Síndrome de Guillain-Barré como forma de presentación de lupus eritematoso sistémico en paciente masculino / Guillain Barré Syndrome as a form of presentation of systemic lupus erythematosus in a male patient

El lupus eritematoso sistémico es una enfermedad reumática, autoinmune, inflamatoria, sistémica y crónica que se caracteriza por afectar principalmente pacientes femeninas en edad fértil. Su mecanismo patogénico se centra en la presencia de un proceso inflamatorio crónico que es el responsable de las manifestaciones clínicas y complicaciones de la enfermedad. Las manifestaciones clínicas se subdividen en articulares y extraarticulares. Entre estas últimas la afectación neurológica cobra especial atención tanto por su frecuencia de presentación elevada como por la gravedad de estas. El síndrome de Guillain-Barré es una enfermedad autoinmune, que puede aparecer como complicación del lupus eritematoso sistémico y se caracteriza por parálisis motora y otros síntomas y signos neurológicos. El objetivo del presente trabajo es reportar el caso de un paciente masculino, de 27 años de edad, que comienza con manifestaciones neuropáticas y se llega a diagnosticar un síndrome de Guillain-Barré que constituyó la forma de presentación del lupus eritematoso sistémico. Por ser una forma de presentación poco común, se considera importante su reporte para conocimiento de la comunidad médica(AU)

Systemic lupus erythematosus is a rheumatic, autoimmune, inflammatory, systemic and chronic disease that is characterized by mainly affecting female patients of childbearing age. Its etiopathogenic mechanism focuses on the presence of a chronic inflammatory process that is responsible for the clinical manifestations and complications of the disease. Clinical manifestations are subdivided into articular and extra-articular. Within the latter, neurological involvement receives special attention both due to its high frequency of presentation and its severity. Guillain-Barré syndrome is an autoimmune disease that can appear as a complication of lupus erythematosus and is characterized by motor paralysis and other neurological signs and symptoms. The objective of this paper is to report the case of a 27-year-old male patient who began with neuropathic manifestations and was diagnosed with Guillain-Barré syndrome, which was the presentation of systemic lupus erythematosus. Because it is an uncommon form of presentation, its report is considered important for the knowledge of the medical community(AU)

Ecocardiografia Tridimensional na avaliação de endocardite de válvula nativa: um relato de caso e semelhança entre achados ecocardiográficos e cirúrgicos / Three-Dimensional transesophageal echocardiography in native valve endocarditis: a case report and similarities between echocardiography and surgical findings

A endocardite de valva nativa é uma doença incomum, complexa, e de alta morbimortalidade. Requer tratamento clínico prolongado, com várias complicações possíveis, e o seu tratamento cirúrgico é complexo e tecnicamente difícil. O ecocardiograma transtorácico e transesofágico são fundamentais na avaliação da doença, inclusive seus achados são parte dos critérios diagnósticos de endocardite. Adicionalmente, o ecocardiograma tridimensional (3D) contribui com detalhamento anatômico na avaliação das estruturas cardíacas acometidas pela doença. Mostramos um caso em que é ilustrado o papel da ecocardiografia no diagnóstico e avaliação de complicações da endocardite, comparando as imagens do ecocardiograma 3D pré-operatórias, com os achados durante o ato cirúrgico. (AU)

Native valve bacterial endocarditis is an uncommon, complex, and highly morbid disease that requires prolonged clinical treatment and challenging surgical interventions. Transthoracic and transesophageal echocardiography are paramount assessment tools whose findings are included in the diagnostic criteria. Three-dimensional echocardiography shows further realistic imaging details. Here we present a case demonstrating the role of echocardiography in the diagnosis of endocarditis and the identification of its complications to show how advanced imaging techniques may have a remarkable resemblance with in vivo surgical findings. (AU)

Síndrome de Guillain Barré asociado a infección SARS-COV-2, reporte de caso clínico / Guillain-Barre syndrome associated with SARS-COV2: a case report

En diciembre de 2019, en Wuhan ­ China, se reportaron varios casos de neumonía de etiología desconocida. A inicios del 2020 se identificó el virus coronavirus 2 del Síndrome Respiratorio Agudo Severo (SARS ­ COV2) como agente causal de la enfermedad. La Organización Mundial de la Salud (OMS) definió la afección por este virus como COVID 19 confirmando su presencia en Sudamérica y en el Ecuador en febrero, por lo cual el 11 de marzo se declaró como una pandemia. Esta patología afecta principalmente al sistema respiratorio, sin embargo, existe el reporte de manifestaciones neurológicas en pacientes con COVID 19. Por lo cual, se informa el primer caso en Ecuador de Síndrome de GuillaIn Barré asociado a la misma.

In December 2019, in Wuhan ­ China, several cases of pneumonia of unknown etiology were reported. At the beginning of 2020, the Severe Acute Respiratory Syndrome coronavirus 2 (SARS ­ COV2) was identified as the disease's causal agent. The World Health Organization (WHO) defined this virus as COVID 19, confirming its presence in South America and Ecuador in February. Later on March 11, it was declared a pandemic. This pathology mainly affects the respiratory system; however, there is a report of neurological manifestations in patients with COVID 19. Therefore, we report the first case of Guillain Barré syndrome associated with COVID 19 in Ecuador.

