Task force of the Brazilian Society of Otology - evaluation and management of peripheral facial palsy.

OBJECTIVE: To review key evidence-based recommendations for the diagnosis and treatment of peripheral facial palsy in children and adults. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on peripheral facial palsy were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 main parts: (1) Evaluation and diagnosis of facial palsy: electrophysiologic tests, idiopathic facial palsy, Ramsay Hunt syndrome, traumatic peripheral facial palsy, recurrent peripheral facial palsy, facial nerve tumors, and peripheral facial palsy in children; and (2) Rehabilitation procedures: surgical decompression of the facial nerve, facial nerve grafting, surgical treatment of long-term peripheral facial palsy, and non-surgical rehabilitation of the facial nerve. CONCLUSIONS: Peripheral facial palsy is a condition of diverse etiology. Treatment should be individualized according to the cause of facial nerve dysfunction, but the literature presents better evidence-based recommendations for systemic corticosteroid therapy.

Task force of the Brazilian Society of Otology — evaluation and management of peripheral facial palsy

Abstract Objective To review key evidence-based recommendations for the diagnosis and treatment of peripheral facial palsy in children and adults. Methods Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on peripheral facial palsy were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results The topics were divided into 2 main parts: (1) Evaluation and diagnosis of facial palsy: electrophysiologic tests, idiopathic facial palsy, Ramsay Hunt syndrome, traumatic peripheral facial palsy, recurrent peripheral facial palsy, facial nerve tumors, and peripheral facial palsy in children; and (2) Rehabilitation procedures: surgical decompression of the facial nerve, facial nerve grafting, surgical treatment of long-term peripheral facial palsy, and non-surgical rehabilitation of the facial nerve. Conclusions Peripheral facial palsy is a condition of diverse etiology. Treatment should be individualized according to the cause of facial nerve dysfunction, but the literature presents better evidence-based recommendations for systemic corticosteroid therapy.

Continuous hemicrania as the initial manifestation of Ramsay Hunt syndrome: a case report / Hemicrania contínua como manifestação inicial da síndrome de Ramsay Hunt: relato de caso

Introduction:Ramsay-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 preeruptive phase. Objective:To present an atypical case of Ramsay Hunt syndrome with continuous unilateral headache preceding the onset of other symptoms and signs of the syndrome. Case report:Report 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. Conclusion:Ramsay-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 preeruptive 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 ótico-zóster, é uma complicação rara do herpes-zóster. A síndrome é caracterizada por paralisia do nervo facial periférico e erupção vesicular eritematosa no ouvido ou na boca. Precedendo o aparecimento das vesículas, pode ocorrer mais comumente otalgia unilateral ou dor cervical. No entanto, a hemicrania persistente é pouco frequente na fase pré-eruptiva. Objetivo: Apresentar um caso atípico de síndrome de Ramsay Hunt com cefaleia unilateral contínua precedendo o aparecimento de outros sintomas e sinais da síndrome. Relato de caso: Relato de uma mulher de 69 anos que apresentou hemicrania esquerda de início subagudo, moderada a grave, sem sinais autonômicos. Oito dias após o início e manutenção contínua da cefaleia, apresentou paralisia facial periférica. Após quatro dias, notou presença de vesículas em orelha esquerda e odinofagia. Ela desenvolveu náuseas com vários episódios de vômitos e desequilíbrio grave que a impossibilitou de andar sem ajuda. Ao exame físico apresentava vesículas em orelha esquerda e orofaringe, paralisia facial periférica esquerda (grau IV de House Brackmann), hipoacusia esquerda, nistagmo e marcha vestibular. Os testes de diagnóstico para rastreio de diversas doenças metabólicas e diagnóstico de infeções (incluindo VIH) não apresentaram resultados dignos de nota. A tomografia computadorizada de cérebro e a análise do líquido cefalorraquidiano não mostraram alterações. Conclusão: A síndrome de Ramsay-Hunt envolve principalmente os nervos facial e vestibulococlear, causando paralisia facial periférica, otalgia, hipoacusia e, menos frequentemente, desequilíbrio. Embora a dor seja uma manifestação frequente da fase pré-eruptiva da SHI, a cefaleia unilateral não é comum neste cenário. Por outro lado, é uma queixa prevalente no serviço de urgência e tem diversas etiologias. Portanto, diagnosticar SHI quando os pacientes apresentam dores de cabeça exclusivamente unilaterais é um desafio para os médicos. Os médicos devem considerar a SHI um diagnóstico diferencial vital de cefaleias laterais, evitando erros de diagnóstico e atrasos no tratamento.

