Graus F, René R
Department of Neurology, Hospital Clinic, Barcelona, Spain.
Rev Neurol (Paris). 1992;148(6-7):496-501.
In the last decade, several features have improved our knowledge of CNS paraneoplastic syndromes. Patients with paraneoplastic cerebellar degeneration (PCD) and breast or ovarian cancer, but not with other tumors, harbor an antibody against Purkinje cells (called anti-Yo). Clinical features of anti-Yo positive and negative PCD are similar but the latter may have a less progressive clinical course with occasional remissions. In addition to the association of opsoclonus with neuroblastoma, this syndrome has been identified in patients with breast or small-cell lung cancer (SCLC). Patients with opsoclonus and breast cancer have an antineuronal antibody (called anti-Ri) not present if opsoclonus is associated with SCLC or neuroblastoma. Paraneoplastic encephalomyelitis (PEM) is almost always associated with SCLC. Most patients present with sensory neuronopathy, limbic or brainstem encephalitis but involvement of multiple levels is usual. An antibody (called anti-Hu) against neuronal nuclear antigens is present in patients with PEM and SCLC. Autopsy studies demonstrate deposits of anti-Hu specific IgG in the neurons and a predominance of T cells in the inflammatory infiltrates. Treatment of the tumor and immunosuppressors are effective in opsoclonus whereas patients with PCD or PEM with circulating antibodies do not improve.
在过去十年中，有几个特点增进了我们对中枢神经系统副肿瘤综合征的了解。患有副肿瘤性小脑变性（PCD）且伴有乳腺癌或卵巢癌而非其他肿瘤的患者，体内存在一种针对浦肯野细胞的抗体（称为抗-Yo）。抗-Yo阳性和阴性PCD的临床特征相似，但后者的临床病程可能进展较慢，偶尔会缓解。除了眼阵挛与神经母细胞瘤相关外，该综合征在患有乳腺癌或小细胞肺癌（SCLC）的患者中也有发现。患有眼阵挛和乳腺癌的患者有一种抗神经元抗体（称为抗-Ri），而当眼阵挛与SCLC或神经母细胞瘤相关时则不存在这种抗体。副肿瘤性脑脊髓炎（PEM）几乎总是与SCLC相关。大多数患者表现为感觉神经元病、边缘叶或脑干脑炎，但通常会累及多个层面。患有PEM和SCLC的患者体内存在一种针对神经元核抗原的抗体（称为抗-Hu）。尸检研究表明，神经元中有抗-Hu特异性IgG沉积，炎症浸润中T细胞占优势。肿瘤治疗和免疫抑制剂对眼阵挛有效，而患有PCD或PEM且有循环抗体的患者病情并无改善。
