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Multiple system atrophy (MSA) is a neurological disorder that has frequently been misdiagnosed as idiopathic Parkinson disease.' A proliferation of articles in recent literature has noted that MSA is a disease that primarily affects the functioning of the autonomic, basal ganglia, and cerebellar systems.l-4
Key Words:
Multiple system atrophy, Olivopontocerebellar atrophy, Orthostatic hypotension, Parkinson disease, Shy-Drager syndrome, Striatonigral degeneration.
[Swan L, Dupont J. Multiple system atrophy. Phys Ther. 1999;79:488-494.]
The History and Nomenclature of MSA
The recognition of MSA as a unique entity has emerged gradually during the last century. Quinn,' in describing several cases occurring in the late 1800s and early 1900s, may have provided the first descriptions of MSA. In 1925, Bradbury and Eggleston5 wrote the first clear case report of 3 patients with postural hypotension. Today, we know that postural hypotension is a characteristic sign of MSA. In 1960, Shy and Drager6 distinguished a separate syndrome from idiopathic Parkinson disease (IPD). They correlated postural hypotension and the neurological signs of parkinsonism and/or cerebellar dysfunction. This obsen-ation was a first red flag for the potential diagnosis of MSA. Graham and Oppenheimer,7 in 1969, described a patient with orthostatic hypotension, impotence, incontinence, and cerebellar ataxia. They advocated the use of a general term for the increasing reports of autonomic dysfunction that appeared with neurological signs. They proposed the use of the term "multiple system atrophy" to avoid any of the many terms then being used to describe various forms of neuronal atrophy, many of which have overlapping characteristics. In 1972, Bannister and Oppenheimer" presented postmortem evidence from 4 patients with MSA and identified the autonomic pathology as more efferent than afferent, more central than peripheral, and more preganglionic than postganglionic. More importantly, they demonstrated that the Lewy bodies, which are eosinophilic cytoplasmic inclusions, are found in the basal ganglia of patients with IPD but are often absent in patients with MSA.
The term "multiple system atrophy" has been used interchangeably with Shy-Drager syndrome, striatonigral degeneration, and olivopontocerebellar atrophy.2 In 1995, a consensus statement was approved by the American Autonomic Society and the American Academy of Neurology to clarify the definitions of MSA, pure autonomic failure (PAF), and orthostatic hypotension.9 Multiple system atrophy is now defined as "a sporadic, progressive, adult-onset...