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

Authors: Korinthenberg, R., Trollmann, R., Felderhoff-Müser, U., Bernert, G., Hackenberg, A., Hufnagel, M., Pohl, M., Hahn, G., Mentzel, HJ., Sommer, C., Lambeck, J., Mecher, F., Hessenauer, M., Winterholler, C., Kempf, U., Jacobs, BC., Rostasy, K., and Müller-Felber, W.

Publication: Eur J Paediatr. Neurol.; 25,5-16.; September 2020

Affiliations: Division of Neuropediatrics and Muscular Disorders, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, University Medical Center (UMC), University of Freiburg, Germany; Department of Neuropediatrics, UMC, Friedrich- Alexander University Erlangen-Nürnberg, Erlangen, Germany; Department of Pediatrics, UMC, University of Essen, Germany; Department of Pediatrics, Kaiser-Franz-Joseph-Hospital with Preyer’s Childrens Hospital, Vienna, Austria; Department of Pediatric Neurology, University Children’s Hospital, Zürich, Switzerland; Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, UMC, University of Freiburg, Germany; Section Pediatric Nephrology, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, UMC, University of Freiburg, Germany; Department of Radiological Diagnostics, UMC, University of Dresden, Germany; Section Pediatric Radiology, Institute of Diagnostic and Interventional Radiology, Universitatsklinikum Jena, Germany; Department of Neurology, UMC, University of Wuerzburg, Germany; Department of Neurology and Neurophysiology, UMC, University of Freiburg, Germany; Physio Deutschland, German Federal Association for Physiotherapy, Germany; Centre for Pediatric Neurology, Neurorehabilitation and Epileptology, Schoen Clinic Vogtareuth, Germany; German Federal Association of Logopedics (dbl e.V. Deutscher Bundesverband, Germany; Mother of a GBS PPatient, Kraichtal-Neuenbürg, Germany; Departments of Neurology and Immunology, Erasmus MC, UMC Rotterdam, the Netherlands; Department of Neuropediatrics, Children´s Hospital Datteln, University Witten/Herdecke, Germany; Department of Neuropediatrics, UMC, LMU Munich, Germany.

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. The full German version of the Guideline is available on the AWMF website.