Kitahara T, Okumura K, Semba A, Yamaura A, Makino H
J Neurol Neurosurg Psychiatry. 1982 Nov;45(11):1048-52. doi: 10.1136/jnnp.45.11.1048.
The genetic and immunologic abnormalities associated with the pathogenesis of moya-moya were assessed in 23, 13 children and 10 adults with angiographically diagnosed moya-moya. In HLA-A, -B, -C stereotyping, an association was found of AW24, BW46, and BW54 with relative risks of 3.83, 6.50, and 3.58 respectively. Natural T cell toxic autoantibody was detected by FACS analysis in sera from five out of 23 patients. Millipore filter assay for autoantibody against double-stranded DNA revealed higher than normal binding in sera from four out of 18 patients. Anti-vessel antibody which might be responsible for vascular change associated with moya-moya was not detected in any of the 23 patients studied. Significant association of the disease with certain HLA types, in addition to the presence of natural T cell toxic autoantibody and anti-double-stranded DNA antibody in patients' sera, supports the theory that genetic and immunologic disturbances may underly the pathogenesis of moya-moya.
对23例经血管造影诊断为烟雾病的患者（13例儿童和10例成人）进行了与烟雾病发病机制相关的遗传和免疫异常评估。在HLA - A、- B、- C定型分析中，发现AW24、BW46和BW54存在关联，相对风险分别为3.83、6.50和3.58。通过荧光激活细胞分选术（FACS）分析，在23例患者中的5例血清中检测到天然T细胞毒性自身抗体。针对双链DNA的自身抗体的微孔滤膜分析显示，18例患者中的4例血清结合高于正常水平。在所研究的23例患者中，均未检测到可能与烟雾病相关血管变化有关的抗血管抗体。除了患者血清中存在天然T细胞毒性自身抗体和抗双链DNA抗体外，该疾病与某些HLA类型的显著关联支持了遗传和免疫紊乱可能是烟雾病发病机制基础的理论。
