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.

Parálisis facial neonatal: identificación del virus del herpes simple 1 en el líquido cefalorraquídeo. Caso clínico / Neonatal facial palsy: Identification of herpes simplex virus 1 in cerebrospinal fluid. Case report

En los neonatos, la parálisis facial es muy infrecuente y, por lo general, diagnosticada al nacer. Se presenta el primer caso de parálisis facial neonatal con identificación del virus del herpes simple 1 en el líquido cefalorraquídeo. Un varón de 35 días de vida acudió a Urgencias por la desviación de la comisura bucal hacia la izquierda y la ausencia de cierre del ojo derecho, sin sintomatología infecciosa ni antecedentes relevantes. La exploración física fue compatible con parálisis facial periférica. Las exploraciones complementarias de urgencia (hemograma, bioquímica, coagulación y citoquímica de líquido cefalorraquídeo) fueron normales. Fue ingresado con prednisolona oral y aciclovir intravenoso. La resonancia magnética craneal fue normal. A las 48 horas, se recibió el resultado positivo de la reacción en cadena de la polimerasa para el virus del herpes simple 1 en el líquido cefalorraquídeo. Con evolución favorable, completó 7 días de prednisolona oral y fue dado de alta tras 21 días de aciclovir intravenoso, con exploración neurológica previa normal.

Neonatal facial palsy is very uncommon and is generally diagnosed at birth. We present the first published case of neonatal facial palsy with identification of herpes simplex virus 1 in cerebrospinal fluid. A 35-day-old male was presented at the Emergency Department with mouth deviation to the left and impossibility of full closure of the right eye. There were no symptoms of infection or relevant medical history. Physical examination was compatible with peripheral facial palsy. Studies performed at admission were normal (blood count, biochemical analysis and coagulation blood tests and cerebrospinal fluid analysis). The patient was admitted on oral prednisolone and intravenous aciclovir. Cranial magnetic resonance was normal. Polymerase chain reaction test for herpes simplex virus 1 in cerebrospinal fluid was reported positive after 48 hours of admission. Patient followed good evolution and received prednisolone for 7 days and acyclovir for 21 days. At discharge, neurological examination was normal.

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.

Ultraestrutura do nervo facial intratemporal em pacientes com paralisia facial idiopática: estudo de evidências de infecção viral / Intratemporal facial nerve ultrastructure in patients with idiopathic facial paralysis: viral infection evidence study

A etiologia da paralisia facial periférica idiopática (PFPI) ainda é uma incógnita, no entanto, alguns autores aventam a possibilidade de ser uma infecção viral. OBJETIVO: Analisar a ultraestrutura do nervo facial procurando evidências virais que possam nos fornecer dados etiológicos. MATERIAL E MÉTODO: Foram estudados 20 pacientes com PFP, com graus de moderado a severo, de ambos os sexos, entre 18-60 anos, provenientes de Ambulatório de Distúrbios do Nervo Facial. Os pacientes foram divididos em dois grupos: Estudo, onze pacientes com PFPI e Controle, nove pacientes com Paralisia Facial Periférica Traumática ou Tumoral. Foram estudados fragmentos de bainha do nervo facial ou fragmentos de seus cotos, que durante a cirurgia de reparação do nervo facial, seriam desprezados ou encaminhados para estudo anatomopatológico. O tecido foi fixado em glutaraldeído 2 por cento e analisado em Microscopia Eletrônica de Transmissão. RESULTADO: Observamos no grupo estudo atividade celular intensa de reparação com aumento de fibras colágenas, fibroblastos com organelas desenvolvidas, isentos de partículas virais. No grupo controle esta atividade de reparação não foi evidente, mas também não foram observadas partículas virais. CONCLUSÃO: Não foram encontradas partículas virais, no entanto, houve evidências de intensa atividade de reparação ou infecção viral.

The etiology of idiopathic peripheral facial palsy (IPFP) is still uncertain; however, some authors suggest the possibility of a viral infection. AIM: to analyze the ultrastructure of the facial nerve seeking viral evidences that might provide etiological data. MATERIAL AND METHODS: We studied 20 patients with peripheral facial palsy (PFP), with moderate to severe FP, of both genders, between 18-60 years of age, from the Clinic of Facial Nerve Disorders. The patients were broken down into two groups - Study: eleven patients with IPFP and Control: nine patients with trauma or tumor-related PFP. The fragments were obtained from the facial nerve sheath or from fragments of its stumps - which would be discarded or sent to pathology exam during the facial nerve repair surgery. The removed tissue was fixed in 2 percent glutaraldehyde, and studied under Electronic Transmission Microscopy. RESULTS: In the study group we observed an intense repair cellular activity by increased collagen fibers, fibroblasts containing developed organelles, free of viral particles. In the control group this repair activity was not evident, but no viral particles were observed. CONCLUSION: There were no viral particles, and there were evidences of intense activity of repair or viral infection.

Rare adverse events associated with oral poliovirus vaccine in Brazil

Oral poliovirus vaccine (OPV) developed by A. Sabin has been effectively used to control poliomyelitis in Brazil, and the last case with the isolation of a wild poliovirus strain occourred in March 1989. Although the vaccine controlled the circulation ...

Some immunological studies in cases of idiopathic facial palsy [Bell's palsy]

A prospective clinical and immunological study was performed on 20 consecutive Bell's palsy patients in addition to 10 normal controls of matched age and sex, to study some immunological changes that might occur in the blood and sera of the patients. They were subjected to thorough clinical neurological and medical examinations, complete blood picture, Erythrocyte sedimentation rate, glucose tolerance curve and immunological investigations that include estimation of-T-and-B lymphocytes percentage of the peripheral blood. Estimation of serum levels of immunoglobulins [IgG, IgA and IgM] and complement C[3] and C[4]. The immunological investigations were done during the acute phase of the disease [1-7 days] and after 4 weeks from the first investigations and at least 2 weeks after stopping treatment. The study revealed a significant decrease in the peripheral blood -T- lymphocyte percentage as well as an increase in -B-lymphocyte percentage [P < 0.001] in cases of Bell's palsy patients during the acute phase of the illness with a slow return to normal values which began after 4 weeks from the onset. A highly significant positive correlation was found between the increase in-B-lymphocyte percentage and the levels of immunoglobulin IgM during the acute phase of the disease. The clinical and immunological data of Bell's palsy show a similar pattern to those of several demyelinating disease such as in acute exacerbations of multiple sclerosis and the acute stage of Guillian-Barre syndrome and also similar to those found during the clinical course of many viral infections suggesting that Bell's palsy may be an autoimmune demyelinating neuropathy of the facial nerve which may be caused by a preceding viral infection