Melkersson-rosenthal: reporte de caso clínico de una neurodermatosis infrecuente / Melkersson-rosenthal syndrome: case report of an uncommon neurodermatosis

Resumen: El síndrome de Melkersson-Rosenthal (SMR) es una entidad clínica rara, de patogénesis desconocida. Se manifiesta característicamente por edema orofacial recidivante, lengua fisurada y parálisis recurrente del nervio facial. Representando así undesafío diagnóstico y terapéutico, además de generar importante compromiso social al individuo acometido. El presente artículo tiene como objetivo describir el caso de un paciente de 15años de edad que presentó: edema labial, lengua fisurada y queilitis granulomatosa al examen histopatológico, llevándose a consideración la hipótesis del síndrome citado, con resultados satisfactorios al tratamiento establecido.

Abstract: Melkersson-Rosenthal syndrome (MRS) is a rare clinical entity with an unknown pathogenesis. It clinically manifests in orofacial edema, plicated tongue and recurrent paralysis of the facial nerve. It represents a diagnostic and therapeutic challenge, and has an important psycosocial impact on the affected individual. This study describes the case of a 15-year-old patient who presented with labial edema, plicated tongue and granulomatous cheilitis on histopathological examination, for which a diagnosis of MRS was proposed. The patient showed a good response to treatment.

Parotidectomy-related facial nerve lesions: proposal for a modified Sunnybrook Facial Grading System / Lesões do nervo facial relacionadas à parotidectomia: proposta de um sistema Sunnybrook de graduação facial modificado

ABSTRACT Facial nerve injury, affecting mainly the marginal mandibular branch, is the most frequent neurologic complication from parotidectomy. Objective To test a modified Sunnybrook Facial Grading System as a new tool to assess the facial nerve function following parotidectomy, emphasizing the marginal mandibular branch. Methods We reviewed the medical records of 73 post-parotidectomy patients (40 female, 18-84 years old, mean age 53.2 years) with facial nerve sparing, referred to the Department of Physical Therapy. All patients had parotid neoplasms or advanced skin cancer, and were followed by the principal author between 2006 and 2014. Results The muscles innervated by the marginal mandibular branch were the most frequently affected (72.6%), particularly in patients undergoing neck dissection (p = 0.023). The voluntary movement scores obtained with the modified system were significantly lower compared with the original version (p < 0.001). The best and worst scores were observed in patients with benign parotid tumors and skin cancer, respectively. Patients requiring neck dissection (p = 0.031) and resection of other structures (p = 0.021) had the lowest scores, evidenced only with the modified version. Patients with malignant tumors had significantly worse ratings, regardless of the Sunnybrook system version. The post-physiotherapy analysis involved 50 patients. The worst facial rehabilitation outcomes were related to the marginal mandibular branch function. Conclusion The modified Sunnybrook Facial Grading System improved the marginal mandibular branch assessment, preserving the evaluation of other facial nerve branches.

RESUMO A lesão do nervo facial é a principal complicação neurológica relacionada às parotidectomias e, em geral, o ramo marginal mandibular é o mais frequentemente acometido. Objetivo Testar um Sistema Sunnybrook de Graduação Facial modificado (mS-FGS) como uma nova ferramenta para avaliar a função do nervo facial após a parotidectomia, enfatizando o ramo marginal mandibular. Métodos Estudo retrospectivo, baseado em prontuários de 73 casos (40 do sexo feminino, 18-84 anos, idade média = 53,2), submetidos à parotidectomia, com preservação do nervo facial. Todos os pacientes apresentavam neoplasias parotídeas ou câncer de pele avançado, e foram tratados pela autora principal entre 2006 e 2014. Resultados Neste estudo, os músculos inervados pelo ramo marginal mandibular foram os mais acometidos (72,6% dos casos), principalmente nos pacientes que realizaram esvaziamento cervical (p = 0,023). Os Escores de Movimento Voluntário obtidos pelo sistema modificado foram inferiores aos obtidos pelo original (p < 0,001). As melhores pontuações foram observadas em pacientes com tumores benignos parotídeos e os piores resultados, naqueles com câncer de pele. Pacientes que necessitaram de esvaziamento cervical e ressecção de outras estruturas, além da parótida, apresentaram escores menores (p = 0,031 e p = 0,021), evidenciados apenas pelo sistema modificado. Os tumores malignos geraram escores significativamente menores, independentemente do instrumento empregado. A análise pós fisioterapia envolveu 50 casos. Os piores resultados, após a intervenção fisioterapêutica, também foram observados nos músculos inervados pelo ramo marginal mandibular. Conclusão A avaliação da disfunção facial pós-parotidectomia, através do Sistema Sunnybrook com a modificação proposta permitiu uma apreciação mais detalhada do ramo marginal mandibular, sem prejuízo à avaliação dos demais ramos.

