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

What is Dyssynergia Cerebellaris Myoclonica?

Dyssynergia Cerebellaris Myoclonica refers to a collection of rare, degenerative, neurological disorders characterised 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.

Dyssynergia Cerebellaris Myoclonica was formerly known as Ramsay Hunt Syndrome I.

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.

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