Multiple system atrophy
Updates to Article Attributes
Multiple system atrophy (MSA) is a neurodegenerative disease (one of the synucleinopathies)
Epidemiology
Multiple systemic atrophy is a sporadic disease, with a prevalence of 4 per 100,000 2. Typically symptoms begin between 40 and 60 years of age 2.
Clinical presentation
Clinical presentation is typically in one of three patterns (initially described as separate entities) 1-2:
- Shy-Drager syndrome is used when autonomic symptoms predominate
- striatonigral degeneration shows predominant parkinsonian features
- olivopontocerebellar atrophy demonstrates primarily cerebellar dysfunction
Alternatively MSA can divided clinically into 2 forms 3-4:
- MSA-C: predominance of cerebellar symptoms (olivopontocerebellar atrophy)
- MSA-P: predominance of parkinsonian signs and symptoms (striatonigral degeneration)
Pathology
Like other synucleinopathies, multiple systemic atrophy results from abnormalities of alpha-synuclein metabolism, resulting in intracellular deposition. Unlike Parkinson disease and Lewy body dementia (two other synucleinopathies) these intracellular deposits are found not only in neurons but also in oligodendroglia 2.
Radiographic features
MRI is the modality of choice for imaging patients with suspected multiple system atrophy (MSA).
MRI
- T2 hyperintensities: typically present in the pontocerebellar tracts
- pons: hot cross bun sign
- middle cerebellar peduncles
- cerebellum
- putaminal findings in MSA-P 5:
- reduced volume
- reduced GRE and T2 signal relative to globus pallidus
- reduced GRE and T2 signal relative to red nucleus
- abnormal disruption of the normal high T2 linear rim
-
MSA-C
- Disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle)
- ADC values: higher in the pons, cerebellum, and putamen than in Parkinson disease or controls
- fractional aniosotropy (FA): lower in the pons, cerebellum, and putamen than in Parkinson disease or controls
Treatment and prognosis
Unfortunately no effective treatment is currently available. The disease progresses relentlessly culminating in death usually within 10 years of diagnosis 2.
- +<li>MSA-C<ul><li>Disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle)</li></ul>
- +</li>
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