Miao Shuai, Liao Shaohua, Li Heng, Niu Bing, Hu Huaiqiang, Qian Ying, Guo Hongwei, Cao Bingzhen
a Department of Neurology , Graduate School of the Second Military Medical University , Shanghai , China.
b Department of Neurology , General Hospital of Jinan Military Command , Jinan , China.
Int J Neurosci. 2018 Sep;128(9):821-827. doi: 10.1080/00207454.2018.1430693. Epub 2018 Feb 5.
To analyze the clinical features, diagnostic strategies and therapeutic methods associated with paraneoplastic neurological syndromes.
A retrospective study of paraneoplastic neurological syndromes was performed at a single center in Shandong, East China. The medical records and follow-up data of 28 patients were intensively reviewed between February 2011 and December 2014.
Twenty-four (85.7%) patients experienced subacute or chronic onset of disease, and the most common symptoms reported were mild myasthenia and paresthesias. Twenty-five (89.3%) patients presented nervous system lesions prior to occult tumors, and the median time frame between paraneoplastic neurological syndromes onset and the diagnosis of a tumor was 15 weeks. Sensorimotor neuropathy, Lambert-Eaton myasthenic syndrome and limbic encephalitis were the three most common neurological syndromes reported. Elevated serum tumor markers were observed in 44.0% of patients, while 40.7% of patients were positive for onconeural antibodies. Tumors were detected in 21 (75.0%) patients after repeated whole-body screening, and lung carcinomas were the most common primary tumor detected. Seventeen patients received anti-tumor or immunological therapy, and clinical symptoms were relieved in 13 (76.5%) of these patients.
In the majority of paraneoplastic neurological syndromes patients, the onset of disease is subacute or chronic with mild clinical symptoms. Nervous system lesions usually occur prior to occult tumors with complicated and various clinical manifestations. Neither tumor markers nor onconeural antibodies exhibit a high rate of occurrence, while repeated whole-body screening is helpful in identifying occult tumors. Early diagnosis and treatment are crucial to these patients.
分析副肿瘤性神经系统综合征的临床特征、诊断策略及治疗方法。
在中国东部山东省的一个单中心对副肿瘤性神经系统综合征进行回顾性研究。对2011年2月至2014年12月期间28例患者的病历和随访数据进行了深入分析。
24例(85.7%)患者呈亚急性或慢性起病,最常见的症状为轻度肌无力和感觉异常。25例(89.3%)患者在隐匿性肿瘤出现之前就出现了神经系统损害,副肿瘤性神经系统综合征发病至肿瘤诊断的中位时间为15周。感觉运动性神经病、兰伯特-伊顿肌无力综合征和边缘叶脑炎是报告的三种最常见的神经系统综合征。44.0%的患者血清肿瘤标志物升高,40.7%的患者肿瘤相关抗体呈阳性。经过反复全身筛查,21例(75.0%)患者检测到肿瘤,其中肺癌是最常见的原发肿瘤。17例患者接受了抗肿瘤或免疫治疗,其中13例(76.5%)患者的临床症状得到缓解。
在大多数副肿瘤性神经系统综合征患者中,疾病呈亚急性或慢性起病,临床症状较轻。神经系统损害通常在隐匿性肿瘤之前出现,临床表现复杂多样。肿瘤标志物和肿瘤相关抗体的出现率均不高,而反复全身筛查有助于发现隐匿性肿瘤。早期诊断和治疗对这些患者至关重要。