Didelot A, Honnorat J
Centre de référence de diagnostic et de traitement des syndromes neurologiques paranéoplasiques, hospices civils de Lyon, hôpital neurologique, 59, boulevard Pinel, 69677 Bron, France.
Rev Med Interne. 2011 Oct;32(10):605-11. doi: 10.1016/j.revmed.2010.09.007. Epub 2010 Oct 30.
Paraneoplastic neurological syndromes (PNS) are uncommon and defined by an acute or subacute neurological syndrome associated with a cancer. These syndromes often antedate the diagnostis of the underlying neoplasia that is usually not clinically evident. An early diagnosis is the main condition for a better neurological and carcinologic outcome. Subacute cerebellar ataxia, subacute sensitive neuropathy, limbic encephalomyelitis, Lambert-Eaton myasthenic syndrome, opsoclonus myoclonus, dermatopolymyositis and intestinal pseudo-obstruction belong to the well-characterized PNS and their presence must lead to onconeuronal antibodies (ONA) detection. Treatment of the underlying neoplasia is the mainstay of treatment to obtain a better outcome. However, recent findings lead to consider immunological specific treatment according to the subtype of associated ONA. PNS associated with ONA targeting membrane antigens are thus usually responsive to immunomodulator therapies. Conversely, PNS associated with ONA targeting intracellular antigens are of poor outcome despite mild improvement under immunosuppressive therapies in some patients.
副肿瘤性神经系统综合征(PNS)并不常见,其定义为与癌症相关的急性或亚急性神经系统综合征。这些综合征往往先于通常在临床上不明显的潜在肿瘤的诊断出现。早期诊断是获得更好的神经学和肿瘤学结果的主要条件。亚急性小脑共济失调、亚急性感觉性神经病、边缘性脑脊髓炎、兰伯特-伊顿肌无力综合征、眼阵挛-肌阵挛、皮肌炎和肠道假性梗阻属于特征明确的PNS,它们的出现必须进行肿瘤神经元抗体(ONA)检测。治疗潜在肿瘤是获得更好结果的主要治疗方法。然而,最近的研究结果促使人们根据相关ONA的亚型考虑进行免疫特异性治疗。因此,与靶向膜抗原的ONA相关的PNS通常对免疫调节疗法有反应。相反,与靶向细胞内抗原的ONA相关的PNS尽管在一些患者中免疫抑制治疗后有轻微改善,但预后较差。