Differentials
- Idiopathic Parkinson's Disease
- Vascular Dementia
Investigations
- MRI abnormalities in the striatum, pons, and cerebellum are highly suggestive. Increased diffusivity in the putamen on diffusion-weighted MRI
- MRI MSA-P: atrophy of stratum and putamen more than caudate. Outer margin of putamen atrophy
- MRI MSA C: pontine atrophy includes middle cerebellar peduncles. T2W hyperintensity with hot cross bun sign
Management
- The parkinsonism is more likely to be bilateral than IPD. Response to L-Dopa is variable and fluctuates but may be worth trying. Cognitive function better preserved than Idiopathic PD
- Orthostatic hypotension - see topic
- Bladder urgency: oxybutinin
- Nocturia: DDAVP intranasal 20-40 mcg nocte watch for hyponatraemia
- Trails ongoing into riluzole, recombinant human growth hormone, and minocycline
References
- The Lancet Neurology, Volume 8, Issue 12, Pages 1172 - 1178, December 2009