Dyssynergia cerebellaris myoclonica

Alternate names

Ramsay Hunt syndrome type 1 (formerly); Myoclonus and ataxia; Dentate Cerebellar Ataxia; Dentatorubral Atrophy; Primary Dentatum Atrophy; Dyssynergia Cerebellaris Progressiva; Progressive myoclonus ataxia; Ramsay Hunt cerebellar syndrome

Definition

• Dyssynergia Cerebellaris Myoclonica refers to a collection of rare, degenerative, neurological disorders characterized by epilepsy, cognitive impairment, myoclonus, and progressive ataxia.
• Tremor may begin in one extremity and later spread to involve the entire voluntary muscular system.
• Arms are usually more affected than legs.

Onset

Onset of the disorder generally occurs in early adulthood.

Cause

Some of the cases are due to mitochondrial abnormalities.

Signs and symptoms

• This condition is characterized by epilepsy, cognitive impairment, myoclonus, and progressive ataxia.
• Symptoms include seizures, tremor, and reduced muscle coordination.

Treatment

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

Prognosis

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

Resources

Frequently asked questions

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NIH genetic and rare disease info

• NIH info on Dyssynergia cerebellaris myoclonica

Dyssynergia cerebellaris myoclonica is a rare disease.