Aicardi症候群（典型例5例と非典型例1例）について

Aicardi's syndrome. Report of five typical and one atypical cases.

　我々は愛知県心身障害者コロニー中央病院において，Aicardi症候群6例（典型例5例および非典型例1例）を経験したので，その臨床症状を報告した．さらに同院で診断された点頭てんかんを有する312名と，脳梁欠損を有する61名について，眼科的所見を検討し，Aicardi症候群の頻度を求め，以下の結論を得た．

（1）点頭てんかんを有する者の2％，脳梁欠損を有する者の10％が本症候群であった．

（2）過去に100例以上が報告されうち2例以外は総て女児であったが，女児で脳梁欠損と点頭てんかんを合併する者が本症候群である可能性は極めて高い．

（3）全前脳胞症と滑脳症は本症候群の類縁疾患である可能性は低い．

　点頭てんかんを有する者や脳梁欠損を有する者についての眼科的検査は極めて重要であり，決して稀ではない本症候群とその類縁疾患の解明に大きく寄与するものと考えられる．

We observed 6 cases of Aicardi's syndrome during the past 5-year period. All were females from 1 to 13 years of age. The 5 typical cases manifested in-fantile spasm, chorioretinopathy showing lacunae and agenesis of the corpus callosum. One atypical case manifested infantile spasm and agenesis of corpus callosum. Her left eye was normal, while the right eye showed microphthalmia with coloboma of the optic nerve.

We analyzed the clinical records of the Welfare Center to evaluate the incidence of Aicardi's syndrome in patients with infantile spasm (312 cases) and age-nesis of the corpus callosum (61 cases). The inci-dence of Aicardi's syndrome was about 2% in patients with infantile spasm and 10% in agenesis of corpus callosum. There was a very high likelihood of pre-sence of Aicardi's syndrome in female subjects with both infantile spasm and agenesis of corpus callosum. Holoprosencephaly and lissencephaly were not asso-ciated with Aicardi's syndrome, even though the both conditions frequently manifested agenesis of corpus callosum.