IntroductionRamsay-Hunt syndrome, also called otic zoster, is a rare complication of herpes zoster. The syndrome is characterized by peripheral facial nerve palsy and an erythematous vesicular eruption in the ear or mouth. Preceding the appearance of the vesicles, unilateral otalgia or neck pain may occur more commonly. However, persistent hemicrania is infrequent in the pre-eruptive phase.ObjectivesTo present an atypical case of Ramsay Hunt syndrome with continuous unilateral headache preceding the onset of other symptoms and signs of the syndrome.Case reportReport of a 69-year-old woman who presented subacute onset of moderate to severe left hemicrania with no autonomic signs. Eight days after the start and continuous headache maintenance, she presented with peripheral facial paralysis. After four days, she noticed the presence of vesicles in the left ear and odynophagia. She developed nausea with several episodes of vomiting and severe imbalance that made it impossible for her to walk unassisted. On physical examination, she presented vesicles in the left ear and oropharynx, left peripheral facial palsy (House Brackmann grade IV), left hypoacusis, nystagmus, and vestibular gait. Diagnostic tests for screening several metabolic diseases and diagnosis of infection (including HIV) were unremarkable. Brain computed tomography and cerebrospinal fluid analysis showed no abnormalities.ConclusionsRamsay-Hunt syndrome mainly involves the facial and vestibulocochlear nerves, causing peripheral facial palsy, otalgia, hypoacusis, and, less frequently, imbalance. Although pain is a frequent manifestation of the pre-eruptive phase of RHS, unilateral headache is not common in this scenario. On the other hand, it is a prevalent complaint in the emergency department and has several different etiologies. Hence, diagnosing RHS when patients present exclusively unilateral headaches is challenging for clinicians. Physicians must consider RHS a vital differential diagnosis of sided-locked headaches, avoiding diagnostic errors and treatment delays.
Introdução A síndrome de Ramsay-Hunt também chamada de zoster otico é uma complicação rara do herpes-zóster em que ocorre reativação de uma infecção latente pelo vírus varicela-zóster no gânglio geniculado. A síndrome é caracterizada por paralisia do nervo facial periférico acompanhada por uma erupção vesicular eritematosa na orelha ou na boca. É a segunda causa mais comum de paralisia facial periférica não traumática. Objetivos Apresentar um caso atípico da síndrome de Ramsay Hunt com presença de cefaleia. Material e Métodos Relato de uma mulher de 69 anos que apresentou início subagudo de hemicrania à esquerda de intensidade moderada a forte sem sinais autonômicos. Oito dias após o início da cefaleia, ela apresentou paralisia facial periférica e após 4 dias, notou o aparecimento de vesículas no lado esquerdo do ouvido e dor ao engolir. Ela desenvolveu náusea com vários episódios de vômito e desequilíbrio importante que tornava a marcha impossível. Resultados No exame físico ela apresentou vesículas na orelha esquerda e orofaringe, paralisia facial periférica esquerda (House Brackmann grau IV), hipoacusia esquerda, nistagmo e marcha vestibular. A tomografia do cérebro e a análise do líquido cefalorraquidiano não mostraram anormalidades. Conclusões A infecção envolve principalmente os nervos facial e vestibulococlear, causando paralisia facial periférica, otalgia e perda auditiva. Os sintomas vestibulares são raros e o início da síndrome com cefaleia persistente, como relatado, é extremamente atípico.
Context: Ramsay Hunt Syndrome (RHS), also named Optic Herpes Zoster is caused by the reactivation of Varicella-Zoster Virus that occurs in the geniculate ganglion. The classic syndrome is composed of vesicular lesions in the ear, ipsilateral otalgia and peripheral facial palsy. The onset of the syndrome with persistent hemicrania is absolutely atypical as a manifestation of RHS. Case report: A 69 year old woman with subacute onset of left exclusive hemicrania in moderate strong intensity without autonomic signs. Eight days after the onset of headache, she presented left peripheral facial paralysis and after 4 days, she noticed the appearance of vesicles in the left ear and pain in swallowing. She has developed nausea with several episodes of vomiting, important imbalance that made impossible gait. On physical examination she presented vesicles in the left ear and oropharynx, left peripheral facial paralysis House Brackmann grade IV, left hypoacusis, nystagmus and vestibular gait. The brain CT and cerebrospinal fluid analysis were normal. The patient has progressive improvement with Acyclovir. Conclusions: Otalgia or even intermediate neuralgia are more frequent painful manifestations of RHS. This case report highlights the need to consider the diagnosis of SRH in patients with persistent hemicrania, even if without initial classic signs of the syndrome.
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