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Multiple system atrophy: Clinical features and diagnosis

Authors
Stewart A Factor, DO
Christine Doss Esper, MD
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

Multiple system atrophy (MSA) is a unifying term that brings together a group of neurodegenerative syndromes (olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager syndrome) with similar brain pathology that are characterized by various degrees of autonomic dysfunction, cerebellar abnormalities, parkinsonism, and corticospinal degeneration.

This topic will review the epidemiology, clinical features, and diagnosis of MSA. The prognosis and treatment of MSA is reviewed separately. (See "Multiple system atrophy: Prognosis and treatment".)

Other forms of parkinsonism are discussed elsewhere. (See "Clinical manifestations of Parkinson disease" and "Diagnosis and differential diagnosis of Parkinson disease" and "Corticobasal degeneration" and "Progressive supranuclear palsy (PSP)".)

HISTORICAL BACKGROUND

In 1900, Dejerine and Thomas provided the first report of sporadic olivopontocerebellar atrophy (OPCA), a disease that later would become a part of the spectrum of MSA [1]. Orthostatic hypotension as a manifestation of autonomic failure was described in 1925 [2]. In 1960, Shy and Drager reported patients with autonomic features of orthostatic syncope, impotence, and bladder dysfunction who went on to develop gait abnormalities, tremor, and fasciculations among other symptoms and signs [3]. This disorder became known as the Shy-Drager syndrome.

Also in 1960, the first cases were reported of a predominantly asymmetric parkinsonian syndrome manifested primarily by akinesia and rigidity [4]. The authors suggested that striatonigral degeneration was the pathologic correlate for these cases.

                 

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Literature review current through: Nov 2016. | This topic last updated: Wed Sep 23 00:00:00 GMT 2015.
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