Arboviruses epidemic associates with Guillain-Barré syndrome: temporal series in northeast Brazil / Epidemia de arboviroses associada à síndrome de Guillain-Barré: série temporal no nordeste brasileiro / Epidemia de arbovirosis asociada al síndrome de Guillain-Barré: serie temporal en el noreste de Brasil

Arboviruses cause public health problems in several countries, and records show that they can generate central and peripheral neurological complications with permanent sequelae. However, it is not certain which arbovirus is responsible for outbreaks of the Guillain-Barré Syndrome (GBS), especially in Brazil. Thus, the objective of this study is to verify if there is a coincidence between the GBS outbreak and the most common arboviruses in Northeastern Brazil, as well as their relationship. An ecological time series study was designed with the federative units of Northeastern Brazil, using hospitalizations for Guillain-Barré syndrome and notifications of arbovirus infections between 2014 and 2019 as a data source. Distribution incidence curves were constructed for the conditions studied, and generalized estimating equations (GEE) models were applied to estimate the relationship between arboviruses and Guillain-Barré. The results showed a similar distribution for the incidences of Chikungunya virus (z=7.82; p=0.001), Zika virus (z=3.69; p=0.03), and Guillain-Barré syndrome (z=2.98; p=0.05) from 2014 to 2019. The GEE model revealed that the distribution of Chikungunya incidence is associated with the distribution of GBS incidence in each year (x2Wald=3,969; p=0.046). This pattern was repeated in seven of the nine states, while the Zika virus had a significant relationship with GBS in only two states. The outbreak of GBS in Northeastern Brazil appears to be probabilistically related to outbreaks of the Chikungunya virus.

As arboviroses são problemas de saúde pública em vários países e há registros de que podem produzir complicações neurológicas centrais e periféricas com sequelas permanentes. Entretanto, não se sabe ao certo qual delas é realmente responsável pelos surtos da Síndrome de Guillain-Barré (SGB), principalmente no Brasil. Assim, o objetivo é verificar se há coincidência entre o surto de SGB e as arboviroses mais comuns no Nordeste do Brasil e suas relações. Foi desenhado um estudo ecológico de série temporal com as unidades federativas do Nordeste do Brasil, adotando como fonte de dados as internações Guillain-Barré e as notificações de infecções por arbovírus entre 2014 e 2019. Curvas de distribuição de incidência foram construídas para as condições estudadas, e foram aplicados modelos de equações generalizadas estimadas (GEE) para estimar a relação entre arbovírus e Guillain-Barré. Evidencia-se que há distribuição semelhante para as incidências do vírus Chikungunya (z=7,82; p=0,001), vírus Zika (z=3,69; p=0,03) e síndrome de Guillain-Barré (z=2,98; p=0,05) entre 2014 e 2019. O modelo GEE revelou que a distribuição da incidência de Chikungunya está associada à distribuição da incidência de SGB em cada ano (x2Wald=3,969; p=0,046). Esse padrão se repetiu em sete dos nove estados, enquanto o zika vírus teve uma relação significativa com o GBS em apenas dois estados. Conclui-se, então, que o surto de SGB no Nordeste do Brasil parece estar probabilisticamente relacionado aos surtos do vírus Chikungunya.

Los arbovirus causan problemas de salud pública en varios países y, según indican los reportes, pueden producir complicaciones neurológicas centrales y periféricas con secuelas permanentes. Sin embargo, no se sabe cuál de ellos es realmente el responsable de los brotes del síndrome de Guillain-Barré (SGB), especialmente en Brasil. Así, el objetivo de este estudio es verificar si existen coincidencias entre el brote del SGB y los arbovirus más comunes en el Noreste de Brasil y sus asociaciones. Se diseñó un estudio de series temporales ecológico en las unidades federativas del Noreste de Brasil, adoptando como fuente de datos las hospitalizaciones y las notificaciones de arbovirosis de Guillain-Barré entre 2014 y 2019. Se construyeron curvas de distribución de incidencia para las condiciones científicas, y se aplicó una ecuación estimada generalizada (GEE) para estimar la relación entre arbovirus y Guillain-Barré. Se encontró que existe una distribución similar en las incidencias de virus del chikunguña (z=7,82; p=0,001), virus del Zika (z=3,69; p=0,03) y síndrome de Guillain-Barré (z =2,98; p=0,05) entre 2014 y 2019. El modelo GEE reveló que la distribución de la incidencia de chikunguña está asociada con la distribución de la incidencia de SGB en cada año (x2Wald=3,969; p=0,046). Este patrón se repitió en siete de los nueve estados, mientras que el virus del Zika presentó una relación significativa con el SGB en solo dos estados. El brote del SGB en el Noreste de Brasil parece estar relacionado probabilísticamente con los brotes del virus del chikunguña.

Diplejía facial en paciente con nefropatía por virus de inmunodeficiencia humana

Introducción: El síndrome de Guillain-Barré comprende un grupo heterogéneo de polirradiculoneuropatías inflamatorias agudas autoinmunes, las cuales se caracterizan por debilidad simétrica de extremidades con pérdida de reflejos miotáticos. Presenta gran variabilidad clínica, donde la afectación facial es habitual, sin embargo, incluye manifestaciones atípicas que dificultan el diagnóstico temprano de la enfermedad. Objetivo: Describir el comportamiento de un caso atípico de diplejía facial en un paciente con nefropatía por virus de inmunodeficiencia humana. Caso clínico: Se presentó el caso de un paciente portador de nefropatía por virus de la inmunodeficiencia humana, que comenzó con parálisis facial bilateral, como forma de presentación atípica de este síndrome. Acudió a los servicios de salud refiriendo decaimiento marcado, náuseas, vómitos, hipo y dos deposiciones líquidas. Se realizaron estudios que corroboran el diagnóstico. El tratamiento empleado facilitó la recuperación paulatina de la afección. Conclusiones: El diagnóstico precoz de las variantes atípicas de presentación del síndrome, permite un tratamiento oportuno, donde las posibilidades de complicaciones en el paciente son reducidas, así como la mortalidad asociada a la enfermedad.