Craniofacial and neurological manifestations in patients with Guillain-Barré syndrome / Manifestaciones neurológicas craneofaciales en pacientes con síndrome de Guillain-Barré

Introducción: Among the broad range of symptoms of the Guillain-Barré Syndrome (GBS), patients can present craniofacial manifestations. Consequently, the participation of the dental surgeon in the evaluation of the stomatognathic system using a multidisciplinary approach model is very important. Objective: To identify the craniofacial and neurological manifestations of the Guillain-Barré Syndrome in patients from three hospitals in the district of Lambayeque, Peru, in 2019. Material and Methods: A descriptive, prospective, and cross-sectional study was carried out in 59 patients diagnosed with GBS by means of a clinical evaluation carried out by previously calibrated neurologists (k=0.911). Additionally, an oral evaluation of the oral hygiene index, the periodontal status, and the oral pH was carried out. Data were processed using frequency distribution tables. Results: 4 4.07% of the patients presented at least one functional alteration, such as unilateral limitation for facial expressions (25.42%), dysphagia (18.64%), alteration in phonation (11.86%), and loss of the sense of taste (6.78%), showing involvement of the corresponding cranial nerves. In addition, unfavorable conditions of oral hygiene and acid pH were observed in some cases, which proved adverse for the periodontal structures of the patients. Conclusion: The study found craniofacial manifestations of GBS, such as cases of unilateral facial palsy, dysphagia, difficulty in phonation, and loss of the sense of taste, due to the alteration of the cranial nerves that command these functions. In addition, poor oral hygiene, and the presence of acidic pH in some patients created an adverse environment for the integrity of the periodontal structures

Introducción: El Síndrome de Guillain-Barré (SGB) dentro de su amplia sintomatología puede presentar manifestaciones craneofaciales, por lo que resulta importante la participación del cirujano dentista en la evaluación del sistema estomatognático desde un modelo de abordaje multidisciplinario. Objetivo: Identificar las manifestaciones neurológicas craneofaciales del Síndrome de Guillain-Barré en pacientes de tres establecimientos de salud de Lambayeque, Perú en el año 2019. Materiales y Metodos: Se realizó un estudio descriptivo, prospectivo y transversal, con 59 pacientes diagnosticados con SGB, mediante una evaluación clínica realizada por médicos neurólogos previamente calibrados (k=0,911). Adicionalmente se realizó una evaluación bucodental del índice de higiene oral, del estado periodontal y del pH bucal, siendo procesados los datos mediante tablas de distribución de frecuencias. Resultados: El 44,07% de pacientes presentaron por lo menos una alteración funcional, identificando limitación unilateral para expresiones faciales (25.42%), disfagia (18.64%), alteración en la fonación (11.86%) y pérdida del sentido del gusto (6.78%), evidenciando compromiso de los pares craneales correspondientes. Además se observaron condiciones desfavorables de higiene oral y de pH ácido en algunos casos, que resultaron adversas para las estructuras periodontales de los pacientes. Conclusión: Fueron encontradas manifestaciones craneofaciales del SGB, identificando casos con parálisis facial unilateral, disfagia, dificultad para la fonación y pérdida del sentido del gusto, debido a la alteración de los pares craneales que comandan esas funciones. Además las condiciones desfavorables de higiene oral y la presencia de pH ácido en algunos casos, propiciaron un ambiente adverso para la integridad de las estructuras periodontales.

Neurological complications in patients with SARS-CoV-2 infection: a systematic review / Complicações neurológicas em pacientes infectados pelo SARS-CoV-2: uma revisão sistemática

ABSTRACT Background: As the COVID-19 pandemic unfolds worldwide, different forms of reports have described its neurologic manifestations. Objective: To review the literature on neurological complications of SARS-CoV-2 infection. Methods: Literature search performed following systematic reviews guidelines, using specific keywords based on the COVID-19 neurological complications described up to May 10th, 2020. Results: A total of 43 articles were selected, including data ranging from common, non-specific symptoms, such as hyposmia and myalgia, to more complex and life-threatening conditions, such as cerebrovascular diseases, encephalopathies, and Guillain-Barré syndrome. Conclusion: Recognition of neurological manifestations of SARS-CoV-2 should be emphasized despite the obvious challenges faced by clinicians caring for critical patients who are often sedated and presenting other concurrent systemic complications.

RESUMO Introdução: À medida que a pandemia da COVID-19 se desenvolve em todo o mundo, diferentes tipos de publicações descreveram suas manifestações neurológicas. Objetivo: Revisar a literatura sobre complicações neurológicas da infecção por SARS-CoV-2. Métodos: A pesquisa bibliográfica foi realizada seguindo diretrizes de revisões sistemáticas, usando palavras-chave específicas baseadas nas complicações neurológicas da COVID-19 descritas até 10 de maio de 2020. Resultados: Foram selecionados 43 artigos, incluindo descrições que variam de sintomas comuns e inespecíficos, como hiposmia e mialgia, a condições mais complexas e com risco de vida, como doenças cerebrovasculares, encefalopatias e síndrome de Guillain-Barré. Conclusão: O reconhecimento das manifestações neurológicas da SARS-CoV-2 deve ser enfatizado apesar dos óbvios desafios enfrentados pelos clínicos que cuidam de pacientes críticos, muitas vezes sedados e apresentando outras complicações sistêmicas concomitantes.

Aumento das internações por síndrome de Guillain-Barré no Brasil: estudo ecológico / Aumento de hospitalizaciones por síndrome de Guillain-Barré en Brasil: un estudio ecológico / Increase in Guillain-Barré syndrome hospitalizations in Brazil: an ecological study

Objetivo: descrever as características demográficas e a dinâmica espaço-temporal das internações por síndrome de Guillain-Barré (SGB) no Brasil, no período 2008-2017. Métodos: trata-se de um estudo ecológico com dados do Sistema de Informações Hospitalares do Sistema Único de Saúde (SIH/SUS); foram calculadas taxas de internações por SGB e construiu-se um diagrama de controle; na análise espacial, utilizou-se o intervalo de quebras naturais. Resultados: foram registradas 15.512 internações por SGB no período do estudo; entre 2008 e 2014, observou-se uma média de 1.344 internações por ano; em 2015, foram registradas 1.953 internações, representando um incremento de 45% em relação à média dos anos anteriores; internações por SGB estiveram presentes em nível epidêmico na região Nordeste, nos anos de 2015 e 2016. Conclusão: houve aumento das internações por SGB a partir de 2015, após a introdução do vírus chikungunya e a rápida propagação do vírus Zika no Brasil.

