Research progress on the relationship between COVID-19 and autoimmune diseases / 中华预防医学杂志

Different autoantibodies can be detected in patients with coronavirus disease 2019 (COVID-19). It is reported that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection could induce autoimmune diseases (AID), including children's multisystem inflammatory syndrome (MIS-C), Guillain Barre syndrome (GBS), Autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP) and thyroid autoimmune diseases. This article mainly reviews the similarities between COVID-19 and AID, the possibility of COVID-19 inducing AID, the risk of AID patients infected or vaccinated against COVID-19. The purpose is to provide strategies for the prevention, management and treatment of AID during the epidemic.

Guía basada en la evidencia. Diagnóstico y manejo del síndrome de Guillain-Barré en diez pasos / Evidence based guidelines. Diagnosis and management of Guillain-Barré syndrome in ten steps

Resumen El síndrome de Guillain-Barré (SGB) es una enfermedad inmunológica del nervio periférico y las raíces nerviosas, poco frecuente, potencialmente mortal y que suele desencadenarse por infecciones. La incidencia del SGB puede aumentar durante el brote de enfermedades infecciosas, tal como se observó en las epidemias del virus Zika en la Polinesia Francesa en 2013 y en América Latina en 2015. El diagnóstico y el manejo clínico del SGB pueden ser complicados ya que su presentación y el curso de la enfermedad son heterogéneos, y actualmente no se cuenta con guías clínicas internacionales. Para respaldar a los médicos, especialmente en el contexto de un brote de una enfermedad infecciosa, hemos desarrollado una guía clínica aplicable en todo el mundo para el diagnóstico y el tratamiento del SGB. La guía se basa en literatura actualizada y el consenso de expertos, y tiene una estructura de diez pasos para facilitar su uso en la práctica clínica. Inicialmente, brindamos una introducción a los criterios de diagnóstico, variantes clínicas y diagnósticos diferenciales del SGB. Los diez pasos luego abordan el reconocimiento y el diagnóstico temprano del SGB, la admisión a la unidad de cuidados intensivos, indicación y selección de tratamiento, seguimiento y tratamiento de la progresión de la enfermedad, predicción del curso clínico, resultados y tratamiento de complicaciones y secuelas.

Abstract Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and in 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diag nostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

Guillain-Barré syndrome: advances and future perspectives / Síndrome de Guillain-Barré: avanços e perspectivas futuras

The first case of Guillain-Barré syndrome was described in 1916. Since then, knowledge about the pathophysiology and immunogenesis of this acquired inflammatory polyradiculoneuropathy has been growing steadily, especially after the advent of nerve conduction studies and the discovery of pathogenic autoantibodies. In the present study, we conducted a review of the main information available in the literature to date about the syndrome, including its diagnosis and management.

A síndrome de Guillain-Barré teve seu primeiro caso descrito em 1916. Desde então, o conhecimento sobre a fisiopatologia e imunogênese dessa polirradiculoneuropatia inflamatória adquirida vem crescendo continuamente, especialmente após o advento dos estudos de condução nervosa e a descoberta de auto-anticorpos patogênicos. No presente estudo, realizamos uma revisão das principais informações disponíveis na literatura até o presente momento sobre a síndrome, incluindo seu diagnóstico e manejo.

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.

Is a second cycle of immunoglobulin justified in axonal forms of Guillain-Barré syndrome? / É justificável utilizar um segundo ciclo de imunoglobulina para as formas axonais da síndrome de Guillain-Barré?

Objective In certain situations, severe forms of Guillain-Barré syndrome (GBS) show no response or continue to deteriorate after intravenous immunoglobulin (IVIg) infusion. It is unclear what the best treatment option would be in these circumstances.Method This is a case report on patients with severe axonal GBS in whom a second cycle of IVIg was used.Results Three patients on mechanical ventilation who presented axonal variants of GBS, with autonomic dysfunction, bulbar impairment and Erasmus score > 6, showed no improvement after IVIg infusion of 400 mg/kg/d for 5 days. After 6 weeks, we started a second cycle of IVIg using the same doses and regimen as in the previous one. On average, 5 days after the second infusion, all the patients were weaned off mechanical ventilation and showed resolution of their blood pressure and heart rate fluctuations.Conclusions A second cycle of IVIg may be an option for treating severe forms of GBS.

