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Ramsay Hunt syndrome: A case report with cranial nerve XII involvement

2007; Elsevier BV; Volume: 57; Issue: 6 Linguagem: Inglês

10.1016/j.jaad.2006.07.020

1097-6787

Allan K. Izumi, Kory H. Kitagawa,

Orthopedic Surgery and Rehabilitation

To the Editor: A 72-year-old white male presented with headaches. He developed pain in his left ear and consulted an otolaryngologist who considered an otitis infection or herpes zoster. He was treated with corticosporin otic suspension and benzocaine/antipyrine otic drops with minimal improvement. He subsequently developed small vesicles in the concha of the auricle of his left ear, hearing loss, and tinnitus. An audiogram revealed bilateral high frequency hearing loss with the left side more affected than the right. Magnetic resonance imaging revealed no signs of tumor or infection. He was treated with methylprednisolone and acyclovir. Three months later, he continued to complain of pain in his left ear and the onset of slurred speech. Physical examination revealed deviation of the tongue to the left with left hemiatrophy of the tongue (Fig 1). A neurological consult confirmed chronic tinnitus and hearing loss of the left ear, left tongue atrophy, deviation of the tongue to the ipsilateral side, and mild left facial paresis. Serology results for herpes simplex virus 1 and 2 were positive for IgG antibodies and negative for IgM antibodies. Varicella-zoster virus (VZV) IgG antibodies were positive, but IgM antibodies were negative. A repeat magnetic resonance imaging scan failed to show an infarct, tumor, or infection. He was started on famciclovir and prednisone with minimal improvement.Herpes zoster oticus (Ramsay Hunt syndrome) was described by James Ramsay Hunt in 1907.1Hunt J.R. On herpetic inflammations of the geniculate ganglion: a new syndrome and its complications.J Nerv Ment Dis. 1907; 34: 73-96Crossref Scopus (302) Google Scholar He concluded that the syndrome resulted from a reactivation of VZV in the geniculate ganglion, which was later confirmed by polymerase chain reaction studies demonstrating the presence of VZV DNA in the geniculate ganglion.2Van de Steene V. Kuhweide R. Vlaminck S. Casselman J. Varicella zoster virus: beyond facial paralysis.Acta Otorhinolaryngol Belg. 2004; 58: 61-66PubMed Google Scholar Originally, Hunt described otalgia, cutaneous and mucosal lesions, and facial paresis involving cranial nerve (CN) VII (the facial nerve). Subsequently, he described involvement of CN VIII (the vestibulocochlear nerve) with hearing loss, tinnitus, vertigo, and nystagmus.The brainstem contains the nuclei for CNs III through XII, which are in close anatomical proximity. Ramsay Hunt syndrome is a common expression of two CN nuclei (VII and VIII) which are adjacent to each other. Other examples of Ramsay Hunt syndrome associated with adjacent CN nuclei include reports involving CNs V, VI, IX, and X.3Shah R.K. Blevins N.H. Otalgia.Otolaryngol Clin North Am. 2003; 36: 1137-1151Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar There is only one other reported case of Ramsay Hunt syndrome associated with CN XII.4Xanthopoulos J. Noussios G. Papaioannides D. Exarchakos G. Assimakopoulos D. Ramsay Hunt syndrome presenting as a cranial polyneuropathy.Acta Otorhinolaryngol Belg. 2002; 56: 319-323PubMed Google Scholar In that case report, CNs V and IX were also involved. Our case represents Ramsay Hunt syndrome associated with CN XII involvement, which is unique but not anatomically unexpected.There are two explanations for multiple CN involvement. Ramsay Hunt proposed that inflammation of a single ganglion could involve surrounding ganglia by contiguous anatomical contact. Others feel that cranial polyneuropathies occur by the spread of a virus through a common blood supply, including small branches of the carotid artery.5Sweeney C.J. Gilden D.H. Ramsay Hunt syndrome.J Neurol Neurosurg Psychiatry. 