Purkinje cell cytoplasmic antibody type-2 (PCA2-IgG) was first defined as a paraneoplastic biomarker based on a unique immunofluorescence pattern seen on a mouse composite brain tissue assay.1 Lung and breast cancer are the most common malignancies associated with PCA2-IgG seropositivity.2 In 2017, microtubule-associated protein 1B (MAP1B) protein was identified as the autoantigen target for PCA2-IgG.2 Clinical presentation associated with MAP1B-IgG is variable including neuropathy, encephalopathy, cognitive dysfunction, brainstem syndrome, ophthalmic involvement and cerebellar ataxia.1 2 Despite neuropathy being the most common neurological accompaniment earlier reports lack details of those neuropathies.2 Here, we describe the neuropathy phenotypes in patients affected by MAP1B-IgG autoimmunity. Although rare, recognition of this paraneoplastic neuropathy phenotype may aid early detection of underlying malignancy. We reviewed the Mayo Clinic Neuroimmunology laboratory database (1 January 1995 and 30 September 2018) for patients tested for paraneoplastic panel by indirect immunofluorescence assay (IFA) and western blot (WB) analysis of rat cerebellar and cortical extracts (online supplementary methods).2 All PCA2-IgG stored samples were also tested and confirmed positive on MAP1B fragment WB. Study inclusion criteria: (1) MAP1B-IgG seropositivity by IFA and MAP1B WB, (2) presence of somatic and autonomic peripheral neuropathies and (3) exclusion of alternative aetiologies including chemotherapy-induced neuropathies, diabetes mellitus, nutritional deficiency, systemic vasculitis, lymphoma and paraproteinemia as deemed clinically appropriate. Clinical outcomes were evaluated by improvement in modified Rankin Scale (≥1) and 5-year mortality rate. Anti-Neuronal Nuclear Antibody type-1 (ANNA1 aka anti-Hu-IgG) seropositive neuropathy cases evaluated at Mayo Clinic (2000–2018), were utilised to evaluate phenotype and survival outcome comparison. All patients with ANNA1-IgG neuropathy in the comparison group were negative for MAP1B-IgG. ### Supplementary data [jnnp-2019-322175supp001.pdf] Indirect IFA using MAP1B-IgG neuropathy patients’ serum showed staining of rat dorsal root ganglia, sciatic nerves, sympathetic ganglia and spinal cord, along with typical staining of cerebellum and myenteric …

中文翻译：

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与MAP1B-IgG相关的副肿瘤性神经病变的表型表现。

浦肯野2型细胞质抗体（PCA2-IgG）首先根据在小鼠复合脑组织测定中观察到的独特免疫荧光模式定义为副肿瘤生物标志物。1肺癌和乳腺癌是与PCA2-IgG血清阳性相关的最常见恶性肿瘤.2在2017年，微管相关蛋白1B（MAP1B）蛋白被确定为PCA2-IgG的自身抗原靶标.2与MAP1B-IgG相关的临床表现是可变的，包括神经病变，脑病，认知功能障碍，脑干综合征，眼部受累和小脑共济失调。12尽管神经病是最常见的神经病伴奏，但较早的报道缺乏这些神经病的细节。2在这里，我们描述了受MAP1B-IgG自身免疫影响的患者的神经病表型。虽然很少见 对这种副肿瘤性神经病表型的认识可能有助于早期发现潜在的恶性肿瘤。我们回顾了Mayo临床神经免疫实验室数据库（1995年1月1日和2018年9月30日），通过间接免疫荧光测定（IFA）和大鼠小脑和皮质提取物的Western blot（WB）分析（在线补充方法）为患者进行了副肿瘤小组测试.2还对所有PCA2-IgG储存的样品进行了测试，并证实其对MAP1B片段WB呈阳性。研究纳入标准：（1）IFA和MAP1B WB产生的MAP1B-IgG血清反应阳性；（2）躯体和自主神经周围神经病变的存在；（3）排除其他病因，包括化疗引起的神经病变，糖尿病，营养缺乏，全身性血管炎，临床认为适当的淋巴瘤和副蛋白血症。通过改良Rankin量表（≥1）和5年死亡率来评估临床结局。在Mayo Clinic（2000–2018）评估的1型抗神经元核抗体（ANNA1 aka抗Hu-IgG）血清反应阳性的神经病病例用于评估表型和生存结果比较。对照组中所有ANNA1-IgG神经病患者均MAP1B-IgG阴性。###补充数据[jnnp-2019-322175supp001.pdf]使用MAP1B-IgG神经病患者血清的间接IFA显示大鼠背根神经节，坐骨神经，交感神经节和脊髓染色，以及小脑和肠系膜的典型染色… 在Mayo Clinic（2000–2018）评估的1型抗神经元核抗体（ANNA1 aka抗Hu-IgG）血清反应阳性的神经病病例用于评估表型和生存结果比较。对照组中所有ANNA1-IgG神经病患者均MAP1B-IgG阴性。###补充数据[jnnp-2019-322175supp001.pdf]使用MAP1B-IgG神经病患者血清的间接IFA显示大鼠背根神经节，坐骨神经，交感神经节和脊髓染色，以及小脑和肠系膜的典型染色… 在Mayo Clinic（2000–2018）评估的1型抗神经元核抗体（ANNA1 aka抗Hu-IgG）血清反应阳性的神经病病例用于评估表型和生存结果比较。对照组中所有患有ANNA1-IgG神经病的患者MAP1B-IgG阴性。###补充数据[jnnp-2019-322175supp001.pdf]使用MAP1B-IgG神经病患者血清的间接IFA显示大鼠背根神经节，坐骨神经，交感神经节和脊髓染色，以及小脑和肠系膜的典型染色…