Objetivo Em determinadas situações, as formas graves da síndrome de Guillain-Barré (GBS) não mostram resposta ou continuam a deteriorar após a infusão endovenosa de imunoglobulina (IVIg). Não está claro qual seria a melhor opção de tratamento nestas circunstâncias.Método Este é o relato de caso de pacientes com grave comprometimento axonal em GBS, nos quais um segundo ciclo de IVIg foi utilizado.Resultados Três pacientes em ventilação mecânica que apresentavam variantes de GBS com disfunção autonômica, comprometimento bulbar e valores de Erasmus > 6, não mostraram melhora após infusão de IVIg 400 mg/kg/d por 5 dias. Após 6 semanas, foi iniciado um segundo ciclo de IVIg utilizando as mesmas doses e esquema feitos previamente. Em média, após 5 dias da segunda infusão, todos os pacientes haviam sido retirados da ventilação mecânica e mostravam resolução de suas flutuações de pressão arterial e frequência cardíaca.Conclusões O segundo ciclo de IVIg pode ser uma alternativa para tratamento de formas graves de GBS.

Síndrome de Guillain-Barré. Experiencia de doce años / Guillain-Barré syndrome. Twelve years of experience

Introducción: el diagnóstico y el tratamiento temprano del síndrome de Guillain-Barré (SGB) es importante para reducir la morbimortalidad de la enfermedad, por lo cual es capital conocer las manifestaciones clínicas iniciales y el rendimiento de las pruebas diagnósticas. Objetivos: describir las características de los pacientes menores de 15 años hospitalizados por SGB en el Centro Hospitalario Pereira Rossell entre el 1 de enero de 2000 al 31 de diciembre de 2011. Método: estudio descriptivo, retrospectivo, en base a revisión de historias clínicas. Resultados: se incluyeron 82 pacientes, 52 eran de sexo masculino. La mediana de edad fue de 6 años. 59 pacientes requirieron internación en unidad de cuidados intensivos. El síntoma más frecuente fue la paresia de miembros inferiores (80 pacientes), seguido del dolor en miembros inferiores (64 pacientes). El 29% de nuestra serie se presentó clínicamente como variantes atípicas de SGB. La mitad de los pacientes presentó un Hughes 4 en el nadir de la enfermedad. A 77 pacientes se les realizó estudio del líquido cefalorraquídeo, reiterándose en 17 pacientes, mostrando disociación albúmino citológica en 19/53 (36%) de los estudios realizados en los primeros 7 días de evolución, y en 39/41 (95%) de los estudios realizados luego de los 7 días. Conclusiones: se destacan las diversas manifestaciones clínicas del SGB y la baja sensibilidad del estudio del líquido cefalorraquídeo en la primera semana de evolución.

Introduction: early diagnosis and treatment of Guillain-Barré is important to reduce morbimortality of the condition and thus, it is essential to learn about initial clinical presentations and the yield of diagnostic tests. Objectives: to describe the characteristics of patients under 15 years of age who were hospitalized due to Guillain-Barré syndrome at the Pereira Rossell Hospital Center from January 1, 2000 through December 31, 2011. Method: descriptive, retrospective study based on a review of the clinical records. Results: eighty two patients were included, 52 of them were male. Fifty nine patients required to be admitted to the intensive care unit. The most frequent symptom was paresia of the lower limbs (80 patients), followed by pain in the lower limbs (64 patients). Twenty nine percent of our series evidenced clinical presentations that were not typical of Guillain-Barré syndrome. Half of the patients presented disease severity at nadir grade 4 by Hughes. Seventy seven patients underwent a study of the spinal fluid, and this was repeated in 17 patients, what showed albuminocytologic dissociation in 19 out of 53 (36%) of the tests performed in the first seven days of evolution, and in 39 out of 41 (95%) of the tests performed after seven days. Conclusions: we point out the diverse clinical presentations of the Guillain-Barré syndrome and the low sensitivity of the spinal fluid in the first week of evolution.

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 ...

