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Ramsay Hunt syndrome Martha R Neagu, Sashank Prasad Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA Correspondence to Dr Sashank Prasad, Department of Neurology, Brigham and Women’s Hospital and Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA; [email protected] Accepted 15 January 2016
A 66-year-old man presented with complete right facial paralysis (figure 1). Three days before, he had experienced a burning sensation in the right ear and developed oral lesions. On examination, there was a peripheral right facial palsy with hyperacusis and loss of taste sensation. He had herpetic vesicles on the right anterior tongue and palate (figure 1). We diagnosed Ramsay Hunt syndrome. Following treatment with valacyclovir and corticosteroids, he made a near-complete recovery. The Ramsay Hunt syndrome describes peripheral facial palsy with an accompanying ipsilateral vesicular rash on the tongue, palate or ear.1 It is attributed to reactivation of the varicella zoster virus.2 To understand the facial nerve’s pattern of cutaneous sensory innervation and taste sensation, Hunt used what he called the ‘herpes zoster method’, carefully analysing the distribution of vesicular eruption in cases of zoster-associated facial palsy.3 He concluded, ‘That portion of the facial sensory system which still persists in man is concerned in the innervation of the internal ear, the middle ear, its prolongations into the Eustachian tube and mastoid cells, and the skin on certain protuberances of the external ear. There is also evidence that a vestigial remnant occasionally participates in the innervation of a strip on the postero-medial surface of the auricle and within the buccal cavity, in the chorda tympani distribution and on the palate near the anterior pillar of the fauces’.
Thus, herpetic involvement of the facial nerve may make vesicles visible around the external ear and on the anterior two-thirds of the tongue and the soft palate. In patients with facial palsy, clinicians should examine the mouth and ear for a vesicular rash to distinguish this entity from idiopathic Bell’s palsy. Contributors MRN provided clinical care and drafted the manuscript. SP provided mentorship and revised the manuscript for intellectual content. Competing interests None declared. Provenance and peer review Not commissioned; externally peer reviewed. Referee’s commentary Neagu and Prasad provide a neat and valuable lesson in facial nerve anatomy, based on the distribution of the rash in a patient with a lower motor neurone facial nerve palsy associated with herpes zoster infection. The viral infection elegantly uncovers areas, not routinely tested on clinical examination, as belonging to the boundary zones of the involved facial nerve. This is a compelling example of a treatable disease briefly delineating, for observant clinicians, the normal but little known anatomy of an otherwise familiar nerve. Tom Hughes, Cardiff, UK Tom. [email protected]
REFERENCES 1 Hunt JR. On herpetic inflammations of the geniculate ganglion. A new syndrome and its complications. J Nerv Ment Dis 1907;34:73–96. 2 Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 2001;71:149–54. 3 Hunt JR. The sensory field of the facial nerve: a further contribution to the symptomatology of the geniculate ganglion. Brain 1915;38:418–46.
▸ http://dx.doi.org/10.1136/ practneurol-2015-001304
To cite: Neagu MR, Prasad S. Pract Neurol Published Online First: [ please include Day Month Year] doi:10.1136/ practneurol-2015-001303
Figure 1 Ramsay Hunt Syndrome. (A) Complete right facial palsy. (B) Vesicular rash on tongue. (C) Vesicular rash on the palate. Neagu MR, Prasad S. Pract Neurol 2016;0:1. doi:10.1136/practneurol-2015-001303
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Ramsay Hunt syndrome Martha R Neagu, Sashank Prasad Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA Correspondence to Dr Sashank Prasad, Department of Neurology, Brigham and Women’s Hospital and Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA; [email protected] Accepted 15 January 2016
A 66-year-old man presented with complete right facial paralysis (figure 1). Three days before, he had experienced a burning sensation in the right ear and developed oral lesions. On examination, there was a peripheral right facial palsy with hyperacusis and loss of taste sensation. He had herpetic vesicles on the right anterior tongue and palate (figure 1). We diagnosed Ramsay Hunt syndrome. Following treatment with valacyclovir and corticosteroids, he made a near-complete recovery. The Ramsay Hunt syndrome describes peripheral facial palsy with an accompanying ipsilateral vesicular rash on the tongue, palate or ear.1 It is attributed to reactivation of the varicella zoster virus.2 To understand the facial nerve’s pattern of cutaneous sensory innervation and taste sensation, Hunt used what he called the ‘herpes zoster method’, carefully analysing the distribution of vesicular eruption in cases of zoster-associated facial palsy.3 He concluded, ‘That portion of the facial sensory system which still persists in man is concerned in the innervation of the internal ear, the middle ear, its prolongations into the Eustachian tube and mastoid cells, and the skin on certain protuberances of the external ear. There is also evidence that a vestigial remnant occasionally participates in the innervation of a strip on the postero-medial surface of the auricle and within the buccal cavity, in the chorda tympani distribution and on the palate near the anterior pillar of the fauces’.
Thus, herpetic involvement of the facial nerve may make vesicles visible around the external ear and on the anterior two-thirds of the tongue and the soft palate. In patients with facial palsy, clinicians should examine the mouth and ear for a vesicular rash to distinguish this entity from idiopathic Bell’s palsy. Contributors MRN provided clinical care and drafted the manuscript. SP provided mentorship and revised the manuscript for intellectual content. Competing interests None declared. Provenance and peer review Not commissioned; externally peer reviewed. Referee’s commentary Neagu and Prasad provide a neat and valuable lesson in facial nerve anatomy, based on the distribution of the rash in a patient with a lower motor neurone facial nerve palsy associated with herpes zoster infection. The viral infection elegantly uncovers areas, not routinely tested on clinical examination, as belonging to the boundary zones of the involved facial nerve. This is a compelling example of a treatable disease briefly delineating, for observant clinicians, the normal but little known anatomy of an otherwise familiar nerve. Tom Hughes, Cardiff, UK Tom. [email protected]
REFERENCES 1 Hunt JR. On herpetic inflammations of the geniculate ganglion. A new syndrome and its complications. J Nerv Ment Dis 1907;34:73–96. 2 Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 2001;71:149–54. 3 Hunt JR. The sensory field of the facial nerve: a further contribution to the symptomatology of the geniculate ganglion. Brain 1915;38:418–46.
▸ http://dx.doi.org/10.1136/ practneurol-2015-001304
To cite: Neagu MR, Prasad S. Pract Neurol Published Online First: [ please include Day Month Year] doi:10.1136/ practneurol-2015-001303
Figure 1 Ramsay Hunt Syndrome. (A) Complete right facial palsy. (B) Vesicular rash on tongue. (C) Vesicular rash on the palate. Neagu MR, Prasad S. Pract Neurol 2016;0:1. doi:10.1136/practneurol-2015-001303
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