Ramsay Hunt syndrome, type I.

Ramsay Hunt syndrome is a neurologic abnormality with two subtypes. (1-3) Ramsey Hunt syndrome type I is known as herpes zoster oticus, geniculate neuralgia, and nervus intermedius neuralgia. Type II syndrome is known as dyssynergia cerebellaris myoclonica; it is an autosomal-recessive, progressive, myoclonic epilepsy with ataxia secondary to mitochondrial encephalopathy.

The type I syndrome was first reported in 1907 by J. Ramsay Hunt in patients with otalgia and cutaneous and mucosal lesions; the lesions led to a varicella zoster virus (human herpesvirus 3) infection of the geniculate ganglion. (4) Varicella zoster virus is diagnosed by serologic tests, anti-body determinations, the presence of vesicular rashes, and polymerase chain reaction testing of tear fluid samples. (5) Furuta et al detected varicella zoster virus DNA in 11 of 14 trigeminal ganglia and 9 of 13 geniculate ganglia in adults with Ramsay Hunt syndrome type I. (6) A finding of varicella-zoster--seronegative ganglia indicates that varicelia has become latent in the human geniculate ganglion following a primary infection and that reactivation of the latent virus from the geniculate ganglion precipitates Ramsey Hunt syndrome type I. (6,7)

Type I syndrome produces acute ipsilateral facial paralysis, which can lead to difficulty closing the eye on the affected side, otalgia, hearing loss, vertigo, tinnitus, and multiple cutaneous herpetic blisters. Herpetic blisters may manifest on the skin of the auricle, pinna, external ear canal, anterior two-thirds of the tongue, and palate. (1-3,8)

In some patients, taste perception and lacrimation are also affected. (9)

Ramsey Hunt syndrome type I can be diagnosed clinically by the presence of herpetic lesions with their characteristic distribution, sensorineural hearing loss, and facial paralysis, as occurred in a 39-year-old woman whose case is illustrated here. T1-weighted magnetic resonance imaging (MRI) with gadolinium contrast can demonstrate enhancement of the cochleovestibular complex, the facial nerve, and the geniculate ganglion (figure).

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Treatment of Ramsey Hunt syndrome type I includes antiviral medications such as acyclovir or valacyclovir for 7 to 10 days, corticosteroids such as prednisone for 3 to 5 days with subsequent tapering regimens, antihistamines such as meclizine to reduce vertigo and tinnitus, anticon-vulsants, and anticholinergics such as scopolamine to suppress conduction of the cochleovestibular system and to treat motion sickness. (3) An eye patch should be worn to prevent corneal abrasions and any damage to an eye that does not close completely. (10)

When there is minimal nerve damage, the patient usually recovers completely. However, when cranial nerve damage is severe, the patient will experience a permanent neurologic deficit. Treatment initiated within 3 days is associated with a 70% recovery rate, while treatment initiated after 3 days is associated with only a 50% recovery rate. (10)

References

(1.) Evans RW, ed. Saunders Manual of Neurologic Practice. Philadelphia: W.B. Saunders; 2003:73.

(2.) Goetz CG, ed. Textbook of Clinical Neurology. Philadelphia: W.B. Saunders; 2003:744, 910-11, 1169.

(3.) Awasthi D. Ramsay Hunt syndrome. Available at www.emedicine. com/neuro/topic420.htm (access verified Feb. 6, 2007).

(4.) Hunt JR. On herpetic inflammations of the geniculate ganglion: A new syndrome and its complications. J Nerv Ment Dis 1907;34: 73-96.

(5.) Diaz GA, Rakita RM, Koelle DM. A case of Ramsay Hunt-like syndrome caused by herpes simplex virus type 2. Clin Infect Dis 2005;40:1545-7.

(6.) Furuta Y, Takasu T, Fukuda S, et al. Detection of varicella-zoster virus DNA in human geniculate ganglia by polymerase chain reaction. J Infect Dis 1992;166:1157-9.

(7.) Harnsberger HR, ed. Diagnostic Imaging: Head and Neck. Salt Lake City: Amirsys; 2004.

(8.) Adour KK. Otological complications of herpes zoster. Ann Neurol 1994;35(suppl):S62-4.

(9.) Mishell JH, Applebaum EL. Ramsay-Hunt syndrome in a patient with HIV infection. Otolaryngol Head Neck Surg 1990;102: 177-9.

(10.) University of Pennsylvania Health System. Encyclopedia: Ramsay Hunt syndrome. Available at http://pennhealth.com/ency/ article/001647.htm (access verified Feb. 6, 2007).

Jagan Gupta, MD; Troy Hutchins, MD; Enrique Palacios, MD

From the Department of Radiology, Tulane University Hospital and Clinics, New Orleans.