Dyssynergia Cerebellaris Myoclonica

Nervous system disorder

Dyssynergia cerebellaris myoclonica (formerly called "Ramsay Hunt syndrome type I") refers to a degenerative neurological disorder. It may be inherited in an autosomal recessive pattern or may occur in someone who has no history of the disorder in his or her family. It is not known how often dyssynergia cerebellaris myoclonica occurs. Symptoms of the disorder, which worsen over time, include:

• seizures
• cognitive impairment
• jerking muscle spasms or tremors (myoclonus)
• problems with muscle coordination (ataxia)

Dyssynergia cerebellaris myoclonica usually begins in early adulthood but may also occur in children. Muscle tremors may begin in one arm or leg and later spread to involve all the voluntary muscles.

Diagnosis and treatment

Dyssynergia cerebellaris myoclonica is diagnosed based on the symptoms. There is no cure, so treatment is based on relieving symptoms. Drugs like Depakene (valproate) may be used to control seizures and muscle spasms.

_Sources:

"NINDS Dyssynergia Cerebellaris Myoclonica Information Page." Disorders. 12 Feb 2007. National Organization for Neurological Disorders and Stroke. 16 Jul 2008.

Tassinari, C.A., R. Michelucci, P. Genton, J.F. Pellissier, & J. Roger. "Dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome): a condition unrelated to mitochondrial encephalomyopathies." J Neurol Neurosurg Psychiatry 52(1989): 262-5.