title: Beneficial plasma exchange response in central nervous system inflammatory demyelination.
abstract: BACKGROUND
  Plasma exchange (PLEX) is a beneficial rescue therapy for acute, steroid-refractory central nervous system inflammatory demyelinating disease (CNS-IDD). Despite the approximately 45% PLEX response rate reported among patients with CNS-IDD, determinants of interindividual differences in PLEX response are not well characterized.
  
  
  OBJECTIVE
  To perform an exploratory analysis of clinical, radiographic, and serological features associated with beneficial PLEX response.
  
  
  METHODS
  Historical cohort study.
  
  
  METHODS
  Neurology practice, Mayo Clinic College of Medicine, Rochester, Minnesota. Patients All Mayo Clinic patients treated with PLEX between January 5, 1999, and November 12, 2007, for a steroid-refractory CNS-IDD attack.
  
  
  METHODS
  The PLEX response in attack-related, targeted neurological deficit(s) assessed within the 6-month period following PLEX.
  
  
  RESULTS
  We identified 153 patients treated with PLEX for a steroid-refractory CNS-IDD, of whom 90 (59%) exhibited moderate to marked functional neurological improvement within 6 months following treatment. Pre-PLEX clinical features associated with a beneficial PLEX response were shorter disease duration (P = .02) and preserved deep tendon reflexes (P = .001); post-PLEX variables included a diagnosis of relapsing-remitting multiple sclerosis (P = .008) and a lower Expanded Disability Status Scale score (P < .001) at last follow-up. Plasma exchange was less effective for patients with multiple sclerosis who subsequently developed a progressive disease course (P = .046). Radiographic features associated with a beneficial PLEX response were presence of ring-enhancing lesions (odds ratio = 4.00; P = .03) and/or mass effect (odds ratio = 3.00; P = .02). No association was found between neuromyelitis optica-IgG serostatus and PLEX response.
  
  
  CONCLUSIONS
  We have identified clinical and radiographic features that may aid in identifying patients with fulminant, steroid-refractory CNS-IDD attacks who are more likely to respond to PLEX.
authors.affiliation: Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
authors.forename: Setty M
authors.initials: SM
authors.lastname: Magaña
authors.forename: B Mark
authors.initials: BM
authors.lastname: Keegan
authors.forename: Brian G
authors.initials: BG
authors.lastname: Weinshenker
authors.forename: Bradley J
authors.initials: BJ
authors.lastname: Erickson
authors.forename: Sean J
authors.initials: SJ
authors.lastname: Pittock
authors.forename: Vanda A
authors.initials: VA
authors.lastname: Lennon
authors.forename: Moses
authors.initials: M
authors.lastname: Rodriguez
authors.forename: Kristine
authors.initials: K
authors.lastname: Thomsen
authors.forename: Stephen
authors.initials: S
authors.lastname: Weigand
authors.forename: Jay
authors.initials: J
authors.lastname: Mandrekar
authors.forename: Linda
authors.initials: L
authors.lastname: Linbo
authors.forename: Claudia F
authors.initials: CF
authors.lastname: Lucchinetti
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002493:Central Nervous System Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D002648:Child; D015331:Cohort Studies; D016001:Confidence Intervals; D003711:Demyelinating Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016015:Logistic Models; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D010951:Plasma Exchange / Q000379:methods*; D011859:Radiography; D013116:Spinal Cord / Q000000981:diagnostic imaging / Q000473:pathology; D055815:Young Adult