レヴィ小体型認知症の全身病理

Multi-Organ Distribution of Alpha-Synuclein Pathology in Dementia with Lewy Bodies

パーキンソン病（PD）とレヴィ小体型認知症（DLB）ではαシヌクレインの蓄積は中枢神経系のみならず，交感神経節，消化管，心臓，副腎，皮膚にも広がっている。さらに，パーキンソン症状も認知症も認められないが，剖検により偶発的にレヴィ小体が認められた例でも中枢および末梢におけるレヴィ小体の広がりはPDやDLBとほぼ同様である。これより中枢と末梢はほぼ同時的におかされると思われる。このことはレヴィ小体病の病変が多中心性に起こっていることを意味する。

Abstract

In Parkinson's disease and dementia with Lewy bodies, neuronal α-synuclein aggregates (Lewy bodies and Lewy neurites) are distributed throughout the nervous system, including the brain, spinal cord, sympathetic ganglia, enteric nervous system, cardiac and pelvic plexuses, submandibular gland, adrenal medulla, and skin. Lewy bodies also occur in 10-20% of neurologically asymptomatic individuals older than 60 years. These cases are called incidental Lewy body disease (ILBD). In ILBD, Lewy bodies can be found in the brain, spinal cord, sympathetic ganglia, visceral autonomic nervous system and skin. In addition, neuronal loss in the substantia nigra is observed in ILBD. Thus, ILBD represents pre-symptomatic Parkinson's disease and/or dementia with Lewy bodies. The pathological process of Lewy body disease may affect the peripheral and central nervous systems at the same time.