Simulation of Brain Death from Fulminant De-efferentation

Abstract

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.