Widespread Demyelination in the Cerebellar Cortex in Multiple Sclerosis
Open Access
- 22 January 2007
- journal article
- Published by Wiley in Brain Pathology
- Vol. 17 (1), 38-44
- https://doi.org/10.1111/j.1750-3639.2006.00041.x
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.Keywords
This publication has 18 references indexed in Scilit:
- Cortical demyelination in multiple sclerosis: A substrate for cognitive deficits?Journal of the Neurological Sciences, 2006
- Remyelination is extensive in a subset of multiple sclerosis patientsBrain, 2006
- A new focal EAE model of cortical demyelination: multiple sclerosis-like lesions with rapid resolution of inflammation and extensive remyelinationBrain, 2006
- Cortical demyelination and diffuse white matter injury in multiple sclerosisBrain, 2005
- Demyelinated neocortical lesions in marmoset autoimmune encephalomyelitis mimic those in multiple sclerosisBrain, 2005
- Subpial Demyelination in the Cerebral Cortex of Multiple Sclerosis PatientsJournal of Neuropathology and Experimental Neurology, 2003
- The normal appearing grey matter in primary progressive multiple sclerosisZeitschrift für Neurologie, 2003
- Thalamic neurodegeneration in multiple sclerosisAnnals of Neurology, 2002
- Multiple SclerosisNew England Journal of Medicine, 2000
- The distribution of plaques in the cerebrum in multiple sclerosisJournal of Neurology, Neurosurgery & Psychiatry, 1962