Anti-neuronal nuclear autoantibody type 2: Paraneoplastic accompaniments
Anti-neuronal nuclear autoantibody type 2: Paraneoplastic accompaniments
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DOI：
10.1002/ana.10518
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发表时间：
2003-05-01
影响因子：
11.2
通讯作者：
Lennon, VA
中科院分区：
文献类型：
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作者：
Pittock, SJ;Lucchinetti, CF;Lennon, VA
我们在 12 年的时间里，通过对约 75,000 名疑似副肿瘤性亚急性神经系统疾病患者的血清进行免疫荧光筛查，在 34 名患者中鉴定出了 IgG 自身抗体 ANNA-2（“抗 Ri”）。提供了 28 名患者（10 名男性，18 名女性）的详细临床信息。 24 名患者 (86%) 被诊断出癌症； 21 例经组织学证实患有癌症（10 例肺癌、9 例乳腺癌、1 例宫颈癌、1 例膀胱癌），3 例胸腔内影像学异常。 28 名患者中有 4 名在癌症出现之前出现神经系统症状。随着持续监测，癌症检测频率有所增加。频率递减的神经系统疾病包括脑干综合征（包括视阵挛、肌阵挛或两者兼而有之）、小脑综合征、脊髓病、周围神经病、颅神经病、运动障碍、脑病、兰伯特-伊顿综合征和癫痫发作。 4 名患者患有喉痉挛，4 名患者患有张口肌张力障碍（2 名患者患有颈部肌张力障碍）。神经系统症状出现 1 个月后，九人 (32%) 坐轮椅。大多数人在免疫调节或肿瘤定向治疗后神经系统得到改善。 73% 的血清中存在伴随自身抗体，包括 ANNA-1、ANNA-3、CRMP-5-IgG、P/Q 型和 N 型 Ca2+ 通道抗体以及肌肉型乙酰胆碱受体抗体。 ANNA-2 的一些神经学伴发可能反映了对共免疫肿瘤抗原的潜在致病性不道德或细胞介导的反应，例如 Lambert-Eaton 综合征（P/Q 型 Ca2+ 通道抗体）和周围神经病变（ANNA-1 效应 T 细胞）。
We identified the IgG autoantibody ANNA-2 ("anti-Ri") in 34 patients in a 12-year period by immunofluorescence screening of sera from approximately 75,000 patients with subacute neurological disorders that were suspected to be paraneoplastic. Detailed clinical information was available for 28 patients (10 men, 18 women). Cancer was diagnosed in 24 patients (86%); 21 had histologically proven carcinoma (10 lung, 9 breast, 1 cervical, 1 bladder), and 3 had an intrathoracic imaging abnormality. Cancer anteceded neurological symptoms in 4 of 28 patients. Cancer detection frequency increased with continued surveillance. Neurological disorders, in decreasing frequency, were brainstem syndrome (including opsoclonus, myoclonus, or both), cerebellar syndrome, myelopathy, peripheral neuropathy, cranial neuropathy, movement disorder, encephalopathy, Lambert-Eaton syndrome, and seizures. Four patients had laryngospasm and four had jaw opening dystonia (two with neck dystonia). Nine (32%) were wheelchair-bound 1 month after neurological symptom onset. Most improved neurologically after immunomodulatory or tumor-directed therapy. Accompanying autoantibodies, found in 73% of sera, included ANNA-1, ANNA-3, CRMP-5-IgG, P/Q type and N-type Ca2+ channel antibodies, and muscle-type acetylcholine receptor antibody. Some neurological accompaniments of ANNA-2 may reflect potentially pathogenic Immoral or cell-mediated responses to coimmunogenic tumor antigens, for example, Lambert-Eaton syndrome (P/Q type Ca2+ channel antibody) and peripheral neuropathy (ANNA-1 effector T cells).