Posner J B, Dalmau J O
Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
Annu Rev Med. 1997;48:157-66. doi: 10.1146/annurev.med.48.1.157.
Paraneoplastic syndromes affecting the nervous system are rare neurologic syndromes caused by cancer but not ascribable to metastases. Any portion of the nervous system may be involved in a paraneoplastic syndrome. The pathogenesis of these disorders appears to be an immune reaction against antigens shared by the cancer and the nervous system. Some disorders, such as the Lambert-Eaton myasthenic syndrome, are effectively treated by removal of autoantibodies directed against the presynaptic cholinergic synapse. In other disorders, such as paraneoplastic cerebellar degeneration or paraneoplastic sensory neuronopathy, neither removal of the autoantibody nor treatment of the cancer is effective.
影响神经系统的副肿瘤综合征是由癌症引起但不能归因于转移的罕见神经综合征。神经系统的任何部分都可能参与副肿瘤综合征。这些疾病的发病机制似乎是针对癌症和神经系统共有的抗原的免疫反应。一些疾病,如兰伯特-伊顿肌无力综合征,通过去除针对突触前胆碱能突触的自身抗体可得到有效治疗。而在其他疾病中,如副肿瘤性小脑变性或副肿瘤性感觉神经元病,去除自身抗体或治疗癌症均无效。