title: Simulation of brain death from fulminant de-efferentation.
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.
authors.affiliation: Division of Neurology, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Yael
authors.initials: Y
authors.lastname: Friedman
authors.forename: Liesly
authors.initials: L
authors.lastname: Lee
authors.forename: John R
authors.initials: JR
authors.lastname: Wherrett
authors.forename: Peter
authors.initials: P
authors.lastname: Ashby
authors.forename: Stirling
authors.initials: S
authors.lastname: Carpenter
mesh_terms: D000328:Adult; D000368:Aged; D001926:Brain Death / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D003937:Diagnosis, Differential; D004525:Efferent Pathways / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011115:Polyneuropathies / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D012189:Retrospective Studies