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运动神经元病(MND)可影响一个家族中的多个成员。作者从诊断为MND的4,071例中,发现116例为家族性。通过复习病史及死亡证书等资料证实在72个家族中有多个成员存在MND。在1983年12月完成本研究时仍然活着的6例获得追踪资料,其它病例均已死亡。从死亡的97例中86例获得死亡证书。在83例死者中73例MND作为主要的死因,另外13例的死因列为:多数性硬化5例,肌营养不良4例,自杀、弥散性硬化、弥散性关节炎及脊髓肿瘤各1例。在20个不同的家族尸解报告中34例都证实了MND的诊断。66个先证者影响一个或多个一级亲属:包括29个父亲,21个母亲,30个兄弟,25个姐妹,6个儿子和8个女儿。在72个
Motor neuron disease (MND) can affect multiple members of a family. Of 4,071 patients diagnosed with MND, 116 were found to be familial. Review of medical history and death certificates confirm that there are multiple members in 72 families that have MND. Six patients who were still alive at the time of completion of the study in December 1983 were followed up and all other cases were fatal. 86 death certificates were obtained from 97 deaths. Among the 83 deceased patients, 73 were MND as the main cause of death. The other 13 deaths were classified as 5 cases of most sclerosis, 4 cases of muscular dystrophy, 1 case of suicide, diffuse sclerosis, diffuse arthritis and spinal cord tumors. Thirty-four of 20 different autopsy reports confirmed the diagnosis of MND. The 66 probands affected one or more first-degree relatives: 29 fathers, 21 mothers, 30 brothers, 25 sisters, 6 sons and 8 daughters. At 72