摘要
目的分析国人散发性Creutzfeldt-Jakob病(sCJD)的临床特点及对辅助检查的敏感性和特异性,以期提高该病的早期诊断率。方法回顾性分析55例sCJD患者的临床表现、14-3-3蛋白检测、脑电图、影像学及病理资料。结果55例sCJD患者中病理确诊10例,临床很可能38例,临床可能7例;患者发病年龄为36—75岁,平均发病年龄55.8岁;发病至死亡的病程为5~22个月,平均病程为11.4个月;绝大多数患者为亚急性起病,最常见的首发症状为进行性痴呆。9例患者行脑脊液14-3-3蛋白检测,其中3例为阳性(33.3%);53例患者行脑电图检查,其中40例(75.5%)患者的脑电图检查记录到典型的周期性尖慢复合波(PSWC);头颅核磁共振弥散加权成像(DWI)显示异常高信号的阳性率为94.0%(47/50);16例行18F-脱氧葡萄糖正电子发射计算机断层显像(18F-FDG PET/CT),其中15例显示脑组织低代谢(93.8%),13例患者PET/CT检fH的脑组织低代谢区域大于其DWI显示的异常高信号区。结论与其他国家文献报道相比,国人scJD患者起病年龄早,总体病程较长。临床以亚急性起病为主,常见的首发症状为进行性痴呆。对于临床怀疑sCJD的患者,典型脑电图、头颅DWI表现可为临床诊断sCJD提供重要的参考依据。PET/CT对sCJD有重要的早期诊断及鉴别诊断价值。
Objectives To analyze the clinical features of sporadic Creutzfeldt-Jakob disease (sCJD) in China, and the sensitivity and specificity of auxiliary examinations to improve the early diagnostic rate of sCJD. Methods Clinical manifestations, 14-3-3 protein test, electroencephalography (EEG), neuroimaging and neuropathology of 55 patients with sCJD were retrospectively analyzed. Results Among 55 patients with sCJD, 10 were neuropathologically confirmed, 38 clinically probable and 7 clinically possible. The average age was 55.8 years old (ranging from 36-75 years) and the interval between onset of symptoms and death ranged 5 to 22 months (average 11.4 months). The majority of patients showed subacute onset and the most frequent initial symptom was progressive dementia. Nine patients received 14-3-3 protein detection and 3 out of them had positive results (33.3%). Fifty-three patients underwent EEG examinations, 40 out of them showed typical periodic sharp wave complexes (PSWC, 75.5%). Abnormal hyperintense lesions on diffusion-weighted images (DWI) were observed in 47/50 (94%) patients. Of 16 patients who underwent lSF-fluorodeoxyglucose positron emission tomography/CT ('SF-FDG PET/CT) scan, 15 (93.8%) showed hypometabolism, especially 13 patients showed abnormal high-intensity zone on PET/CT in addition to the hyper-intense areas observed in DWI. Conclusions Chinese sCJD patients have a much earlier onset age and longer disease duration than those reported in the foreign literatures. The majority of patients have subacute onset, and the most frequent initial symptom is progressive dementia. For patients suspected of sCJD, typical EEG and brain DWI changes could provide important reference for clinical diagnosis of sCJD. PET/CT is of great value in early diagnosis and differential diagnosis of sCJD.
出处
《解放军医学杂志》
CAS
CSCD
北大核心
2013年第10期855-858,共4页
Medical Journal of Chinese People's Liberation Army