Objetivo: describir las características demográficas y la dinámica espacio-temporal de las hospitalizaciones por síndrome de Guillain-Barré (GBS) en Brasil, en el período 2008-2017. Métodos: este es un estudio ecológico con datos del Sistema de Información Hospitalaria del SUS (SIH/SUS); se calcularon las tasas de hospitalización por GBS y se construyó un diagrama de control; en el análisis espacial, se utilizó el rango de rupturas naturales. Resultados: se registraron 15.512 ingresos por GBS durante el período de estudio. Entre 2008-2014, se observó un promedio de 1.344 hospitalizaciones por año, en el año siguiente (2015), se registraron 1.953 hospitalizaciones, lo que representa un aumento del 45% en relación con el promedio de años anteriores; las hospitalizaciones por GBS estuvieron presentes a nivel epidémico en la Región Nordeste en los años 2015 y 2016. Conclusión: hubo un aumento en las hospitalizaciones por GBS a partir de 2015, después de la introducción del virus chikungunya y la rápida propagación del virus Zika en Brasil.

Objective: to describe the demographic characteristics and the spatio-temporal dynamics of Guillain-Barré syndrome (GBS) hospitalizations in Brazil between 2008 and 2017. Methods: this is an ecological study using data from the Hospital Information System of the Brazilian National Health System (SIH/SUS); GBS hospitalization rates were calculated and a control diagram was built; natural break ranges were used in the spatial analysis. Results: 15,512 GBS hospitalizations were recorded during the study period; between 2008-2014 there were 1,344 hospitalizations per year on average, in the following year (2015), 1,953 hospitalizations were registered, representing an increase of 45% in relation to the average of previous years; GBS hospitalizations reached an epidemic level in the Northeast region in 2015 and 2016. Conclusion: GBS hospitalizations increased with effect from 2015, following the introduction of chikungunya virus and the rapid spread of Zika virus in Brazil.

Mycoplasma Pneumoniae, asociado a polineuropatía axonal autoinmune, ¿variante clínica del síndrome Guillain-Barré en niños? / Mycoplasma Pneumoniae, associated to autoimmune axonal polyneuropathy, clinical variant of Guillain-Barré Syndrome in children?

Introducción: Mycoplasma penumoniae es un patógeno reconocido como principal agente causal de neumonía atípica, así como también por generar diferentes tipos de complicaciones extrapulmonares, especialmente de carácter neurológico y afectar directamente el sistema nervioso, gracias a sus mecanismos de virulencia, mimetismo y de inmunomodulación en el huésped. Causa afecciones como neuropatías, polineuropatías, encefalopatías, síndrome de Guillain Barré y otros. Objetivo: Reforzar en el área pediátrica la necesidad de modificar criterios diagnósticos e incorporar variantes clínicas del síndrome de Guillain Barre, además de instrumentos para diagnóstico de afecciones neuropáticas. Presentación del caso: Paciente masculino, 9 años 8 meses de edad, quien consulta en repetidas ocasiones por: dispepsias, episodios de diarrea, constipación y fiebre. Se constató según consulta: disbiosis, resfriado común, y finalmente, neumonía atípica por Mycoplasma Pneumoniae. Paciente evoluciona, con debilidad muscular, paresia, hiperalgesia y alodinia de extremidades superiores e inferiores. Acude a neurólogo, quien indica exámenes neurofisiológicos (velocidad de conducción nerviosa, potenciales evocados y se descartó una electromiografía, debido a la hiperalgesia). Se diagnosticó una polineuropatía axonal, la que se caracterizó por presentar ciertos aspectos del síndrome de Guillain-Barré. Tanto la evolución clínica de este síndrome, así como sus variantes clínicas, tienen un curso en adultos, caracterizado por un comienzo y signos distintos, lo que puede retrasar y errar el diagnóstico en pacientes pediátricos. Conclusiones: Hace falta nuevos criterios diagnósticos y su amplitud y herramientas de abordaje, para hacer un diagnóstico rápido y eficaz, y contribuir a la recuperación optima del paciente(AU)

Introduction: Mycoplasma pneumoniae is a pathogen know as to the main causal agent of atypical pneumonia, as well as to generate different extrapulmonary sickness, especially in neurological ways, directing to the nervous system, thanks to all its different mechanisms, like: virulence, mimetysm and immunomodulation in to the host. Producing, pathologies like neuropathies, polyneuropathies, encephalopathies, Guillain Barré Syndrome. Objetives: To highlight in the pediatric area, the need to modificate diagnosis criteria and incorporate Guillain-Barre Syndrome clinicals variants, also instruments to diagnosis of neuropathic pathologies. Case presentation: Male patient, 9 years, 8 months old, who consulted in repeated occasions for: dyspepsia, diarrhea and constipation episodes and fiber. Confirmed according to consultation: dysbiosis, common cold, and finally, atypical pneumonia by Mycoplasma Pneumoniae. The patient evolves with: muscular weakness, hyperalgesia and allodynia of upper and inferior extremities. Then, the Neurologist, indicates neurophysiological exams (nerve conduction velocity, evoked potentials, discarding an electromyography, due to hyperalgesia). Diagnosing an axonal polyneuropathy. Which was characterized to present some same aspects, from clinical course of Guillain-Barre Syndrome. Highlighting that the clinical evolution, as also, the syndrome clinical variants, has it a course in adults, characterized by a different beginning and signs, than in children. Retarding and do a wrong diagnosis in pediatric patients. Conclusion: Lack of new diagnosis criteria, the amplitude of these and tools of approach to give a fast and effective diagnosis, and contribute to the optimal recovery of the patient(AU)

