august 2020 • European Journal of Paediatric Neurology

Diagnosis and treatment of Guillain-Barré Syndrome in childhood and adolescence: An evidence- and consensus-based guideline

Content curated by:David Rodrigues

Key message

O diagnóstico de SGB deve basear-se nas características clínicas, distribuição de fraqueza, eletrofisiologia típica e características do LCR. Procedimentos diagnósticos dolorosos só devem ser repetidos se isso for necessário para um diagnóstico claro e indicação de tratamento. Imunoglobulina de alta dose ou plasmaferese é a modalidade de tratamento de primeira escolha suportadas por um nível de evidência moderado. No caso de ausência de respostas impõem-se decisões individuais, em função das características da evolução clínica. Programas de reabilitação serão necessários para todos os pacientes com incapacidade significativa.

Abstract

This evidence- and consensus-based practical guideline for the diagnosis and treatment of Guillain-Barré Syndrome (GBS) in childhood and adolescence has been developed by a group of delegates from relevant specialist societies and organisations; it is the result of an initiative by the German-Speaking Society of Neuropediatrics (GNP), and is supported by the Association of Scientific Medical Societies (AWMF, Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften). A systematic analysis of the literature revealed that only a few adequately-controlled studies exist for this particular age group, while none carries a low risk of bias. For this reason, the diagnostic and therapeutic recommendations largely rely on findings in adult patients with GBS, for which there are a higher number of suitable studies available. Consensus was established using a written, multi-step Delphi process. A high level of consensus could be reached for the crucial steps in diagnosis and treatment. We recommend basing the diagnostic approach on the clinical criteria of GBS and deriving support from CSF and electrophysiological findings. Repetition of invasive procedures that yield ambiguous results is only recommended if the diagnosis cannot be ascertained from the other criteria. For severe or persistently-progressive GBS treatment with intravenous immunoglobulin (IVIG) is recommended, whereas in cases of IVIG intolerance or inefficacy we recommended treatment with plasmapheresis. Corticosteroids are ineffective for GBS but can be considered when acute onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is suspected due to a prolonged disease course.