Ramsay Hunt Syndrome I

Ramsay Hunt syndrome I was the name once given to a collection of rare, degenerative neurological disorders. Today this collection of disorders is known as dyssynergia cerebellaris myoclonica. Ramsay Hunt syndrome I is not related to the disorder once known as Ramsay Hunt syndrome II (or simply Ramsay Hunt syndrome).

 

What Is Ramsay Hunt Syndrome I?

Now referred to as dyssynergia cerebellaris myoclonica, Ramsay Hunt syndrome I was the name given to a collection of rare, degenerative neurological disorders characterized by:
 
  • Epilepsy
  • Cognitive impairment
  • Myoclonus (a sudden, involuntary jerking of a muscle or group of muscles)
  • Progressive ataxia (clumsiness).
     
Ramsay Hunt syndrome II (or simply Ramsay Hunt Syndrome) is a different condition. It is now called herpes zoster oticus.
 

Symptoms Associated With Ramsay Hunt Syndrome I

Symptoms of Ramsay Hunt syndrome I include:
 
  • Seizures
  • Tremor
  • Reduced muscle coordination.
     
Onset of Ramsay Hunt syndrome I generally occurs in early adulthood. Tremor may begin in one extremity and later spread to involve the entire voluntary muscular system. In most people with Ramsay Hunt syndrome I, their arms are usually more affected than their legs. Some of the cases are due to mitochondrial abnormalities.
 

Treatment of Ramsay Hunt Syndrome I

Treatment of Ramsay Hunt syndrome I is symptomatic. Myoclonus and seizures may be treated with drugs like valproate.
 

Ramsay Hunt Syndrome I: Prognosis

The progression of Ramsay Hunt syndrome I is usually 10 years or longer.
 
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Written by/reviewed by: Arthur Schoenstadt, MD
Last reviewed by: Arthur Schoenstadt, MD