Ramsay Hunt syndrome type I

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.



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 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]


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]


It is named for James Ramsay Hunt[9] who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921.[2]


  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

Wikimedia Foundation. 2010.

Игры ⚽ Поможем написать реферат

Look at other dictionaries:

  • Ramsay Hunt syndrome type II — DiseaseDisorder infobox Name = Ramsay Hunt syndrome (RHS) type 1 ICD10 = ICD10|B|02|2|b|00 ICD9 = ICD9|053.11 ICDO = Caption = Geniculate ganglion is #4 OMIM = OMIM mult = MedlinePlus = 001647 eMedicineSubj = neuro eMedicineTopic = 420 DiseasesDB …   Wikipedia

  • Ramsay Hunt syndromes — At least three separate neurological syndromes carry the name of Ramsay Hunt syndrome (RHS), their only connection being that they were all first described by James Ramsay Hunt (1872 1937): * Ramsay Hunt syndrome type I * Ramsay Hunt syndrome… …   Wikipedia

  • James Ramsay Hunt — (born 1872 in Philadelphia; died July 22, 1937 in Katonah, New York) was an American neurologist. He graduated M.D. from the University of Pennsylvania School of Medicine in 1893. He then studied in Paris, Vienna, and Berlin and returned to… …   Wikipedia

  • Severe acute respiratory syndrome — SARS redirects here. For other uses, see SARS (disambiguation). Further information: Progress of the SARS outbreak Severe Acute Respiratory Syndrome Classification and external resources SARS coronavirus (SARS CoV) is causative of the syndrome …   Wikipedia

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Hunt — Cette page d’homonymie répertorie les différents sujets et articles partageant un même nom. Hunt signifie chasse en anglais. C est aussi un nom de famille très répandu dans le monde anglophone. Sommaire 1 Personnalités 2 …   Wikipédia en Français

  • Liste der Syndrome — Diese Seite listet in alphabetischer Reihenfolge und ohne Anspruch auf Vollständigkeit Syndrome und Komplexe aus unterschiedlichen medizinischen Fachgebieten auf. Bitte nur Verweise auf den tatsächlichen Titel des Beitrags und keine… …   Deutsch Wikipedia

  • List of neurological disorders — This is a list of major and frequently observed neurological disorders (e.g., Alzheimer s disease), symptoms (e.g., back pain), signs (e.g., aphasia) and syndromes (e.g., Aicardi syndrome). Contents: Top · 0–9 A B C D E F G H I J K L M N O P …   Wikipedia

  • Poliomyelitis — Polio redirects here. For the virus, see Poliovirus. Not to be confused with poliosis, a condition of the hair being or becoming white or grey. Poliomyelitis Classification and external resources A man with an atrophie …   Wikipedia

  • Influenza — Flu redirects here. For other uses, see Flu (disambiguation). This article is about the disease influenza. For the family of viruses that cause the disease, see Orthomyxoviridae. Influenza Classification …   Wikipedia

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”