Hydroencephalodysplasiaの経験

Six Cases of Hydroencephalodysplasia

Ⅰ．はじめに

　小児神経疾患の中でその原因が先天性奇形に由来するものは多数あるがhydroencephalodysplasiaもその1つである．Hydroencephalodysplasiaとは1955年Picaza8）によって命名されたものであり大部分がhydrocephalusの一部に含まれている．しかしいわゆる狭義のhydrocephalusに比べて一般に予後が悪く，臨床症状，病理解剖所見等の点において明らかに区別される．

　われわれは昭和39年以降6例のhydroencephalodysplasiaを経験したが，今回は臨床症状，診断上の問題について検討を加えたので報告する．

Six cases of hydroencephalodysplasia were evaluated from the points of clinical signs and symptoms and neuroradiological procedures and diagnostic problems were discussed.

All of our cases were characterized by extensive dysplasia of the brain and increased cerebrospinal fluid pressure. Deformity of the head and developmental arrest of the psychomotor function were observed in five cases. The side of the skull deformity was consisted with that of the brain defect.