Ramsay Hunt syndrome type I

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.

Contents

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

Inflammation
Both/either
Brain/
encephalopathy
Episodic/
paroxysmal
Other
Spinal cord/
myelopathy
Both/either

M: CNS

anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp

noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr

proc, drug(N1A/2AB/C/3/4/7A/B/C/D)