Iivanainen M, Leinikki P, Taskinen E, Shekarchi I C, Madden D, Sever J
Arch Neurol. 1981 Apr;38(4):206-8. doi: 10.1001/archneur.1981.00510040032003.
We studied CSF and serum samples from 16 patients with progressive myoclonus epilepsy (PME). These patients had juvenile-onset PME with evidence of autosomal recessive inheritance and no Lafora bodies. Twelve of the 16 patients with PME had immunologic abnormalities. Oligoclonal gamma bands were seen in six of the eight patients from whom sufficient CSF was available. The CSF albumin and serum/CSF albumin ratios were normal in all 16 patients, indicating the presence of intact blood-brain barriers. Six of the 16 patients showed increased CSF IgG levels and five had an increased CNS IgG synthesis. All patients had normal serum and CSF IgM and IgA levels. Three patients, all with bands, had reduced measles and/or vaccinia serum/CSF antibody ratios. The findings suggest altered immune response of the CNS of some patients with PME apparently caused by nonspecific immunostimulation.
我们研究了16例进行性肌阵挛癫痫（PME）患者的脑脊液（CSF）和血清样本。这些患者为青少年起病的PME，具有常染色体隐性遗传证据且无Lafora小体。16例PME患者中有12例存在免疫异常。在有足够脑脊液样本的8例患者中，6例可见寡克隆γ带。所有16例患者的脑脊液白蛋白以及血清/脑脊液白蛋白比值均正常，表明血脑屏障完整。16例患者中有6例脑脊液IgG水平升高，5例中枢神经系统IgG合成增加。所有患者的血清和脑脊液IgM及IgA水平均正常。3例均有带型的患者，其麻疹和/或牛痘血清/脑脊液抗体比值降低。这些发现提示，部分PME患者的中枢神经系统免疫反应改变显然是由非特异性免疫刺激所致。
