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快速进展型可能散发性克雅氏病

Rapidly Progressive Probable Sporadic Creutzfeldt-Jakob Disease.

作者信息

Elziny Moustafa M, Elsaid Shaimaa S

机构信息

Department of Academic Internal Medicine and Geriatrics, University of Illinois at Chicago, Chicago, USA.

Department of Diagnostic and Interventional Radiology, National Cancer Institute, Cairo University, Cairo, EGY.

出版信息

Cureus. 2022 Mar 17;14(3):e23245. doi: 10.7759/cureus.23245. eCollection 2022 Mar.

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain infection caused by a human prion. Because CJD is associated with rapidly progressive neurological degeneration, it requires high suspicion for diagnosis. We report the case of a 79-year-old patient who presented with a rapidly progressive neurological clinical picture. The patient had positive 14-3-3 proteins in cerebrospinal fluid, electroencephalography was significant for periodic discharges, and magnetic resonance imaging of the brain showed both diffusion restriction and increased fluid-attenuated inversion recovery signal in different cortical regions, consistent with probable sporadic CJD infection. The patient was enrolled under hospice and palliative care. The patient passed away two months after the onset of her symptoms. We discuss the probable sporadic CJD diagnostic criteria and possible risk factors that might have led to a faster progressive course.

摘要

克雅氏病(CJD)是一种由人类朊病毒引起的罕见致命性脑部感染。由于CJD与快速进展的神经退行性变有关,因此诊断时需要高度怀疑。我们报告了一例79岁患者,其临床表现为快速进展的神经系统症状。该患者脑脊液中14-3-3蛋白呈阳性,脑电图显示有周期性放电,脑部磁共振成像显示不同皮质区域既有弥散受限又有液体衰减反转恢复信号增强,符合可能的散发性CJD感染。该患者接受了临终关怀和姑息治疗。患者在症状出现两个月后去世。我们讨论了可能的散发性CJD诊断标准以及可能导致病程进展更快的危险因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0f7/9012942/4429a8cc910a/cureus-0014-00000023245-i01.jpg

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