Ramsay Hunt syndrome type I
Encyclopedia
Ramsay Hunt syndrome type 1 is a rare, degenerative, neurological disorder characterized by myoclonus
epilepsy, intention tremor
, progressive ataxia
and occasionally cognitive impairment
It has also been alternatively called dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.
, convulsions, and myoclonic epileptic jerks.
Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system. Overall, the lower extremity is usually disturbed less often than the upper extremity.
Additional features of the syndrome include: an unsteady gait, seizures, muscular hypotonia, reduced muscular coordination, asthenia, adiadochokinesia and errors with estimating range, direction, and force of voluntary movements. Mental deterioration can occur, however it is rare.
Some have described this condition as difficult to characterize.
who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921.
Myoclonus
Myoclonus is brief, involuntary twitching of a muscle or a group of muscles. It describes a medical sign and, generally, is not a diagnosis of a disease. Brief twitches are perfectly normal. The myoclonic twitches are usually caused by sudden muscle contractions; they also can result from brief...
epilepsy, intention tremor
Intention tremor
Intention tremor, also known as cerebellar tremor, is a dyskinetic disorder characterized by a broad, course, and low frequency tremor. The amplitude of an intention tremor increases as an extremity approaches the endpoint of deliberate and visually guided movement...
, progressive ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
and occasionally cognitive impairment
It has also been alternatively called dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.
Presentation
Onset of symptoms usually occur in early adulthood and is characterized by intention tremor, progressive ataxiaAtaxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
, convulsions, and myoclonic epileptic jerks.
Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system. Overall, the lower extremity is usually disturbed less often than the upper extremity.
Additional features of the syndrome include: an unsteady gait, seizures, muscular hypotonia, reduced muscular coordination, asthenia, adiadochokinesia and errors with estimating range, direction, and force of voluntary movements. Mental deterioration can occur, however it is rare.
Treatment
Treatment of Ramsay Hunt Syndrome Type 1 is specific to individual symptoms. 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 body disease, dentatorubropallidoluysian atrophy, and celiac disease.Eponym
It is named for James Ramsay HuntJames Ramsay Hunt
James Ramsay Hunt was an American neurologist.He graduated M.D. from the University of Pennsylvania School of Medicine in 1893. He then studied in Paris, Vienna, and Berlin and returned to practise neurology in New York, working at Cornell University Medical School from 1900 - 1910 with Charles...
who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921.