Guillain-Barré syndrome (GBS) is a subacutely evolving paralysis with variable sensory involvement accompanied by hyporeflexia, and typically, albuminocytologic dissociation in cerebrospinal fluid. Abnormal nerve conductions and pathological descriptions of lymphocytic and macrophage infiltration of the peripheral nerve with segmental myelin destruction 1-3 have been well-described. Axonal loss can be present, and is usually secondary to demyelination. Among the intriguing aspects of this syndrome are the variations in clinical course, with severity ranging from trivial limb weakness to total paralysis requiring ventilatory assistance. The majority of patients (75-80%) recover fully (or almost so), but a small percentage (3-8%) still die secondary to sepsis, pulmonary ABSTRACT: Background: Guillain-Barré syndrome (GBS) classically presents with a subacutely evolving areflexic paralysis, with typical laboratory findings of elevated cerebrospinal fluid protein and abnormal nerve conduction studies. There is now an increasing recognition of GBS variants that differ in clinical presentation, prognosis, electrophysiology and presumed pathogenesis. Fulminant cases of GBS have been reported in which a rapid deterioration evolves to a clinical state resembling "brain death". Methods: A retrospective analysis of two such cases of fulminant neuropathy are described, that includes the clinical course, electrophysiology and neuropathology where available. Results: We describe two patients that presented with a rapid course of neurological deterioration, lapsing into what resembled a "clinically brain-dead" state that was subsequently ascribed to a fulminant polyneuropathy. Investigations (electrophysiological, pathological) and the clinical course suggested an axonal neuropathy. Conclusions: A fulminant neuropathy can result in a clinical state resembling "brain death" through diffuse de-efferentation. Although generally attributed to aggressive demyelination with secondary axonal degeneration, a primary axonopathy can also lead to a similar clinical presentation. RÉSUMÉ: Simulation de la mort cérébrale par de-efférentation fulminante. Introduction: La présentation classique du syndrome de Guillain-Barré (SGB) consiste en une paralysie aréflexique évoluant de façon subaiguë accompagnée d'anomalies biochimiques typiques, soit une augmentation des protéines dans le liquide céphalorachidien et des anomalies de conduction nerveuse. On reconnaît maintenant qu'il existe des variantes du SGB qui diffèrent par leur présentation clinique, leur pronostic, les constatations électrophysiologiques et la pathogenèse présumée. Des cas fulminants de SGB chez qui une détérioration rapide évolue vers un état clinique ressemblant à la mort cérébrale ont été rapportés. Méthodes: Nous présentons une analyse rétrospective de deux cas de neuropathie fulminante, leur évolution clinique, ainsi que les constatations électrophysiologiques et neuropathologiques si elles étaient disponibles. Résultats: Nous décrivons deux patients qui ont consulté pour...
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.