Guillain-Barré syndrome associated with arboviruses in the state of Pernambuco in 2016 / Síndrome de Guillain-Barré associada à arboviroses no estado de Pernambuco em 2016 / Síndrome de Guillain-Barré asociado a arbovirosis en el Estado de Pernambuco en 2016

Abstract Introduction: In 2016, Brazil presented an increase in the notifications related to neurological syndromes with previous register of a febrile illness compatible with arboviruses, including Guillain-Barré syndrome (GBS). Such disease is responsible for the most frequent occurrence of flaccid paralysis in the world, causing bilateral ascending muscle weakness that might affect the respiratory tract. Objective: To investigate the incidence of Guillain-Barré syndrome associated with arboviruses in Pernambuco in 2016 and to describe the confirmed/probable cases. Method: This is an observational retrospective study of GBS cases with a background of infection from dengue, chikungunya, or Zika virus notified to the Pernambuco Health State Secretariat (SES-PE). Cases notified from January 1st to December 31st, 2016 were included, which were considered as possible GBS at the time of notification. The suspect cases were investigated and classified as confirmed/probable by the positive laboratory test result, excluding the possibility of infection. Results: Forty-three suspect cases of GBS after previous arbovirus infection were notified. From these, 23 were classified as confirmed/probable for the etiological agents chikungunya and/or dengue. The mean age of the affected individuals was 37.22 ± 21.29 years and they were mostly female (56.5%). The annual incidence of GBS after arbovirus infection was 0.24 cases per 100 thousand inhabitants in the state. The neurological condition was mainly characterized by the presence of movement (91.3%) and walking (78.3%) alterations. Conclusion: The findings of this research confirm the current set of evidence and show the likelihood of GBS being a severe neurological complication of these arboviruses.

Resumo Introdução: No Brasil, em 2016, houve aumento das notificações relacionadas às síndromes neurológicas com registro anterior de doença febril compatível com infecção por arboviroses, dentre elas a Síndrome de Guillain- Barré (SGB). Trata-se da ocorrência mais frequente de paralisia flácida no mundo, causa fraqueza muscular bilateral ascendente e pode atingir as vias respiratórias. Objetivo: Analisar a incidência da Síndrome de Guillain- Barré associada à arboviroses em Pernambuco no ano de 2016 e descrever os casos confirmados/prováveis. Método: Trata-se de um estudo observacional retrospectivo dos casos de SGB com histórico de infecção prévia por dengue, chikungunya ou vírus Zika, notificados à Secretaria Estadual de Saúde de Pernambuco (SES-PE). Foram incluídos casos notificados de 1º de janeiro a 31 de dezembro de 2016 considerados suspeitos de SGB no momento da notificação. Os casos suspeitos foram investigados e classificados como confirmados/prováveis através de resultado de exame laboratorial positivo, excluída a possibilidade de infecção. Resultados: Foram notificados 43 casos suspeitos de SGB após infecção prévia por arbovírus. Destes, 23 foram classificados como confirmados/prováveis para os agentes etiológicos chikungunya e/ou dengue, sendo a média de idade igual a 37,22 ± 21,29 anos e maioria do sexo feminino (56,5%). A incidência anual de SGB após infecção por arbovírus foi de 0,24 casos por 100 mil habitantes no estado. O quadro neurológico foi caracterizado prioritariamente pela presença de alterações motoras (91,3%) e na marcha (78,3%). Conclusão: Os achados da presente pesquisa acrescentam ao conjunto de evidências sobre a possibilidade de a SGB se apresentar como uma grave complicação neurológica dessas arboviroses.

Resumen Introducción: En Brasil, en 2016, hubo aumento de las notificaciones relacionadas con los síndromes neurológicos con registro anterior de enfermedad febril compatible con infección por arbovirosis, entre ellas el Síndrome de Guillain-Barré (SGB). Se trata de la aparición más frecuente de parálisis flácida en el mundo, causa debilidad muscular bilateral ascendente y puede alcanzar las vías respiratorias. Objetivo: Analizar la incidencia del Síndrome de Guillain-Barré asociada a los arbovirosis en Pernambuco en el año 2016 y describir los casos confirmados / probables. Método: Se trata de un estudio observacional retrospectivo de los casos de SGB con antecedentes de infección previa por dengue, chikungunya o virus Zika, notificados a la Secretaría Estatal de Salud de Pernambuco (SES-PE). Se incluyeron casos notificados del 1 de enero al 31 de diciembre de 2016 considerados sospechosos de SGB en el momento de la notificación. Los casos sospechosos fueron investigados y clasificados como confirmados / probables a través del resultado de un examen de laboratorio positivo, excluida la posibilidad de infección por otros agentes virales. Resultados: Se han notificado 43 casos sospechosos de SGB tras la infección previa por arbovirus. De estos, 23 fueron clasificados como confirmados / probables para los agentes etiológicos chikungunya y / o dengue, siendo la media de edad igual a 37,22 ± 21,29 años y la mayoría del sexo femenino (56,5%). La incidencia anual de SGB tras infección por arbovirus fue de 0,24 casos por 100 mil habitantes en el estado. El cuadro neurológico fue caracterizado prioritariamente por la presencia de alteraciones motoras (91,3%) y en la marcha (78,3%). Conclusión: Los hallazgos de la presente investigación añaden al conjunto de evidencias sobre la posibilidad de que la SGB se presente como una grave complicación neurológica de esas arbovirosis.

