Department of Neurochemistry and Neuropathology, Poznan University of Medical Sciences, Poznan, Poland.
Eur J Med Res. 2009 Dec 7;14 Suppl 4(Suppl 4):156-61. doi: 10.1186/2047-783x-14-s4-156.
Onconeuronal antibodies are important diagnostic tool in patients with suspicion of paraneoplastic neurological syndromes (PNS). However, their role in PNS pathophysiology and specificity for particular neurological manifestation remains unclear. The aim of this study was to evaluate onconeuronal and antineuronal antibodies in patients with pulmonary pathologies and suspected for PNS.
Twenty one patients with pulmonary pathologies were selected from the database of 525 consecutive patients with suspicion of PNS. Patients' sera were screened for the presence of onconeuronal and antineuronal antibodies by means of indirect immunofluorescence; the presence was confirmed by Western blotting. Clinical data were obtained from medical records, hospital data base, and questionnaire-based direct telephone contact with patients.
Among 21 patients, aged 54 +/-11, with pulmonary pathologies, the most frequent neurological manifestations were neuropathies. Typical PNS included paraneoplastic cerebellar degeneration (PCD) and limbic encephalitis (LE). We found cases with multiple onconeuronal antibodies (anti-Ri and anti-Yo) and coexisting PNS (PCD/LE). Well-defined onconeuronal antibodies were identified in 23.8% of patients. Among antineuronal antibodies, the most frequent were anti-MAG (23.8%). ROC curves analysis revealed high sensitivity of onconeuronal and antineuronal antibodies for typical PNS and lower for pulmonary malignancies.
Tests for antibodies are highly sensitive for the diagnosis of typical paraneoplastic neurological syndromes. Anti-myelin and anti-MAG antibodies are associated with non-neoplastic pulmonary diseases. Patients with well-defined onconeuronal antibodies require careful screening and follow-up, because the PNS diagnosis indicates a high probability of an underlying malignancy.
在疑似副肿瘤神经系统综合征(PNS)的患者中,神经元抗体是重要的诊断工具。然而,其在 PNS 病理生理学中的作用以及对特定神经表现的特异性仍不清楚。本研究旨在评估患有肺部疾病且疑似 PNS 的患者的神经元抗体和神经元抗体。
从 525 例疑似 PNS 的连续患者数据库中选择 21 例患有肺部疾病的患者。通过间接免疫荧光法筛选患者血清中神经元抗体和神经元抗体的存在;通过 Western blot 确认存在。临床数据来自病历、医院数据库和基于问卷的直接电话联系患者。
在 21 名年龄为 54 +/-11 岁的患有肺部疾病的患者中,最常见的神经系统表现为神经病变。典型的 PNS 包括副肿瘤性小脑变性(PCD)和边缘性脑炎(LE)。我们发现了具有多种神经元抗体(抗 Ri 和抗 Yo)和共存 PNS(PCD/LE)的病例。在 23.8%的患者中确定了明确的神经元抗体。在神经元抗体中,最常见的是抗 MAG(23.8%)。ROC 曲线分析显示,神经元抗体和神经元抗体对典型 PNS 的敏感性较高,对肺部恶性肿瘤的敏感性较低。
抗体检测对典型副肿瘤性神经系统综合征的诊断具有高度敏感性。抗髓鞘和抗 MAG 抗体与非肿瘤性肺部疾病有关。具有明确神经元抗体的患者需要仔细筛查和随访,因为 PNS 诊断表明存在潜在恶性肿瘤的可能性很高。