本文报告1例先天性风疹综合征的儿童发生慢性进行性全脑炎。其母妊娠早期曾接触风疹。2岁前智力发育正常,但言语迟钝,4岁出现听力障碍,8岁开始出现进行痴呆、共济失调及舞蹈性运动,11岁出现黄斑区色素沉着及肌阵挛发作,脑脊液细胞9/立方毫米,蛋白156毫克％,52％为γ-球蛋白,脑电图为普遍中度慢波,偶有高波幅慢活动,气脑示第四脑室对称性扩大,脑血管造影正常,11岁时血清的风疹病毒 This article reports a case of congenital rubella syndrome in children with chronic progressive total encephalitis. Her mother had been exposed to rubella early in her pregnancy. 2 years before normal intelligence development, but slow speech, 4-year-old hearing impairment, dementia began at the age of 8, ataxia and dance exercise, 11-year-old macular pigmentation and myoclonus episodes, cerebrospinal fluid cells 9 / cubic Mm, protein 156 mg%, 52% of γ-globulin, EEG is generally moderate slow wave, occasional high amplitude slow activity, the fourth cerebral ventricle to expand Symmetry, cerebral angiography normal, 11 years old Serum rubella virus