Olivopontocerebellar Atrophy, Hereditary

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 olivae. 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 olivae 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

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

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This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated: 5/23/2003
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