Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Int J Neurosci. 2013 Aug;123(8):557-62. doi: 10.3109/00207454.2013.782025. Epub 2013 Apr 15.
We aimed to investigate various anti-neuronal antibodies in sera of amyotrophic lateral sclerosis (ALS) patients to detect possible autoimmune encephalitis patients imitating ALS findings and to delineate the validity of routine screening of well-characterized anti-neuronal antibodies in ALS. The patients fulfilling the revised El Escorial diagnostic criteria for definite ALS were included. Their serum samples were investigated for antiganglioside (IgM/IgG) and onconeural (IgG) antibodies by immunoblotting, for ion channel antibodies (IgG) by a cell-based assay and for IgG binding patterns to the rat brain by indirect immunohistochemistry. Thirty-five patients with definite ALS and 30 healthy individuals were included. Ganglioside antibodies were detected in 2 out of 35 (5.7%) patients with ALS. The onconeural and ion channel antibodies were negative in our series. Varied serum IgG binding patterns were identified in eight (22.9%) patients. Although autoimmune encephalitis patients may occasionally present with atypical motor neuron disease findings, definite ALS patients do not appear to exhibit onconeural or ion channel antibodies, suggesting that routine analysis of these antibodies in typical ALS is not mandatory. By contrast, some ALS patients display anti-neuronal antibodies against undetermined target antigens, prompting investigation of these novel antibodies with more advanced methods.
我们旨在研究肌萎缩侧索硬化症(ALS)患者血清中的各种抗神经元抗体,以检测可能模仿 ALS 发现的自身免疫性脑炎患者,并阐明在 ALS 中常规筛查特征明确的抗神经元抗体的有效性。纳入符合修订后的 El Escorial 明确 ALS 诊断标准的患者。通过免疫印迹法检测抗神经节苷脂(IgM/IgG)和神经原性(IgG)抗体,通过细胞测定法检测离子通道抗体(IgG),并通过间接免疫组织化学法检测 IgG 与大鼠脑的结合模式。纳入 35 例明确的 ALS 患者和 30 名健康个体。在 35 例 ALS 患者中有 2 例(5.7%)检测到神经节苷脂抗体。在我们的研究中,未见神经原性和离子通道抗体阳性。在 8 例(22.9%)患者中鉴定出不同的血清 IgG 结合模式。尽管自身免疫性脑炎患者偶尔可能出现不典型运动神经元病表现,但明确的 ALS 患者似乎不表现出神经原性或离子通道抗体,这表明在典型 ALS 中常规分析这些抗体并非强制性的。相比之下,一些 ALS 患者显示针对未确定靶抗原的抗神经元抗体,提示用更先进的方法研究这些新型抗体。