Tiroidectomía en polineuropatía desmielinizante aguda para paciente con enfermedad de Graves: a propósito de un caso / Thyroidectomy in acute demyelinating polyneuropathy for a patient with Graves' disease: a case report

RESUMEN Existen pocos estudios que describan las complicaciones neuromusculares del hipertiroidismo. Presentamos el caso de una mujer de 36 años con antecedente de enfermedad de Graves en manejo médico, quien presentó compromiso motor y sensitivo progresivo en extremidades inferiores hasta comprometer la marcha. La electromiografía fue compatible con polineuropatía desmielinizante aguda, la resonancia nuclear magnética sugirió síndrome de Guillain-Barré. Se proporcionó manejo con plasmaféresis y se ajustó tratamiento tiroideo presentando mejoría transitoria. Posteriormente presentó deterioro neurológico con debilidad ascendente y compromiso deglutorio; a pesar de inicio de ciclo de inmunoglobulina persistió empeoramiento clínico con requerimiento de soporte ventilatorio; se decidió realizar tiroidectomía, que resultó en mejoría clínica y resolución del cuadro.

ABSTRACT There are few studies that describe the neuromuscular complications of hyperthyroidism. We present the case of a 36-year-old woman with a history of Graves' disease in medical management, who presented motor and sensitive involvement in the lower limbs until compromising gait. Electromyography was compatible with acute demyelinating polyneuropathy, nuclear magnetic resonance suggested Guillain-Barré syndrome. Management with plasmapheresis was indicated, and thyroid therapy was adjusted with transient improvement. Subsequently, she presented neurological deterioration with ascending weakness and swallowing compromise; despite an immunoglobulin cycle regimen, clinical worsening persisted with the requirement of ventilatory support; thyroidectomy was performed resulting in clinical improvement and resolution of the condition.

A Korean patient with Guillain-Barré syndrome following acute hepatitis E whose cholestasis resolved with steroid therapy

Autochthonous hepatitis E virus (HEV) is an emerging pathogen in developed countries, and several cases of acute HEV infection have been reported in South Korea. However, there have been no reports on HEV-associated Guillain-Barré syndrome (GBS) in Korea. We recently experienced the case of a 58-year-old Korean male with acute HEV infection after ingesting raw deer meat. Persistent cholestasis was resolved by the administration of prednisolone. At 2.5 months after the clinical presentation of HEV infection, the patient developed weakness of the lower limbs, and was diagnosed with GBS associated with acute hepatitis E. To our knowledge, this is the second report on supportive steroid therapy for persistent cholestasis due to hepatitis E, and the first report of GBS in a Korean patient with acute HEV infection.

Diplejía facial con parestesias: Una variante atípica del síndrome de Guillain-Barré

El síndrome de Guillain-Barré es una enfermedad desmielinizante aguda con una forma clásica que se presenta con debilidad muscular y ausencia de reflejos. Existen múltiples variantes y formas atípicas de la enfermedad, entre otras la diplejía facial con parestesias. Asimismo, la ausencia de reflejos en este síndrome es característico pero no constante, ya que en un 10% de los pacientes los reflejos están presentes. Se presenta aquí el caso de una mujer de 33 años con paresia facial bilateral, parestesias y debilidad de miembros inferiores e hiperreflexia, una forma de presentación infrecuente de este síndrome.

Guillain-Barré syndrome is an acute demyelinating disease which presents in a classic form with muscular weakness and the lack of reflexes. There are multiple variations and atypical forms of the disease, being facial diplegia with paresthesia one of them. Also, the absence of reflexes in this syndrome is typical but not constant, since 10% of patients present reflexes. We describe a case of atypical presentation with bilateral facial palsy, paresthesia, brisk reflexes and weakness in the lower limbs in a 33 year old woman.

Variante faringo-cérvico-braquial del síndrome de Guillain-Barré

El síndrome de Guillain-Barré es una polineuropatía desmielinizante inflamatoria aguda, existiendo variantes locorregionales poco frecuentes como la faringo-cérvico-braquial. Presentamos el caso de un varón de 63 años de edad que acudió a urgencias por debilidad de extremidades superiores y a nivel cervical, con incapacidad para deglutir y masticar, y una insuficiencia respiratoria aguda rápidamente progresiva por debilidad de músculos respiratorios, todo ello secundario a la variante faringo-cérvico-braquial del síndrome de Guillain-Barré. Esta variante, aunque poco frecuente, presenta un patrón clínico y unos criterios diagnósticos bien definidos, importantes de reconocer para poder así iniciar de forma precoz el tratamiento y mejorar el pronóstico, no siempre favorable, de estos pacientes.

Guillain-Barré syndrome is an acute inflammatory demyelinating polyneuropathy. Infrequent loco-regional variants, like the pharyngeal-cervical-brachial, have been described. We report the case of a 63-year-old male admitted to the emergency department with cervical and upper limb weakness, inability to swallow and chew, he also presented a rapidly progressive acute respiratory failure due to weakness of the respiratory muscles secondary to the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. This variant, although unusual, presents a well-defined clinical pattern and diagnostic criteria, which is important in order to start an early treatment to improve the prognosis, not always favorable, to these patients.

