CONE DYSTROPHY AND SUPERNORMAL DARK-ADAPTED B-WAVES IN THE ELECTRORETINOGRAM
CONE DYSTROPHY AND SUPERNORMAL DARK-ADAPTED B-WAVES IN THE ELECTRORETINOGRAM
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DOI：
10.1007/bf02764303
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发表时间：
1990-01-01
影响因子：
2.7
通讯作者：
WESSING, A
中科院分区：
文献类型：
--
作者：
FOERSTER, MH;KELLNER, U;WESSING, A
对一名患有视锥细胞营养不良的男性患者进行了9年以上的随访。除了典型的临床和电生理体征外，暗适应视网膜电图中还发现了异常的b波。我们的情况下，与文献中描述的12个类似的患者进行了比较。我们的患者与其他患者的不同之处在于：他是男性，患有先天性静止性疾病，仅在左眼有一个小的色素上皮瘢痕，直到22岁时没有其他眼底变化。他没有抱怨夜盲症。我们的病人暗适应视网膜电图显示b波阈值正常，b波振幅增加，b波潜伏期和内隐时间明显延长。迄今为止，在任何其他患者中均未报告这种体征组合，并指出神经元间连接的postreceptoral缺陷。
A male patient suffering from cone dystrophy was followed over 9 years. In addition to the typical clinical and electrophysiologic signs, supernormal b-waves were found in the dark-adapated electroretinogram. Our case is compared with 12 similar patients described in the literature. Our patient differed from the other patients in the following aspects: he was male and had a congenital stationary disease with a small pigment epithelial scar in the left eye only and no other fundus changes up to the age of 22 years. He did not complain of night blindness. The dark-adapted electroretinogram of our patient showed a normal b-wave threshold with increased b-wave amplitudes and markedly prolonged b-wave latencies and implicit times. This combination of signs has not been reported to date in any other patient and points towards a postreceptoral defect of the interneuronal connection.