Eficacia del recambio plasmático terapéutico en el tratamiento del Síndrome de Guillain Barre en el Hospital Nacional Dos de Mayo / Efficacy of therapeutic plasma exchange in the treatment of the Guillain-Barre Syndrome at the National Hospital Dos de Mayo

El Síndrome de Guillain-Barré es un trastorno en el que el sistema inmunológico del cuerpo ataca a parte del sistema nervioso periférico. Los primeros síntomas de esta enfermedad incluyen distintos grados de debilidad o sensaciones de cosquilleo en las piernas. Estos síntomas pueden aumentar en intensidad hasta que los músculos no pueden utilizarse en absoluto y el paciente queda casi totalmente paralizado. En estos casos, el trastorno pone en peligro la vida-potencialmente interfiriendo con la respiración y, a veces, con la presión sanguínea y el ritmo cardíaco-y se le considera una emergencia médica. Formulación del Problema: ¿Es el Recambio Plasmático Terapéutico eficaz en el tratamiento del Síndrome de Guillain Barré?. Hipótesis: El Recambio Plasmático Terapéutico es eficaz en el tratamiento del Síndrome de Guillain-Barré. Objetivo General: Determinar la eficacia del recambio plasmático terapéutico en el tratamiento del Síndrome de Guillain-Barré. Objetivos Específicos: Determinar la mejoría de la función motora según la escala de Huhges posterior a la sesión de recambio plasmático terapéutico. Identificar los efectos secundarios ocasionados por el recambio plasmático terapéutico. Determinar la frecuencia de pacientes según sexo con Síndrome de Guillain-Barré. Determinar la frecuencia de pacientes según grupo etáreo con Síndrome de Guillain-Barré. El diseño de la investigación es analítico experimental, prospectivo y longitudinal. La muestra fue de 56 pacientes con Sd de Guillain-Barré, a quienes se les realizó 208 sesiones de RPT, durante el período comprendido de mayo 2012 a abril 2013, teniendo como criterios de exclusión a aquellas otras polirradiculopatías, así como un tiempo de enfermedad mayor a 15 días. Conclusiones: Este estudio demostró que los pacientes que ingresaron al Hospital Nacional Dos de Mayo durante el periodo de un año y que presentaron un cuadro clínico compatible con síndrome de Guillain-Barré y a quienes se les practicó un Recambio...

Plasmapheresis-induced hypercalcaemia

Guillain-Barre syndrome [GBS] is an acute inflammatory polyradiculoneuropathy that can cause total motor paralysis in severe cases. Reports of hypercalcaemia in patients with GBS are rare. Plasmapheresis, an extracorporeal blood purification procedure for the removal of large molecular weight substances, is a well-established therapy for ventilated GBS patients. Although it has been observed in a few reported cases, theoretically, hypercalcaemia is not described as a plasmapheresis-related problem unless there is an underlying cause. We present a rare case of an 8-year-old child presenting with headache, diplopia, and squint, followed by disturbed conscious levels and paralysis. He was treated with both intravenous immunoglobulin and plasmapheresis, with a favourable outcome. We made a laboratory observation of hypercalcaemia which was associated with the plasmapheresis therapy without any related underlying cause. This raises the need for similar observations and the gathering of other possible acceptable explanations

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.

Avaliação econômica do uso de imunoglobulina intra-venosa e de plasmaferese no tratamento da Síndrome de Guillain-Barré no Hospital de Clínicas de Porto Alegre / Economic analysis of intravenous immunoglobulin and plasma exchange therapies for the treatment of Guillain-Barré syndrome in a university-based hospital in the south of Brazil

Background: direct costs for treating Guillain-Barré Syndrome (GBS) represent a significant financial burden to public hospitals. Few studies compared the cost of plasma exchange (PE) treatment with human intravenous immunoglobulin (IVIg). Aim: to compare the cost of two therapies for GBS: IVIg and PE. Secondary objective was to evaluate compliance to IVIg prescription guidelines of the Pharmacy and Therapeutics Committee (PTC). Methods: a cross-sectional study included 25 patients with GBS admitted in a university affiliated hospital from June, 2003 through June, 2008. The costs of IVIg (N=20) and PE (N=5) were evaluated through the cost minimization method, considering direct medical costs yield by the management of the institution. Patients receiving treatments other than PE or IVIg were excluded. Data were collected by medical records review. Clinical endpoint was disability on discharge, established by the 7-point scale of Hughes. Compliance to the PTC guidelines was evaluated considering the dose and prescription regime of IVIg. Results: twenty-five participants, ranging from 2 to 70 years of age, were included. No difference occurred in any medical variables related to the treatment or in the main clinical outcome measured by the Hughes’ scale. The mean direct cost of PE treatment was US$ 6,059± 1,701 per patient, and the same expense for IVIg was US$ 18,344±12,259 (P= 0.035). Total inpatient cost was US$ 25,730± 18,714 in the PE group, and 34,768± 27,766 (P=0.530) in the IVIg group. Conclusion: in a university-based hospital, PE is less expensive than IVIg to treat GBS.

