Ramsay Hunt syndrome type I

For other uses, see Ramsay Hunt syndrome.

Ramsay Hunt syndrome type I
Classification and external resources
ICD-10	G11.1
ICD-9	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 body 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. ^ ^{a b c} "Ramsay Hunt Syndrome". http://www.ramsayhunt.org/epon.shtml. Retrieved 2011-05-12. 
2. ^ ^{a b c d e f g h i} "National Institute of Neurological Disorders and Stroke". February 14, 2011. http://www.ninds.nih.gov/disorders/dyssynergia/dyssynergia.htm. 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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1032517. 
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. 
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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1014111. 
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. PMID 9339701. 
9. ^ synd/2245 at Who Named It?

External links

• dyssynergia at NINDS