Multiple system atrophy

Robert Fekete MD (Dr. Fekete of New York Medical College received consultation fees from Acadia, Impax, Lundbeck, Neurocrine, and Teva.)
Joseph Jankovic MD, editor. (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released October 4, 1993; last updated October 14, 2016; expires October 14, 2019

This article includes discussion of multiple system atrophy, Shy-Drager syndrome, multiple system atrophy-cerebellar type, multiple system atrophy-parkinsonian type, MSA-C, and MSA-P. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Multiple system atrophy is a sporadic degenerative disorder with a highly variable anatomic distribution and clinical picture. It typically features some combination of motor parkinsonism, ataxia, and dysautonomia, along with many other less constant signs. Average age at onset is 53 years, and average survival is only 8 years. Although there is no specific treatment, clinicians can palliate many aspects of the syndrome, particularly the dysautonomia and the sleep disturbances. The defining pathologic feature is aggregates of alpha-synuclein in oligodendroglia. In this article, the author describes the essential features of multiple system atrophy, including advances in distinguishing it from the other parkinsonian disorders and in managing its many disabling clinical features.

Key points

 

• The hallmark of multiple system atrophy pathology is the presence of glial cytoplasmic inclusions.

 

• “Red flags” for multiple system atrophy include postural instability within 3 years of disease onset and resulting recurrent falls, wheelchair dependency within 10 years of onset, Pisa syndrome, stridor, inspiratory sighs, severe dysphonia, severe dysarthria, severe dysphagia, and emotional incontinence.

 

• Signal loss in dorsolateral putamen on T2 MRI sequences with the presence of a hyperintense lateral rim in fluid attenuated inversion recovery (FLAIR) sequences has a specificity of 0.97 for discriminating between multiple system atrophy and Parkinson disease.

 

• The “hot cross bun” sign, a hyperintensity in the pons on T2 MRI images, is not specific for multiple system atrophy.

Historical note and terminology

Like many heterogeneous disorders, multiple system atrophy was described piecemeal over a period of decades and engenders nosologic controversy. The sporadic form of "olivopontocerebellar atrophy," a term often applied to the cerebellar-predominant variety of multiple system atrophy, was first described by Dejerine and Thomas (Dejerine and Thomas 1900). A dysautonomia-predominant type was described much later (Shy and Drager 1960), as was the parkinsonism-predominant variety, striatonigral degeneration (van der Eecken et al 1960; Adams et al 1964; Adams and Salam-Adams 1986). No less historic a contribution was the observation that all varieties of multiple system atrophy share a specific glial cytoplasmic inclusion (Papp et al 1989) and that these inclusions are principally composed of alpha-synuclein (Gai et al 1998; Dickson et al 1999).

The current classification scheme based on the first consensus statement on the diagnosis of multiple system atrophy divides the disorder into a parkinsonian type (MSA-P) and a cerebellar type (MSA-C) (Gilman et al 1999). These terms replaced the striatonigral degeneration, sporadic olivopontocerebellar atrophy, and Shy-Drager syndrome designations. In the second consensus statement in 2008, definite, probable, and possible multiple system atrophy diagnostic criteria were introduced (Gilman et al 2008).

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