Introduction: Guillain-Barré Syndrome comprises a heterogeneous group of autoimmune acute inflammatory polyradiculoneuropathies, which are characterized by symmetrical limb weakness with loss of stretch reflexes. It presents great clinical variability, where facial involvement is common; however, it includes atypical manifestations that make early diagnosis of the disease difficult. Objective: To describe the behavior of an atypical case of facial displejía in a patient with nephropathy due to Human Immunodeficiency Virus. Case report: A case of a patient with HIV nephropathy is presented, which begins with bilateral facial paralysis, as an atypical presentation of this syndrome. The patient went to the health services reporting marked weakness, nausea, vomiting, hiccups and two liquid stools. Studies were performed that corroborate the diagnosis. The treatment used facilitated the gradual recovery of the condition. Conclusions: Early diagnosis of the atypical presentation variants of the syndrome allows timely treatment, where the chances of complications in the patient are reduced, as well as the mortality associated with the disease.

Características clínicas y laboratoriales de los diez primeros pacientes con diagnóstico de Guillain-Barré en Piura, 2023

El síndrome de Guillain Barré es una enfermedad derivada del compromiso en las neuronas del sistema nervioso periférico por una respuesta descontrolada del sistema inmune que conduce daño axonal y/o desmielinización. El objetivo de este reporte fue describir los 10 primeros casos sospechosos de Síndrome de Guillain Barré en Piura. Se logró identificar la presencia de Campylobacter jejuni en las muestras de heces del 80% de los pacientes reportados. Es muy importante reconocer rápida y oportunamente al paciente con diagnóstico sospechoso de Guillain Barré, y realizar los estudios necesarios en un brote para identificar los agentes desencadenantes del cuadro.

Guillain Barré syndrome is a disease derived from compromise in neurons of the peripheral nervous system by an uncontrolled response from the immune system that leads to axonal damage and/or demyelination. The objective of this report was to describe the first 10 suspected cases of Guillain Barre Syndrome in Piura. It was possible to identify the presence of Campylobacter jejuni in the stool samples of 80% of the reported patients. It is very important to quickly and opportunely recognize the patient with a suspected diagnosis of Guillain Barré, and to carry out the necessary studies in an outbreak to identify the triggering agents of the condition.

Síndrome de Guillain-Barré recurrente asociado a vacuna anti-COVID-19. Informe de caso

El Síndrome de Guillain-Barré constituye la causa más frecuente de parálisis flácida aguda en el mundo. Su diagnóstico es clínico y debe ser respaldado por estudios del líquido cefalorraquídeo y de conducción nerviosa. Se produce como consecuencia de una respuesta inmune cruzada ante antígenos de diferentes patógenos, y se ha asociado también a inmunizaciones previas. Existe escasa literatura sobre su recurrencia asociado a la vacunación anti-COVID-19 en el mundo, y hasta donde los autores tienen conocimiento, este es el único informe asociado a la vacuna Abdala (CIGB-66). Se presenta el caso de un paciente masculino de 55 años de edad que refirió pérdida aguda de la fuerza muscular, el cual presentó complicaciones durante su estancia en la Terapia Intensiva, asociadas a la ventilación mecánica. La dificultad en realizar un diagnóstico rápido y certero, especialmente en países subdesarrollados, donde los estudios especializados no se encuentran siempre disponibles y debe hacerse un diagnóstico eminentemente clínico, motivó a los autores a presentar este caso atípico.

Guillain-Barré Syndrome is the most frequent cause of acute flaccid paralysis in the world. Its diagnosis is clinical and must be supported by studies of the cerebrospinal fluid and nerve conduction. It is produced as a consequence of a crossed immune response to antigens from different pathogens, and it has also been associated with previous immunizations. There is little literature on its recurrence associated with anti-COVID-19 vaccination in the world, and as far as the authors are aware, this is the only report associated with the Abdala vaccine (CIGB-66). We present the case of a 55-year-old male patient who reported acute loss of muscle strength, who presented complications during his stay in the Intensive Care Unit, associated with mechanical ventilation. The fact that it is so difficult to give a quick and accurate diagnosis, especially in underdeveloped countries, where specialized studies are not always available and an eminently clinical diagnosis must be made motivated the authors to present this atypical case.

A Síndrome de Guillain-Barré é a causa mais frequente de paralisia flácida aguda no mundo. Seu diagnóstico é clínico e deve ser apoiado por estudos do líquido cefalorraquidiano e da condução nervosa. É produzida como consequência de uma resposta imune cruzada a antígenos de diferentes patógenos, e também tem sido associada a imunizações anteriores. Há pouca literatura sobre sua recorrência associada à vacinação anti-COVID-19 no mundo e, até onde os autores sabem, este é o único relato associado à vacina Abdala (CIGB-66). Apresentamos o caso de um paciente do sexo masculino, 55 anos, que relatou perda aguda de força muscular, que apresentou complicações durante sua internação na Unidade de Terapia Intensiva, associada à ventilação mecânica. A dificuldade de um diagnóstico rápido e preciso, principalmente em países subdesenvolvidos, onde nem sempre há estudos especializados e deve ser feito um diagnóstico eminentemente clínico, motivou os autores a apresentarem este caso atípico.

