Clinical characteristics of sporadic Creutzfeldt-Jakob disease in China
10.3760/cma.j.cn115354-20200413-00268
- VernacularTitle:国内散发性克雅病的临床特征分析
- Author:
Li ZHONG
1
;
Junchen ZHANG
;
Wuhua XU
Author Information
1. 贵州医科大学临床医学院，贵阳 550004
- Keywords:
Sporadic Creutzfeldt-Jakob disease;
Clinical feature;
Diffusion-weighted imaging;
Prion protein;
Summary analysis
- From:
Chinese Journal of Neuromedicine
2020;19(11):1128-1133
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical characteristics of domestic sporadic Creutzfeldt-Jakob disease (sCJD).Methods:One patient diagnosed with probable sCJD in our hospital in October 2019 was firstly reported; the clinical data of this patient were retrospectively analyzed combining with those of 82 patients publicly reported in domestic core journals on China Biology Medicine disc, CNKI and WANFANG databases from 2009 to 2019 and met with MRI-CJD consortium criteria for sCJD.Results:Of the total 83 patients, 78 were diagnosed as clinically probable sCJD and other 5 as definite sCJD. There were 45 males and 38 females; the median age was 63 (58, 68) years. The course of disease was recorded in 56 patients, and the time from onset to death was 4.3 (2.9, 7.0) months. The onset and main symptoms were diverse and non-specific, however, progressive cognitive impairments, myoclonus, ataxia, visual dysfunction, akinetic mutism frequently occurred during the whole disease course. As compared with those in patients less than or equal to 65 years old, cerebellar symptoms and visual impairment occurred more frequently in patients over 65 years old ( P< 0.05). High diffusion weighted imaging (DWI) signals were detected on the cortex and/or the basal ganglia and/or the thalamus of 92.4% patients (73/79). Meanwhile, akinetic mutism was more likely to appear in patients with basal ganglia involvements than the unaffected ones (62.5% vs. 25.8%, P<0.05). Abnormal cortical DWI high signals in one patient were detected 7 months before the appearance of first symptom, and four patients who were clinically confirmed by dynamic follow-up DWI presented clinical symptoms highly corresponding to changes in DWI signals in brain regions. Conclusions:The sCJD tends to occur in the middle-aged and elderly patients, with high heterogeneity in clinical symptoms, durations and abnormal DWI signals, and symptoms caused by damage in posterior cranial fossa occur more frequently in patients over 65 years old; the DWI lesions in basal segment might be seen as a sign for deterioration of disease. Dynamic DWI follow-up may be helpful for early detection and objective reflection of sCJD intracerebral lesions.
