BRAIN and NERVE－神経研究の進歩69巻1号

2017年01月発行

特集 近年注目されている白質脳症

Neuronal Intranuclear Inclusion Disease（NIID）—エオジン好性核内封入体病

著者： 曽根淳¹² 祖父江元³

所属機関： ¹名古屋大学大学院医学系研究科神経内科学 ²名古屋大学大学院医学系研究科難治性神経疾患治療学 ³名古屋大学大学院医学系研究科脳と心の研究センター

ページ範囲：P.5 - P.16

文献概要

NIID（neuronal intranuclear inclusion disease）は，近年，診断数が増加し注目されている疾患である。孤発性NIIDでは，多数の症例で認知機能障害，髄液蛋白質の上昇，頭部MRIでの白質脳症，および拡散強調画像での皮髄境界の高信号が認められ，家族性NIIDでは，認知症を初発症状とする群と，四肢筋力低下から発症する群の2群が認められる。多くのNIID症例が，いまだ正確に診断されていない可能性があり，NIID診断フローチャートの活用が有効である。

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