Síndrome de Guillain-Barré antes y durante la pandemia de COVID-19 en un centro de referencia de México / Guillain-Barre syndrome before and during the COVID-19 pandemic in a referral center of Mexico

Resumen Antecedentes: Durante la pandemia de COVID-19 se ha reportado incremento de casos de síndrome de Guillain-Barré (SGB). Objetivo: Describir características clínicas y pronóstico de pacientes con SGB antes y durante la pandemia de COVID-19. Material y métodos: Cohorte prospectiva de pacientes con SGB estratificados en dos subgrupos: antes (2018-2019) y durante (2020-2021) la pandemia de COVID-19. Se registraron características clínicas, paraclínicas y defunciones. Se definió como buen pronóstico a la recuperación de la marcha independiente a los tres meses. Resultados: Se incluyeron 201 pacientes (123 durante la pandemia y 78 antes), 69 % del sexo masculino, edad de 45 ± 16 años, 2.5 % de muertes intrahospitalarias. Durante la pandemia se observó mayor frecuencia de la variante desmielinizante (50 %), afección de nervios craneales bulbares (44 % versus 28 %), antecedente de vacunación (16 % versus 0 %) y menor puntuación en la escala MRC (30 ± 16.7 versus 34.3 ± 17.7); se observó aumento de casos de julio a septiembre (38 versus 13). No existieron diferencias significativas en la recuperación de la marcha independiente y número de defunciones. Conclusiones: Durante la pandemia se atendió mayor número de casos de SGB, 16 % asociado a la vacuna contra SARS-CoV-2; los pacientes no presentaron peor pronóstico.

Abstract Background: During the COVID-19 pandemic, an increase in the number of Guillain-Barre syndrome (GBS) cases has been reported. Objective: To describe the clinical characteristics and prognosis of patients with GBS before and during the COVID-19 pandemic. Material and methods: Prospective cohort of GBS patients divided in two subgroups: before (2018-2019) and during (2020-2021) the COVID-19 pandemic. Clinical and paraclinical characteristics, as well as deaths, were recorded. A good prognosis was defined as independent ambulation recovery at three months. Results: Two-hundred and one patients were included (123 during and 78 before the pandemic), out of whom 69 % were males; age was 45 ± 16 years, and there was 2.5 % of in-hospital deaths. During the pandemic, a higher frequency of the demyelinating variant (50 %), bulbar cranial nerves involvement (44 % vs. 28 %), prior history of vaccination (16 % vs. 0 %), and a lower MRC score (30 ± 16.7 vs. 34.3 ± 17.7) were documented. An increase in the number of cases was observed from July to September (38 vs. 13). There were no significant differences in independent ambulation recovery or in the number of deaths. Conclusions: During the COVID-19 pandemic, a higher number of GBS cases were treated, out of which 16 % were associated with the SARS-CoV-2 vaccine; patients treated during the pandemic did not have a worse prognosis.

Independência funcional de pacientes com síndrome de Guillain-Barré internados para reabilitação / Functional independence of patients with Guillain-Barré syndrome hospitalized for rehabilitation

Objetivo: Analisar a evolução da independência funcional de pacientes com Síndrome de Guillain-Barré (SGB) internados para reabilitação. Método: Estudo retrospectivo e longitudinal. Foram analisados prontuários de pacientes com diagnóstico de SGB internados para reabilitação, de janeiro de 2015 a março de 2020, que possuíam a Medida de Independência Funcional (MIF) na admissão e alta hospitalar devidamente preenchida, sem distinção de idade. A comparação da MIF antes e depois da internação para reabilitação foi feita por meio do teste t de Student e McNemar. Resultados: A amostra foi composta por 26 pacientes, com média de idade de 41,96 ± 19,67 anos. Os participantes tinham em média 66,07 ± 69,56 dias entre os primeiros sintomas e a admissão para internação para reabilitação. O tempo médio de internação foi de 38,96± 28,36 dias. Houve diferença significativa entre as médias das pontuações, na admissão e alta, nos domínios motor (37,58 - 59,62; p<0,001) e cognitivo (23,19 - 33,35; p<0,001) e escore total da MIF (60,77 - 92,96; p<0,001). Observou-se também aumento do número de pacientes com independência completa após internação para reabilitação (2 - 15; p<0,001). Conclusão: Pacientes com SGB internados para reabilitação apresentam melhora da independência funcional. É importante acesso a esta modalidade terapêutica multiprofissional aos pacientes com SGB.

Objective: To analyze the evolution of the functional independence of patients with Guillain-Barré Syndrome (GBS) hospitalized for rehabilitation. Method: Retrospective and longitudinal study. Medical records of patients with diagnosis of GBS hospitalized for rehabilitation were analyzed, from January 2015 to March 2020, who had the Functional Independence Measure (FIM) at admission and hospital discharge duly filled, regardless of age. The comparison of FIM before and after hospitalization for rehabilitation was performed using Student's t-test and McNemar's test. Results: The sample consisted of 26 patients, with a mean age of 41.96 ± 19.67 years. Participants had a mean of 66.07 ± 69.56 days between the first symptoms and hospital admission for rehabilitation. The mean length of stay was 38.96 ± 28.36 days. There was a significant difference between the mean scores, at admission and discharge, in the motor domain (37.58-59.62; p<0.001) and cognitive domain (23.19-33.35; p<0.001) and total FIM score (60.77-92.96; p<0.001). There was also an increase in the number of patients with complete independence after hospitalization for rehabilitation (2-15; p<0.001). Conclusion: Patients with GBS hospitalized for rehabilitation show improved functional independence. Access to this multiprofessional therapeutic modality is important for patients with GBS.

