Clinical Manifestations of Sporadic Creutzfeldt-Jakob Disease in a Public Neurological Hospital in Thailand.

IF 1.8 4区医学 Q3 CLINICAL NEUROLOGY

Alzheimer Disease & Associated Disorders Pub Date : 2025-04-01 Epub Date: 2025-05-12 DOI:10.1097/WAD.0000000000000671

Jedsada Khieukhajee, Arada Rojana-Udomsart

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引用次数: 0

Abstract

Introduction: Sporadic Creutzfeldt-Jakob disease (sCJD) is one of the common causes of rapidly progressive dementia. However, because of the variety of its clinical presentations, a definite diagnosis in public hospitals is not always possible. Therefore, this study will provide more information about the clinical data of probable sCJD cases in Thailand.

Methods: This case series of probable sCJD patients who visited the Neurological Institute of Thailand during 2018 to 2023 was conducted. Demographic data, clinical presentations, brain MRI, EEG, CSF analyses, and clinical course were reviewed.

Results: A total of 17 probable sCJD cases were studied. Eleven patients were female (64.71%) with a median age of 62 (IQR: 14.5) years. The median onset of symptoms was 2 months (IQR: 2) before hospital visit, in which cognitive impairment was the most common first presentation (29.41%) followed by ataxia (23.53%), and visual disturbances (17.65%). Most patients had typical high signal intensities at both caudate/putamen and cortical regions (76.47%). Generalized periodic discharges with triphasic morphology were found in 11/14 patients with EEG results (78.57%).

Conclusion: sCJD has a diverse clinical presentation that resembles other cognitive disorders. Detailed assessment of signs and symptoms, together with proper investigations, could help to differentiate this condition in a resource-limited setting.

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泰国一家公立神经医院散发性克雅氏病的临床表现

散发性克雅氏病（sCJD）是快速进行性痴呆的常见病因之一。然而，由于其临床表现的多样性，公立医院的明确诊断并不总是可能的。因此，本研究将为泰国sCJD可能病例的临床资料提供更多信息。方法：收集2018 - 2023年在泰国神经病学研究所就诊的疑似sCJD患者。回顾了人口统计资料、临床表现、脑MRI、脑电图、脑脊液分析和临床过程。结果：共分析了17例疑似sCJD病例。女性11例（64.71%），中位年龄62岁（IQR: 14.5）岁。中位发病时间为就诊前2个月（IQR: 2），其中认知障碍是最常见的首发症状（29.41%），其次是共济失调（23.53%）和视力障碍（17.65%）。大多数患者在尾状核/壳核区和皮质区均有典型的高信号强度（76.47%）。11/14例患者的脑电图结果显示有三相形态的广泛性周期性放电（78.57%）。结论：sCJD具有与其他认知障碍相似的多种临床表现。在资源有限的情况下，对体征和症状进行详细评估，并进行适当的调查，有助于区分这种情况。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Alzheimer Disease & Associated Disorders 医学-病理学

CiteScore

3.10

自引率

4.80%

发文量

期刊介绍： Alzheimer Disease & Associated Disorders is a peer-reviewed, multidisciplinary journal directed to an audience of clinicians and researchers, with primary emphasis on Alzheimer disease and associated disorders. The journal publishes original articles emphasizing research in humans including epidemiologic studies, clinical trials and experimental studies, studies of diagnosis and biomarkers, as well as research on the health of persons with dementia and their caregivers. The scientific portion of the journal is augmented by reviews of the current literature, concepts, conjectures, and hypotheses in dementia, brief reports, and letters to the editor.