Introdução: os custos diretos do tratamento de Síndrome de Guillain-Barré (SGB) representam parcela significativa dos gastos dos hospitais públicos. Poucos estudos compararam os custos de imunoglobulina intravenosa(IGI) e plasmaferese (PE). Objetivos: comparar os custos de duas terapias para SGB: imunoglobulina intravenosa(IGI) e plasmaferese. Objetivo secundário foi avaliar a adesão à recomendação para uso de imunoglobulina intravenosa da Comissão de Medicamentos da instituição. Métodos: estudo transversal com análise econômica incluindo 25 pacientes com SGB admitidos em um hospital universitário de junho de 2003 a junho de 2008. O custo do uso de IGI (N=20) e plasmaferese (N=5) foi avaliado pelo método de custo-minimização, considerando custos diretos praticados na instituição. Excluíram-se pacientes que receberam outros tratamentos além dos estudados. Os dados foram coletados do prontuário hospitalar. Incapacidade na alta foi avaliada através da escala de sete pontos de Huges. Adesão às recomendações da Comissão de Medicamentos foi avaliada quanto à dose e regime de IGI prescritos. Resultados: incluíram-se 25 participantes, com idade entre 2 e 70 anos. As características clínicas basais foram semelhantes entre os grupos de tratamento, assim como a pontuação na escala de Huges na alta. O custo direto do por paciente foi de US$ 6,059± 1,701 com plasmaferese e de US$ 18,344±12,259 com IGI (P= 0,03). O custo total de internação foi US$ 25,730± 18,714 e US$ 34,768± 27,766 respectivamente ( P=0,53).Conclusão: plasmaferese tem menor custo que IGI no tratamento de pacientes com SGB em um hospital universitário do sul do Brasil.

Guillain-Barré syndrome in child with prolong intubation.

Guillain-Barré syndrome (GBS) is an acute demyelinating disorder of the peripheral nervous system that results from an aberrant immune response directed at peripheral nerves. A typical GBS patient presents with rapidly ascending symmetrical weakness, which may progress to respiratory failure in 30% of patients. There are no definite criteria exists in GBS in children regarding prolonged ventilation. Here we report a child of GBS requiring prolonged intubation and ventilation for 60 days who afterward had a complete recovery. We present this case to highlight the importance that even in children prolonged intubation and ventilation of GBS case prognosis can be good.

Outcome in childhood guillain-barré syndrome.

To assess the outcome of children diagnosed with Guillain-Barré syndrome (GBS), followed up for a median duration of 25 months. Methods. Tertiary center, prospective follow up of children with GBS enrolled from Dec 2003 through Sep 2006. Functional recovery was determined at 12 months and later using Hughes scale (0-6). Clinical, electrophysiological variables were compared between the good outcome (grade 0/1) and bad outcome groups (died or functional grade >1). Results. Among 52 children with a median age of five yr there was male preponderance (75.4%). Mortality during acute phase was 11.5% (6/52). Among the survivors long term data was obtainable in 40 of the 46 children. At one year follow up 87.5% children had fully recovered or had minimal symptoms, beyond one year this rose to 95%. Only 2 among 40 had significant symptoms at last follow up (1 grade-2 and 1 grade-3). Factors significantly associated with poor outcome were: need for artificial ventilation, inexitable nerves on nerve conduction testing and delayed independent walking. Conclusion. Children needing ventilation have the worst short-term prognosis. However, irrespective of severity during acute phase, good long-term recovery can be expected in most children.

Childhood Guillain Barre syndrome

The Guillan-Barre syndrome [GBS] is characterized by the acute onset of rapidly progressive, symmetric muscle weakness with absent or decreased deep tendon reflexes. GBS is the most common cause of acute flaccid paralysis in childhood, with an incidence of 0.6-4 per 100000 Population per year. The clinical features are district and obtaining patient's history and conducting and examination generally lead to the diagnosis that can be confirmed by supportive laboratory tests and electrodiagnostic studies. The major considerations in differential diagnosis include transverse myelities, toxic neuropathy, tick paralysis, infantile butolism and myasthenia gravis. Although most, children with GBS have a relatively care monitoring. Immunomodulating treatment should be used for any child who loses the ability to walk

