Características clínicas y respuesta al recambio plasmático terapéutico en los pacientes con síndrome de Guillain Barré / Clinical characteristics and plasma exchange response in Guillain-Barré patients

RESUMEN El objetivo del estudio fue describir las características clínicas, la respuesta al tratamiento y posibles factores asociados de los pacientes con síndrome de Guillain Barré en el Instituto Nacional de Ciencias Neurológicas. Se realizó un estudio descriptivo sobre egresos hospitalarios durante el periodo 2017-2019. La respuesta al tratamiento se evaluó mediante la escala de discapacidad de Hughes. De los 31 pacientes el 61,3% eran varones, y la edad promedio fue de 50 años. Al ingreso, el 87,1% de pacientes se encontraban en el grado 3 o 4 de la escala de Hughes, la mayoría con compromiso axonal, el cual estuvo asociado a discapacidad. Solo 22 pacientes recibieron recambio plasmático; luego de seis meses el 90,9% disminuyó al menos en un grado en la escala de Hughes y el 42,8% quedaron sin discapacidad. En conclusión, se encontró un predominio del sexo masculino y del compromiso axonal, este último asociado a discapacidad.

ABSTRACT The objective of the study was to describe the clinical characteristics, treatment response and possible associated factors of patients with Guillain-Barré syndrome at the National Institute of Neurological Sciences. A descriptive study on hospital discharges was conducted during the period 2017-2019. Treatment response was evaluated based on Hughes' disability scale. From 31 patients 61.3% were males and the mean age was 50 years. At admission, 87.1% of patients were on grade 3 or 4 of Hughes scale, most of them with axonal compromise which was associated to disability. Only 22 patients received plasma exchange; 6 months thereafter, 90.9% of patients decreased by at least one degree in Hughes scale and 42.8% were left without disability. In conclusion, a male and axonal subtype predominance was found, been the latter associated to disability.

Características clínicas y respuesta al recambio plasmático terapéutico en los pacientes con síndrome de Guillain Barré / Clinical characteristics and plasma exchange response in Guillain-Barré patients

RESUMEN El objetivo del estudio fue describir las características clínicas, la respuesta al tratamiento y posibles factores asociados de los pacientes con síndrome de Guillain Barré en el Instituto Nacional de Ciencias Neurológicas. Se realizó un estudio descriptivo sobre egresos hospitalarios durante el periodo 2017-2019. La respuesta al tratamiento se evaluó mediante la escala de discapacidad de Hughes. De los 31 pacientes el 61,3% eran varones, y la edad promedio fue de 50 años. Al ingreso, el 87,1% de pacientes se encontraban en el grado 3 o 4 de la escala de Hughes, la mayoría con compromiso axonal, el cual estuvo asociado a discapacidad. Solo 22 pacientes recibieron recambio plasmático; luego de seis meses el 90,9% disminuyó al menos en un grado en la escala de Hughes y el 42,8% quedaron sin discapacidad. En conclusión, se encontró un predominio del sexo masculino y del compromiso axonal, este último asociado a discapacidad.

ABSTRACT The objective of the study was to describe the clinical characteristics, treatment response and possible associated factors of patients with Guillain-Barré syndrome at the National Institute of Neurological Sciences. A descriptive study on hospital discharges was conducted during the period 2017-2019. Treatment response was evaluated based on Hughes' disability scale. From 31 patients 61.3% were males and the mean age was 50 years. At admission, 87.1% of patients were on grade 3 or 4 of Hughes scale, most of them with axonal compromise which was associated to disability. Only 22 patients received plasma exchange; 6 months thereafter, 90.9% of patients decreased by at least one degree in Hughes scale and 42.8% were left without disability. In conclusion, a male and axonal subtype predominance was found, been the latter associated to disability.

