肌强直性营养不良(MD)常伴发白内障、秃发、性功能障碍及神经系统病变等,作者用智能检查、头颅CT,EEG 和视觉诱发电位(VEP)来研究本病的CNS 损害。10侧MD,男6,女4,平均40(31～55)岁,病期21.3(9—42)年,7例两眼有白内障(1例已手术),除1例左眼失明外,矫正视力均为0.5～1.5。因肌病致生活不能自理之重症1例,生活自理有困难的中度患者4例,生活基本自理之轻症5例。检查结果如下:①智能障碍共9例;②CT 异常者7例,其中广泛的大脑皮层萎缩6例,脑室扩大5例,主要发生在侧脑室前角附近之脑室周围低密度改变5例。CT 有无异常及异常程度与病期无关,但与病情相关,即CT 正常或只有1种异常CT 表现者 Myotonic dystrophy (MD) is often accompanied by cataracts, alopecia, sexual dysfunction and neurological disease, the authors use the smart test, head CT, EEG and visual evoked potential (VEP) to study the disease CNS damage. 10 MD, male 6, female 4, average 40 (31-55) years old, the duration of 21.3 (9-42) years, 7 eyes had cataract (1 had surgery), except for 1 case of left eye blindness, Corrected visual acuity were 0.5 to 1.5. 1 case of severe illness due to myopathy and unable to take care of themselves, 4 cases of moderate patients who had difficulties in taking care of themselves, and 5 cases of mild cases of basic self-care. The examination results were as follows: (1) There were 9 cases of mental retardation; (7) 7 cases of abnormal CT, including 6 cases of atrophic cortical atrophy and 5 cases of ventricular enlargement, mainly occurred in 5 cases of low-density changes around the ventricle around the anterior horn of lateral ventricle. CT with or without abnormalities and the extent of the disease has nothing to do, but with the disease that CT is normal or only 1 abnormal CT manifestations