Widespread demyelination in the cerebellar cortex in multiple sclerosis

Alexandra Kutzelnigg, Jens C. Faber-Rod, Jan Bauer, Claudia F. Lucchinetti, Per S. Sorensen, Henning Laursen, Christine Stadelmann, Wolfgang Brück, Helmut Rauschka, Manfred Schmidbauer, Hans Lassmann

Research output: Contribution to journalArticle

219 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)38-44
Number of pages7
JournalBrain Pathology
Volume17
Issue number1
DOIs
StatePublished - Jan 1 2007

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neuroscience(all)
  • Clinical Neurology

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    Kutzelnigg, A., Faber-Rod, J. C., Bauer, J., Lucchinetti, C. F., Sorensen, P. S., Laursen, H., Stadelmann, C., Brück, W., Rauschka, H., Schmidbauer, M., & Lassmann, H. (2007). Widespread demyelination in the cerebellar cortex in multiple sclerosis. Brain Pathology, 17(1), 38-44. https://doi.org/10.1111/j.1750-3639.2006.00041.x