抗Hu副肿瘤综合征病例2例分析并文献复习

Analysis of Clinical Characteristics of anti-Hu Antibody Associated Paraneoplastic Neurological Syndrome:2 Cases Report and Literature Review

目的:探讨抗Hu神经系统副肿瘤综合征的临床特点及预后。方法:报道我院2例抗Hu副肿瘤综合征并结合文献进行回顾性分析。结果:其中1例表现为严重的中枢性低通气,同时伴有周围神经病变,但全身PET-CT并未发现肿瘤;另1例表现为非特异性症状,肿瘤筛查中发现头颈部肿块,主要分布在鼻窦和腮腺,病理活检为小细胞癌。鼻窦肿块切除后短期内患者症状缓解明显。搜索既往报道的抗Hu相关副肿瘤综合征患者477例,最常见的表现是亚急性感觉性周围神经病,颅脑的MRI表现无特异性,最常见合并小细胞肺癌;在所有的病例中,有2.5%的患者表现为中枢性低通气。结论:抗Hu副肿瘤综合征应作为中枢性低通气的鉴别诊断之一。副肿瘤综合征相关抗体的检测,有利于更早发现肿瘤。

Objective：To analyze the clinical characteristic and prognosis of anti-Hu antibody associated para- neoplastic neurological syndrome. Methods： Two cases of anti-hu associated paraneoplastic neurological syn- drome were reported and the relevant literature was reviewed. Results： One of the patients showed severe cen- tral hypoventilation, accompanied by peripheral neuropathy. But systemic PET-CT did not detect tumors. Anoth- er patient showed nonspecific presentation, but the tumor was found in the head and neck, which were mainly dis- tributed in the sinus and parotid gland. Nasal sinus mass was confirmed as small cell carcinoma by pathological biopsy. The symptoms of the patient were relieved after resection of the sinus lumps. We have reviewed data of 477 cases of anti-Hu associated paraneoplastic neurological syndrome from the MEDLINE. Most of patients pre- sented with subacute sensory neuropathy. Most of cerebral MR[ manifestation did not show abnormalities. Small cell lung cancer was the most common combined type of tumors. In all the cases, 2.5% of the patients presented with central hypoventilation. Conclusion： Paraneoplastic neurological syndrome constitutes a reason for central hypoventilation without underlying causes. Anti-hu and other paraneoplastic syndrome-related antibodies detec- tion is helpful to detect the tumors, and may provide a more favorable time for the treatment of cancers.