2001; 71: 149-154Crossref PubMed Scopus (331) Google Scholar This case demonstrates the importance of recognizing the possible manifestations of Ramsay Hunt syndrome as noted by our patient with otalgia, vesicles in the concha, facial paresis (CN VII), hearing loss, tinnitus (CN VIII) and slurred speech with hemiatrophy and ipsilateral deviation of the tongue (CN XII). To the Editor: A 72-year-old white male presented with headaches. He developed pain in his left ear and consulted an otolaryngologist who considered an otitis infection or herpes zoster. He was treated with corticosporin otic suspension and benzocaine/antipyrine otic drops with minimal improvement. He subsequently developed small vesicles in the concha of the auricle of his left ear, hearing loss, and tinnitus. An audiogram revealed bilateral high frequency hearing loss with the left side more affected than the right. Magnetic resonance imaging revealed no signs of tumor or infection. He was treated with methylprednisolone and acyclovir. Three months later, he continued to complain of pain in his left ear and the onset of slurred speech. Physical examination revealed deviation of the tongue to the left with left hemiatrophy of the tongue (Fig 1). A neurological consult confirmed chronic tinnitus and hearing loss of the left ear, left tongue atrophy, deviation of the tongue to the ipsilateral side, and mild left facial paresis. Serology results for herpes simplex virus 1 and 2 were positive for IgG antibodies and negative for IgM antibodies. Varicella-zoster virus (VZV) IgG antibodies were positive, but IgM antibodies were negative. A repeat magnetic resonance imaging scan failed to show an infarct, tumor, or infection. He was started on famciclovir and prednisone with minimal improvement. Herpes zoster oticus (Ramsay Hunt syndrome) was described by James Ramsay Hunt in 1907.1Hunt J.R. On herpetic inflammations of the geniculate ganglion: a new syndrome and its complications.J Nerv Ment Dis. 1907; 34: 73-96Crossref Scopus (302) Google Scholar He concluded that the syndrome resulted from a reactivation of VZV in the geniculate ganglion, which was later confirmed by polymerase chain reaction studies demonstrating the presence of VZV DNA in the geniculate ganglion.2Van de Steene V. Kuhweide R. Vlaminck S. Casselman J. Varicella zoster virus: beyond facial paralysis.Acta Otorhinolaryngol Belg. 2004; 58: 61-66PubMed Google Scholar Originally, Hunt described otalgia, cutaneous and mucosal lesions, and facial paresis involving cranial nerve (CN) VII (the facial nerve). Subsequently, he described involvement of CN VIII (the vestibulocochlear nerve) with hearing loss, tinnitus, vertigo, and nystagmus. The brainstem contains the nuclei for CNs III through XII, which are in close anatomical proximity. Ramsay Hunt syndrome is a common expression of two CN nuclei (VII and VIII) which are adjacent to each other. Other examples of Ramsay Hunt syndrome associated with adjacent CN nuclei include reports involving CNs V, VI, IX, and X.3Shah R.K. Blevins N.H. Otalgia.Otolaryngol Clin North Am. 2003; 36: 1137-1151Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar There is only one other reported case of Ramsay Hunt syndrome associated with CN XII.4Xanthopoulos J. Noussios G. Papaioannides D. Exarchakos G. Assimakopoulos D. Ramsay Hunt syndrome presenting as a cranial polyneuropathy.Acta Otorhinolaryngol Belg. 2002; 56: 319-323PubMed Google Scholar In that case report, CNs V and IX were also involved. Our case represents Ramsay Hunt syndrome associated with CN XII involvement, which is unique but not anatomically unexpected. There are two explanations for multiple CN involvement. Ramsay Hunt proposed that inflammation of a single ganglion could involve surrounding ganglia by contiguous anatomical contact. Others feel that cranial polyneuropathies occur by the spread of a virus through a common blood supply, including small branches of the carotid artery.5Sweeney C.J. Gilden D.H. Ramsay Hunt syndrome.J Neurol Neurosurg Psychiatry. 2001; 71: 149-154Crossref PubMed Scopus (331) Google Scholar This case demonstrates the importance of recognizing the possible manifestations of Ramsay Hunt syndrome as noted by our patient with otalgia, vesicles in the concha, facial paresis (CN VII), hearing loss, tinnitus (CN VIII) and slurred speech with hemiatrophy and ipsilateral deviation of the tongue (CN XII).