Williams L L, Kissel J T, Shannon B T, Wright F S, Mendell J R
Department of Pediatrics, Neurology, Ohio State University College of Medicine, Columbus.
J Neuroimmunol. 1992 Feb;36(2-3):147-55. doi: 10.1016/0165-5728(92)90046-n.
To evaluate possible immune-mediated mechanisms in hereditary motor and sensory neuropathy (HMSN-I, Charcot-Marie-Tooth syndrome), we examined class II major histocompatibility complex antigen expression (MHC-II, HLA-DR) in Schwann cells and peripheral lymphocyte T-cell (Ta1, CD26) activation in five unrelated adults with HMSN-I. Evidence of increased activation expression was found in both compartments but the pattern did not suggest a general state of hyperimmunity or appear related to clinical characteristics of HMSN. Significantly increased CD26+ T-cell activation and greater than normal fluctuation of values occurred intermittently in sequential tests of eight HMSN patients and at single time points in 24 others. The combined data, consistent with repeated stimulations of an immune reaction under normal feedback control, suggest that HMSN-I expresses some characteristics also found in autoimmune polyneuropathies.
为评估遗传性运动和感觉神经病（HMSN-I，夏科-马里-图斯综合征）中可能的免疫介导机制，我们检测了5例无亲缘关系的成年HMSN-I患者雪旺细胞中的II类主要组织相容性复合体抗原表达（MHC-II，HLA-DR）以及外周淋巴细胞T细胞（Ta1，CD26）的活化情况。在这两个部分均发现活化表达增加的证据，但该模式并不提示存在普遍的高免疫状态，也似乎与HMSN的临床特征无关。在8例HMSN患者的连续检测中以及另外24例患者的单个时间点，间歇性出现CD26+ T细胞活化显著增加以及值的波动大于正常情况。综合数据表明，在正常反馈控制下免疫反应反复受到刺激，这提示HMSN-I表现出一些自身免疫性多发性神经病中也存在的特征。
