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Multiple system atrophy 

Multiple system atrophy
Chapter:
Multiple system atrophy
Author(s):

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

DOI:
10.1093/med/9780192619112.003.0303
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date: 06 June 2020

The term multiple system atrophy was originally proposed by Graham and Oppenheimer (1969) in a paper describing a patient with autonomic failure, cerebeller ataxia, and probable pyramidal signs, but no parkinsonism in life. Pathologically, there was neuronal loss and gliosis not only in the intermediolateral cell columns, olives, pons, and cerebellum, but also in the substantia nigra and the putamen. Multiple system atrophy (MSA) was then developed (Bannister and Oppenheimer 1972) as a common label to include many, but not all, cases of idiopatic orthostatic hypotension (Bradbury and Eggleston 1925), progressive autonomic failure or Shy–Drager syndrome (Shy and Drager 1960), olivopontocerebellar atrophy (OPCA) (Déjerine and Thomas 1900), and striatonigral degeneration (SND) (Adams et al. 1964). At autopsy, whatever the mode of clinical presentation, these cases showed involvement of most or all of the above structures to varying degrees (Oppenheimer 1983; Gray et al. 1988 ).

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