Multiple system atrophy

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By Robert Fekete MD

Multiple system atrophy is also known as or subsumes Shy-Drager syndrome. -ed.

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 clinical article, Dr. Robert Fekete of Baylor College of Medicine describes the essential features of multiple system atrophy, including recent 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.