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对同一家系11名显性遗传CSNB 患者进行全视野明视ERG 分析,发现8名患者出现异常,表现为:1.明视白光刺激的b 波峰时延长或振幅降低;2.白光刺激出现负电反应;3.对闪烁光刺激的反应振幅降低甚至无波。认为CSNB 的主要病损在视网膜杆体系统,但锥体系统也受到不同程度的损害。提出根据电生理和心理物理检查可将本病分为仅累及杆体系统的单纯型和杆体、锥体均受到损害的混合型。临床上这两种不同的亚型是CSNB 基因不同表现度的反映。
A total of 11 dominant CSNB patients in the same pedigree were subjected to full-field bright-field ERG analysis and found that 8 patients had abnormalities manifested as: 1. prolonged or decreased amplitude of peak b in bright white light stimuli; 2. negative reaction in white light stimulated ; 3 response to flashing light stimulus amplitude reduction or even no wave. It is believed that the major lesion of CSNB is in the retinal rod system, but the cone system is also damaged to varying degrees. Proposed according to electrophysiological and psychophysical examination can be divided into the disease only involving the rod system simple and rod body, cone are compromised hybrid. Clinically, these two different subtypes reflect the different degrees of CSNB gene expression.