Ramsay Hunt syndrome (RHS) type 1
is a rare and nebulous entity that has alternatively been called dyssynergia cerebellaris myoclonica
, dyssynergia cerebellaris progressiva
, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.
It is characterized by seizures, cognitive impairment, action myoclonus
, and progressive ataxia
. Symptoms include seizures, tremor, and reduced muscle coordination. Myoclonus and seizures may be treated with drugs like valproate
Some have described this condition as difficult to characterize.
RHS type 1 is caused by the impairment of a regulatory mechanism between cerebellar and brainstem nuclei and has been associated with a wide range of diseases, including Lafora disease
and celiac disease
It is named for James Ramsay Hunt