Ramsay Hunt syndromes
From Wikipedia, the free encyclopedia
| ICD-9 | 053.11, 334.2 |
|---|---|
| DiseasesDB | 11176 |
| MedlinePlus | 001647 |
| eMedicine | neuro/420 |
At least three separate diseases carry the name of Ramsay Hunt syndrome (RHS), described by James Ramsay Hunt (1872-1937). RHS type 1, also known as dyssynergia cerebellaris myoclonica or dyssynergia cerebellaris progressiva is a rare degenerative neurological disorder. The best known of the three is RHS type 2 (or II), and is caused by a Herpes Zoster inflammation of the geniculate ganglion. A third RHS refers to a compression neuritis of the deep palmar branch of the ulnar nerve. Yet another entity encompasses the clinical features produced by carotid artery occlusion (Hunt, 1914 a).
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[edit] Ramsay Hunt syndrome type 2
Ramsay Hunt syndrome (RHS) type 2, is a disorder that is caused by the reactivation of pre-existing herpes zoster virus in a nerve cell bundle (the geniculate ganglion) (Hunt, 1907). The neurons in this ganglion are responsible for the movements of facial muscles, the touch sensation of a part of ear and ear canal, the taste function of the frontal two-thirds of the tongue, and the moisturization of the eyes and the mouth. The syndrome specifically refers to the combination of this entity with weakness of the muscles activated by the facial nerve. In isolation the latter entity would be called Bell's Palsy.
The symptoms and signs include the paralysis of the facial nerve (see Bell's palsy), pain in the ear, taste loss in the front two-thirds of the tongue, dry mouth and eyes, and eruption of veiscles in the ear canal, the tongue, and/or hard palate. Since the vestibulocochlear nerve is in proximity to the geniculate ganglion, it may also be affected, and patients may also suffer from tinnitus, hearing loss, and vertigo.
RHS type 2 is essentially shingles of the geniculate ganglion. Briefly, the herpes zoster virus, which causes chicken pox, lies dormant in various nerve cells in the body, where it is kept in check by the patient's immune system. Given the opportunity, for example during an illness that suppresses the immune system, the virus is reactivated and travels to the end of the nerve cell, where it causes the symptoms described above.
Like shingles, however, lack of lesions does not definitely exclude the existence of a herpes infection. The virus can be detected, even before the eruption of vesicles, from the skin of the ear (Murakami et al, 1998).
The largest study on the treatment of RHS type 2 has shown that complete recovery can be achieved in 75% of patients if treatment with prednisone and acyclovir is started within the first 3 days of onset of symptoms (Murakami et al, 1997). Chances of complete recovery decrease as treatment is delayed. Studies have shown that half of all patients whose treatment was delayed had complete loss of response to facial nerve stimulation. Treatment apparently has no effect on the recovery of hearing loss.
[edit] Ramsay Hunt syndrome type 1
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 (Hunt, 1914 b; Hunt, 1921). It is characterized by seizures, cognitive impairment, action myoclonus, and pregressive 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 (Lu et al, 1986; Chinnery et al, 1997).
[edit] Ramsay Hunt syndrome type 3
Ramsay Hunt syndrome (RHS) type 3 is an occupational compression neuropathy of the deep palmar branch of the Ulnar nerve.
[edit] See also
[edit] External links
- NINDS ramsay2
- NINDS dyssynergia
- Merck Manual 7-85e
- GPnotebook 2134179853
- Ramsay-Hunt Syndrome. Support site.
[edit] References
- 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
- Hunt JR (1907). "On herpetic inflammations of the geniculate ganglion: a new syndrome and its complications". J Nerv Ment Dis 34: 73-96.
- Hunt JR (1914 a). "The role of the carotid arteries in the causation of vascular lesions of the brain, with remarks on certain special features of the symptomatology". Am J Med Sci 147: 704-713.
- Hunt JR (1914 b). "Dyssynergia cerebellaris progressiva: A chronic progressive form of cerebellar tremor". Brain 37: 247.
- 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.
- 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
- Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara N (1997). "Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment". Ann Neurol 41 (3): 353-7. PMID 9066356
- Murakami S, Honda N, Mizobuchi M, Nakashiro Y, Hato N, Gyo K (1998). "Rapid diagnosis of varicella zoster virus infection in acute facial palsy". Neurology 51 (4): 1202-5. PMID 9781562
- Sweeney CJ, Gilden DH (2001). "Ramsay Hunt syndrome". J Neurol Neurosurg Psychiatry 71 (2): 149-54. PMID 11459884
