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Ramsay Hunt syndrome type 1
Specialty	Neurology

Ramsay Hunt syndrome type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment

It has also been alternatively called dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.

Presentation

Onset of symptoms usually occurs in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks. Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system. Overall, the lower extremity is usually disturbed less often than the upper extremity. 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. Mental deterioration can, rarely, occur.

Causes

Ramsay 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.

Diagnosis

The diagnosis of Ramsay Hunt syndrome type 1 is different to individual people with the syndrome.

Treatment

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

Eponym

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

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". Journal of Neurology, Neurosurgery, and 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?". Movement Disorders. 1 (3): 209–19. doi:10.1002/mds.870010306. PMID 3504245. S2CID 45493928.
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. S2CID 18672452.
7. 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.
8. synd/2245 at Who Named It?

External links

• Dyssynergia Cerebellaris Myoclonica at NINDS

• Neurological disorders
• Rare syndromes