Concurrencia de síndrome de Guillain-Barré y colangitis biliar primaria no vinculado a SARS-CoV-2: reporte de caso / Concurrence of Guillain-Barré syndrome and primary biliary cholangitis not related to SARS-CoV-2: Case report

Introducción El síndrome de Guillain-Barré es una polirradiculoneuropatia de origen autoinmune, considerada la causa más frecuente de parálisis flácida aguda. Se han reportado diversas asociaciones del síndrome de Guillain-Barré con otras enfermedades autoinmunes no neurológicas, algunas de ellas extremadamente raras, como la que ocurre con la colangitis biliar primaria, una enfermedad crónica de etiología autoinmune cuyo diagnóstico se sustenta, además del cuadro clínico, en la alteración de las enzimas hepáticas y la presencia de anticuerpos anti-mitocondriales. Caso clínico Paciente varón de 38 años, sin antecedente de comorbilidades previas, quien luego de presentar enfermedad diarreica dos semanas antes, desarrolló debilidad ascendente de inicio subagudo asociado a parestesias en cuatro extremidades que progresó hasta generar cuadriplejia y dificultad respiratoria. Se le realizó examen citoquímico de líquido cefalorraquídeo que evidenció disociación albumino-citológica y electromiografía que mostró hallazgos compatibles con neuropatía axonal motora aguda. Recibió tratamiento con inmunoglobulina intravenosa a dosis de 0,4 gramos por kilogramo al día, logrando mejoría del cuadro neurológico. Desde su ingreso y durante la hospitalización, presentó alteración persistente de las enzimas hepáticas que seguía un patrón colestásico. Además, se agregó dolor abdominal de leve intensidad y prurito generalizado, por lo cual fue evaluado por gastroenterología, quienes solicitaron anticuerpos anti-mitocondriales que resultaron positivos. Con esta prueba, se comprobó el diagnóstico de colangitis biliar primaria. Conclusión El presente caso muestra una asociación extremadamente rara de dos enfermedades autoinmunes; síndrome de Guillain-Barré y colangitis biliar primaria, tanto así que representa el primer caso reportado, no vinculado a SARS-CoV-2.

Introduction Guillain-Barré syndrome is a polyradiculoneuropathy of autoimmune origin, considered the most frequent cause of acute flaccid paralysis. Various associations of Guillain-Barré syndrome with other non-neurological autoimmune diseases have been reported, some of them extremely rare, such as that which occurs with primary biliary cholangitis, a chronic disease of autoimmune etiology whose diagnosis is also supported by the clinical picture. , in the alteration of liver enzymes and the presence of anti-mitochondrial antibodies. Clinical case A 38-year-old male patient, with no history of previous comorbidities, who, after presenting with diarrheal disease two weeks prior, developed subacute onset ascending weakness associated with paresthesias in four extremities that progressed to quadriplegia and respiratory distress. Cerebrospinal fluid cytochemistry was performed, which showed albuminocytological dissociation and electromyography, which showed findings compatible with acute motor axonal neuropathy, for which he received treatment with intravenous immunoglobulin at 0.4g/kg/day, achieving improvement in the neurological condition. Since admission and during hospitalization, he presented persistent changes in liver enzymes which followed a cholestatic pattern, in addition to mild abdominal pain and generalized itching, for which he was evaluated by gastroenterology, who requested anti-mitochondrial antibodies that were positive. Concluding in the diagnosis of primary biliary cholangitis. Conclusion The present case shows an extremely rare association of two autoimmune diseases Guillain-Barré syndrome and primary biliary cholangitis, so much so that it represents the first case reported, not linked to SARS-CoV-2.

Neuromuscular Alterations Associated with COVID-19. A Scientific Literature Review

Introduction: The COVID-19 pandemic has brought short, medium, and long-term consequences on the quality of life of those affected. Among the complications are those related to the involvement of the nervous system and the structures involved in body movement, with sequelae that may be transitory and/or definitive, and require rehabilitation. Objective: Identify the neuromuscular alterations that affect body movement, associated with COVID-19. Material and methods: A search was made for observational works published in the SCOPUS, PubMed, EBSCO, and Nature databases between January 2020 and June 2022 under the PRISMA methodology, to answer the PICO question: what are the neuromuscular alterations that can potentially affect movement, associated with COVID-19? The established filters were type of study, language, age, availability, publication dates. The MeSH terms were SARS-Cov-2, COVID-19, Long Covid, Motor Activity Neuromuscular Diseases, Neurological Disorders, Guillain-Barré Syndrome, Myelitis Transverse, Stroke, Patient, Peripheral Nervous System Diseases. The methodological quality was evaluated according to STROBE and the level of evidence was established according to CEBM. Results: In the first search, 645 articles were identified. 637 were discarded by filters, titles, duplicate abstracts, methodological quality, and level of evidence. There were 8 articles selected for the present review in which neuromuscular alterations of central and peripheral origin were identified, such as myalgias, fatigue, polyneuroradiculopathies, CNS inflammation, among others, with clinical manifestations that affect movement. Conclusion: COVID-19 is a multisystemic disease that can affect the nervous system with symptoms of neuromuscular alterations that compromise body movement.

