Ramsay Hunt syndrome type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment [1]
It has also been alternatively called dyssynergia cerebellaris myoclonica,[2][3]dyssynergia cerebellaris progressiva,[4] dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.
Onset of symptoms usually occurs 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]
The diagnosis of Ramsay Hunt syndrome type 1 is different to individual people with the syndrome.
Treatmentedit
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]
Eponymedit
It is named for James Ramsay Hunt[8] who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921.[1]
Referencesedit
^ abcdefghi. February 14, 2011. Archived from the original on February 16, 2015. 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): 490–538. 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". Journal of Neurology, Neurosurgery, and Psychiatry. 52 (2): 262–5. doi:10.1136/jnnp.52.2.262. PMC1032517. PMID 2703843.
^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.
^Lu CS, Thompson PD, Quinn NP, Parkes JD, Marsden CD (1986). "Ramsay Hunt syndrome and coeliac disease: a new association?". Movement Disorders. 1 (3): 209–19. doi:10.1002/mds.870010306. PMID 3504245. S2CID 45493928.
^Berkovic SF, Andermann F (January 1990). "Ramsay Hunt syndrome: to bury or to praise". Journal of Neurology, Neurosurgery, and Psychiatry. 53 (1): 89–90. doi:10.1136/jnnp.53.1.89. PMC1014111. PMID 2106013.
ramsay, hunt, syndrome, type, other, uses, ramsay, hunt, syndrome, rare, degenerative, neurological, disorder, characterized, myoclonus, epilepsy, intention, tremor, progressive, ataxia, occasionally, cognitive, impairment, specialtyneurology, also, been, alte. For other uses see Ramsay Hunt syndrome Ramsay Hunt syndrome type 1 is a rare degenerative neurological disorder characterized by myoclonus epilepsy intention tremor progressive ataxia and occasionally cognitive impairment 1 Ramsay Hunt syndrome type 1SpecialtyNeurology It has also been alternatively called dyssynergia cerebellaris myoclonica 2 3 dyssynergia cerebellaris progressiva 4 dentatorubral degeneration or Ramsay Hunt cerebellar syndrome Contents 1 Presentation 2 Causes 3 Diagnosis 4 Treatment 5 Eponym 6 References 7 External linksPresentation editOnset of symptoms usually occurs 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 editRamsay Hunt syndrome 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 Diagnosis editThe diagnosis of Ramsay Hunt syndrome type 1 is different to individual people with the syndrome Treatment editTreatment 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 editIt is named for James Ramsay Hunt 8 who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921 1 References edit a b c d e f g h i National Institute of Neurological Disorders and Stroke February 14 2011 Archived from the original on February 16 2015 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 4 490 538 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 Journal of Neurology Neurosurgery and Psychiatry 52 2 262 5 doi 10 1136 jnnp 52 2 262 PMC 1032517 PMID 2703843 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 Lu CS Thompson PD Quinn NP Parkes JD Marsden CD 1986 Ramsay Hunt syndrome and coeliac disease a new association Movement Disorders 1 3 209 19 doi 10 1002 mds 870010306 PMID 3504245 S2CID 45493928 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 S2CID 18672452 Berkovic SF Andermann F January 1990 Ramsay Hunt syndrome to bury or to praise Journal of Neurology Neurosurgery and Psychiatry 53 1 89 90 doi 10 1136 jnnp 53 1 89 PMC 1014111 PMID 2106013 synd 2245 at Who Named It External links editDyssynergia Cerebellaris Myoclonica at NINDS Retrieved from https en wikipedia org w index php title Ramsay Hunt syndrome type 1 amp oldid 1224452331, wikipedia, wiki, book, books, library,