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 POEMS: a propósito de un caso y revisión de la literatura / POEMS syndrome: report of a case and review of literature

El síndrome POEMS es un síndrome paraneoplásico raro asociado a discrasia de células plasmáticas. Se presenta el caso de un paciente masculino de 37 años de edad, quien consultó por presentar de 38.5 C°, precedida de escalofríos, predominio nocturno y frecuencia diaria/interdiaria, de tres meses de evolución; concomitante parestesias en región plantar bilateral, ascendente, con disminución de fuerza muscular simétrica en miembros inferiores. Durante su estancia hospitalaria se evidencia trombocitosis y eritrocitosis. Se realizó ecosonograma abdominal que evidencia hepatomegalia; tomografía computarizada (TC) de cuello que reportó adenopatías submandibulares, laterocervicales y parayugulares; TC de tórax donde se evidencia lesión ocupante de espacio de características osteolíticas expansivas a nivel de segunda costilla izquierda; electromiografía de miembros superiores e inferiores con radiculopolineuropatía de tipo mixto en miembros inferiores y polineuropatía sensitivo motora de tipo mixto en miembros superiores; electroforesis de proteínas con discreto aumento de gammaglobulinas, a expensas de IgG y aspirado de médula ósea con serie plasmática aumentada (3%) de apariencia madura. Se realizó biopsia de lesión en segundo arco costal izquierdo sin evidenciar atipias. En vista de hallazgos clínicos y paraclínicos se planteó síndrome POEMS.

POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. We present a male patient of 37 years old with fever 38.5°C, preceded by chills, nocturnal predominance and daily frequency of three months of evolution, as well as paresthesias in plantar region, bilateral, ascendant with decrease in muscle strength of legs. During his hospitalization, he presented thrombocytosis and erythrocytosis. The abdominal ecosonography evidenced hepatomegaly; thorax computerized tomography evidenced space osteolytic occupying lesion in second left rib. The electromyography demonstrated radiculo polineuropathy in legs, and sensory motor polineuropathy in upper limbs. The protein electrophoresis with elevated of gammaglobulins ;bone marrow aspirate evidenced (3%) mature increased plasma cells. The biopsy of second rib evidenced no atypia.

Guillain-Barré syndrome in children: clinic, laboratorial and epidemiologic study of 61 patients / Síndrome de Guillain-Barré em crianças: estudo clínico, laboratorial e epidemiologico em 61 pacientes

The aim of the study was to analyze the epidemiologic, clinical, laboratory and development profile of Guillain-Barré syndrome series studied at the Child Institute, between 1989 and 2000. From the 61 patients that fulfilled the selection criteria, aged between 7 months and 13 years old, no sexual or seasonal variation was observed. Clinical events prior to neurological symptoms (with an average time gap of 20.7 days) were observed in 62.3 percent, 55 percent had cranial nerve disturbances, 27.9 percent dysautonomic symptoms, and 27.9 percent respiratory dysfunction. Installation time varied from 2-40 days, plateau from 0-28 days and recuperation from 30-480 days; 94 percent of patients had a complete clinical recuperation. Electrophysiology in 20 patients disclosed an abnormal demyelination pattern in 15, an exclusively motor axonal pattern in 4 and a mixed pattern in 1 patient. The results obtained did not differ from those in the literature but it was observed that boys and older children had a longer recuperation time. It was not possible to correlate electroneurography with clinical abnormalities and evolution due to the reduced number of patients.

O objetivo do estudo foi analisar retrospectivamente, quanto ao perfil clínico, laboratorial, epidemiológico e evolutivo a casuística da síndrome de Guillain-Barré no Instituto da Criança, entre 1989 e 2000. Preencheram os critérios de seleção 61 pacientes, com idades entre 7 meses e 13 anos, e não se observou variação entre sexos nem sazonal. Em 62,3 por cento houve uma afecção precedendo o quadro neurológico, num tempo médio de 20,7 dias; 55 por cento apresentaram acometimento de nervos cranianos, 27,9 por cento distúrbios disautonômicos e 27,9 por cento comprometimento respiratório. A fase de progressão variou de 2 a 40 dias, a de platô de 0 a 28 dias e a de recuperação de 30 a 480 dias e 94 por cento tiveram recuperação total. A eletroneurografia, em 20 pacientes, mostrou padrão desmielinizante em 15, padrão axonal puramente motor em 4 e padrão misto em 1. Os resultados não diferiram dos encontrados na literatura, mas os meninos e os pacientes na faixa etária superior apresentaram maior tempo de recuperação total. Não foi possível estabelecer relação entre resultados da ENMG e o quadro clínico e evolutivo em vista do pequeno numero de pacientes com esse exame.