Introducción: La pandemia por la COVID-19 ha traído consecuencias a corto, mediano y largo plazo sobre la calidad de vida de los afectados. Entre las complicaciones se encuentran aquellas relacionadas con la afectación del sistema nervioso y las estructuras involucradas en el movimiento corporal, con secuelas que pueden ser transitorias y/o definitivas, y requieren rehabilitación Objetivo: Identificar las alteraciones neuromusculares que afectan el movimiento corporal, asociadas a la COVID-19. Material y métodos: Se hizo una búsqueda de trabajos observacionales publicados en las bases de datos SCOPUS, PubMed, EBSCO y Nature entre enero de 2020 y junio de 2022 bajo metodología PRISMA, para dar respuesta a la pregunta PICO: ¿cuáles son las alteraciones neuromusculares que potencialmente pueden afectar el movimiento, asociadas a la COVID-19? Los filtros establecidos fueron tipo de estudio, idioma, edad, disponibilidad y fechas de publicación. Los términos MesH fueron SARS-Cov-2, COVID-19, Long Covid, Motor Activity Neuromuscular Diseases, Neurological Disorders, Guillain-Barré Syndrome, Myelitis Transverse, Stroke, Patient, Peripheral Nervous System Diseases. La calidad metodológica se evaluó según STROBE y el nivel de evidencia se estableció según CEBM. Resultados: En la primera búsqueda se identificaron 645 artículos. Posteriormente se descartaron 637 por filtros, títulos, resúmenes duplicados, calidad metodológica y nivel de evidencia. Así, quedaron seleccionados 8 para la presente revisión, en los cuales se identificaron alteraciones neuromusculares de origen central y periférico, como mialgias, fatiga, polineuroradiculopatías, inflamación del SNC, entre otras, con manifestaciones clínicas que afectan el movimiento. Conclusión: La COVID-19 es una enfermedad multisistémica que puede afectar el sistema nervioso con síntomas de alteraciones neuromusculares que comprometen el movimiento corporal.

Miositis viral aguda asociada a síndrome de Guillain-Barré

Introducción: Las manifestaciones clínicas del síndrome de Guillain Barré y de la miositis viral aguda son diferentes. Ambos cuadros suelen ser autolimitados y pueden manifestarse tras un cuadro infeccioso. Objetivo: Informar acerca de una presentación inusual de dos afecciones en un mismo paciente. Presentación del caso: Niña de 4 años de edad, quien presenta síntomas de una miositis viral aguda, la que al mejorar, hizo más evidente la sintomatología característica del síndrome de Guillain Barré. El caso se confirmó a través de estudios hematológicos, citología de líquido cefalorraquídeo y estudios neurofisiológicos. La paciente recibió tratamiento con inmunoglobulina G humana intravenoso durante 5 días, tras lo cual presentó mejoría de los síntomas neurológicos. Conclusiones: La afectación del músculo y del sistema nervioso periférico pueden coexistir en la población pediátrica, pero no es condición usual, por lo que se necesita realizar más investigación para poder describir mejor esta entidad y su pronóstico.

Introduction: The clinical manifestations of Guillain Barré syndrome and acute viral myositis are different. Both conditions are usually self-limiting and can manifest after an infectious process. Objective: To report an unusual presentation of two conditions in the same patient. Case presentation: A 4-year-old girl presented with symptoms of acute viral myositis, which, with improvement, made more evident the characteristic symptoms of Guillain Barré syndrome. The case was confirmed through hematological studies, cerebrospinal fluid cytology and neurophysiological studies. The patient was treated with intravenous human immunoglobulin G for 5 days, after which she showed improvement of the neurological symptoms. Conclusions: The involvement of the muscle and the peripheral nervous system may coexist in the pediatric population, but it is not a usual condition, so more research is needed to better describe this entity and its prognosis.

Síndrome de Guillain barre asociado a infección por sars-cov-2: a propósito de un caso / Guillain-Barré Syndrome Associated with SARS-CoV-2 infection: A pediatric presentation

Introducción: la pandemia de SARS-CoV-2 exige una medicina fiable, basada en evidencia para poder comprender y tratar oportunamente las nuevas manifestaciones de esta enfermedad, previniendo así las complicaciones y la mortalidad infantil. Materiales y método: femenina de seis años ingresada en urgencias con síntomas neurológicos de entumecimiento en extremidades inferiores y superiores, ataxia, somnolencia y disartria. Después de un análisis de sangre, un examen del líquido cefalorraquídeo y una prueba de electromiografía, la paciente fue diagnosticada con síndrome de Guillain-Barré e infección concomitante por COVID-19. Resultados y discusión: se obtuvieron resultados positivos, tanto para el anticuerpo IgM como para la RT-PCR, para infección por SARS-CoV-2. No se encontró evidencia de celularidad en el examen del líquido cefalorraquídeo, pero se observó un alto nivel de proteína de 100 mg/dL y un nivel anormal de glucosa (58 mg/dL) en este paciente. Por último, se administró una terapia consistente en dexametasona 3,5 mg/kg, paracetamol 315 mg/kg, azitromicina 105 mg/kg y 50 mg/kg de inmunoglobulina intravenosa. Conclusión: considerando este caso clínico, reforzamos la hipótesis de la asociación entre el síndrome de Guillain-Barré y la infección por el virus SARS-CoV-2, como ya han documentado otros autores, tanto en adultos como en pacientes pediátricos.

Introduction: Ongoing SARS-CoV-2 pandemic calls for trustworthy, evidence-based medicine to be able to comprehend and treat opportunely new manifestations of this disease, therefore preventing complications and children mortality. Materials and methods: This case report adresses a unique presentation of Guillain-Barre syndrome (GBS) & COVID-19. Results: The patient was a 6-year-old girl admitted to the emergency room with neurological symptoms numbness in lower and upper extremities, ataxia, drowsiness, and dysarthria. After blood work, cerebrospinal fluid examination, and electromyography test, the patient was diagnosed with Guillain-Barre syndrome and concomitant COVID-19 infection. Both IgM antibody and RT-PCR were positive for SARS-coV-2 infection and no evidence of cellularity was found in cerebrospinal fluid examination, but a high protein level of 100 mg/dL and an abnormal glucose level (58mg/dL) was observed within this patient. Lastly, a therapy consisted of dexamethasone 3.5mg/kg, acetaminophen 315 mg/kg, azithromycin 105mg/kg and 50 mg/kg of intravenous immune globulin was administered. Conclusion: Considering this clinical case, we reinforce the hypothesis of the association between Guillain-Barré syndrome and SARS-CoV-2 virus infection, as has already been documented by other authors in both adults and pediatrics patients.

