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家族遗传性视神经病(Leber’s optic neuropat-hy)的病史特点为:在几周至数月内,患者先单眼后继发另一眼的双侧中央视觉迅速丧失。其典型病变表现为视乳头周毛细血管扩张性微血管病和神经纤维层水肿。本病多见于男青年,有阳性家族史,常合并心血管症状、心电图异常,但伴发二尖瓣脱垂则是首次报道。患者男,44岁,既往健康。1986年工作中突发胸痛,当时心电示:Ⅰ、Ⅲ、aVL、aVF、V5和V6导联T波倒置,左心室肥厚电压,V1-V3ST段压低。就诊时心电无改变,连续心源酶测值正常。心脏听诊无异常,但二维超声(2D echo)和心导管插入证实存在二尖瓣脱垂,冠状动脉无异常。1988年3月患者出现左眼视力模糊两月,否认心脏任何症状。
The history of Leber’s optic neuropat-hy is characterized by a rapid loss of bilateral central visual acuity in patients with monocular following secondary to within a few weeks to several months. The typical lesions manifested as papillary capillary dilated microvascular disease and nerve fiber layer edema. The disease more common in young men, a positive family history, often associated with cardiovascular symptoms, abnormal ECG, but with mitral valve prolapse is the first report. Male patient, 44 years old, formerly healthy. In 1986, the work had sudden chest pain. At that time, ECG showed T wave inversion of Ⅰ, Ⅲ, aVL, aVF, V5 and V6 leads, voltage of left ventricular hypertrophy, and depression of V1-V3ST. ECG no change at the time of treatment, continuous cardiac enzyme test normal. Cardiac auscultation was normal, but 2D echo and cardiac catheterization confirmed mitral valve prolapse and no abnormalities in the coronary arteries. March 1988 Left eye vision blur in two patients, denied any symptoms of the heart.