進行性の痴呆・行動異常を呈した40歳女性

A 40-year-old Woman with Progressive Dementia and Abnormal Behavior

症例呈示

　症例　H．E．，40歳女性（1957年3月20日生）

　家族歴　両親はいとこ婚。3人兄弟（姉，本人，弟）で弟も類症発症し1995年7月，死亡。

We report a 40-year-old Japanese woman who died after 12 years history of progressive dementia and abnorinal behaviors. She was well until 1985 at her age of 28 years old, when she had an onset of behavioral change in which she drank much, neg-lected house-keeping works, and her life style became sloppy. At age 30, she became unable to understand written sentences, and paced up^- and down in and out of her house.