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患有肌萎缩侧索硬化症和帕金森病痴呆症的关岛人的细胞免疫

Cellular immunity in Guamanians with amyotrophic lateral sclerosis and Parkinsonism-dementia.

作者信息

Hoffman P M, Robbins D S, Nolte M T, Gibbs C J, Gajdusek D C

出版信息

N Engl J Med. 1978 Sep 28;299(13):680-5. doi: 10.1056/NEJM197809282991302.

Abstract

To test the hypothesis that host resistance factors may be abnormal in Guamanians in whom amyotrophic lateral sclerosis and Parkinsonism-dementia develop, cellular immunity was evaluated in both diseases and compared to that of Guamanians with other nervous-system diseases, normal adult Guamanians and non-Guamanians with amyotrophic lateral sclerosis and Parkinsonism. Diminished responses to skin-test antigens, lymphopenia, diminished per cent and total T cells and, less frequently, decreased mitogen responses were seen in Guamanian patients with amytorophic lateral sclerosis and Parkinsonism-dementia but not in the other patient or normal groups. Guamanian patients with amyotrophic lateral sclerosis and diminished cellular immunity had an increased frequency of HLA-Bw35 (P less than 0.005) and shorter mean duration of disease (P less than 0.05) than those with normal cellular immunity. In Parkinsonism dementia diminished cellular immunity was less strongly associated with HLA-BW35 (P less than 0.05) and was not associated with differences in duration of disease. Normal Guamanians and those with other nervous-system diseases showed no association of diminished cellular immunity with HLA-Bw35. The association appeared disease-related, with onset concomitant with the neurologic expression of Guamanian amyotrophic lateral sclerosis and Parkinsonism-dementia.

摘要

为验证在患肌萎缩侧索硬化症和帕金森痴呆症的关岛人中宿主抵抗因子可能异常这一假说,对这两种疾病患者的细胞免疫功能进行了评估,并与患有其他神经系统疾病的关岛人、关岛正常成年人以及患有肌萎缩侧索硬化症和帕金森症的非关岛人进行了比较。在患有肌萎缩侧索硬化症和帕金森痴呆症的关岛患者中,观察到对皮肤试验抗原的反应减弱、淋巴细胞减少、T细胞百分比和总数降低,以及较少见的丝裂原反应降低,但在其他患者或正常组中未观察到这些情况。与细胞免疫功能正常的关岛肌萎缩侧索硬化症患者相比,细胞免疫功能减弱的患者中HLA - Bw35频率增加(P小于0.005),疾病平均持续时间较短(P小于0.05)。在帕金森痴呆症中,细胞免疫功能减弱与HLA - BW35的相关性较弱(P小于0.05),且与疾病持续时间差异无关。关岛正常人和患有其他神经系统疾病的人未显示细胞免疫功能减弱与HLA - Bw35有关联。这种关联似乎与疾病相关,且与关岛肌萎缩侧索硬化症和帕金森痴呆症的神经学表现同时出现。

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