报告2例(兄妹)脑皮质-纹状体-脊髓变性,中年发病,主要为精神异常,锥体束及锥体外系症状,脑皮质呈海绵状,神经元的坏变和消失,星形细胞的肥大和增生。认为本病可能同某种遗传性缺陷使病毒对中枢神经系统有较高的易伤性有关,推测本病与运动神经元病可能有一定关系,脑血液循环障碍或代谢障碍在发病机制中可能起某种作用,故应用抗病毒、改善脑血液循坏和代谢的治疗。 Reported in 2 cases (siblings) cerebral cortex - striatum - spinal cord degeneration, middle-aged onset, mainly mental disorders, pyramidal tract and extrapyramidal symptoms, cerebral cortex was cavernous, neuronal degeneration and disappearance of stars Cell hypertrophy and hyperplasia. That the disease may be associated with some genetic defects of the virus on the central nervous system has a higher vulnerability, speculated that the disease and motor neuron disease may have a certain relationship, cerebral blood circulation disorders or metabolic disorders in the pathogenesis may be Play a role, so the application of anti-virus, improve cerebral blood circulation and metabolism of the treatment.