Anti-Hu antibody neuropathy:: a clinical, electrophysiological, and pathological study
Anti-Hu antibody neuropathy:: a clinical, electrophysiological, and pathological study
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DOI：
10.1016/j.clinph.2004.07.012
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发表时间：
2005-01-01
影响因子：
4.7
通讯作者：
Claussen, GC
中科院分区：
文献类型：
--
作者：
Oh, SJ;Gürtekin, Y;Claussen, GC
目的：报告16例抗Hu抗体神经病患者的临床、电生理和组织病理学特征。方法：分析16例抗Hu抗体阳性患者（11例女性，5例男性）的临床和电生理学数据以及9例神经活检数据。结果：11例患者检出癌症，其中9例为小细胞肺癌。在 7 名患者 (44%) 中观察到经典副肿瘤性亚急性感觉神经元病 (SSN) 和/或脑脊髓炎 (EM)，其中 5 名患者患有 SSN。最常见的临床特征是感觉运动神经病（SMN），占病例的 50%。尽管感觉神经传导异常是 14 例（88%）病例的突出特征，但所有病例的感觉和运动神经传导均异常。运动神经传导检查结果是典型的轴突变性。最常见的神经传导模式是 SMN，仅在 3 例病例中观察到感觉神经元病模式。 9例患者的腓肠神经活检均显示轴突变性，4例出现炎症细胞。结论：无论是临床上还是电生理学上，经典感觉神经元病都比预期罕见。运动受累并不少见，运动神经传导异常也很常见。在这种神经病中观察到了多样化的临床、电生理学和组织病理学谱。意义：建议选择抗 Hu 抗体检测患者的新指南。 (C) 2004 年国际临床神经生理学联合会。由爱思唯尔爱尔兰有限公司出版。保留所有权利。
Objective: The objective is to report the clinical, electrophysiological, and histopathological features of 16 patients with anti-Hu antibody neuropathy.Methods: Clinical and electropbysiological data in 16 patients (11 females and 5 males) with positive anti-Hu antibody and nerve biopsy data in 9 cases were analyzed.Results: Cancer was detected in 11 patients, including 9 with small-cell lung cancer. Classical paraneoplastic subacute sensory neuronopathy (SSN) and/or encephalomyelitis (EM) was observed in 7 patients (44%), including 5 with SSN. The most common clinical feature was sensory-motor neuropathy (SMN), accounting for 50% of cases. Though sensory nerve conduction abnormality was the prominent feature in 14 (88%) cases, sensory and motor nerve conduction was abnormal in all cases. Motor nerve conduction findings were typical of axonal degeneration. The most common nerve conduction pattern was that of SMN, with a sensory neuronopathy pattern being observed in only 3 cases. Sural nerve biopsy in 9 patients showed axonal degeneration in all cases and inflammatory cells in 4 cases.Conclusions: Classical sensory neuronopathy is rarer than expected, both clinically and electrophysiologically. Motor involvement is not uncommon and motor nerve conduction abnormality is frequently seen. A diverse clinical and electrophysiological, and histopathoiogical spectrum was observed in this neuropathy.Significance: New guidelines for the selection of patients for anti-Hu antibody test are recommended. (C) 2004 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.