Syndrome of inappropriate antidiuretic hormone secretion related to Guillain-Barré syndrome after laparoscopic cholecystectomy / Síndrome da secreção inapropriada do hormônio antidiurético relacionada à síndrome de Guillain-Barré após colecistectomia videolaparoscópica / Síndrome de secreción inapropiada de hormona antidiurética relacionado con el síndrome de Guillain-Barré tras colecistectomía laparoscópica

Background and objectives: Guillain-Barré Syndrome is one of the most common causes of acute polyneuropathy in adults. Recently, the occurrence of Guillain-Barré Syndrome after major and minor surgical operations has been increasingly debated. In Guillain-Barré syndrome, syndrome of inappropriate antidiuretic hormone secretion and dysautonomy are generally observed after maximal motor deficit. Case report: A 44-year-old male patient underwent a laparoscopic cholecystectomy for acute cholecystitis. After the development of a severe headache, nausea, diplopia, and attacks of hypertension in the early postoperative period, a computer tomography of the brain was normal. Laboratory tests revealed hyponatremia linked to syndrome of inappropriate antidiuretic hormone secretion, the patient's fluids were restricted, and furosemide and 3% NaCl treatment was initiated. On the second day postoperative, the patient developed numbness moving upward from the hands and feet, loss of strength, difficulty swallowing and respiratory distress. Guillain-Barré syndrome was suspected, and the patient was moved to intensive care. Cerebrospinal fluid examination showed 320 mg/dL protein, and acute motor-sensorial axonal neuropathy was identified by electromyelography. Guillain-Barré syndrome was diagnosed, and intravenous immune globulin treatment (0.4 g/kg/day, 5 days) was initiated. After 10 days in the intensive care unit, at which the respiratory, hemodynamic, neurologic and laboratory results returned to normal, the patient was transferred to the neurology service. Conclusions: Our case report indicates that although syndrome of inappropriate antidiuretic hormone secretion and autonomic dysfunction are rarely the initial characteristics of Guillain-Barré syndrome, the possibility of postoperative syndrome of inappropriate antidiuretic hormone secretion should be kept in mind. The presence of secondary hyponatremia ...

Justificativa e objetivos: a síndrome de Guillain-Barré é uma das causas mais comuns de polineuropatia aguda em adultos. Recentemente, a ocorrência após grandes e pequenas intervenções cirúrgicas tem sido cada vez mais debatida. Na Guillain-Barré, a síndrome da secreção inapropriada do hormônio antidiurético e a disautonomia são geralmente observadas após déficit motor máximo. Relato de caso: paciente do sexo masculino, 44 anos, submetido a colecistectomia videolaparoscópica para colecistite aguda. Após desenvolver uma forte dor de cabeça, náusea, diplopia e ataques de hipertensão no período pós-operatório imediato, uma tomografia computadorizada do cérebro revelou-se normal. Os exames laboratoriais revelaram hiponatremia associada à síndrome de secreção inadequada de hormônio antidiurético; os líquidos foram restritos e tratamento com furosemida e NaCl a 3% foi iniciado. No segundo dia pós-operatório, o paciente desenvolveu dormência que se propagava a partir das mãos e dos pés, perda de força, dificuldade para engolir e respirar. Suspeitou-se de síndrome de Guillain-Barré e o paciente foi transferido para a unidade de tratamento intensivo. Exame do líquido cefalorraquidiano revelou 320 mg/dL de proteína e neuropatia axonal sensório-motora aguda foi identificada por eletromiografia. Síndrome de Guillain-Barré foi diagnosticada e tratamento intravenoso com imunoglobuliva (0,4 g/kg/dia, cinco dias) foi iniciado. Após 10 dias na unidade de terapia intensiva, durante os quais os parâmetros respiratório, hemodinâmico, neurológicos e laboratoriais voltaram ao normal, o paciente foi transferido para o serviço de neurologia. Conclusões: nosso relato de caso indica que, ...

Justificación y objetivos: el síndrome de Guillain-Barré es una de las causas más comunes de polineuropatía aguda en adultos. Recientemente, la aparición del síndrome de Guillain-Barré después de cirugías mayores o menores se ha convertido en objeto de debate cada vez mayor. En el síndrome de Guillain-Barré, generalmente se observan síndrome de secreción inapropiada de hormona antidiurética y disautonomía después de un déficit motor máximo. Relato de caso: paciente de sexo masculino, 44 años, sometido a colecistectomía laparoscópica por colecistitis aguda. Después de comenzar con un fuerte dolor de cabeza, náuseas, diplopía y ataques de hipertensión en el período postoperatorio inmediato, una tomografía computadorizada del cerebro del paciente se reveló normal. Las pruebas de laboratorio revelaron hiponatremia asociada con el síndrome de secreción inadecuada de hormona antidiurética; los líquidos se restringieron y se inició tratamiento con furosemida y NaCl al 3%. En el segundo día del postoperatorio, el paciente presentó adormecimiento que se propagaba desde las manos y los pies, pérdida de fuerza, dificultad para tragar y para respirar. Se sospechó síndrome de Guillain-Barré y el paciente fue derivado a la unidad de cuidados intensivos. El examen del líquido cefalorraquídeo reveló 320 mg/dL de proteína y mediante electromiografía se identificó neuropatía axonal sensorial y motora aguda. Se diagnosticó síndrome de Guillain-Barré y se inició el tratamiento intravenoso con inmunoglobulina (0,4 g/kg/día, durante 5 días). Después de 10 días en la unidad de cuidados intensivos, durante los cuales los parámetros respiratorio, hemodinámico, neurológico ...

