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 = 11176
MeshID = D016697

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). [cite journal | author=Hunt JR | title= On herpetic inflammations of the geniculate ganglion: a new syndrome and its complications| journal= J Nerv Ment Dis| year= 1907| pages= 73–96| volume= 34 | doi= 10.1097/00005053-190702000-00001 ] 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.

Eponym

It is named for James Ramsay Hunt. [WhoNamedIt|synd|2246] cite journal |author= |title=THE RAMSAY HUNT syndrome |journal=Proc. R. Soc. Med. |volume=47 |issue=5 |pages=371–84 |year=1954 |month=May |pmid=13167057 |pmc=1918846 |doi= |url=]

ymptoms and signs

The symptoms and signs include acute facial nerve paralysis, pain in the ear, taste loss in the front two-thirds of the tongue, dry mouth and eyes, and eruption of a erythematous vesicular rashcite journal |author=Sweeney CJ, Gilden DH |title=Ramsay Hunt syndrome |journal=J. Neurol. Neurosurg. Psychiatr. |volume=71 |issue=2 |pages=149–54 |year=2001 |month=August |pmid=11459884 |pmc=1737523 |doi= |url=http://jnnp.bmj.com/cgi/pmidlookup?view=long&pmid=11459884] 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.

Pathophysiology

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. [cite journal | author=Murakami S, Honda N, Mizobuchi M, Nakashiro Y, Hato N, Gyo K | title=Rapid diagnosis of varicella zoster virus infection in acute facial palsy | journal=Neurology | year=1998 | pages=1202–5 | volume=51 | issue=4 PMID 9781562]

Prognosis

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. [cite journal | author=Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara N | title=Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment | journal=Ann Neurol | year=1997 | pages=353–7 | volume=41 | issue=3 | doi = 10.1002/ana.410410310 PMID 9066356] 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.

Diazepam is sometimes used to treat the vertigo. [NINDS|ramsay2]

References

External links

*
* [http://www.ramsayhunt.org Ramsay-Hunt Syndrome] . Support site.
* [http://www.drtbalu.com/Ramsay%20hunt%20syndrome.html drtbalu otolaryngology on line]