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NINDS Dyssynergia Cerebellaris Myoclonica Information Page

Synonym(s):  Dyssynergia Cerebellaris Progressiva, Dentate Cerebellar Ataxia, Dentatorubral Atrophy, Primary Dentatum Atrophy, Ramsay Hunt Syndrome I

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What is Dyssynergia Cerebellaris Myoclonica?

Dyssynergia Cerebellaris Myoclonica refers to a collection of rare, degenerative, neurological disorders characterized by epilepsy, cognitive impairment, myoclonus, and progressive ataxia.  Symptoms include seizures, tremor, and reduced muscle coordination.  Onset of the disorder generally occurs in early adulthood.  Tremor may begin in one extremity and later spread to involve the entire voluntary muscular system.  Arms are usually more affected than legs.  Some of the cases are due to mitochondrial abnormalities.

Is there any treatment?

Treatment of Dyssynergia Cerebellaris Myoclonica is symptomatic.  Myoclonus and seizures may be treated with drugs like valproate.

What is the prognosis?

The progression of the disorder is usually 10 years or longer.

What research is being done?

The NINDS supports a broad range of research on neurodegenerative disorders such as Dyssynergia Cerebellaris Myoclonica.  The goals of this research are to find ways to prevent, treat, and cure these kinds of disorders.

NIH Patient Recruitment for Dyssynergia Cerebellaris Myoclonica Clinical Trials


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National Institute on Deafness and Other Communication Disorders (NIDCD)
National Institutes of Health, DHHS
31 Center Drive, MSC 2320
Bethesda, MD 20892-2320
Tel: 301-496-7243; 800-241-1044; 800-241-1055 (TTY)

National Ataxia Foundation (NAF)
2600 Fernbrook Lane North
Suite 119
Minneapolis, MN 55447-4752
Tel: 763-553-0020
Fax: 763-553-0167

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT 06810
Tel: 203-744-0100; Voice Mail: 800-999-NORD (6673)
Fax: 203-798-2291


Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last Modified February 14, 2011