Swelling of the Intratemporal Facial Nerve in Ramsay Hunt Syndrome

Although Ramsay Hunt syndrome is one of the most important diseases causing peripheral facial palsy, the detailed pathology of the disease in the intratemporal facial nerve remains unclear. The purpose of this study was to increase knowledge of the pathogenesis of the syndrome by means of surgical findings. Between April 1976 and March 1997 we performed subtotal decompression of the facial nerve in 74 patients with severe Ramsay Hunt syndrome. The grade of nerve swelling was assessed using a microscope and recorded in a standardized form. The relationships between nerve swelling, the timing of surgery and the swelling of each segment were analyzed. Pronounced neural swelling, involving the geniculate ganglion and the horizontal segment, was consistent finding in the acute phase. Although the incidence of pronounced swelling of the horizontal segment gradually declined with time after onset, in most cases nerve swelling persisted even beyond the 16th week after onset. These data suggest that diffuse viral neuritis occurs throughout the intratemporal facial nerve. We assume that the viral inflammatory swelling involving the geniculate ganglion and horizontal segment is mostly responsible for the acute facial palsy in the acute phase.

[1]  D. Gilden,et al.  Ramsay Hunt syndrome , 2001, Journal of neurology, neurosurgery, and psychiatry.

[2]  S. Kollias,et al.  Ramsay Hunt syndrome associated with brain stem enhancement. , 1999, AJNR. American journal of neuroradiology.

[3]  N. Hato,et al.  Rapid diagnosis of varicella zoster virus infection in acute facial palsy , 1998, Neurology.

[4]  P. A. Wackym Molecular Temporal Bone Pathology: II. Ramsay Hunt Syndrome (Herpes Zoster Oticus) , 1997, The Laryngoscope.

[5]  K. Adour Otological complications of herpes zoster , 1994, Annals of neurology.

[6]  A. Valavanis,et al.  Idiopathic, herpetic, and HIV-associated facial nerve palsies: abnormal MR enhancement patterns. , 1994, AJNR. American journal of neuroradiology.

[7]  W. Grody,et al.  Varicella-zoster DNA in temporal bones of patients with ramsay hunt syndrome , 1993, The Lancet.

[8]  Y. Furuta,et al.  Detection of varicella-zoster virus DNA in human geniculate ganglia by polymerase chain reaction. , 1992, The Journal of infectious diseases.

[9]  B. Connelly,et al.  Animal models of varicella. , 1992, The Journal of infectious diseases.

[10]  M. May,et al.  Gadolinium-enhanced magnetic resonance imaging of the facial nerve in herpes zoster oticus and Bell's palsy: clinical implications. , 1991, The American journal of otology.

[11]  R. P. Mehta,et al.  Ramsay - Hunt Syndrome. , 1980, Indian journal of dermatology, venereology and leprology.

[12]  A. Lieberman,et al.  Herpes zoster oticus and facial paralysis (Ramsay Hunt syndrome). Clinico-pathologic study and review of literature. , 1973, Journal of the neurological sciences.

[13]  J. Dawes,et al.  Herpes zoster of the head and neck , 1972, The Journal of Laryngology & Otology.

[14]  I. Friedmann,et al.  Herpes zoster auris associated with facial nerve palsy and auditory nerve symptoms: a case report with histopathological findings. , 1968, Acta oto-laryngologica.

[15]  I. Friedmann,et al.  Herpes Zoster Auris Associated with Facial Nerve Palsy and Auditory Nerve Symptoms: A Case Report with Histopathological Findings , 1967 .

[16]  K. Kettel,et al.  Histopathology of the facial nerve in herpes zoster oticus. , 1959, A.M.A. archives of otolaryngology.

[17]  R. K. House,et al.  The Ramsay Hunt Syndrome , 1956, Neurology.

[18]  Findlay Jp Facial paralysis, a clinical review with an autopsy report of the histo-pathology in a case of infection of the geniculate ganglion by the virus of cephalic herpes zoster. , 1952 .

[19]  D. Denny-Brown,et al.  PATHOLOGIC FEATURES OF HERPES ZOSTER: A NOTE ON GENICULATE HERPES , 1944 .

[20]  J. G. Druss,et al.  GENICULATE GANGLIONITIS (HUNT'S SYNDROME): CLINICAL FEATURES AND HISTOPATHOLOGY , 1934 .

[21]  J. Hunt ON HERPETIC INFLAMMATIONS OF THE GENICULATE GANGLION. A NEW SYNDROME AND ITS COMPLICATIONS , 1907 .

[22]  J. Bethlem Herpetic geniculate ganglionitis , 2004, Acta Neuropathologica.

[23]  N. Hato,et al.  Edematous swelling of the facial nerve in Bell's palsy. , 2000, Acta oto-laryngologica.

[24]  N. Hato,et al.  Varicella-zoster virus distribution in Ramsay Hunt syndrome revealed by polymerase chain reaction. , 1998, Acta oto-laryngologica.

[25]  Y. Koike,et al.  Gd-DTPA enhanced MRI in Ramsay Hunt syndrome. , 1994, Acta oto-laryngologica. Supplementum.

[26]  H. Schuknecht,et al.  Pathological findings and surgical implications in herpes zoster oticus. , 1983, Advances in oto-rhino-laryngology.

[27]  A. Aviel,et al.  Ramsay Hunt syndrome: a cranial polyneuropathy. , 1982, American journal of otolaryngology.