CAJ | 학술논문

目的了解副肿瘤综合征患儿的临床基本特征.方法回顾性分析12例副肿瘤综合征患儿的临床资料并加以总结,对部分患儿进行术后随访.结果 12例副肿瘤综合征患儿男11例,女1例,平均发病年龄(30.5±15.3)个月,病程平均(112.7±154.4)d,急性起病2例,亚急性起病3例,7例慢性起病(2例为缓解-复发或加重过程).神经系统症状11例表现为小脑共济失调,其中3例伴有眼阵挛(眼阵挛-肌阵挛综合征),1例表现为肢体无力、逐渐出现共济失调症状.12例患儿9例接受手术,病理为神经母细胞瘤或节细胞神经瘤.对6例患儿进行8 -21个月随访,1例眼阵挛-肌阵挛综合征患儿症状较术前轻微改善,余5例明显改善(但无1例恢复至正常).结论儿童副肿瘤综合征可仅以神经系统症状首发,且缺乏肿瘤其他相关表现,亦缺乏相关特异性实验室诊断指标,临床容易误诊,儿童副肿瘤综合征预后不佳.
목적 료해부종류종합정환인적림상기본특정.방법 회고성분석12례부종류종합정환인적림상자료병가이총결,대부분환인진행술후수방.결과 12례부종류종합정환인남11례,녀1례,평균발병년령(30.5±15.3)개월,병정평균(112.7±154.4)d,급성기병2례,아급성기병3례,7례만성기병(2례위완해-복발혹가중과정).신경계통증상11례표현위소뇌공제실조,기중3례반유안진련(안진련-기진련종합정),1례표현위지체무력、축점출현공제실조증상.12례환인9례접수수술,병리위신경모세포류혹절세포신경류.대6례환인진행8 -21개월수방,1례안진련-기진련종합정환인증상교술전경미개선,여5례명현개선(단무1례회복지정상).결론 인동부종류종합정가부이신경계통증상수발,차결핍종류기타상관표현,역결핍상관특이성실험실진단지표,림상용역오진,인동부종류종합정예후불가.
Objective To investigate the basic clinical characteristics of paraneoplastic neurological syndrome (PNS) in children.Method To retrospectively analyze the clinical data of 12 PNS children who were hospitalized in neurology department in Beijing Children's Hospital from 2010 to 2011.Some patients were followed up after surgery.Result In 12 patients with PNS,11 were male and 1 was female.The mean onset age were (30.5 ± 15.3) months.The mean duration from neurological symptom onset to finding out of tumor was ( 112.7 ± 154.4) days.The onset of the disease in 2 patients was acute,in 3 was subacute and in the other 7 was chronic ( 2 of 7 had 2 to 3 relapses ).Of 12 patients,1 1 had symptoms of ataxia ( 3 patients also had opsoclonus and myoclonus,OMS),1 had weakness of limbs at onset and then had ataxia.Nine of 12 patients had surgery,and pathologic diagnosis was neuroblastoma and ganglioneuroma.Six patients were followed-up for 8 to 21 months.One patient had a little improvement and 5 almost recovered.Conclusion The PNS children can have neurological symptoms only at the onset and there were no particular evidence of tumor.It is prone to misdiagnosis.The prognosis of PNS in children was poor.