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dyssynergia cerebellaris myoclonica; Ramsay-Hunt syndrome type 1; Ramsay-Hunt cerebellar syndrome
Epidemiology:
- rare
Pathology:
- mitochondrial abnormalities
Clinical manifestations:
- onset generally occurs in early adulthood
- progression of disease >= 10 years or longer
- epilepsy, seizures
- cognitive impairment
- myoclonus
- progressive ataxia
- tremor
- arms are usually more affected than legs
Management:
- symptomatic
- myoclonus & seizures may respond to anticonvulsants
Related
Herpes zoster oticus; Ramsay Hunt syndrome type 2
General
neurologic disease
Ramsay-Hunt syndrome
References
NINDS Dyssynergia Cerebellaris Myoclonica Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Dyssynergia-Cerebellaris-Myoclonica-Information-Page