第50回日本神経学会総会
＜シンポジウム11―2＞プリオン病の最新トピックス
プリオン病サーベイランスの現状と成果
山田 正仁1）6）, 野崎 一朗1）, 浜口 毅1）, 篠原 もえ子1）, 北本 哲之2）6）, 中村 好一3）6）, 佐藤 猛4）6）, 水澤 英洋5）6）
1）金沢大学大学院脳老化・神経病態学（神経内科）〔〒920-8640 金沢市宝町13-1〕
2）東北大学大学院プリオン蛋白研究部門
3）自治医科大学公衆衛生学
4）東大和病院神経内科
5）東京医科歯科大学大学院脳神経病態学（神経内科）
6）厚生労働省難治性疾患克服研究事業・プリオン病及び遅発性ウイルス感染症に関する調査研究班・CJDサーベイランス委員会
The Creutzfeldt-Jakob Disease (CJD) Surveillance Committee has identified 1,241 patients with prion diseases during 1999-2009, including 953 with sporadic CJD (sCJD)(76.8%), 207 with genetic prion diseases (16.7%), 78 with environmentally acquired prion diseases (6.3%), and 3 with unclassified CJD. Among atypical cases of sCJD, most common was MM2 type including the cortical and thalamic forms. The genetic cases included 84 with a PrP V180I mutation (40.6%), 37 with a P102L mutation (17.9%), 34 with a E200K mutation (16.4%), 32 with a M232R mutation (15.5%), 4 with a P105L mutation (1.9%), and so on. The environmentally acquired cases included 77 with dura mater graft-associated CJD (dCJD) and one with variant CJD (vCJD). Combined with the results by the previous surveillance systems, a total number of dCJD in Japan was 135. The vCJD patient had a history of short stay in the UK and presented with periodic electroencephalogram in the late stage. Although there was no evidence of association of surgical procedures or blood transfusion with sCJD, 4.5% of the sCJD patients underwent operations after the onset of sCJD, including neurosurgical for 0.8% and ophthalmic for 1.9%, requiring more attention on prion diseases to reduce the iatrogenic risk.
Full Text of this Article in Japanese PDF (454K)
(臨床神経, 49：939−942, 2009)
key words：プリオン病, Creutzfeldt-Jakob病（CJD）, 硬膜移植後CJD, 変異型CJD, 日本
（受付日：2009年5月22日）