A case of recurrent Miller-Fisher syndrome with multiple anti-ganglioside antibodies / 中国综合临床

Guillain-Barré syndrome (GBS) defines a kind of Immune-mediated acute inflammatory peripheral neuropathy. Miller-Fisher Syndrome (MFS) is a special variant of GBS, with mostly one-way course and rare clinical recurrence. Only a few recurrent cases have been reported in China. Here we report a case of a young male patient with double vision and progressive aggravation of limb numbness, acute onset, with symptoms of upper respiratory tract infection before onset, accompanied by pupil abnormalities and autonomic nervous dysfunction, who was was admitted to our hospital for similar symptoms 3 years ago and was improved by immunotherapy. The patient had a triad of “ataxia, areflexia and ophthalmoplegia”. Cerebrospinal fluid showed protein-cell separation. Serum anti-Sulfatides antibody IgM, anti-GT1a antibody IgG, anti-GQ1b antibody IgG and anti-GM3 IgM were positive. Recurrent MFS was diagnosed and the symptoms improved after immunotherapy. This case suggests that MFS is clinically heterogeneous, a few patients can present with relapse and generally have a better prognosis with immunotherapy. Pre-existing infection and anti-GQ1b antibody production may be predisposing factors for MFS recurrence.

Guillain-Barré syndrome / 中华神经科杂志

Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots, which is usually triggered by infections. It is characterized by rapidly progressive, symmetrical weakness of the extremities. Some patients develop respiratory insufficiency and many show signs of autonomic dysfunction. Diagnosis can usually be made on clinical grounds, but lumbar puncture and electrophysiological studies can help to substantiate the diagnosis and to differentiate demyelinating from axonal subtypes of GBS. Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is generally accepted pathogenesis of GBS. The treatment of GBS is intravenous immunoglobulin or plasma exchange with general clinical treatment. Most patients have a good prognosis and basically recover within weeks to months. A few patients have persistent neurological dysfunction or even death.

Optic neuritis in Guillain-Barré syndrome: a case report and literature review / 中华神经科杂志

Objective:To summarize the clinical characteristics of patients with Guillain-Barré syndrome (GBS) complicated with optic neuritis (ON).Methods:The clinical data of a patient with GBS complicated with ON, who admitted to the Department of Neurology, the First Hospital of Shanxi Medical University in December 2021, were collected, including demographic characteristics, clinical symptoms and signs, laboratory and electrophysiological data, and results of fundus color films. The patients with GBS complicated with ON reported in the literature were also reviewed.Results:A 40-year-old female patient with GBS was diagnosed by the results of electromyography and cerebrospinal fluid tests combining with the history and signs, who was treated with intravenous immunoglobulin on the 3rd day after onset. On the 8th day, her muscle strength improved significantly. However, on the 12th day, the visual field darkened, and on the 19th day, the vision decreased significantly (oculus dexter visual acuity 0.2, oculus sinister visual acuity 0.1 +1) with bilateral papilloedema, a relative afferent pupillary defect and delayed P100 response of the visual evoked potential. Obvious abnormality was not noted in optic nerve magnetic resonance imaging. Thus ON was diagnosed and treated with pulse methylprednisolone therapy. After 8 days of treatment, the visual acuity was completely recovered and there was no abnormality in the ocular fundus. A total of 28 cases of GBS complicated with ON (including the present patient) were reported in the literature. The age of onset was mostly 20-60 years, and there was no gender preference. Mycoplasma pneumoniae was the most common premorbid pathogen and was identified in 7 of the 10 cases in which the causative agent was described. ON usually involved both sides, and 21 of 28 patients had bilateral optic nerves involved. GBS preceded ON or both occurred simultaneously in the majority of patients; GBS preceded ON in 14 of 28 patients, and both occurred simultaneously in 10 of 28 patients. All patients responded well to immunotherapy, and vision was completely recovered in 20 patients. Conclusions:GBS complicated with ON is rare. Attention should be paid to the loss of vision in patients with GBS. Relevant examinations should be completed as soon as possible and immunotherapy should be given.

The autopsy is not dead: ongoing relevance of the autopsy

ABSTRACT Background Autopsy requests have been trending downward for a variety of factors. There are differences between pre- and postmortem diagnoses. Autopsies remain a tool for education, public health research, quality control, and closure for families. Objective We report two cases that illustrate the utility of autopsy for uncovering contributing factors in the death of these patients and highlight their ongoing importance. Design Clinical and autopsy investigation of two individuals and illustration of the importance of autopsy findings which, had they been diagnosed premortem, could have changed the outcome. Cases were evaluated using the Goldman criteria for discrepancies between premortem clinical diagnoses and postmortem autopsy findings. Results In the first case, the patient had been previously admitted due to a non-ST elevation myocardial infarction months before the fatal event. The autopsy showed an undiagnosed clear cell carcinoma of the ovary. She expired due to a massive myocardial infarction secondary to neoplasm induced hypercoagulable state. The degree of pre-mortem/postmortem diagnostic discrepancy makes this a Goldman Class I error. In the second case, the patient presented to the emergency department with symptoms of Guillain-Barré Syndrome (GBS), for which he was treated. Abdominal masses were discovered; however, the patient decompensated before workup was completed. A high-grade B-cell lymphoma was confirmed but would not have altered the outcome, making this a Goldman class II error. Conclusions The autopsy remains a relevant and necessary tool for physicians and society. It assists in the establishment of diagnoses, measurement of treatment quality, the providence of public health metrics, and closure to the survivors.

