神经型棘红细胞增多症(Neuroacanthocytosis,NA)是一种罕见的常染色体隐性遗传病。作者报告一名NA且血清中无前β-脂蛋白血症的患者。 患者为女性,30岁。入院前一年出现全身性强直一阵挛性惊厥发作,首次发病后一个月,发生进行性口面运动障碍、流涎、咽下困难及吞咽困难、躯干和四肢的舞蹈动作。患者双亲为一级表姻亲,其侄儿自18岁起手足有异常姿势。据诉,患者之姐因罹患同样疾病于38岁死亡。患者之母由于肝炎及肝功衰竭 Neuroacanthocytosis (NA) is a rare autosomal recessive disease. The authors report a patient with NA and no pre-beta-lipoproteinemia in the serum. Patient is female, 30 years old. One year before admission, there was generalized tonic-clonic convulsive seizures. One month after the first episode, progressive facial dyskinesia, salivation, dysphagia and dysphagia, and dance movements of the trunk and extremities occurred. Patient’s parents as a table in-laws, his nephew since the age of 18 hands and feet have unusual posture. According to the complaint, the sister of the patient died at 38 years of age suffering from the same disease. The mother of the patient due to hepatitis and liver failure