Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford University, Oxford, UK.
J Neurol Neurosurg Psychiatry. 2012 Apr;83(4):437-40. doi: 10.1136/jnnp-2011-301506. Epub 2012 Feb 15.
Relatively few studies have searched for potentially pathogenic antibodies in non-paraneoplastic patients with cerebellar ataxia.
We first screened sera from 52 idiopathic ataxia patients for binding of serum IgG antibodies to cerebellar neurons. One strong-binding serum was selected for immunoprecipitation and mass spectrometry, which resulted in the identification of contactin-associated protein 2 (CASPR2) as a major antigen. CASPR2 antibodies were then found by a cell-based assay in 9/88 (10%) ataxia patients, compared to 3/144 (2%) multiple sclerosis or dementia controls (p=0.011). CASPR2 is strongly expressed in the cerebellum, only partly in association with voltage-gated potassium channels.
Prospective studies are now needed to see whether identification of CASPR2 antibodies has relevance for the diagnosis and treatment of idiopathic cerebellar ataxia.
相对较少的研究在非副肿瘤性小脑共济失调患者中寻找潜在致病性抗体。
我们首先筛选了 52 例特发性共济失调患者的血清，以检测血清 IgG 抗体与小脑神经元的结合情况。选择一种强结合的血清进行免疫沉淀和质谱分析，结果鉴定出接触蛋白相关蛋白 2 (CASPR2) 为主要抗原。然后通过基于细胞的检测方法在 88 例（10%）共济失调患者中发现了 CASPR2 抗体，而多发性硬化症或痴呆对照组为 144 例（2%）（p=0.011）。CASPR2 在小脑中有强烈表达，仅部分与电压门控钾通道相关。
目前需要进行前瞻性研究，以确定 CASPR2 抗体的鉴定是否与特发性小脑共济失调的诊断和治疗有关。
