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对一个AD遗传型CSNB家系的11名患者22眼检测、分析了全视野暗视ERG。结果所有患眼的暗视ERG均有异常,并发现三种明显不同的类型。即以a波正常b波降低甚至出现负电反应为特征的Schubert-Bornschein型,以a波、b波振幅均下降为特征的Riggs型和以暗视白光和蓝光刺激下均无波为特征的无波型。认为不同类型的ERG反应并不表示不同的遗传本质,只代表CSNB基因的不同表现度。指出全视野暗视ERG检查对于CSNB的临床诊断、分型及与其它视网膜病的鉴别诊断均有重要价值。
Twenty-two eyes of 11 patients with an AD-linked CSNB pedigree were analyzed for total-field-under-scotopic ERG. Results All eyes with strabismus ERG were abnormal and found three distinct types. That is, the Schubert-Bornschein type characterized by the decrease or even the negative b-wave of the normal a-wave, the Riggs-type characterized by a-wave and b-wave amplitudes, and the wave- Wave type. It is considered that different types of ERG responses do not represent different genetic backgrounds and only represent different degrees of CSNB gene expression. It is pointed out that ERG examination of total scotopic eyesight has important value in the clinical diagnosis, classification of CSNB and differential diagnosis of other retinopathy.