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

More general terms

• neurologic disease
• Ramsay-Hunt syndrome

Additional terms

• Herpes zoster oticus; Ramsay Hunt syndrome type 2