Gannushkina I V, Zhirnova I G, Toropova N G, Toporkina T D, Markova E D
Zh Nevropatol Psikhiatr Im S S Korsakova. 1981;81(7):1009-13.
In 37 patients with some hereditary diseases of the nervous system (deforming muscular dystonia, hepatocerebral dystrophy, essential tremor, etc.), as well as in 22 healthy subjects (donors), the percentage and the absolute content of the T- and B-lymphocytes in the blood were determined. Use was made of the reactions of spontaneous, active, and complementary rosette formation and determination of the B-cells from superficial immunoglobulins. In all the hereditary diseases listed a drop of the content of thymus-dependent lymphocytes and an increase of the capacity of lymphocytes for complementary rosette formation were revealed. The data obtained can be, probably, interpreted as evidences of a secondary immunodeficient state, possibly, caused by metabolic disturbances which are known to play an essential role in the pathogenesis of hereditary diseases of the nervous system.
对37例患有某些神经系统遗传性疾病（变形性肌营养不良、肝脑性营养不良、特发性震颤等）的患者以及22名健康受试者（供体）测定了血液中T淋巴细胞和B淋巴细胞的百分比及绝对含量。采用了自发、活性和补体玫瑰花结形成反应以及通过表面免疫球蛋白测定B细胞。在所列出的所有遗传性疾病中，均发现胸腺依赖性淋巴细胞含量下降以及淋巴细胞形成补体玫瑰花结的能力增强。所获得的数据或许可解释为继发性免疫缺陷状态的证据，这可能是由代谢紊乱所致，而代谢紊乱在神经系统遗传性疾病的发病机制中起着重要作用。