The blink reflex test does not show abnormalities in a large group of patients with chronic migraine / O teste do reflexo do piscamento nao mostra alteracoes em um grande grupo de pacientes com enxaqueca cronica

The blink reflex – a simple, non-invasive and inexpensive test – may be indicative of lesions or dysfunctions of the brainstem, and particularly assesses the trigeminal-facial arch. Results from alterations of the blink reflex in patients with headaches have provided controversial data. Method Registration of the waves R1 and R2 (ipsilateral to the stimulus) and R2c (contralateral to the stimulus) by electroneuromyography. Results A large number of controls (n=160) and patients with chronic migraine (n=160) were studied. No significant differences were observed between the two groups. Conclusion It is possible that this relatively simple and primitive reflex is not affected unless there is significant damage to the brainstem. .

O reflexo do piscamento é um teste simples, não invasivo de baixo custo que pode direcionar o raciocínio para lesões ou disfunções do traonco cerebral, avaliando particularmente o arco trigêmeo-facial. Resultados de reflexo do piscamento em pacientes com cefaleias têm mostrado dados controversos. Método Registro das ondas R1 e R2 (ipsilaterais ao estímulo) e R2c (contralateral ao estímulo) por eletroneuromiografia. Resultados Um grande número de controles (n=160) e pacientes com enxaqueca crônica (n=160) foi avaliado. Não foram encontradas diferenças significativas no reflexo do piscamento entre os dois grupos. Conclusão É possível que este reflexo, por ser relativamente simples e primitivo, não esteja alterado a menos que ocorram danos significativos no tronco cerebral. .

Treatment of large vestibular schwannomas: towards a compromise between recurrence and facial function preservation

Ideal treatment of large vestibular schwannomas [VS] is complete removal without significant morbidity. The aim of this study is to assess the long-term outcome of surgery, focusing on factors influencing facial function and recurrence. Large VS were removed from 76 consecutive patients, through the retrosigmoid approach, from 1990-2004. Prognostic factors evaluated were age, size hydrocephalus, IX[th] and X[th] nerve signs, extent of surgical removal, and tumours's consistency. Statistics were taken by assessment of Chi-square, Fisher, student t-test. The mean age was 40 +/- 14 years, and the mean diameter was 45 +/- 9 mm. Preoperatively, 93% had significant hearing loss, 74% had cerebellar ataxia, 77% had high intracranial pressure and 40% had IX[th] and X[th] nerve signs. The perioperative mortality dramatically decreased from 17.6% in the earlier period [1990-1996] to 4.5% in the latter period [1997-2004]. At long-term follow-up, 84% of the patients had a Karnofsky score superior to 80%. Mean facial function was better for solid tumours [mean House-Brackmann [HB] standard deviation [SD], 3.7 [1.7] vs. 4.5 [1.7], p = 0.035]. Facial anatomical continuity was preserved in 76% of the cases [n = 58] and was correlated with a better facial function [mean HB [sd], 3.1 [0.7] vs. 5.8 [1.3], p < 0.001]. Excluding patients with a preoperative facial palsy, good facial function was achieved in 58% of the patients, without statistical difference between gross total and subtotal resection [mean HB [SD], 4.3 [1.4] vs. 4.8 [1.9], p=0.229]. The method resulted in improved outcome. Subtotal resection is a good strategy with high likelihood of preserving facial nerve function, without significantly increasing the risk of recurrence. New strategies associating subtotal resection and radiosurgery must be evaluated

