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Simulation of Brain Death from Fulminant De-efferentation

Published online by Cambridge University Press:  02 December 2014

Yael Friedman ,
Liesly Lee ,
John R. Wherrett ,
Peter Ashby  and
Stirling Carpenter
Yael Friedman
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
Liesly Lee
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
John R. Wherrett
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
Peter Ashby
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
Stirling Carpenter
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
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Abstract

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Background:

Guillain-BarrÈ 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 ìbrain deathî.

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 ìclinically brain-deadî state that was subsequently ascribed to a fulminant polyneuropathy. Investigations (electrophysiological, pathological) and the clinical course suggested an axonal neuropathy.

Conclusion:

A fulminant neuropathy can result in a clinical state resembling ìbrain deathî 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:

RÉSUMÉ: 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 une détérioration neurologique rapide, les patients ayant éventuellement sombré dans un état qui ressemblait au point de vue clinique à la mort cérébrale, attribuée ultérieurement à une polyneuropathie fulminante. L'investigation électrophysiologique et anatomopathologique et l'évolution clinique suggéraient qu'il s'aggissait d'une neuropathie axonale.

Conclusions:

Une neuropathie fulminante peut induire un état clinique ressemblant à la mort cérébrale par de-efférentation diffuse. Bien qu'attribuée généralement à une démélinisation agressive avec dégénéressence axonale secondaire, une axonopathie primaire peut aussi entraîner une telle présentation clinique.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 30 , Issue 4 , November 2003 , pp. 397 - 404
Copyright
Copyright © The Canadian Journal of Neurological 2003

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