Review
Multiple system atrophy
H U RehmanHull Royal
Infirmary, Anlaby Road, Hull HU3 2JZ, UK
Correspondence to: Dr Rehman
Submitted 12
July 2000;
Accepted 28 November 2000
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Introduction |
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Multiple system atrophy (MSA) is a degenerative disease of the
central nervous system. Dejerine and Thomas in 1900 were the first to use the term olivopontocerebellar atrophy (OPCA) in
two sporadic cases,1 although Menzel described the first
case in 1891. Shy and Drager in 1960 described four cases of a
"neurological syndrome with orthostatic hypotension".2
They defined the full syndrome comprising of orthostatic hypotension,
urinary and rectal incontinence, loss of sweating, iris atrophy,
external ocular palsies, rigidity, tremor, loss of associated
movements, impotence, atonic bladder, loss of rectal sphincter tone,
fasciculations, wasting of distal muscles, evidence of a neuropathic
lesion in the electromyogram suggesting involvement of the anterior
horn cells, and the finding of a neuropathic lesion in the muscle
biopsy. Adams et al first described a
syndrome designated striatonigral degeneration in the early
1960s.3 These patients had mild autonomic failure and
ataxia and were shown to have lesions in the olivopontocerebellar system.
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