Multiple System Atrophy
You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.
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
- progressive autonomic failure with multiple system atrophy
- Shy-Drager syndrome (SDS)
- sporadic olivopontocerebellar atrophy (sOPCA)
- striatonigral degeneration (SND
- MSA-C (cerebellar phenotype)
- MSA-P (parkinsonian phenotype)
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.
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.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
Copyright 1986, 1987, 1988, 1991, 1992, 1994, 1998, 1999, 2000, 2003, 2013
NORD's Rare Disease Information Database is copyrighted and may not be published without the written consent of NORD.