multiple system atrophy (MSA)
Multiple system atrophy (MSA) is a fatal, adult-onset, neurodegenerative disease with rapid progression and limited symptomatic treatment options
- clinical features include parkinsonism and cerebellar and autonomic signs
and symptoms in various combinations
- two major motor variants are distinguished according to the predominant
- MSA parkinsonian variant (MSA-P) and
- MSA cerebellar variant (MSA-C)
- two major motor variants are distinguished according to the predominant motor presentation:
- in Europe and North America, the distribution is in favor of the MSA-P variant,
whereas MSA-C is more prevalent in Japan.
- neuropathological correlates of MSA-P and MSA-C are striatonigral degeneration and olivopontocerebellar atrophy, respectively
Shy-Drager syndrome is considered another form of MSA.
Pathologically multiple system atrophy is characterised by widespread neuronal loss and gliosis.
Clinically there are three distinct pictures according to which parts of the brain are most severely affected:
- cerebellar ataxia in OPCA (MSA-C)
- Parkinsonism in strionigral degeneration (MSA-P)
- autonomic failure in Shy-Drager syndrome
- Wenning GK, Ben Shlomo Y, Magalhaes M, Daniel SE, Quinn NP. Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain. 1994;117:835-845
- Kollensperger M, Geser F, Ndayisaba JP, et al. Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. Mov Disord. 2010;25:2604-2612
- Tolosa, E. & Berciano, J. (1993). Choreas, hereditary and other ataxias, tics, myoclonus and other movement disorders. Curr Op Neurol Neurosurg. 6, 358-368.
Last reviewed 01/2018