Guillain-Barré syndrome and its variants: a case of acute motor-sensory axonal neuropathy in Jamaica / Síndrome de Guillain-Barré y sus variantes: un caso de la neuropatía axonal sensorial motor aguda en Jamaica

This paper reports a case of a Jamaican young woman who experienced flaccid quadriparesis and bulbar weakness over a three-week period after a gastrointestinal illness. Nerve conduction studies confirmed an axonal type neuropathy consistent with the acute motor-sensory axonal neuropathy variant of the Guillain-Barré syndrome. Recovery, although evident, was slow and was augmented after a course of intravenous immunoglobulin. The patient was discharged from hospital after three months but was re-admitted one week later and eventually succumbed to complications of the illness. This case serves as a reminder that Guillain-Barré syndrome is now the most common cause of acute flaccid paralysis and should be considered early in all patients presenting with flaccid quadriparesis.

El presenta trabajo reporta el caso de una joven jamaicana que experimentó debilidad bulbar y cuadriparesiaflácida por un período de tres semanas después de una enfermedad gastrointestinal. Los estudios de conducción nerviosa confirmaron una neuropatía de tipo axonal en correspondencia con la variante de la neuropatía axonal sensorial motora aguda del síndrome de Guillain-Barré. La recuperación, aunque evidente, fue lenta, y aumentó después de que se le aplicara inmunoglobulina intravenosa. La paciente fue dada de alta del hospital después de tres meses, pero fue ingresada de nuevo una semana más tarde, falleciendo finalmente a causa de las complicaciones de la enfermedad. Este caso sirve como recordatorio de que el síndrome de Guillain-Barré es ahora la causa más común de parálisis flácida aguda, y debe tenerse en cuenta temprano en todos los pacientes que acuden con cuadriparesia flácida.

Clinical features, role of mobile plasmapharesis unit and outcome in patients with acute inflammatory demyelinating polyradiculoneuropathy [Guillain-Barr‚ syndrome]

To study the clinical features, role of mobile plasmapharesis unit and outcome in patients with acute inflammatory demyelinating polyradiculoneuropathy [AIDP]. Retrospective Cross Sectional Study. This study was conducted at Neurology Department, KEMU, Lahore from July 2008 till June 2012. Patients from various hospitals [both public and private] fulfilling the Ashbury and Cornblath's Clinical Diagnostic Criteria for GBS and requiring plasmapharesis were included in the study. For this purpose a special proforma was designed to be filled by the primary physician at the time of request for mobile plasmapharesis service. This service was provided by a donor organization namely Pakistan Myasthenia Gravis Welfare Organization [PMWO] based at a public hospital in Lahore. Plasmapharesis was started according to the guidelines, as soon as possible after admission, if patient had history of progressive weakness. The protocol of this treatment was to exchange 200 to 250 ml of plasma per kilogram of body weight in five sessions within 7 to 10 days. The replacement fluids most often consist of 0.9% normal saline, haemaccel and/or albumin. Recovery was assessed by modified Hughes Guillain-Barr‚ syndrome disability scale. A total of 152 patients were included in the study with 94 [61.8%] males and 58 [38.2%] females and M: F ratio of 1.62:1. The mean age was 32.66 [SD 15.89] with range from 7-80 years. One hundred and nine [72%] cases presented between 11-40 years of age. All patients were treated with five sessions of plasmapharesis. Drop out rate for plasmapharesis was 1.5% implying its good tolerability. Out of the total of 152 cases, 149 [98%] cases presented with progressive areflexic weakness and 3 [2%] patients with bilateral external Ophthalmoplegia, areflexia and ataxia [Miller-Fisher variant]. Sensory symptoms were present in 31[20.4%], bulbar weakness in 29[19.1%], and bilateral facial weakness in 25[16.4%] cases. Severe respiratory distress requiring ventilatory support occurred in 36[23.7%] cases. Pearson's correlation revealed that gender and age were not risk factors for the development of ventilatory failure [p=0.354; 0.803], bilateral facial weakness [p = 0.121; 0.473] or bulbar weakness [p= 0.383; 0.745] respectively. Overall mortality was 5% and all these cases developed severe respiratory distress and needed ventilatory support. Complete recovery occurred in 90% cases and 5% had residual deficit [Hughes disability scale severity 1 and 2] at mean follow up of six months. Our study showed that GBS is statistically more frequent in males than females in our local population with maximum frequency between 11-40 years of age range. However, the two factors i.e. gender and age has no significant association with the development of ventilatory failure, bilateral facial weakness and bulbar weakness. Areflexic motor weakness was the commonest presenting feature. Plasmapharesis remained very effective therapeutic option which is cheaper and affordable in our poor socio-economic setting. Mobile unit service provided an excellent opportunity to treat most of these patients at their native hospitals. We recommend that government and donor organizations should develop more mobile plasmapharesis services in all major cities which can cover nearby district and tehsil hospitals

Encefalopatía posterior reversible como primera manifestación del Síndrome de Guillain-Barré / Posterior reversible encephalopathy as the first manifestation of Guillain-Barré syndrome. Report of one case

Background: We report a 56year old male hypertensive, who presented with a posterior reversible encephalopathy syndrome (PRES) as an initial manifestation of Guillain-Barré syndrome (GBS). His first symptoms were right hemiparesis and hemihypoesthesia, followed by headache, dizziness, dysarthria and a general feeling of discomfort. On the third day, flaccid tetraparesis, impairment of consciousness, epileptic seizures and respiratory failure appeared, along with severe hypertension. Cerebral Magnetic Resonance Imaging showed the characteristic PRES lesions. Cerebrospinal fluid analyses revealed albumin-cytological dissociation and nerve conduction studies showed an axonal demyelinating polyradiculoneuropathy, which confirmed the diagnosis of GBS. Treatment with intravenous immunoglobulin was given together with antihypertensive therapy and mechanical ventilation, achieving an important clinical and imaging remission of PRES, but maintaining tetraparesis during the hospitalization. Twelve months after discharge and regular motor rehabilitation, the patient achieved complete autonomy on the activities of daily living. It has been postulated that the autonomic failure and the elevation of circulating pro-inflammatory cytokines in GBS may be the cause of a breach in the blood-brain barrier, thus causing PRES, that can completely remit with an adequate management.

