Introduction: Sporadic Creutzfeldt Jakob Disease, the most common reported prion disease, is a fatal neurodegenerative disease caused by the misfolding of protein PrP^C to PrP^SC. EEG is the first in vivo test to support the clinical diagnosis of sporadic CJD (sCJD). This study is carried out to describe the comprehensive electroencephalography changes in a cohort of patients with probable sCJD from south India. Methodology: This retrospective observational study is conducted by reviewing case records from 2013–2020. Demographic, clinical, imaging details were noted. Electroencephalogram (EEG) recordings were retrieved and reviewed independently by two qualified epileptologists and the findings were visually analyzed and correlated with clinical and imaging abnormalities. Results: Fifty patients with probable sCJD with the availability of artefact-free EEG were included in the study. The mean age was 59.76 ± 8.17 years and M: F was 31:19. Magnetic resonance imaging (MRI) brain showed abnormality defining CJD in 48/50, i.e., 96%patients. EEG showed specific and or nonspecific abnormalities in 88%. The specific abnormalities, i.e., periodic sharp wave complexes (PSWC), were noted 66%. PSWC were mostly asymmetric (90%) and more frequently seen in the right hemisphere (57.6%). Amplitude maximum in the occipital region was noted in 42.4% and in frontal region in 36.4%. The sensitivity of EEG specific abnormalities to diagnose probable sCJD according to CDC 2018 criteria with positive MRI findings was 68.75%. Conclusion: This study showed that EEG is a relatively inexpensive and sensitive tool and assists in the diagnosis of sCJD. However, it can be normal or show nonspecific abnormalities in the early stages of the disease.

中文翻译：

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可能的散发性 CJD 的脑电图观察

简介：散发性克雅氏病是最常见的朊病毒病，是一种致命的神经退行性疾病，由蛋白质 PrP ^C错误折叠为PrP ^SC引起。EEG 是第一个支持散发性 CJD (sCJD) 临床诊断的体内测试。本研究旨在描述来自印度南部的一组可能患有 sCJD 的患者的综合脑电图变化。方法：这项回顾性观察研究是通过回顾 2013-2020 年的病例记录进行的。记录了人口统计学、临床、影像学细节。脑电图 (EEG) 记录由两名合格的癫痫专家独立检索和审查，并对结果进行视觉分析，并将其与临床和影像学异常相关联。结果：该研究包括 50 名可能患有 sCJD 且可获得无伪影 EEG 的患者。平均年龄为 59.76 ± 8.17 岁，M:F 为 31:19。48/50 即 96% 的患者脑部磁共振成像 (MRI) 显示异常定义 CJD。EEG 显示 88% 的特异性和或非特异性异常。特定异常，即周期性尖波复合波 (PSWC)，占 66%。PSWC 大多是不对称的（90%），更常见于右半球（57.6%）。枕区振幅最大值为 42.4%，额叶区为 36.4%。根据 CDC 2018 标准和 MRI 阳性结果，EEG 特异性异常对诊断可能的 sCJD 的敏感性为 68.75%。结论：本研究表明，EEG 是一种相对便宜且敏感的工具，有助于诊断 sCJD。然而，在疾病的早期阶段，它可能是正常的或表现出非特异性异常。