Síndrome de Guillain-Barré como manifestación neurológica autoinmune asociado a COVID-19: Una revisión de la literatura

La enfermedad por coronavirus (COVID-19) producida por el SARS-CoV-2 ha sido un reto para los servicios de salud en todo el mundo. La pandemia se ha extendido ampliamente con más de 80 millones de casos confirmados y más de un millón de muertes a nivel mundial, por lo que ha estado bajo constante investigación para entender todos los aspectos de la enfermedad. Recientemente se han reportado varios casos de pacientes con síndrome de Guillain-Barré asociado a COVID-19 como manifestación principal, convirtiéndola en la primera enfermedad neurológica autoinmune desencadenada por SARS-CoV-2; sin embargo, es necesario obtener más información para entender completamente los mecanismos inmunopatogénicos implicados en esta asociación.

The coronavirus disease (COVID-19) caused by SARS-CoV-2 has been a challenge for health services around the world. The pandemic has spread widely, with more than 80 million confirmed cases and more than one million deaths globally. There have been many studies to understand all aspects of the disease. Recently, several cases have been reported of patients with Guillain-Barré syndrome associated with COVID-19 as the main manifestation. As it may be one of the first autoimmune neurological diseases triggered by SARS-CoV-2, it is necessary to obtain more information to fully understand the immunopathogenic mechanisms involved in this association.

Guillain Barré syndrome in the paediatric population. Consequence of active infection or long Covid?

Background: Guillain-Barre syndrome is a polyradiculoneuropathy that has been associated with infectious diseases as triggers. There is currently little medical evidence exploring the relationship between the development of Guillain-Barre syndrome caused by SARS-CoV-2 infection and long Covid. Objective: To synthesize the medical evidence that describes the relationship between post Covid syndrome and Guillain-Barre syndrome in the paediatric population. Methodology: A scoping review was developed using Scopus and PubMed databases, including analytical and/or descriptive experimental and observational studies. Results: The main clinical manifestations presented by paediatric patients were distal and ascending weakness in the lower limbs and myalgia. The diagnostic approach was based on clinical findings, imaging findings on spinal magnetic resonance and electromyography. The therapeutic strategy is based on the use of intravenous human immunoglobulins. Conclusion: Guillain-Barre syndrome is a frequent disease in the paediatric population with active SARS-CoV-2 infection or in survivors, however, it is necessary to encourage further clinical studies that increase the medical literature that describes this association.

Introducción: El síndrome de Guillain-Barré es una polirradiculoneuropatía que se ha asociado con enfermedades infecciosas como desencadenantes. En la actualidad es escasa la evidencia médica que explore la relación entre el desarrollo del síndrome de Guillain-Barré causado por la infección por SARS-CoV-2 y la COVID prolongada. Objetivo: Sintetizar la evidencia médica que describe la relación entre el síndrome pos-COVID y el síndrome de Guillain-Barré en la población pediátrica. Metodología: Se realizó una revisión exploratoria utilizando las bases de datos de Scopus y PubMed, incluyendo estudios experimentales y observacionales analíticos o descriptivos. Resultados: Las principales manifestaciones clínicas presentadas por los pacientes fueron debilidad distal y ascendente en miembros inferiores y mialgias. El enfoque diagnóstico se apoyó en los hallazgos clínicos, hallazgos imagenológicos por resonancia magnética de columna y electromiografía. La estrategia terapéutica se basó en el uso de inmunoglobulinas humanas intravenosas. Conclusión: El síndrome de Guillain-Barré es una enfermedad frecuente en la población pediátrica con infección activa por SARS-CoV-2 o en sobrevivientes, sin embargo, es necesario incentivar el desarrollo de estudios clínicos que incrementen la literatura médica que describe esta asociación.

Scrub Typhus Associated with Guillain–Barré Syndrome (GBS)

Scrub typhus is a vector-borne disease caused by Orientia tsutsugamushi. Clinical manifestations generally occur due to vasculitis and infammation and can have variable degrees of systemic involvement. Meningoencephalitis and cerebellitis are well-known neurological manifestations of scrub typhus, but the occurrence of Guillain–Barré syndrome is extremely rare. The authors report a 7-y-old boy who developed fever followed by rapidly progressive ascending quadriparesis with arefexia and whose etiological workup revealed positive IgM scrub typhus antibody, as well as, a high OXK titer (1:80). Nerve-conduction studies in all four limbs were suggestive of demyelinating neuropathy. He showed complete recovery after treatment with intravenous immunoglobulin (2 g/kg) and azithromycin.

