Goldstein D S, McRae A, Holmes C, Dalakas M C
Clinical Neuroscience Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD 20892, USA.
Clin Auton Res. 1996 Feb;6(1):17-21. doi: 10.1007/BF02291401.
We report here the case of a patient with the Shy-Drager syndrome and multiple myeloma who had evidence consistent with a central neural autoimmune basis for sympathetic autonomic failure. Autonomic function testing showed no recordable peroneal skeletal muscle sympathoneural traffic, normal arterial norepinephrine (NE) spillover during supine rest and no increment in NE spillover during exposure to lower body negative pressure. The patient's cerebrospinal fluid and serum contained an immunoglobulin G that bound to rat locus ceruleus (LC) in an in vitro test system. The myeloma protein was of the lambda subtype and bound in the rat LC, without binding in the substantia nigra, as demonstrated with anti-lambda antiserum. Since in this case the monoclonal antibody produced by the myeloma bound specifically to LC cells, the results are consistent with the hypothesis that in this patient the Shy-Drager syndrome may have had an immune-mediated basis.
我们在此报告一例患有夏伊-德雷格综合征和多发性骨髓瘤的患者，其证据表明交感神经自主神经功能衰竭存在中枢神经自身免疫基础。自主神经功能测试显示，腓骨骨骼肌交感神经传导无记录，仰卧休息时动脉去甲肾上腺素（NE）溢出正常，下身负压暴露期间NE溢出无增加。在体外测试系统中，患者的脑脊液和血清中含有一种与大鼠蓝斑（LC）结合的免疫球蛋白G。骨髓瘤蛋白为λ亚型，如用抗λ抗血清所示，其在大鼠LC中结合，而在黑质中不结合。由于在该病例中骨髓瘤产生的单克隆抗体特异性结合LC细胞，结果与该患者夏伊-德雷格综合征可能有免疫介导基础的假说一致。
