Pediatric Neurology 52 (2015) 554e555

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Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu

Visual Diagnosis

Ramsay Hunt Syndrome Enrico Valerio MD a, *, Mario Cutrone MD b, Chiara Gentilomo MD c, Eugenio Baraldi MD a, Enrico Ravagnan MD c a

Department of Woman and Child Health, Medical School, University of Padua, Padova, Italy Department of Pediatric Dermatology, Ospedale Dell’Angelo, Mestre, VE, Italy c Department of Pediatric Neurology, Ospedale Dell’Angelo, Mestre, VE, Italy b

Patient Description A 14-year-old boy developed Ramsay Hunt syndrome after varicellazoster virus infection. He experienced right earache and palatal pain. Two days later, a whitish, exuding vesiculobullous rash developed on the right hard palate (Figure) along with swelling and erythema of the right ear canal, similarly exuding and covered with vesicles (Figure). A right peripheral facial palsy (Figure) was also evident. High-dose antiviral (acyclovir) and corticosteroid (prednisone) therapy was started, with remission of fever and slow improvement of vesicobullous lesions. Right facial palsy and slight right hypoacusis persisted at the time of discharge.

Discussion

In early twentieth century, James Ramsay Hunt described herpetiformis lesions in the oral mucosa and/or ear canal in association with various neurological symptoms. Almost 10% of all peripheral facial nerve palsies occur in children, and idiopathic Bell’s palsy accounts for 24% to 70% of these. Varicella-zoster reactivation with Ramsay Hunt syndrome is responsible for 16.7% of the children with facial paralysis.1 Varicella-zoster virus leads to hospitalization in 1% of affected children, of whom 9.5% experience Ramsay Hunt syndrome as a complication, with a two-fold risk ratio for immunocompetent (10%) versus immunocompromised (5%) patients. When a diagnosis of Ramsay Hunt syndrome from varicella-zoster virus reactivation is made, either clinically or by laboratory confirmation, timely (within 3 days of symptom onset) antiviral therapy with acyclovir should be started (oral: 80 mg/kg/day; intravenous: 45 mg/kg/day) to combat further viral replication and spread. Corticosteroid therapy (prednisone) helps to reduce inflammation and edema when paralysis has * Communications should be addressed to: Dr. Valerio; Department of Woman and Child Health; Medical School; University of Padua; Via Giustiniani, 3; 35128 Padova, Italy. E-mail addresses: [email protected], [email protected] 0887-8994/$ e see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2015.01.017

FIGURE. At the time of admission, a whitish, exuding vesiculobullous rash was evident in the right ear canal and right hard palate (insert). He also had a right peripheral facial palsy (lower pictures). (The color version of this figure is available in the online edition).

E. Valerio et al. / Pediatric Neurology 52 (2015) 554e555

occurred but corticosteroids should be reserved for more severely affected patients and always administered in conjunction with antiviral treatment.2 The incidence of herpes zoster seems to be lower in children who receive the varicella vaccine than in those who contract varicella infection.3 Both in adults and children, Ramsay Hunt syndrome has a worse prognosis for complete recovery of facial nerve function than idiopathic facial palsy. Individuals with Ramsay Hunt syndrome whose initial electromyographic pattern is more severely abnormal (i.e., affected side neural response