Ramsay Hunt syndrome type 1 | |
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Specialty | Neurology |
Ramsay Hunt syndrome type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment [1]
It has also been alternatively called dyssynergia cerebellaris myoclonica, [2] [3] dyssynergia cerebellaris progressiva, [4] dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.
Onset of symptoms usually occurs in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks. [1] Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system. [1] Overall, the lower extremity is usually disturbed less often than the upper extremity. [1] 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. [1] Mental deterioration can, rarely, occur. [1]
Ramsay Hunt syndrome 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, dentatorubropallidoluysian atrophy, and celiac disease. [5] [6]
The diagnosis of Ramsay Hunt syndrome type 1 is different to individual people with the syndrome.
Treatment of Ramsay Hunt syndrome type 1 is specific to individual symptoms. [1] Myoclonus and seizures may be treated with drugs like valproate. [1] Some have described this condition as difficult to characterize. [7]
It is named for James Ramsay Hunt [8] who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921. [1]
Ramsay Hunt syndrome type 1 | |
---|---|
Specialty | Neurology |
Ramsay Hunt syndrome type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment [1]
It has also been alternatively called dyssynergia cerebellaris myoclonica, [2] [3] dyssynergia cerebellaris progressiva, [4] dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.
Onset of symptoms usually occurs in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks. [1] Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system. [1] Overall, the lower extremity is usually disturbed less often than the upper extremity. [1] 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. [1] Mental deterioration can, rarely, occur. [1]
Ramsay Hunt syndrome 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, dentatorubropallidoluysian atrophy, and celiac disease. [5] [6]
The diagnosis of Ramsay Hunt syndrome type 1 is different to individual people with the syndrome.
Treatment of Ramsay Hunt syndrome type 1 is specific to individual symptoms. [1] Myoclonus and seizures may be treated with drugs like valproate. [1] Some have described this condition as difficult to characterize. [7]
It is named for James Ramsay Hunt [8] who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921. [1]