O uso de cola de fibrina humana na anastomose de lesões traumáticas parciais do nervo facial / The use of human fibrin glue in the anastomosis of partial traumatic injuries of the facial nerve

Introdução: O uso de cola de fibrina humana na anastomose do nervo facial tem se mostrado uma opção bastante segura e rápida para os cirurgiões. Objetivo: Avaliar o uso da cola de fibrina humana na realização de anastomose do nervo facial em uma série de 42 pacientes que sofreram lesão parcial do nervo facial no segmento intratemporal e foram tratados por três diferentes técnicas de anastomose. Método: Estudo retrospectivo de 42 pacientes apresentavam lesão parcial no segmento intratemporal do nervo facial o qual as anastomoses foram fixadas com cola de fibrina humana. Dividiu-os em 3 grupos: interposição de enxerto parcial na parte lesada do nervo(Grupo 1=12 pacientes); manter a parte preservada e realizar tubolização (Grupo 2= 8 pacientes); seccionar as partes do nervo lesionado (proximal e distal) e interpor enxerto total de nervo sural(Grupo3 =22 pacientes). Resultados: Pacientes com resultado menor ou igual a III na escala House-Brackmann somaram 8,3% (1) no grupo 1, 0,0% (0) no grupo 2 e 68.2% (15) no grupo 3 (p<0.001). Discussão: Ainda existe controvérsia quanto a melhor técnica cirúrgica no tratamento da lesão parcial do nervo facial. O uso da cola de fibrina tem mostrado resultados semelhantes a sutura com pontos. Conclusões: O uso de cola de fibrina humana mostrou ser eficaz na realização destes três tipos diferentes de anastomose do nervo facial sendo que nesta série de 42 pacientes, os indivíduos do grupo 3 (enxerto total) tiveram melhores resultados estatisticamente comprovados se compararmos aos dos grupos 1 (reconstrução parcial) e 2 (tubolização).

Introduction: The use of human fibrin glue in anastomosis of the facial nerve have been an option rather safe and quick for surgeons. Objective: To evaluate the use of the glue of human fibrin glue in the anastomosis of the facial nerve in a series of 42 patients who had suffered partial injury from the facial nerve in the intratemporal segment and they had been treated by three different techniques of anastomosis. Method: A retrospective study on 42 patients who presented partial lesion on intratemporal segment of facial nerve. Patients were divided into three groups: interposition of partial graft on the injured area of the nerve (group 1 - 12 patients); to keep the preserved part and perform tubulization (group 2 - 8 patients); to divide parts of injured nerve (proximal and distal) and place total graft of sural nerve (group 3 - 22 patients). Results: Results lower or equal III on House-Brackmann scale were: 1 (8.3%) patient on group 1; 0 (0.0%) patient on group 2 and 15 (68.2%) of patients on group 3 (p>0.001). Discussion: The best surgery technique for partial lesion therapy of facial nerve is still questionable. The use of the fibrin glue has shown resulted similar to the suture with points. Conclusion: The use The use of human fibrin glue showed to be efficient in the accomplishment of these three different anastomosis types of the facial nerve. Among these 42 patients, the best achieved results were from total graft of facial nerve when comparing to others.

Parálisis facial en otitis media: revisión bibliográfica / Facial paralysis in otitis media: literature review

La parálisis facial secundaria a una otitis media es una conocida complicación de infecciones agudas y crónicas. Su fisiopatología es desconocida pero la presión elevada en el oído medio, la osteítis, la invasión bacteriana y la neurotoxicidad, podrían estar involucradas. Los exámenes de laboratorio no entregan mayor información para determinar la etiología de la parálisis facial y la tomografía computada de oídos sería el estudio radiológico de elección ya que permite identificar el compromiso óseo, la extensión de la enfermedad y posibles alteraciones anatómicas existentes, además de ser utilizado para el diagnóstico de eventuales complicaciones intracraneales. El tratamiento de la parálisis en el curso de una otitis media aguda es médico, con antibióticos y esteroides, con buen pronóstico de recuperación; mientras en una otitis media crónica el tratamiento es quirúrgico y el pronóstico global peor.

