卵黄様黄斑変性症の母子例の電気生理学的所見

Electrophysiological features of vitelliform macular dystrophy in a mother and her son

　卵黄様黄斑変性症では，EOGでのL／D比の著明な低下が知られている。筆者らは母子に認められた症例に諸種電気生理学的検討を行った。症例1は13歳男児，右眼黄斑部にニボーを伴う黄色斑，左眼黄斑部にいり卵状黄色斑を認めた。症例2は38歳女性，症例1の母で，両眼黄斑部に網脈絡膜萎縮巣を認めた。電気生理学的検査では2例ともscotopic, photopic, flicker, single flash ERG, pattern-reversalVECPは正常であったが，EOGでL／D比の著しい低下を認め，pattern-reversal ERGは消失型で，網膜内層の障害を示唆した。

We observed vitelliform macular dystrophy in a 13-year-old boy and his mother aged 38 years.The macula showed pseudohypopyon appearance in the right eye and scrambled-egg one in the left in the first case. Chorioretinal atrophy had developed in the macula in his mother. Both cases showed normal findings by electroretinogram (ERG) and visual evoked cortical potential (VECP). Both cases showed reduced light/dark ratio (Arden index) in electrooculogram and abnormal pattern ERG (PERG) in both eyes. The abnormal PERG seemed to indicated that a dysfunction in the inner retinal layers had set in both cases.