Ramsay Hunt syndrome type 1

For other uses, see Ramsay Hunt syndrome.
Ramsay Hunt syndrome type 1
Classification and external resources
Specialty neurology
ICD-10 G11.1
ICD-9-CM 334.2
OMIM 213400
DiseasesDB 30054
MeSH D002527

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.

Presentation

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]

Treatment

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]

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 disease, dentatorubropallidoluysian atrophy, and celiac disease.[7][8]

Eponym

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

References

  1. 1 2 3 "Ramsay Hunt Syndrome". Retrieved 2011-05-12.
  2. 1 2 3 4 5 6 7 8 9 "National Institute of Neurological Disorders and Stroke". February 14, 2011. Retrieved 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. doi:10.1093/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. doi:10.1136/jnnp.52.2.262. PMC 1032517. PMID 2703843.
  5. ↑ Hunt JR (1914 b). "Dyssynergia cerebellaris progressiva: A chronic progressive form of cerebellar tremor". Brain 37 (2): 247. doi:10.1093/brain/37.2.247. Check date values in: |date= (help)
  6. ↑ Berkovic SF, Andermann F (January 1990). "Ramsay Hunt syndrome: to bury or to praise". J. Neurol. Neurosurg. Psychiatr. 53 (1): 89–90. doi:10.1136/jnnp.53.1.89. PMC 1014111. PMID 2106013.
  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. doi:10.1002/mds.870010306. PMID 3504245.
  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. doi:10.1212/wnl.49.4.1131. PMID 9339701.
  9. ↑ synd/2245 at Who Named It?

External links

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