Síndrome de Ramsay Hunt: revisión narrativa / Ramsay Hunt Syndrome: Narrative Review

El síndrome de Ramsay Hunt (SRH) corresponde a la asociación de la parálisis facial periférica con una erupción vesicular localizada en el pabellón auricular, causada por el compromiso del ganglio geniculado secundario a una infección por el virus de la varicela-zóster (VVZ). Este síndrome es la segunda causa más común de parálisis facial atraumática y representa aproximadamente el 10 %-12 % de las parálisis faciales agudas, con una incidencia anual de 5 por cada 100 000 habitantes en Estados Unidos. El diagnóstico es principalmente clínico y entre las manifestaciones más destacadas se encuentran síntomas neurológicos como otalgia, tinnitus, hipoacusia asociada con parálisis facial junto a lesiones herpéticas características. Dentro de las complicaciones que se pueden presentar en esta entidad se encuentra, principalmente, la neuralgia posherpética, seguida de otras menos frecuentes como la encefalitis, el herpes zóster oftálmico y la mielitis. El manejo actual del SRH se basa en la aplicación de terapias duales con corticosteroides asociados a terapia antiviral, lo cual ha demostrado que el inicio temprano del tratamiento mejora el pronóstico y disminuye la aparición de complicaciones. El pronóstico de esta patología es inferior en comparación a patologías menos severas que comprometen el nervio facial (como la parálisis de Bell) y se ve impactado por varios factores como el inicio oportuno de tratamiento, el grupo etario y la presencia de comorbilidades.

Ramsay Hunt syndrome corresponds to the association of peripheral facial paralysis with a vesicular eruption located in the pinna, caused by the involvement of the geniculate ganglion secondary to infection by the varicella zoster virus. This syndrome is the second causes of atraumatic facial paralysis, representing approximately 10 %-12 % of acute facial paralysis, with an annual incidence of 5 per 100,000 inhabitants. The diagnosis is mainly clinical and among the most prominent manifestations are neurological symptoms such as otalgia, tinnitus, hypoacusis associated with facial paralysis together with characteristic herpetic lesions. Among the complications that may occur in this entity is mainly postherpetic neuralgia, followed by less frequent ones such as encephalitis, ophthalmic herpes zoster and myelitis. Current management of Ramsay Hunt syndrome is based on the application of dual therapies consisting of corticosteroids associated with antiviral therapy, showing that early initiation of treatment improves prognosis and reduces the appearance of complications. The prognosis of this pathology is inferior compared to less severe pathologies that compromise the facial nerve (Bell's palsy) and is impacted by several factors such as the timely initiation of treatment, the age group, and the presence of comorbidities.

Facial paralysis due to Ramsay Hunt syndrome - A rare condition / Paralisia facial secundária à síndrome de Ramsay Hunt - Uma condição rara

Summary Ramsay Hunt syndrome (or herpes zoster oticus) is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion occurs. Usually, there are auricular vesicles and symptoms and signs such otalgia and peripheral facial paralysis. In addition, rarely, a rash around the mouth can be seen. Immunodeficient patients are more susceptible to this condition. Diagnosis is essentially based on symptoms. We report the case of a diabetic female patient who sought the emergency department with a complaint of this rare entity.

Resumo A síndrome de Ramsay Hunt (ou zóster auricular) é uma complicação rara do herpes-zóster em que ocorre reativação de uma infecção latente pelo vírus varicela-zóster no gânglio geniculado. Geralmente, estão presentes vesículas auriculares e sintomas como otalgia e paralisia facial periférica. Além disso, mais raramente pode haver rash ao redor da boca. Pacientes com imunodeficiência apresentam maior susceptibilidade para essa condição. O diagnóstico é essencialmente pelo quadro clínico. É apresentado o caso de uma paciente diabética que compareceu ao setor de emergência com essa manifestação rara.