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QJM: An International Journal of Medicine, 2016, 693

doi: 10.1093/qjmed/hcw113
Advance Access Publication Date: 6 August 2016
Clinical picture

CLINICAL PICTURE

Ramsay Hunt syndrome


A 78-year-old female presented at an outside hospital with
complaints of drooping of the mouth on left side and dehydra-
tion due to inability to eat. On examination patient was noticed
to have sagging left eyebrow, inability to close the left eye, ab-
sent left nasolabial fold and drooping of the mouth on the left
side. A clinical diagnosis of Bell’s palsy was made and patient
was discharged on a 5-day oral prednisone taper.
After 1 week, the patient presented to our hospital with no
improvement in facial weakness (Figure 1a) and a new vesicular
rash affecting the left external ear and ipsilateral hard palate
and anterior two-thirds of the tongue (Figure 1b). Patient was
diagnosed with House-Brackman grade V facial palsy and a clin-
ical diagnosis of Ramsay-Hunt syndrome (RHS) was made.
Patient was started on a treatment with prednisone 60 mg/day
and valacyclovir 1000 mg/three times a day for 1 week. Patient
also required a temporary Tarsorrhaphy due to complete inabil-
ity to close the eye.

Discussion
The pathogenesis of RHS involves the reactivation of latent vari-
cella zoster virus (VZV) in the geniculate ganglion of the facial
nerve.1 About 12% of all peripheral facial nerve palsies are
caused by VZV.2 Pathognomonic for the syndrome are periph-
eral facial nerve palsy and vesicular rash on the external ear
and/or the mucosa of the ipsilateral part of the tongue or palate.
The RHS diagnosis is purely clinical, but in some cases a blood
test for VZV antibodies may be useful. When compared with
Bell’s palsy (facial paralysis without rash), patients with RHS Figure 1. (a) Left-sided facial palsy. (b) Vesicular rash affecting external ear,
often have more severe paralysis at onset and are less likely to tongue and palate on the left side of the face.

recover completely. Studies suggest that treatment with pred-


nisone and antiviral agents may improve outcome, although a References
prospective randomized treatment trial remains to be under-
taken.1,3 For persons of 60 years or older, vaccinating them 1. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol
against VZV effectively reduces the risk of Herpes zooster and Neurosurg Psychiatry 2001; 71:149–54.
post-herpetic pain.4 2. Peitersen E. Bell’s palsy: the spontaneous course of 2,500 per-
ipheral facial nerve palsies of different etiologies. Acta
Otolaryngol Suppl 2002; 549:4–30.
Photographs and text from: N.M. Gupta and M.P. Parikh,
3. Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara
Division of Hospital Medicine, Cleveland Clinic Foundation,
N. Treatment of Ramsay Hunt syndrome with acyclovir-
9500 Euclid Ave, Cleveland, OH 44114, USA; S. Panginikkod and
prednisone: significance of early diagnosis and treatment. Ann
V. Gopalakrishnan, Division of Internal Medicine, Presence
Neurol 1997; 41:353–7.
Saint Francis Hospital, 355 Ridge Ave, Evanston, IL 60602, USA.
4. Rasmussen ER, Lykke E, Toft JG, Mey K. Ramsay Hunt syn-
email: niyati_gupta@yahoo.com.
drome revisited–emphasis on Ramsay Hunt syndrome with
Conflict of interest: None declared. multiple cranial nerve involvement. Virol Discov 2014; 2:1.

C The Author 2016. Published by Oxford University Press on behalf of the Association of Physicians.
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