title: Widespread demyelination in the cerebellar cortex in multiple sclerosis.
abstract: Neocortical demyelination in the forebrain has recently been identified as an important pathological feature of multiple sclerosis (MS). Here we describe that the cerebellar cortex is a major predilection site for demyelination, in particular in patients with primary and secondary progressive MS. In these patients, on average, 38.7% of cerebellar cortical area is affected, reaching in extreme examples up to 92%. Cerebellar cortical demyelination occurs mainly in a band-like manner, affecting multiple folia. The lesions are characterized by primary demyelination with relative axonal and neuronal preservation, although some axonal spheroids and a moderate reduction of Purkinje cells are present. Although cortical demyelination sometimes occurs together with demyelination in the adjacent white matter (leukocortical lesions), in most instances, the cortex was affected independently from white matter lesions. We found no correlation between demyelination in the cortex and the white matter, and in some cases, extensive cortical demyelination was present in the near absence of white matter lesions. Our data identify cortical demyelination as a potential substrate of cerebellar dysfunction in MS.
authors.affiliation: Center for Brain Research, Medical University of Vienna, Austria.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Kutzelnigg
authors.forename: Jens C
authors.initials: JC
authors.lastname: Faber-Rod
authors.forename: Jan
authors.initials: J
authors.lastname: Bauer
authors.forename: Claudia F
authors.initials: CF
authors.lastname: Lucchinetti
authors.forename: Per S
authors.initials: PS
authors.lastname: Sorensen
authors.forename: Henning
authors.initials: H
authors.lastname: Laursen
authors.forename: Christine
authors.initials: C
authors.lastname: Stadelmann
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Brück
authors.forename: Helmut
authors.initials: H
authors.lastname: Rauschka
authors.forename: Manfred
authors.initials: M
authors.lastname: Schmidbauer
authors.forename: Hans
authors.initials: H
authors.lastname: Lassmann
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001369:Axons / Q000473:pathology*; D002525:Cerebellar Cortex / Q000473:pathology*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000145:classification / Q000473:pathology*; D009186:Myelin Sheath / Q000473:pathology*; D018709:Statistics, Nonparametric