Widespread Demyelination in the Cerebellar Cortex in Multiple Sclerosis

  title={Widespread Demyelination in the Cerebellar Cortex in Multiple Sclerosis},
  author={Alexandra Kutzelnigg and Jens C. Faber‐Rod and Jan Bauer and Claudia F. Lucchinetti and Per S. S{\o}rensen and Henning Laursen and Christine Stadelmann and Wolfgang Br{\"u}ck and Helmut Rauschka and Manfred Schmidbauer and Hans Lassmann},
  journal={Brain Pathology},
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… 
Meningeal and cortical grey matter pathology in multiple sclerosis
Early cortical lesions in multiple sclerosis are highly inflammatory, suggesting that neurodegeneration in MS occurs on an inflammatory background and raising interesting questions regarding the role of cortical demyelination and meningeal inflammation in initiating and perpetuating the disease process in early MS.
Cerebellar Cortical Demyelination in the Murine Cuprizone Model
In summary, cuprizone feeding provides an excellent model for the investigation of de‐ and remyelination processes in the cerebellar cortex and white matter, and demyelinations, microglia and astrocyte changes were different in the Cerebellum as compared with the cerebrum, indicating region‐dependent pathomechanisms.
Grey matter pathology in multiple sclerosis
The most common lesion type consists of purely cortical lesions extending inward from the surface of the brain, this lesion subgroup is grossly underestimated by standard histochemical myelin staining methods.
Neuronal and Axonal Loss in Normal-Appearing Gray Matter and Subpial Lesions in Multiple Sclerosis
It is suggested that neurodegeneration in the cerebral cortex of patients with MS may be independent of cortical demyelination, and both NAGM and Type III lesions showed Neurodegenerative changes, but they had no consistent differences in neuronal and axonal alterations.
Cortical pathology in multiple sclerosis
Studies on the pathogenesis of cortical demyelination, cortical damage, and repair will elucidate basic principles of multiple sclerosis lesion formation, however, more sensitive imaging tools are required to study the impact of cortical lesions on clinical symptoms, disability, and disease progression.
Cortical lesions in multiple sclerosis: inflammation versus neurodegeneration.
  • H. Lassmann
  • Biology, Psychology
    Brain : a journal of neurology
  • 2012
In the early descriptions of cortical lesions in patients with progressive disease, it was emphasized that they differ from white matter lesions by the absence of perivascular and parenchymal infiltrates of T- and B-lymphocytes, vascular inflammation and blood–brain barrier.
Absence of cortical demyelination in neuromyelitis optica
This study provides a plausible explanation for the absence of a secondary progressive clinical course in N MO and shows that cognitive and cortical neuroimaging abnormalities previously reported in NMO cannot be attributed to cortical demyelination.
Substantial subpial cortical demyelination in progressive multiple sclerosis: have we underestimated the extent of cortical pathology?
Cases of progressive MS with substantial subpial cortical demyelination that is independent of underlying white matter lesion area support the implications that these lesions may in-part arise through different pathogenetic mechanisms.
Purkinje Cell Pathology and Loss in Multiple Sclerosis Cerebellum
Changing pathologic changes occurring within Purkinje cells of the cerebellum have important implications for the study of cerebellar involvement in multiple sclerosis and may help design therapies to reduce the burden of ataxia in the condition.


Subpial Demyelination in the Cerebral Cortex of Multiple Sclerosis Patients
The results indicate that the cerebral cortex is likely to be a predilection site for MS lesions and identify general cortical subpial demyelination as a distinct pattern occurring in a significant subpopulation of MS patients.
Grey Matter Pathology in Multiple Sclerosis
Grey matter demyelination and neuronal loss could contribute to disability and cognitive dysfunctions in MS.
Cortical demyelination in multiple sclerosis: A substrate for cognitive deficits?
Transected neurites, apoptotic neurons, and reduced inflammation in cortical multiple sclerosis lesions
The hypothesis that demyelination, axonal transection, dendritic tran section, and apoptotic loss of neurons in the cerebral cortex contribute to neurological dysfunction in MS patients is supported.
A new focal EAE model of cortical demyelination: multiple sclerosis-like lesions with rapid resolution of inflammation and extensive remyelination.
The data suggest that cortical inflammation is a transient phenomenon, and that remyelination of cortical inflammatory-demyelinating lesions may occur rapidly, as well as that cortical demyelinated lesions in the neocortex was readily compensated by rapid remyElination.
Intracortical multiple sclerosis lesions are not associated with increased lymphocyte infiltration
The data indicate that areas of intracortical demyelination in chronic MS are not associated with an increased number of lymphocytes, or an altered distribution of lymphocyte subsets, when compared with control areas in MS and control patients.
Differential Macrophage/Microglia Activation in Neocortical EAE Lesions in the Marmoset Monkey
The findings indicate that the inflammatory response, especially macrophage and microglia activation, may be regulated differently in gray matter areas in primate brain.
The Pathology of Multiple Sclerosis Is Location-Dependent: No Significant Complement Activation Is Detected in Purely Cortical Lesions
The role of complement in MS pathogenesis seems lesion location-dependent, with less frequent activation in the gray matter part of combined gray matter/white matter lesions and low in purely cortical lesions.
The distribution of plaques in the cerebrum in multiple sclerosis
The present study was undertaken to determine with some precision the position and relative frequency of plaques seen macroscopically in the cerebral hemispheres in a series of necropsied cases of multiple sclerosis.
Evidence of early cortical atrophy in MS
The extent of the changes suggests that neocortical GM pathology may occur early in the course of the disease in both RR and PP MS patients and contribute significantly to neurologic impairment.