The Japanese journal of neuropsychology

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ArticleTitle	Clinical features and neuropathological background of Lewy body disease; Parkinson's disease, Parkinson disease (with) dementia and dementia with Lewy bodies
Language	J
AuthorList	Shigeki Kuzuhara
Affiliation	Department of Neurology, Mie University Graduate School of Medicine
Publication	Japanese Journal of Neuropsychology: 22 (3), 200-211, 2006
Received
Accepted
Abstract	Lewy body disease refers to a group of neurodegenerative diseases that are neuropathologically characterized by abundant Lewy bodies in the central nervous system. Lewy bodies and Lewy neurites are most sensitively demonstrated with α-synuclein immunohistochemistry. Three core clinical phenotypes include Parkinson's disease (PD), Parkinson disease (with) dementia (PDD) and dementia with Lewy bodies (DLB). As to the clinicopathological correlation, PD corresponds to the brainstem type, PDD, to the limbic type, and DLB, to the diffuse neocortical type of Lewy body disease. Full-blown clinical features as well as neuropathological findings are similar between PDD and DLB although the temporal sequence of appearance of parkinsonian symptoms and dementia are reversed between them. Recent gene studies have demonstrated that either mutation or increase in dose of the α-synuclein gene causes Lewy body disease, and the clinical phenotypes of PD, PDD and DLB may be related to the quality or quantity of the protein. Whether PDD and DLB are different clinical phenotypes of one disease or apparent resemblance of different diseases at the late stage remains to be clarified.
Keywords	Lewy body disease, dementia, Parkinson's disease, dementia with Lewy bodies, α-synucleis