Compromiso vesical en síndrome de Guillain-Barré: a propósito de un caso / Bladder involvement in Guillain-Barre syndrome: a case study

Introducción: El Síndrome de Guillain-Barré (SGB) es una enfermedad autoinmune caracterizada por debilidad muscular, arreflexia y disociación albúmino-citológica en líquido cerebroespinal, cuya incidencia clásica a nivel internacional suele uniformarse alrededor de 0,6 a 4 casos por 100.000 al año. Presentación del caso: Paciente de 45 años, sexo femenino, quien ingresó al Servicio de Urgencia del Hospital de Temuco derivada desde Lonquimay, por debilidad progresiva de las cuatro extremidades. En Lonquimay la paciente requirió sonda vesical por retención urinaria. Es diagnosticada con SGB, hospitalizándose en Unidad de Cuidados Intensivos, requiriendo ventilación asistida y tratada mediante plasmaféresis, presentando neumonía como complicación relacionada a la ventilación mecánica y recuperando íntegramente función vesical. Discusión: El compromiso vesical no es descrito como un factor importante en el diagnóstico, siendo controversial aquella afirmación por varios autores.

Introduction: Guillain-Barre syndrome (GBS) is an autoimmune disease characterized by muscle weakness, areflexia and albumin-cytological dissociation in cerebrospinal fluid, the incidence has been reported to be relatively uniform between 0.6 to 4 cases per 100.000 per year. Case report: Patient of 45 years old, female, admitted to the emergency department of Hospital de Temuco derived from Lonquimay by progressive weakness of four extremities. In Lonquimay the patient required bladder catheterization for urinary retention. It was diagnosed with GBS, hospitalized in intensive care unit, requiring assisted ventilation and treated with plasmapheresis, developing pneumonia as a complication related to mechanical ventilation and fully recovered bladder function. Discussion: The bladder involvement is not described as an important factor in diagnosis and remains controversial this statement by several authors.

Asociación de encefalomielitis diseminada aguda y síndrome de Guillain-Barré en un adulto / Association between acute disseminated encephalomyelitis and Guillain Barré syndrome in an adult

La encefalomielitis diseminada aguda (EMDA) y el síndrome de Guillain-Barré (SGB) son reconocidas como entidades distintas, que afectan diferentes sectores del sistema nervioso, pero que comparten varias características tales como la patogenia autoinmune, el impacto sobre la mielina y el antecedente de infección viral o vacunación una a cuatro semanas previas al cuadro clínico. Se presenta un paciente varón de 41 años de edad que consultó por presentar fiebre, debilidad en miembros inferiores y somnolencia dos semanas posteriores a episodio agudo de gastroenteritis. Al ingreso se constató deterioro del sensorio (obnubilación) hiperreflexia patelar, Babinski bilateral y vejiga neurogénica. Veinticuatro horas después desarrolló paraplejía flácida y arreflexia generalizada, requiriendo asistencia respiratoria mecánica por insuficiencia respiratoria. El líquido cefalorraquídeo mostró pleocitosis mononuclear e hiperproteinorraquia. El estudio electrofisiológico evidenció importante disminución de las velocidades de conducción en ambos nervios ciáticos poplíteos externos, compatible con polineuropatía desmielinizante. La resonancia magnética nuclear mostró imágenes compatibles con desmielinización en cerebro, protuberancia y segmentos medulares dorsales. Se realizó diagnóstico de ASEMDA-SGB e inició tratamiento con metilprednisolona e inmunoglobulina intravenosa. Evolucionó favorablemente, recuperando las funciones motoras, vesical y la sensibilidad, siendo capaz de deambular luego de seis meses. La asociación de EMDA y SGB (ASEMDA-SGB) es una condición infrecuente, generalmente señalada como de mal pronóstico, en la cual un diagnóstico precoz y un rápido y enérgico tratamiento pueden mejorar substancialmente la evolución.

Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré Syndrome (GBS) are commonly recognized as separated entities involving different parts of the nervous system. However, they share some features such as: autoimmune pathogenesis, myelin injury and previous history of viral infections or vaccination. We report the case of a 41 year-old man who developed fever, lower limbs weakness and obtundation fifteen days after an acute gastroenteritis. Neurological examination showed patellar hypereflexia, bilateral Babinski and neurogenic bladder. Twenty-four hours later he developed flaccid paraparesis, generalized areflexia and respiratory failure that was supported by mechanical ventilation. Cerebrospinal fluid showed mononuclear pleocytosis and elevated proteins. Electrodiagnosis showed important reduction of conduction velocity on both peroneal nerves. Magnetic Resonance Imaging revealed white matter lesions in brain, pons and thoracic levels of the spinal cord. Diagnosis of the association between ADEM and GBS (ASADEM-GBS) was made and treatment with corticosteroids and intravenous immunoglobulin was started. The patient recovered motor, sensory and bladder functions and he was able to walk six months later. ASADEM-GBS is an uncommon entity generally considered of poor outcome; however a rapid diagnosis and treatment can substantially improve the prognosis.