Controversias en neuroinmunología: esclerosis múltiple, vacunación, SARS-CoV-2 y otros dilemas / Controversies in neuroimmunology: multiple sclerosis, vaccination, SARS-CoV-2 and other dilemas

La neuroinmunología es una disciplina que cada vez amplía más sus horizontes en la comprensión de las enfermedades neurológicas. Contemporáneamente, y a la luz de los nexos fisiopatológicos de las enfermedades neurológicas y la inmunología, se han planteado enfoques diagnósticos y terapéuticos específicos. A pesar de los importantes avances de esta disciplina, existen múltiples dilemas que le conciernen y se filtran en la práctica clínica. En esta revisión, se presentan y discuten 15 controversias, las cuales se construyen con la información clínica disponible más actualizada. Los temas incluidos son: disminución de esteroides en recaídas de esclerosis múltiple; recomendaciones terapéuticas en esclerosis múltiple a la luz de la pandemia por el SARS-CoV-2; evidencia de vacunación en esclerosis múltiple y en otras enfermedades desmielinizantes; panorama actual del síndrome clínico y radiológico aislado; y fallas terapéuticas en esclerosis múltiple; además, criterios para suspender las terapias modificadoras de la enfermedad; evidencia del manejo en recaídas leves; recomendaciones para la profilaxis contra Strongyloides stercolaris; utilidad de un segundo ciclo de inmunoglobulina en el síndrome de Guillain-Barré; criterios para diferenciar una polineuropatía crónica desmielinizante inflamatoria de inicio agudo de un síndrome de Guillain-Barré y, utilidad de la enzima convertidora de angiotensina en neurosarcoidosis. En cada una de las controversias, se presenta la problemática general y se ofrecen recomendaciones específicas que pueden adoptarse en la práctica clínica diaria.

Neuroimmunology is a discipline that increasingly broadens its horizons in the understanding of neurological diseases. At the same time, and in front of the pathophysiological links of neurological diseases and immunology, specific diagnostic and therapeutic approaches have been proposed. Despite the important advances in this discipline, there are multiple dilemmas that concern and filter into clinical practice. This article presents 15 controversies and a discussion about them, which are built with the most up-to-date evidence available. The topics included in this review are: steroid decline in relapses of multiple sclerosis; therapeutic recommendations in MS in light of the SARS-CoV-2 pandemic; evidence of vaccination in multiple sclerosis and other demyelinating diseases; overview current situation of isolated clinical and radiological syndrome; therapeutic failure in multiple sclerosis, as well as criteria for suspension of disease-modifying therapies; evidence of the management of mild relapses in multiple sclerosis; recommendations for prophylaxis against Strongyloides stercolaris; usefulness of a second course of immunoglobulin in the Guillain-Barré syndrome; criteria to differentiate an acute-onset inflammatory demyelinating chronic polyneuropathy versus Guillain-Barré syndrome; and, the utility of angiotensin-converting enzyme in neurosarcoidosis. In each of the controversies, the general problem is presented, and specific recommendations are offered that can be adopted in daily clinical practice.

Síndrome de Guillain-Barré postinfección por SARS-CoV-2 y postvacunación contra covid-19: una revisión sistemática de casos / Guillain-Barré syndrome post-SARS-CoV-2 infection and post-vaccination against COVID-19: a systematic review of cases

RESUMEN INTRODUCCIÓN: El síndrome de Guillain-Barré (SGB) se ha asociado recientemente con la infección por SARS-CoV-2 y su vacunación. El objetivo de esta revisión es describir los datos sociodemográficos y la presentación clínica del SGB asociado con la infección y la vacunación contra covid--19. MATERIALES Y MÉTODOS: Se realizó una búsqueda sistemática en las bases de datos Medline, Embase y LILACS. Se incluyeron reportes de casos publicados entre los años 2021 y 2022. De los artículos seleccionados se extrajo edad, sexo, comorbilidades, laboratorio de la vacuna, tiempo de inicio de síntomas, manifestaciones clínicas, método diagnóstico, tratamiento recibido y desenlace. RESULTADOS: Se seleccionaron 93 artículos, en los que se reporta un total de 131 casos: 82 asociados con la infección por SARS-CoV-2 y 49 asociados con la vacunación contra el covid-19. La población más afectada fue el sexo masculino y se encontró entre los 31 y los 65 años. Las principales manifestaciones descritas fueron: paresia de extremidades, compromiso de la sensibilidad, arreflexia, compromiso de los pares craneales. DISCUSIÓN: El cuadro clínico del SGB presentó características similares a la presentación clásica. La disautonomía y la falla respiratoria son las principales complicaciones mortales de esta entidad. CONCLUSIÓN: El SGB se puede presentar de manera postinfecciosa o parainfecciosa. Se debe sospechar cuando se consulta por cuadro clínico de neuropatía desmielinizante con antecedentes de inmunización o síntomas concomitantes sugestivos de covid-19. Se necesitan más estudios para establecer una asociación entre el virus y el SGB.

ABSTRACT INTRODUCTION: Guillain-Barre syndrome (GBS) has recently been associated with SARS-CoV-2 infection and vaccination. The objective of this review is to describe the sociodemographic data and the clinical presentation of GBS associated with infection and vaccination against COVID-19. MATERIALS AND METHODS: A systematic search was performed in the Medline, Embase and LILACS databases. Case reports published in 2021-2022 are included. From the selected articles, age, sex, comorbidi-ties, vaccine brand, symptom onset time, clinical manifestations, diagnostic method, treatment received and outcome were extracted. RESULTS: 93 articles were selected, reporting a total of 131 cases: 82 associated with SARS-CoV-2 infection and 49 associated with vaccination against COVID-19. The most affected population was the male sex and was between 31 and 65 years old. The main manifestations described were: limb paresis, compromised sensitivity, areflexia, compromised cranial nerves. DISCUSSION: The clinical picture of GBS presented characteristics similar to the classic presentation. Dys-autonomia and respiratory failure are the main fatal complications of this entity. CONCLUSION: GBS can present in a postinfectious, parainfectious manner. It should be suspected when consulting for a clinical picture of demyelinating neuropathy with a history of immunization or concomitant symptoms suggestive of COVID-19. More studies are needed to establish an association between the virus and GBS.

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)