Ramsay Hunt syndrome type 1

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]

For other uses, see Ramsay Hunt syndrome.
Ramsay Hunt syndrome type 1
SpecialtyNeurology 

It has also been alternatively called dyssynergia cerebellaris myoclonica,[2][3] dyssynergia cerebellaris progressiva,[4] 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] Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system.[1] Overall, the lower extremity is usually disturbed less often than the upper extremity.[1] 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] Mental deterioration can, rarely, occur.[1]

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.[5][6]

Treatment

Treatment of Ramsay Hunt Syndrome Type 1 is specific to individual symptoms.[1] Myoclonus and seizures may be treated with drugs like valproate.[1]Some have described this condition as difficult to characterize.[7]

Eponym

It is named for James Ramsay Hunt[8] who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921.[1]

References
  1. "National Institute of Neurological Disorders and Stroke". February 14, 2011. Archived from the original on February 16, 2015. Retrieved 2011-05-12.
  2. 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): 490–538. doi:10.1093/brain/44.4.490.
  3. 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. Psychiatry. 52 (2): 262–5. doi:10.1136/jnnp.52.2.262. PMC 1032517. PMID 2703843.
  4. Hunt JR (1914). "Dyssynergia cerebellaris progressiva: A chronic progressive form of cerebellar tremor". Brain. 37 (2): 247–268. doi:10.1093/brain/37.2.247.
  5. 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.
  6. 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.
  7. Berkovic SF, Andermann F (January 1990). "Ramsay Hunt syndrome: to bury or to praise". J. Neurol. Neurosurg. Psychiatry. 53 (1): 89–90. doi:10.1136/jnnp.53.1.89. PMC 1014111. PMID 2106013.
  8. synd/2245 at Who Named It?
Classification
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