Important
Important
It is possible that the main title of the report Olivopontocerebellar Atrophy, Hereditary is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
- Hereditary OPCA
Disorder Subdivisions
- OPCA1
- OPCA, Menzel type
- Spinocerebellar Ataxia 1
- SCA1
- OPCA2
- OPCA, Holguin type
- Spinocerebellar Ataxia 2
- SCA2
- SCA, Cuban type
- OPCA3
- Spinocerebellar Ataxia 7
- SCA7
- OPCA with retinal degeneration
- OPCA, Fickler-Winkler type
- OPCA4
- OPCA, Schut-Haymaker type
- OPCA5
- OPCA with dementia and extrapyramidal signs
General Discussion
Hereditary olivopontocerebellar atrophy (OPCA) is a rare group of disorders characterized by progressive balance problems (disequilibrium), progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia), and difficulty speaking or slurred speech (dysarthria). There are at least five distinct forms of hereditary OPCA. All forms of hereditary OPCA, except one, are inherited as autosomal dominant traits.
The term olivopontocerebellar atrophy has generated significant controversy and confusion in the medical literature because of its association with two distinct groups of disorders, specifically multiple system atrophy (MSA) and spinocerebellar ataxia (SCA). OPCA may refer to a specific form of MSA or one of several types of SCA. Hereditary OPCA refers to the group of disorders that overlaps with SCA. Both forms of OPCA are characterized by progressive degeneration of certain structures of the brain, especially the cerebellum, pons, and inferior olives. The cerebellum is the part of the brain that plays a role in maintaining balance and posture as well as coordinating voluntary movement. The pons is part of the brainstem and contains important neuronal pathways between the cerebrum, spinal cord, and cerebellum. The pons serves as a relay point for messages between these structures. The inferior olives are two round structures that contain nuclei that are involved with balance, coordination and motor activity.
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Resources
WE MOVE (Worldwide Education and Awareness for Movement Disorders)
204 West 84th Street
New York, NY 10024
USA
Tel: 2128758312
Fax: 2128758389
Email: wemove@wemove.org
Internet: http://www.wemove.org
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com
National Ataxia Foundation
2600 Fernbrook Lane
Suite 119
Minneapolis, MN 55447-4752
USA
Tel: 7635530020
Fax: 7635530167
Email: naf@ataxia.org
Internet: http://www.ataxia.org
National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/
National Parkinson Foundation, Inc.
1501 NW 9th Ave/Bob Hope Road
Miami, FL 33136-1494
Tel: (305)243-6666
Fax: (305)243-5595
Tel: (800)327-4545
Email: contact@parkinson.org
Internet: http://www.parkinson.org
