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越南散发性克雅氏病病例的描述性分析:来自四个三级医疗中心的31名患者。

A descriptive analysis of sporadic Creutzfeldt-Jakob cases in Vietnam: 31 patients from four tertiary care centers.

作者信息

Vo Khoi Hong, Bui Nga Thi, Hoang Dung Thi, Nguyen Thanh Vinh, Nguyen Binh Thanh, Tran Tai Ngọc, Nguyen Anh Hai, Le My Thi, Do Ha Thi, Nguyen Thuan Duc, Ta Hai Van, Phan Toan Van, Phan Phuc Duy, Pham Thang Xuan, Xing Frank, Truong Daniel

机构信息

Neurology Center, Bach Mai Hospital, Hanoi, Vietnam.

Department of Neurology, Hanoi Medical University, Hanoi, Vietnam.

出版信息

Clin Park Relat Disord. 2025 May 30;12:100351. doi: 10.1016/j.prdoa.2025.100351. eCollection 2025.

Abstract

BACKGROUND

Accurate diagnosis of sCJD remains challenging in developing countries such as Vietnam, as clinical and research efforts focus on treatable diseases. Several cases of sCJD have been diagnosed in Vietnam but data from formal analyses are lacking. This is the first systematic analysis of patients with sCJD in Vietnam.

METHODS

This was a systematic retrospective review of medical records from patients with probable sCJD (N = 31) seen between April 2021 to April 2024 at four tertiary care centers. Clinical, laboratory, neuroimaging, and EEG findings were included in the analysis.

RESULTS

Data from 16 men and 15 women with sCJD were analyzed. The average age of onset was 63.4 years (range 50-83 years). Twenty-one of the patients had died after a mean survival of 7.6 months (range 3-20 months). All patients initially presented with rapidly progressive dementia. Other associated symptoms included myoclonus (77 %), extrapyramidal symptoms (80 %), pyramidal symptoms (58 %), akinetic mutism (55 %), visual disturbance (45 %), and cerebellar ataxia (32 %). Neuroimaging revealed abnormal fluidattenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI) sequences in 28/31 patients. EEG revealed periodic sharp wave complexes (PSWCs) in 26/31 patients. Only 12 patients had been tested for 14-3-3 protein in CSF and all were positive.

CONCLUSIONS

Clinical, neuroimaging, laboratory, and EEG features are consistent with global findings.

摘要

背景

在越南等发展中国家,散发性克雅氏病(sCJD)的准确诊断仍然具有挑战性,因为临床和研究工作主要集中在可治疗的疾病上。越南已诊断出数例sCJD病例,但缺乏正式分析的数据。这是越南首例对sCJD患者的系统分析。

方法

这是一项对2021年4月至2024年4月期间在四个三级医疗中心就诊的疑似sCJD患者(N = 31)的病历进行的系统回顾性研究。分析包括临床、实验室、神经影像学和脑电图检查结果。

结果

对16例男性和15例女性sCJD患者的数据进行了分析。平均发病年龄为63.4岁(范围50 - 83岁)。21例患者在平均存活7.6个月(范围3 - 20个月)后死亡。所有患者最初均表现为快速进展性痴呆。其他相关症状包括肌阵挛(77%)、锥体外系症状(80%)、锥体束症状(58%)、运动不能性缄默(55%)、视觉障碍(45%)和小脑共济失调(32%)。神经影像学显示28/31例患者的液体衰减反转恢复(FLAIR)和扩散加权成像(DWI)序列异常。脑电图显示26/31例患者有周期性尖波复合波(PSWC)。仅12例患者进行了脑脊液14 - 3 - 3蛋白检测,结果均为阳性。

结论

临床、神经影像学、实验室和脑电图特征与全球研究结果一致。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4897/12171541/635a0b1b8ff8/gr1.jpg

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