dyssynergia cerebellaris progressiva

Ramsay Hunt syndrome type I

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.

Presentation

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.

Causes

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.

Eponym

It is named for James Ramsay Hunt.

References

External links

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