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目的 研究迪谢内 /贝克肌营养不良 (DMD/BMD)患者视网膜眼电图 (ERG)的改变特征 ,探讨ERG对DMD/BMD的诊断价值及dystrophin在视网膜电信号传导上的作用。方法 对 2 2例临床确诊的DMD/BMD患者进行详细的眼科检查和ERG检测 ,采用ERG国际测量标准记录结果。 结果 全部患者眼科检查无明显异常。 2 2例患者中 16例 (72 7% )出现异常ERG改变 ;其中 15例为暗适应蓝光b波波幅下降或 (和 )消失 (P <0 0 0 1)。 13例暗适应白光b波波幅下降或 (和 )潜伏期延长(P <0 0 0 1)。 12例b/a波波幅比≤ 1(P <0 0 0 1)。 16例振荡电位OPs1～ 4 波波幅下降。结论 ERG可作为DMD/BMD诊断和症状前判断的一项有价值的检查。dystrophin及其同源蛋白在视网膜电信号的传导上起着重要作用。
Objective To study the changes of retinal electroretinography (ERG) in patients with Dickinson / Becker muscular dystrophy (DMD / BMD) and to investigate the diagnostic value of ERG in DMD / BMD and the role of dystrophin in the transmission of retinal signals. Methods Twenty-two clinically diagnosed DMD / BMD patients underwent detailed ophthalmic examination and ERG test, and ERG international measurement standards were used to record the results. Results All patients ophthalmoscopy no obvious abnormalities. Abnormal ERG was found in 16 (72.7%) of the 2 2 patients; in 15 of them, the amplitude of dark adaptation b wave decreased or disappeared (P <0.01). Thirteen patients underwent ambiguous white light wave amplitude reduction or (and) latency (P <0 001). The amplitude of b / a wave in 12 cases was ≤ 1 (P <0 0 0 1). 16 cases of oscillation potential OPs1 ~ 4 wave amplitude decreased. Conclusion ERG can be used as a valuable diagnostic and pre-symptomatic diagnosis of DMD / BMD. Dystrophin and its cognate proteins play an important role in the transmission of retinal electrical signals.