Retrospective study of paraneoplastic neurological syndromes in a Chinese Han population from Shandong, East China

Retrospective study of paraneoplastic neurological syndromes in a Chinese Han population from Shandong, East China
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山东汉族人群副肿瘤性神经综合征的回顾性研究

DOI:
10.1080/00207454.2018.1430693
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发表时间:
2018-01-01
影响因子:
2.2
通讯作者:
Cao, Bingzhen
Cao, Bingzhen
中科院分区:
医学4区
文献类型:
--
作者:
Miao, Shuai;Liao, Shaohua;Cao, Bingzhen

文献摘要

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摘要目的:分析神经系统副肿瘤综合征的临床特点、诊断策略和治疗方法。方法:在中国东部山东省的一个中心对副肿瘤性神经系统综合征进行回顾性研究。2011年2月至2014年12月期间,对28例患者的病历和随访数据进行了深入审查。结果如下:24例(85.7%)患者出现亚急性或慢性起病,最常见的症状为轻度肌无力和感觉异常。25例(89.3%)患者在隐匿性肿瘤之前出现神经系统病变,副肿瘤性神经系统综合征发作和肿瘤诊断之间的中位时间范围为15周。感觉运动神经病、Lambert-Eaton肌无力综合征和边缘系统脑炎是最常见的三种神经系统综合征。在44.0%的患者中观察到血清肿瘤标志物升高,而40.7%的患者肿瘤神经抗体阳性。21例(75.0%)患者在重复全身筛查后检出肿瘤,肺癌是最常见的原发性肿瘤。17例患者接受抗肿瘤或免疫治疗,其中13例(76.5%)临床症状缓解。结论:在大多数副肿瘤性神经综合征患者中,起病为亚急性或慢性,临床症状轻微。神经系统病变常先于隐匿性肿瘤发生,临床表现复杂多样。肿瘤标记物和肿瘤神经抗体的发生率都不高,而重复的全身筛查有助于识别隐匿性肿瘤。早期诊断和治疗对这些患者至关重要。
ABSTRACT Objective: To analyze the clinical features, diagnostic strategies and therapeutic methods associated with paraneoplastic neurological syndromes. Methods: 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. Results: 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. Conclusions: 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.