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家族性成人运动神经元病:肌萎缩侧索硬化症

Familial adult motor neuron disease: amyotrophic lateral sclerosis.

作者信息

Mulder D W, Kurland L T, Offord K P, Beard C M

出版信息

Neurology. 1986 Apr;36(4):511-7. doi: 10.1212/wnl.36.4.511.

DOI:10.1212/wnl.36.4.511
PMID:3960325
Abstract

We analyzed the medical records of 103 patients with familial adult motor neuron disease (MND). In the 72 families, 329 members were known to be affected. Observations were compared with the sporadic and Mariana forms of MND. Clinical and laboratory examinations of all three forms were similar in clinical course and findings, but there were minor variations in age at onset, sex ratio, survival, and the frequency with which onset occurred in the lower extremities. Recognition of the familial form still depends on diagnosis of the disease in more than one member of a family.

摘要

我们分析了103例家族性成人运动神经元病(MND)患者的病历。在这72个家庭中,已知有329名成员患病。将观察结果与散发性和马利阿纳型MND进行了比较。所有三种类型的临床和实验室检查在临床病程和检查结果方面相似,但在发病年龄、性别比例、生存率以及下肢发病频率方面存在细微差异。家族性类型的确诊仍然依赖于一个家族中不止一名成员被诊断出患有该病。

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