2023 Guideline Update on Diagnosis and Treatment of Guillain–Barré Syndrome

A task force from the European Academy of Neurology (EAN) and Peripheral Nerve Society (PNS) used the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) methodology to develop an evidence-based guideline for diagnosing and treating Guillain–Barré syndrome (GBS). 

GBS is an acute immune-mediated polyradiculoneuropathy with diverse symptoms and severity, making diagnosis and treatment challenging. Several published criteria exist for diagnosing GBS, with the National Institute of Neurological Disorders and Stroke (NINDS) criteria, revised by Asbury and Cornblath, being the most frequently used. At the 2024 PNS Annual Meeting in Montreal, Canada, Dr. Pieter van Doorn, a neurologist and Professor of Neuromuscular Diseases at Erasmus MC, presented recent updates on GBS diagnosis and treatment.

"It was a huge work conducted by 21 colleagues from all over the world." Dr Doorn highlighted the global collaborative approach to translating evidence into clinical practice. The task force constructed 14 PICO (Population/Intervention/Comparison/Outcome) questions covering the diagnosis, treatment, and prognosis of GBS. Dr. Doorn presented several key diagnostic questions and supporting evidence regarding the relevance of antecedents, lumbar puncture, antibody testing, and nerve imaging to underscore the importance of these questions in clinical practice by healthcare professionals. Flowcharts in the guideline detailing diagnostic criteria are designed to offer clinicians distinction for motor GBS versus what makes GBS less likely. He acknowledged that ethnic variation in the presentation of motor versus motor-sensory GBS also creates a challenge.

Dr. Doorn presented a quick overview of the available treatments and stressed the importance of early intervention with intravenous immunoglobulins, particularly within two weeks of weakness onset. He stressed the need for a cost-effective, straightforward, and timely start of adequate treatment, especially for GBS patients in resource-poor countries like Bangladesh. Addressing treatment dilemmas in scenarios such as definitive GBS, nodopathy, and acute-onset chronic inflammatory demyelinating polyradiculoneuropathy, he recommended consulting the 2023 guideline flowchart created based on the onset of weakness and severity. Ongoing clinical trials are assessing complement blockers, Imlifidase, neonatal Fc receptor blockers, Phase II Immunoabsorption trials, and small-volume plasma exchange trials. However, these trials usually span years. "Clinical trials in GBS last an awful lot of time, many, many years, and patients can't wait," noted Dr. Doorn. He recommended that the patient data in the international GBS Outcome Study (IGOS) data bank is a unique opportunity to leverage for developing clinical trials and guidelines and identify subgroups with different treatment responses.

Dr. Doorn echoed Richard Hughes' sentiment, asking, "Are we moving in the right direction?" He assured many of the action steps Richard Hughes recommended in his 2024 European Journal of Neurology article were already under work, such as research to seek biomarkers, identify subgroups with different treatment responses, study new populations, and understand disease pathology through autopsies. Dr. Doorn concluded by summarizing the current situation as ideal: "Currently, there's a kind of golden triangle. We have patient organizations, the GBS/CIDP foundation in one corner, basic and clinical researchers in another, and we do have the treatment strategies."