Síndrome de Guillain Barré en pediatría / Guillain-Barré syndrome in pediatrics

Este trabajo revisa el conocimiento actual sobre el síndrome de Guillain-Barré (SGB) en niños. El SGB se define como una parálisis flácida arrefléxica aguda y se clasifica en 4 subgrupos: polirradiculopatía aguda inflamatoria desmielinizante (AIDP), neuropatía axonal sensitivo-motora aguda (AMSAN), neuropatía axonal motora aguda (AMAN) y síndrome de Miller-Fisher (SMF). La AIDP se asocia en un 30-50% a compromiso de pares craneales, lo cual no se observa en la AMAN. El SMF se caracteriza por ataxia, oftalmoplejía y arreflexia, pero puede presentar también compromiso de pares craneales. Datos recientes de la anatomía patológica y la fisiopatología del SGB destacan la importancia de la infección por Campylobacter jejuni en la generación de anticuerpos anti-gangliósidos (GM1 en AIDP, GQ1b en SMF y GD1a en AMAN) que lesionan la mielina en AIDP y SMF y el axón en AMAN. El diagnóstico diferencial debe descartar enfermedades del sistema nervioso central (SNC) (encefalitis, encefalomielitis, mielitis), síndromes miasténicos, neuropatías tóxicas por metales pesados, fármacos, substancias químicas o toxinas animales y cuadros miopáticos, especialmente la miositis aguda infecciosa benigna y la neuromiopatía del paciente en la unidad de cuidados intensivos. Es importante el tratamiento con inmunoglobulina en dosis total de 2 gramos por kilogramo a administrar en 48 horas. La plasmaféresis puede ser igualmente eficaz. El SGB tiene buen pronóstico en niños, con una recuperación total en el 85% de los casos. La rehabilitación es fundamental para lograr una recuperación más rápida e integral.

This paper reviews the current knowledge about Guillain- Barré syndrome (GBS). GBS is defined as an acute, areflexic, flaccid paralysis, which is classified into 4 subgroups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor-sensory axonal neuropathy (AMSAN), acute motor axonal neuropathy (AMAN) and Miller-Fisher syndrome (MFS). AIDP is associated in 30-50% of cases with cranial nerve involvement, which is not observed in AMAN. MFS is characterized by ataxia, ophthalmoplegia and areflexia, but it may also present cranial nerve dysfunction. Recent data on the pathology and pathophysiology of GBS emphasize the important role of Campylobacter jejuni infection in generating anti-ganglioside antibodies (GM1 in AIDP, GQ1b in MFS and GD1a in AMAN), which damage myelin in AIDP and MFS and axons in AMAN. The differential diagnosis must rule out other disorders of the central nervous system (encephalitis, encephalomyelitis, myelitis), myasthenic syndromes, toxic neuropathies induced by heavy meals, drugs, chemical substances or animal toxins, and myopathic conditions, especially acute benign infectious myositis and neuromyopathy of the intensive care unit patient. It is important the treatment with immune globulin, at a total dose of 2 grams per kilogram administered over 48 hours. Plasmapheresis can be equally effective. GBS has a good prognosis in children with a total recovery in 85% of cases. Rehabilitation is crucial to attain a more rapid and global improvement.

Clinical experience with Gullain Barre Syndrome over a 6-year period in one hospital in the Middle East

Gulain Barre Syndrome [GBS] is the most common cause of sporadic acute flaccid paralysis but is still relatively rare. No series describing the full clinical course of patients with GBS were reported form the Arab world. Here we report on our experience with GBS over a 6-years-old period in one large medical facility form January 1999 till December 2004. We encountered 12 patients with GBS. The median age was 47 years and all had generalized weakness and areflexi. One patient started as Miller Fisher Syndrome [MFS] and ended up with GBS. More than 80% of patients were admitted to Intensive Care Unit [ICU], and 75% were intubated. All patients received supportive care. Specific treatment included intravenous immunoglubin IVIG in all patients [100%], plasma exchange [PE] in 2 patients [17%], and intravenous methyl prednisolone [IVMP] in 1 patient [9%]. The median time to walk independently was 62 days. Transient complications occurred in the majority of patent but the overall outcome was good in the majority of patients; with one single [8%] and probably unrelated mortality. Compared to other series, no significant difference in clinical presentation or outcome was noted

Le syndrone de Guillain-Barre en bref

On voit souvent mentionne parmi les effets indesirables de nombreux vaccins ''Sydrome de Guillan-Barre''.De quoi s'agit-il exactement?