title: Neuromyelitis optica with extensive active brain involvement: an autopsy study.
abstract: OBJECTIVE
  To describe the clinical, molecular, and neuropathological findings of a patient with aquaporin 4-positive relapsing myelitis who developed extensive brain involvement followed by death.
  
  
  METHODS
  Case report.
  
  
  METHODS
  Foothills Medical Center, Calgary, Alberta, Canada.
  
  
  METHODS
  A 51-year-old woman with neuromyelitis optica spectrum disorder.
  
  
  RESULTS
  Neuropathological examination disclosed neuromyelitis optica lesions, even in areas that appeared normal radiologically and grossly. Immunostaining confirmed the massive disintegration of astrocytes in the acute and chronic lesions, indicating that astrocytes are targeted early in the disease process. Induction of the immune response was demonstrated by reverse-transcriptase polymerase chain reaction analysis of relevant immune response genes.
  
  
  CONCLUSIONS
  This article supports and supplements current concepts of astrocyte disintegration in neuromyelitis optica and of immune mechanisms in its pathogenesis.
authors.affiliation: Department of Internal Medicine, King Abdulaziz University, Jeddah, Saudi Arabia. malmekhl@ucalgary.ca
authors.forename: Mohammed A
authors.initials: MA
authors.lastname: Almekhlafi
authors.forename: Arthur W
authors.initials: AW
authors.lastname: Clark
authors.forename: Claudia F
authors.initials: CF
authors.lastname: Lucchinetti
authors.forename: Yunyan
authors.initials: Y
authors.lastname: Zhang
authors.forename: Christopher
authors.initials: C
authors.lastname: Power
authors.forename: Robert B
authors.initials: RB
authors.lastname: Bell
mesh_terms: D001253:Astrocytes / Q000276:immunology / Q000473:pathology*; D001344:Autopsy; D001921:Brain / Q000276:immunology / Q000473:pathology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009413:Nerve Fibers, Myelinated / Q000276:immunology / Q000473:pathology*; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000473:pathology*