Ramsay Hunt syndrome type 1
| Ramsay Hunt syndrome type 1 | |
|---|---|
| Specialty | Neurology  |
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
- ^ a b c "Ramsay Hunt Syndrome". Retrieved 2011-05-12.
- ^ a b c d e f g h i "National Institute of Neurological Disorders and Stroke". February 14, 2011. Retrieved 2011-05-12.
- ^ 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.
- ^ 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.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ 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.
{{cite journal}}: Check date values in:|year=(help)CS1 maint: year (link) - ^ 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.
- ^ 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.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ 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.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ synd/2245 at Who Named It?
External links
- dyssynergia at NINDS