Delattre J Y, Davila L, Vega F, Poisson M
Clinique Neurologique, Hôpital de la Salpêtrière, Paris.
Rev Neurol (Paris). 1991;147(8-9):549-56.
Paraneoplastic syndromes affecting the nervous system are rare and their diagnosis is often difficult when the original cancer is unknown. Recently, high levels of antineuronal antibodies (AB) have been found in serum and CSF of some patients with paraneoplastic syndromes. The anti-Yo AB recognizes 2 proteins of 34 and 62 kd in the cytoplasm of Purkinje cells and in malignant cells of patients suffering from paraneoplastic cerebellar degeneration associated with ovarian and breast cancer. The anti-Hu AB recognizes a 37-40 kd protein in nuclei of neurons and in tumor cells of patients suffering from subacute sensory neuronopathy and encephalomyelitis associated with small cell lung cancer. Other antineuronal AB have been more rarely identified. The presence of high titer of one of these AB in a patient with suspected paraneoplastic syndrome is of great practical interest since it confirms the neurological diagnosis and strongly suggests the location of the primary tumor when the malignancy is unknown. The pathogenetic role of the antineuronal AB is unknown but it is likely that some paraneoplastic syndromes affecting the nervous system are due to an immune reaction against antigens shared by the tumor and the nervous system. To date, no efficient treatment has been found.
影响神经系统的副肿瘤综合征很罕见,当原发癌不明时,其诊断往往很困难。最近,在一些副肿瘤综合征患者的血清和脑脊液中发现了高水平的抗神经元抗体(AB)。抗Yo抗体可识别浦肯野细胞胞质以及患有与卵巢癌和乳腺癌相关的副肿瘤性小脑变性患者的恶性细胞中的两种分子量分别为34 kd和62 kd的蛋白质。抗Hu抗体可识别患有与小细胞肺癌相关的亚急性感觉神经元病和脑脊髓炎患者的神经元细胞核以及肿瘤细胞中的一种37 - 40 kd的蛋白质。其他抗神经元抗体则较少被识别。在疑似副肿瘤综合征的患者中,若存在这些抗体之一的高滴度,具有重要的实际意义,因为这证实了神经学诊断,并在恶性肿瘤不明时强烈提示了肿瘤的位置。抗神经元抗体的致病作用尚不清楚,但一些影响神经系统的副肿瘤综合征可能是由于针对肿瘤和神经系统共有的抗原的免疫反应所致。迄今为止,尚未找到有效的治疗方法。