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Ramsay Hunt syndrome type I

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Ramsay Hunt syndrome type I
ICD-10 G111
ICD-9 334.2
OMIM 213400
DiseasesDB 30054
MedlinePlus [1]
eMedicine /
MeSH {{{MeshNumber}}}

Ramsay Hunt syndrome (RHS) type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment [1][2]

It has also been alternatively called dyssynergia cerebellaris myoclonica,[3][4] dyssynergia cerebellaris progressiva,[5] dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.

PresentationEdit

Onset of symptoms usually occur in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks.[1][2] Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system.[2] Overall, the lower extremity is usually disturbed less often than the upper extremity.[2] 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][2] Mental deterioration can occur, however it is rare.[2]

TreatmentEdit

Treatment of Ramsay Hunt Syndrome Type 1 is specific to individual symptoms.[2] Myoclonus and seizures may be treated with drugs like valproate.[2]

Some have described this condition as difficult to characterize.[6]

CausesEdit

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.[7][8]

EponymEdit

It is named for James Ramsay Hunt[9] who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921.[2]

ReferencesEdit

  1. 1.0 1.1 1.2 Ramsay Hunt Syndrome. URL accessed on 2011-05-12.
  2. 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.
  3. 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.
  4. 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.
  5. Hunt JR (1914 b). Dyssynergia cerebellaris progressiva: A chronic progressive form of cerebellar tremor. Brain 37 (2): 247.
  6. Berkovic SF, Andermann F (January 1990). Ramsay Hunt syndrome: to bury or to praise. J. Neurol. Neurosurg. Psychiatr. 53 (1): 89–90.
  7. 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.
  8. 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.
  9. Who Named It synd/2245

External linksEdit


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