臨床神経学

<シンポジウム33―2>パーキンソン病の病態から臨床update

病理

齊藤 祐子1), 村山 繁雄2)

1)国立精神・神経医療研究センター病院臨床検査部〔〒187―8551 東京都小平市小川東町4―1―1〕
2)東京都健康長寿医療センター高齢者ブレインバンク

This research is based on the brain bank project, which combines prospective clinical follow ups and retrospective neuropathological studies. Pathology of idiopathic Parkinson disease (PD) can be summarized as a spectrum of Lewy body (LB) disease (LBD) comprising PD, dementia with LB (DLB) and pure autonomic failure (PAF). Recently core protein component of LB is proved to be alpha-synuclein, which is truncated, phosphorylated and ubiquitinated. Immunohistochemistry with anti-phosphorylated alpha-synuclein (psyn) antibody visualizes LB pathology with previously unattained high sensitivity and specificity, and enables pathological studies of peripheral autonomic nervous system as well as central nervous system. Recently Braak et al proposed ascending extension hypothesis of LB pathology, based on consecutive autopsy cases of PD and normal controls without dementia. However, not excluding demented cases, we found olfactoryamygdala extension pathway of LB pathology, which is independent from Braak's ascending pathway. We propose that abnormal seeding and aggregation of alpha-synuclein could be formed in peripheral autonomic nervous system or olfactory bulb and extend via neural network and form clinical phenotype of LBD.
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(臨床神経, 51:1168−1171, 2011)
key words:リン酸化αシヌクレイン,末梢自律神経,嗅球,扁桃核,Braak仮説

(受付日:2011年5月20日)