Greiner A, Schmausser B, Petzold K, Krüger H, Marx A
Institut für Pathologie, Universität Würzburg, Germany.
Acta Neuropathol. 1996;91(1):67-71. doi: 10.1007/s004010050393.
Sera and cerebrospinal fluid (CSF) from 25 patients with amyotrophic lateral sclerosis (ALS) were tested by immunofluorescence on fetal, juvenile and adult central and peripheral neuronal (CNS/PNS) tissues and on nerve biopsy material from affected patients for the presence of autoantibodies. Results were compared with control sera from normal blood donors (n = 45) and patients with other neurological diseases (OND) (n = 11). Three different types of tissue reactivity (glial, axonal, and small blood vessels) were found. Antibodies binding to glial and axonal structures were found in 32% of ALS patients as compared to 12% in normal and 27% in OND controls. In contrast, staining of endothelial cells was found with 24% of ALS sera and CSF but not with normal and OND control sera and was demonstrated only with fetal and juvenile nervous tissue and with suralis nerve biopsies of two of five ALS patients. However, normal or inflamed adult CNS/PNS tissue was not stained with these sera. We conclude that ALS is most likely a heterogeneous group of diseases and only a subgroup of ALS may have an autoimmune pathogenesis. These findings may, therefore, have implications for the evaluation of any immunosuppressive treatment in ALS.
采用免疫荧光法，对25例肌萎缩侧索硬化症（ALS）患者的血清和脑脊液（CSF）进行检测，观察其在胎儿、青少年和成人中枢及外周神经组织（CNS/PNS）以及ALS患者神经活检材料上自身抗体的存在情况。将结果与正常献血者（n = 45）和其他神经系统疾病（OND）患者（n = 11）的对照血清进行比较。发现了三种不同类型的组织反应性（胶质、轴突和小血管）。与正常对照组的12%和OND对照组的27%相比，32%的ALS患者血清中存在与胶质和轴突结构结合的抗体。相反，24%的ALS患者血清和脑脊液能使内皮细胞染色，而正常对照组和OND对照组血清则不能，且仅在胎儿和青少年神经组织以及5例ALS患者中2例的腓肠神经活检组织中出现这种情况。然而，这些血清不能使正常或发炎的成人CNS/PNS组织染色。我们得出结论，ALS很可能是一组异质性疾病，只有一小部分ALS患者可能具有自身免疫发病机制。因此，这些发现可能对ALS的任何免疫抑制治疗评估具有启示意义。
