副肿瘤性脊髓病的临床和影像特点

目的报道副肿瘤性脊髓病的临床表现、影像和血清学特征。方法检索宣武医院2010年1月至2019年6月期间的副肿瘤性脊髓病住院患者,系统分析其临床表现、血清学和影像学数据。结果10例患者中符合确定的副肿瘤综合征7例,可能的副肿瘤综合征3例。患者中亚急性起病3例,慢性起病7例,脊髓病起病后确诊时间平均为10.1个月。10例患者中7例表现为单纯副肿瘤性脊髓病,2例伴有周围神经病,1例伴有僵人综合征。10例患者中9例患者行腰穿检查脑脊液,其中8例有异常,5例淋巴细胞增多,5例蛋白升高,6例寡克隆区带阳性;7例患者神经肿瘤抗体阳性,分别为抗amphiphysin抗体1例、抗CRMP-5抗体1例、抗Hu抗体3例、抗Yo抗体1例、抗Ri抗体1例;7例患者病理证实为恶性肿瘤。10例患者中,脊髓磁共振异常5例,其中长脊髓病变4例(其中累及椎体节段14个1例,6个1例,3个2例),短脊髓病变1例,病灶均对称性累及传导束或者脊髓灰质。10例患者中9例接受治疗,其中单纯手术治疗3例,单纯糖皮质激素治疗1例,手术+糖皮质激素治疗1例,糖皮质激素+肿瘤化疗治疗2例,手术+糖皮质激素+肿瘤化疗治疗3例,患者临床症状均无明显改善。结论副肿瘤性脊髓病罕见,多表现为慢性进行性脊髓病且多先于肿瘤出现;多数患者脑脊液表现异常,影像学可表现为脊髓长节段病灶。该病治疗上以肿瘤治疗为主,免疫治疗为辅。

Clinical and radiological characteristics of patients with paraneoplastic myelopathy

Objective To report the clinical,radiological and serological characteristics of patients with paraneoplastic myelopathy.Methods We reviewed patients through our medical records system and screened patients who presented with myelopathy,and/or coexisting cancer,and/oronconeural antibodies.Results Ten patients were identified as paraneoplastic myelopathy presenting with progressive subacute(3/9)or insidious(7/9)myelopathy.CSF abnormalities included elevated protein(5 cases);pleocytosis(5 cases);excess oligoclonal bands(6 cases).Seven patients had onconeural antibody.Cancer was confirmed histopathologically in 7 patients and diagnosed by PET-CT in 1 patient.Four patients had symmetric,longitudinally extensive grey matter or tract-specificchanges on spinal cord MRI.One patient had short spinal cord lesion.Conclusions Paraneoplastic myelopathy is a rare and progressive spinal cord disease.CSF has inflammatory characteristics in most of patients.Nearly half of the patients have symmetric,longitudinally extensive grey matter or tract-specific changes on their spinal cord.It is associated with significant morbidity and has a poor response to treatment.