第48回日本神経学会総会
＜シンポジウム11-2＞西太平洋地域の筋萎縮性側索硬化症/パーキンソン痴呆複合（ALS/PDC）と関連神経変性疾患
紀伊ALS/PDC（牟婁病）の歴史，概念，疫学
葛原 茂樹
三重大学神経内科〔〒514-8507 三重県津市江戸橋2-174〕
現 国立精神・神経センター武蔵病院〔〒187-8551 東京都小平市小川東町4-1-1〕
The Muro district includes the southern coastal mountainous areas of the Kii peninsula of Japan where high incidence foci of ALS and parkinsonism-dementia complex (PDC) exist. "Muro disease" refers to the endemic ALS in Muro, and the oldest description is in a book published in 1689. Miura reported high prevalence of ALS in Muro in 1911, and the first epidemiological survey by Kimura and Yase in 1960s disclosed extremely high prevalence of ALS in Hohara and Kozagawa. The high incidence was, however, reported to have disappeared by early 1980s as in Guam. In 1990s we resurveyed and found not only continuous high ALS incidence but also neuropathologically-verified PDC in Hohara. ALS and PDC here frequently affected one individual simultaneously and members in a family. Neuropathological changes were common to ALS and PDC, showing a combination of ALS changes and many neurofibrillar tangles (NFTs) in the brainstem and cerebral cortex, suggesting ALS/PDC may be a single entity with different clinical manifestations, "ALS-parkinsonism-dementia complex". TDP-43-positive inclusions were confirmed in all cases examined. Age-adjusted incidence rates during 1950 and 2000 have showed that incidence of ALS gradually declined for 50 years while that of PDC rose up steeply in 1990s, suggesting changing pattern of ALS/PDC that had occurred in Guam in 1970s. Continuing high incidence of ALS/PDC and high familial occurrence suggest that primary cause of Kii ALS/PDC may be genetic rather than environmental.
(臨床神経, 47：962−965, 2007)
key words：筋萎縮性側索硬化症, パーキンソン認知症複合, 紀伊半島, 疫学, 歴史
（受付日：2007年5月16日）
