Senties-Madrid H, Vega-Boada F
Department of Neurology, National Institute of Medical Sciences and Nutrition Salvador Zubiran, Mexico City, Mexico.
Isr Med Assoc J. 2001 Feb;3(2):94-103.
Paraneoplastic syndromes are disorders associated with cancer but without a direct effect of the tumor mass or its metastases on the nervous system. Small cell carcinoma of lung associated with paraneoplastic sensory neuronopathy and/or paraneoplastic encephalomyelitis with the presence of anti-Hu antibodies has been termed "anti-Hu syndrome." Anti-Hu associated PSN-PEM is an immune disorder in which both cell-mediated and humoral mechanisms are involved. Patients are considered affected by Anti-Hu associated PSN-PEM when they develop clinical signs and symptoms of CNS dysfunction and/or sensory neuropathy not caused by metastases or other disorders, and serum or cerebrospinal fluid is positive for Hu abs. SCLC is found in more than 90% of patients with cancer and positive Hu abs. Individual patients with Hu abs associated to SCLC may suffer PSN-PEM, limbic encephalitis, brainstem encephalopathy, opsoclonus-myoclonus, paraneoplastic cerebellar degeneration or myelopathy. Hu abs have a specificity of 99% and sensitivity of 82% in detecting paraneoplastic neurological syndromes. There are two types of treatment: the first is to treat the cancer, the second is to suppress the immune reaction with the use of corticosteroids, cyclophosphamide, azathioprine, plasma exchange, intravenous immunoglobulin and immunoadsorption; however, treatment of paraneoplastic syndromes is generally unsatisfactory.
副肿瘤综合征是与癌症相关的疾病，但肿瘤块或其转移灶对神经系统无直接影响。与副肿瘤性感觉神经元病和/或伴有抗Hu抗体的副肿瘤性脑脊髓炎相关的肺小细胞癌被称为“抗Hu综合征”。抗Hu相关的PSN-PEM是一种免疫紊乱，涉及细胞介导和体液机制。当患者出现并非由转移或其他疾病引起的中枢神经系统功能障碍和/或感觉神经病变的临床体征和症状，且血清或脑脊液Hu抗体呈阳性时，即被认为患有抗Hu相关的PSN-PEM。超过90%患有癌症且Hu抗体呈阳性的患者被发现患有小细胞肺癌。与小细胞肺癌相关的Hu抗体个体患者可能会患PSN-PEM、边缘叶脑炎、脑干脑病、眼阵挛-肌阵挛、副肿瘤性小脑变性或脊髓病。Hu抗体在检测副肿瘤性神经综合征时的特异性为99%，敏感性为82%。有两种治疗方法：第一种是治疗癌症，第二种是使用皮质类固醇、环磷酰胺、硫唑嘌呤、血浆置换、静脉注射免疫球蛋白和免疫吸附来抑制免疫反应；然而，副肿瘤综合征的治疗通常并不令人满意。
