Dubas F
Service de Neurologie A, INSERM U 298, CHU rue Larrey, Angers.
Presse Med. 1991 Jun 8;20(22):1041-6.
Paraneoplastic neurological syndromes are of two types: some are more often seen without than with cancers and may therefore be called "occasionally para neoplastic" (e.g. chronic sensorimotor polyneuropathy and polymyositis), while others are fairly regularly associated with cancers, and particularly with small cell lung carcinoma. In this category falls subacute encephalomyelitis, an entity of broad anatomico-clinical spectrum including limbic encephalitis and subacute sensory neuronopathy; the patient's serum and cerebrospinal fluid may contain neuronal antinuclear antibodies. One type of subacute cerebellar degeneration is characterized by the presence of antibodies specifically directed against Purkinje cell cytoplasmic antigens, and it is associated with ovarian and mammary cancers. The other type shows no antibodies or different antibodies and sometimes neuronal antinuclear antibodies; the latter case may represent the cerebellar form of subacute encephalopathy. Because it may be either a true autoimmune disease or a true paraneoplastic syndrome, Lambert-Eaton myasthenic syndrome, caused by autoantibodies that block the voltage-dependent calcium channels, stands out as the most convincing argument in support of the autoimmune paraneoplastic syndrome theory. The theory, which refers to cross-antigenicity, cannot be extended to the other syndromes without reservation: there is no evidence that autoantibodies are neurotoxic, and specific autoantibodies in high levels are sometimes detected in patients with cancer but without any neurological symptom. Nevertheless, the finding of circulating antineuronal antibodies in patients with a suggestive clinical syndrome should prompt investigations for cancer perhaps at an early stage.
有些在无癌症时比有癌症时更常见,因此可称为“偶发性副肿瘤性”(如慢性感觉运动性多神经病和多发性肌炎),而另一些则相当规律地与癌症相关,尤其是与小细胞肺癌相关。亚急性脑脊髓炎就属于这一类别,它是一种具有广泛解剖学 - 临床谱的疾病,包括边缘叶脑炎和亚急性感觉神经元病;患者的血清和脑脊液中可能含有神经元抗核抗体。一种亚急性小脑变性的特征是存在针对浦肯野细胞胞质抗原的特异性抗体,它与卵巢癌和乳腺癌相关。另一种类型则未显示抗体或有不同的抗体,有时还会出现神经元抗核抗体;后一种情况可能代表亚急性脑病的小脑形式。由阻断电压依赖性钙通道的自身抗体引起的兰伯特 - 伊顿肌无力综合征,因其可能是真正的自身免疫性疾病或真正的副肿瘤综合征,成为支持自身免疫性副肿瘤综合征理论最有说服力的论据。这种涉及交叉抗原性的理论不能毫无保留地扩展到其他综合征:没有证据表明自身抗体具有神经毒性,而且有时在患有癌症但没有任何神经症状的患者中也能检测到高水平的特异性自身抗体。然而,在具有提示性临床综合征患者中发现循环抗神经元抗体应促使对癌症进行检查,或许应在早期进行。