Widespread demyelination in the cerebellar cortex in multiple sclerosis.
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.
Kutzelnigg
Alexandra
A
Center for Brain Research, Medical University of Vienna, Austria.
Faber-Rod
Jens C
JC
Bauer
Jan
J
Lucchinetti
Claudia F
CF
Sorensen
Per S
PS
Laursen
Henning
H
Stadelmann
Christine
C
Brück
Wolfgang
W
Rauschka
Helmut
H
Schmidbauer
Manfred
M
Lassmann
Hans
H
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