Guillain-Barré Syndrome

Kazim A. Sheikh, MBBS Peripheral Nerve and Motor Neuron Disorders p. 1184-1204 October 2020, Vol.26, No.5 doi: 10.1212/CON.0000000000000929
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PURPOSE OF REVIEW This article reviews the clinical features, diagnosis and differential diagnosis, prognosis, pathogenesis, and current and upcoming treatments of Guillain-Barré syndrome (GBS).

RECENT FINDINGS GBS is an acute inflammatory neuropathic illness with striking clinical manifestations and significant morbidity. A substantial proportion of patients with GBS do not respond to current immunomodulatory therapies (ie, plasma exchange and IV immunoglobulin [IVIg]), highlighting the need for new therapies. Prognostic models that can accurately predict functional recovery and the need for artificial ventilation have emerged. These models are practical, and online calculators are available for clinical use, facilitating early recognition of patients with poor outcome and the opportunity to personalize management decisions. Clinical and experimental studies have identified innate immune effectors (complement, macrophage lineage cells, and activating Fcγ receptors) as important mediators of inflammatory nerve injury. Two complement inhibitors are undergoing clinical testing for efficacy in GBS.

SUMMARY GBS is the most common cause of acute flaccid paralysis in the United States and worldwide. New treatments for GBS have not emerged since the 1990s. Our understanding of the pathogenesis of this disorder has progressed, particularly over the past decade; as a result, new therapeutic agents targeting different components of the complement cascade are at advanced stages of clinical development.

Address correspondence to Dr Kazim Sheikh, Department of Neurology, University of Texas-Medical School at Houston, 6431 Fannin St, Houston, TX 77030, [email protected].

RELATIONSHIP DISCLOSURE: Dr Sheikh serves on the medical advisory board of the GBS/CIDP Foundation International and on the editorial board of Scientific Reports. Dr Sheikh has received personal compensation for speaking engagements from CSL Behring and research/grant support from the Department of Defense (W81XWH-18-1-0422) and the National Institute of Neurological Disorders and Stroke (R21NS107961).

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Sheikh reports no disclosure.

© 2020 American Academy of Neurology.