Facial paralysis secondary to otitis media is a well known complication of acute and chronic infection. The physiopathology of facial nerve paralysis secondary to otitis media of not known, but high middle ear pressure, osteitis, direct bacterial invasion and neurotoxicity could be involved. Laboratory studies have failed to provide clues as to determining the etiology of facial nerve palsy. Ear CT is the radiologic study of choice, as it allows the identification of bone destruction, disease extension and possible anatomic alterations, and may also be used as a screening for intracranial complications. Acute otitis media treatment is medical with antibiotics and steroids, with good prognosis, whereas in chronic otitis media the treatment is surgical, with worst global prognosis.

Doenças infecciosas específicas manifestando paralisia facial: revisão da literatura / Specific infectious diseases manifesting facial paralysis: a literature review

A paralisia facial otogênica decorre devido à interrupção do influxo nervoso no nervo facial em seu trajeto pelo interior do osso temporal, em consequência de diferentes e variados fatores etiológicos. A etiologia infecciosa não é uma das mais frequentes em nosso meio, no entanto deve ser levada em consideração e bem investigada. Existem várias doenças infecciosas que manifestam paralisia facial. Neste trabalho, foi realizada uma revisão da literatura de doenças infecciosas que manifestam paralisia facial.

Lepromatous leprosy with bilateral facial nerve palsy and hyperthyroidism.

Bilateral lagophthalmos secondary to facial nerve is extremely uncommon. Further, the aetiology in most of these cases is of central origin unlike the peripheral involvement in leprosy. A patient of lepromatous leprosy (LL) may be euthyroid or hypothyroid on account of leprous involvement of the thyroid gland. A case of LL with bilateral lagophthalmos and hyperthyroidism is reported.

Avaliação funcional da mímica na paralisia facial central por acidente cerebrovascular / Mime functional evaluation in facial paralysis following a stroke

TEMA: avaliação funcional da mímica facial de pacientes com paralisia facial central por acidente cerebrovascular. OBJETIVO: avaliar os aspectos funcionais da musculatura facial em pacientes com paralisia facial central após acidente cerebrovascular. MÉTODO: foram avaliados nove pacientes do Serviço de Neurologia. A avaliação enfocou movimentos espontâneos, reflexos e voluntários. RESULTADOS: todos os pacientes apresentaram movimentação voluntária e involuntária das pálpebras e testa, mas a movimentação dos lábios e de nariz estava prejudicada em ambos os movimentos, dependendo da localização e extensão da lesão. CONCLUSÃO: a sintomatologia da paralisia facial central, na qual se espera a paralisia da movimentação da parte inferior somente para os movimentos voluntários, ocorre na minoria dos pacientes.

Lagoftalmos bilateral en paciente con lepra lepromatosa. Caso clínico / Bilateral lagophthalmos inlepromatous leprosy. Case report

CASE REPORT: A case of bilateral facial palsy with paralytic ectropion, lagophthalmos and corneal damage secondary to corneal exposure in a long-standing patient with lepromatous leprosy is presented. Correction of paralytic ectropion was performed by medial cantoplasty, tarsal strip and Medpor lower eyelid spacer implantation. Lagophthalmos was corrected by gold weight implant in the upper tarsus. DISCUSSION: Ocular findings in leprosy appear in 72 per cent of patients. Facial nerve palsy occurs in 3-19.8 per cent, being bilateral in 5 per cent. In long standing cases with corneal complications secondary to exposure, surgical treatment is required.

