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Multiple System Atrophy

Abstract

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NORD is very grateful to Niall P. Quinn, MD, Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London, UK, for assistance in the preparation of this report.

Synonyms of Multiple System Atrophy

  • MSA
  • progressive autonomic failure with multiple system atrophy
  • Shy-Drager syndrome (SDS)
  • sporadic olivopontocerebellar atrophy (sOPCA)
  • striatonigral degeneration (SND

Disorder Subdivisions

  • MSA-C (cerebellar phenotype)
  • MSA-P (parkinsonian phenotype)

General Discussion

Summary
Multiple system atrophy (MSA) is a rare sporadic progressive neurological disorder characterized by a varying combination of symptoms and signs. Onset is during adulthood (>30 years). Affected individuals may experience symptoms similar to those found in Parkinson’s disease (parkinsonism); cerebellar signs such as progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia); and impaired functioning of the portion of the nervous system (autonomic nervous system) that regulates certain involuntary body functions (autonomic failure) such as heart rate, blood pressure, sweating, and bowel and bladder control. When parkinsonian symptoms predominate, the disorder may be referred to as MSA-P (parkinsonian phenotype); when the cerebellar symptoms predominate the disorder may be referred to as MSA-C (cerebellar phenotype). The exact cause of MSA is unknown.

Introduction
The term multiple system atrophy was first introduced in the medical literature in 1969. It encompasses three presentations of a single disease formerly thought to be separate disorders, specifically Shy-Drager syndrome (which emphasized autonomic dysfunction), striatonigral degeneration (which emphasized parkinsonian symptoms), and sporadic olivopontocerebellar atrophy (which emphasized cerebellar symptoms), although the cases of each of these that were originally described presented a combination of all three clinical features, and brain pathology was found in both the striatonigral and olivopontocerebellar structures. Additionally, there is a hereditary form of olivopontocerebellar atrophy that is not part of the multiple system atrophy spectrum.

Organizations related to Multiple System Atrophy

NORD offers an online community for this rare disease. RareConnect was created by EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders) to provide a safe space where individuals and families affected by rare diseases can connect with each other, share vital experiences, and find helpful information and resources. You can view these international, rare disease communities at www.rareconnect.org.

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