title: Neuropathologic changes of multiple system atrophy and diffuse Lewy body disease.
abstract: The accumulation of fibrillar α-synuclein protein is the pathological hallmark occurring in both multiple system atrophy (MSA) and diffuse Lewy body disease (DLBD). The oligodendrocytes are especially involved in MSA, while subtypes of neurons are the targets in DLBD. In both instances, the changes are widespread within the central nervous system, but with distinct and topistic vulnerability.Two diagnostic subtypes of MSA are currently applied: multiple system atrophy-cerebellar (MSA-C), formerly designated olivopontocerebellar atrophy, and multiple system atrophy-parkinsonian (MSA-P), formerly called striatonigral degeneration. However, overlaps exist between them, notably during the late stages of the illness. The brainstem and cerebellum are especially vulnerable and dysfunctional in MSA-C, but the striatum and mesencephalon in MSA-P, wherein parkinsonism usually prevails.In DLBD, Lewy-body-containing neurons (LBCN) involve the cerebral cortex in addition to the sites affected in Parkinson disease (PD). Thus, dementia and parkinsonism occur, often without brain atrophy. Then, two main pathological subtypes are used: the limbic or the cortical type. Possibly, DLBD, either the limbic or cortical-type, is the ultimate stage of PD, which corresponds to the brainstem type. The epidemiologic and genetic traits causing sporadic MSA and sporadic DLBD are not known.
authors: Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York, New York.
authors: Etty Paola Cortes
authors: EP
authors: Ramirez
authors: Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York, New York.
authors: Jean Paul G
authors: JP
authors: Vonsattel
mesh_terms: D001921:Brain / Q000473:pathology*; D006801:Humans; D020961:Lewy Body Disease / Q000473:pathology*; D019578:Multiple System Atrophy / Q000473:pathology*; D009474:Neurons / Q000473:pathology