中枢神経系海綿状変性症（van Bogaert-Bertrand Type；Canavan病）

Spongy degencration of the central nervous system (van Bogacrt-Bcrtrand type ; Canavan's discase)

　中枢神経系海綿状変性症（van Bogacrt-Bertrand型）は，稀であり，初期の報告では，汎発性白質硬化症の一つとされていた^1〜5）。

　van BogaertとBertrand（1949）^6）が，この病気の特有な臨床的，および病理的所見を確立したので，最近，この病名が，用いられている。

　The historical, clinical, genetic, pathological and biochemical features of spongy degeneration of the central nervous system (van Bogaert and Bertrand type; Canavan's disease), including our 13 cases, are reviewed.

　Although the high incidence of the disease in off-spring of Jews has been stressed by earlier reports, recent studies have shown an increasing frequency in non-Jewish families. The infantile form of the disorder is most commonly observed and shows characteristic megalencephaly by 4 months of age.