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Onset of symptoms usually occur in early adulthood and is characterized by intention tremor, progressive ataxia, 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.
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.
- ↑ 1.0 1.1 1.2 Ramsay Hunt Syndrome. URL accessed on 2011-05-12.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 National Institute of Neurological Disorders and Stroke. URL accessed on 2011-05-12.
- ↑ Hunt JR (1921). Dyssynergia cerebellaris myoclonica—Primary atrophy of the dentate system: A contribution to the pathology and symptomatology of the cerebellum. Brain 44 (4): 490.
- ↑ Tassinari CA, Michelucci R, Genton P, Pellissier JF, Roger J (February 1989). Dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome): a condition unrelated to mitochondrial encephalomyopathies. J. Neurol. Neurosurg. Psychiatr. 52 (2): 262–5.
- ↑ Hunt JR (1914 b). Dyssynergia cerebellaris progressiva: A chronic progressive form of cerebellar tremor. Brain 37 (2): 247.
- ↑ Berkovic SF, Andermann F (January 1990). Ramsay Hunt syndrome: to bury or to praise. J. Neurol. Neurosurg. Psychiatr. 53 (1): 89–90.
- ↑ Lu CS, Thompson PD, Quinn NP, Parkes JD, Marsden CD (1986). Ramsay Hunt syndrome and coeliac disease: a new association?. Mov Disord 1 (3): 209–19.
- ↑ Chinnery PF, Reading PJ, Milne D, Gardner-Medwin D, Turnbull DM (1997). CSF antigliadin antibodies and the Ramsay Hunt syndrome. Neurology 49 (4): 1131–3.
- ↑ Who Named It synd/2245
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