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Ramsay Hunt syndrome type I



Ramsay Hunt syndrome type I
Classification & external resources
ICD-10 G11.1
ICD-9 334.2

Ramsay Hunt syndrome (RHS) type 1 is a rare and nebulous entity that has alternatively been called dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.[1][2]

It is characterized by seizures, cognitive impairment, action myoclonus, and progressive ataxia. Symptoms include seizures, tremor, and reduced muscle coordination. Myoclonus and seizures may be treated with drugs like valproate. 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 and celiac disease.[3][4]

See also

  • James Ramsay Hunt

References

  1. ^ Hunt JR (1914 b). "Dyssynergia cerebellaris progressiva: A chronic progressive form of cerebellar tremor". Brain 37: 247.
  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: 490.
  3. ^ 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. PMID 3504245
  4. ^ 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
 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Ramsay_Hunt_syndrome_type_I". A list of authors is available in Wikipedia.
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