Nervo facial: aspectos anatômicos e semiológicos / Facial nerve: anatomic and semiologic features

Os autores revisam a anatomia e a semiologia do nervo facial. Comentários são feitos a respeito de afecções ou síndromes neurológicas que possam envolver o nervo facial

Carcinoma adenocístico da parótida: disseminaçäo perineural através do nervo facial - tomografia computadorizada e ressonância magnética / Adenoid cystic carcinoma of the parotid gland: perineural spread through facial nerve-CT and MRI

O carcinoma adenocístico é o tumor maligno mais comum das glândulas parótidas. A invasäo intracraniana do tumor costuma se fazer por disseminaçäo perineural e é característica comumente encontrada neste tumor. Geralmente a disseminaçäo se dá pelos ramos maxilar e mandibular do nervo trigêmio. A proximidade do tumor e do trajeto intratemporal do nervo facial, a propensäo à disseminaçäo perineural através do nervo facial, a propensäo à disseminaçäo perineural e o pior prognóstico associado com o envolvimento do nervo säo importantes no planejamento pré-terapêutico. Três pacientes portadores de carcinoma adenocístico da parótida com disseminaçäo perineural através do nervo facial säo apresentados, sendo avaliados por meio de tomografia computadorizada (TC) e ressonância

Descompresión microvascular del quinto y séptimo nervio craneales: estudio con seguimiento a 10 años / Microvascular descompression of the fifth and seventh cranial nerves: a ten years follow up study

Treinta y siete pacientes (dieciséis con neuralgia del trigémino (NT) y veintiuno con espasmo hemifacial -EHF-) fueron sometidos a descompresión microvascular por la fosa posterior. La causa más frecuente de compresión neruvascular en los casos de neuralgia del trigémino fue la arteria cerebelosa superior y en los casos de espasmo hemifacial el vaso compresor fue la arteria cerebelosa anteroinferior. Los pacientes tuvieron un seguimiento de 1 a 10 años obteniéndose los siguientes resultados: Para la NT; mejoría en 1 caso, curación en 14 y sin cambio en 1 caso. Para el EHF; mejoría en 8 casos y curación en 13 casos. Dos pacientes en cada grupo presentaron recidiva de la sintomatología. Los resultados dan un 73.2 por ciento de curaciones cifra ligeramente menor a la reportada en la literatura. Los resultados podrían estar en relación al tipo de material utilizado para la descompresión microvascular. Se propone utilizar el dacrón corrugado (material inadsorbible) para mantener la descompresión

Otogenic facial nerve palsy

To study the various otogenic conditions responsible for facial nerve palsy in all age groups. Design: It is a retrospective study completed in three years. Settings: Department of E. N. T., King Edward Medical College and Mayo Hospital, Lahore. Subjects: We studied twenty two patients with facial nerve paralysis and cause was somehow related with ear. Otological Trauma was found to be the commonest cause [41%]. Inflammatory pathologies were responsible for 36% cases and malignant tumours in 23% cases. Otological conditions must be thoroughly looked in every patient with lower motor neuron type of facial nerve palsy

Desórdenes del nervio facial: revisión bibliográfica y reporte de casos / Facial nerve disorders: review of the literature and case reports

Múltiples factores pueden lesionar el nervio facial en su trayecto produciendo una parálisis facial, siendo esencial averiguar el sitio de la lesión para comprender el cuadro clínico. El presente artículo considera el valor diagnóstico, terapéutico y pronóstico de pruebas clínicas específicas realizadas en nueve casos de parálisis de Bell, junto con revisión de la literatura

Facial electroneurography in Bell's palsy: variability in the early stage and comparison between interpretation methods

To determine the variability of the abnormalities found in the electroneurography (ENG) of the facial nerve in cases of Bell's palsy during the initial two week period was one of the objectives of the authors. A second one was to investigate the value of ENG as a tool to determine an early prognosis of recovery utilizing two different methods. In the first one the amplitude of the compound muscular action potential (CMAP) obtained on the paralyzed side was compared to this potential on the opposite (normal) side. The second method compared the CMAP on the paralyzed side to normal standardized data from normal individuals. A group of 33 patients with Bell's palsy was followed until total recovery or for at least 4 months, if the recovery was not achieved earlier. It was observed that amplitude of the CMAP become stable towards the sixth day of palsy and this is a good time to establish the prognosis. Another conclusion is that both methods were equivalent to determine the prognosis in Bell's palsy.