title: Clinical course, pathological correlations, and outcome of biopsy proved inflammatory demyelinating disease.
abstract: BACKGROUND
  A pathological classification has been developed of early active multiple sclerosis (MS) lesions that reveals four patterns of tissue injury: I-T cell/macrophage associated; II-antibody/complement associated; III-distal oligodendrogliopathy, and IV-oligodendrocyte degeneration in the periplaque white matter. Mechanisms of demyelination in early MS may differ among the subgroups. Previous studies on biopsied MS have lacked clinicopathological correlation and follow up. Critics argue that observations are not generalisable to prototypic MS.
  
  
  OBJECTIVE
  To describe the clinicopathological characteristics of the MS Lesion Project biopsy cohort.
  
  
  METHODS
  Clinical characteristics and disability of patients with pathologically confirmed inflammatory demyelinating disease (excluding ADEM) classified immunopathologically (n = 91) and patients from the Olmsted County MS prevalence cohort (n = 218) were determined.
  
  
  RESULTS
  Most patients who underwent biopsy and had pathologically proved demyelinating disease ultimately developed definite (n = 70) or probable (n = 12) MS (median follow up 4.4 years). Most had a relapsing remitting course and 73% were ambulatory (EDSS < or =4) at last follow up. Nine patients remained classified as having an isolated demyelinating syndrome at last follow up. Patients with different immunopathological patterns had similar clinical characteristics. Although presenting symptoms and sex ratios differed, the clinical course in biopsy patients was similar to the prevalence cohort. Median EDSS was <4.0 in both cohorts when matched for disease duration, sex, and age.
  
  
  CONCLUSIONS
  Most patients undergoing biopsy, who had pathologically confirmed demyelinating disease, were likely to develop MS and remain ambulatory after a median disease duration of 4.4 years. The immunopathological patterns lacked specific clinical correlations and were not related to the timing of the biopsy. These data suggest that pathogenic implications derived largely from MS biopsy studies may be extrapolated to the general MS population.
authors.affiliation: Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, USA.
authors.forename: S J
authors.initials: SJ
authors.lastname: Pittock
authors.forename: R L
authors.initials: RL
authors.lastname: McClelland
authors.forename: S J
authors.initials: SJ
authors.lastname: Achenbach
authors.forename: F
authors.initials: F
authors.lastname: Konig
authors.forename: A
authors.initials: A
authors.lastname: Bitsch
authors.forename: W
authors.initials: W
authors.lastname: Brück
authors.forename: H
authors.initials: H
authors.lastname: Lassmann
authors.forename: J E
authors.initials: JE
authors.lastname: Parisi
authors.forename: B W
authors.initials: BW
authors.lastname: Scheithauer
authors.forename: M
authors.initials: M
authors.lastname: Rodriguez
authors.forename: B G
authors.initials: BG
authors.lastname: Weinshenker
authors.forename: C F
authors.initials: CF
authors.lastname: Lucchinetti
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D002648:Child; D015331:Cohort Studies; D003711:Demyelinating Diseases / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007249:Inflammation; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000473:pathology*; D011379:Prognosis; D011788:Quality of Life