陶氏病
病理
医学
神经病理学
萎缩
疾病
高强度
神经退行性变
痴呆
海马硬化
回顾性队列研究
神经影像学
尸检
额颞叶变性
磁共振成像
失智症
颞叶
癫痫
精神科
放射科
作者
Nikol Jankovská,Robert Rusina,Jiří Keller,Jaromír Kukal,Magdalena Brůžová,Eva Parobková,Tomáš Olejár,Radoslav Matěj
出处
期刊:Biomedicines
[Multidisciplinary Digital Publishing Institute]
日期:2022-03-16
卷期号:10 (3): 680-680
被引量:6
标识
DOI:10.3390/biomedicines10030680
摘要
Creutzfeldt–Jakob disease (CJD), the most common human prion disorder, may occur as “pure” neurodegeneration with isolated prion deposits in the brain tissue; however, comorbid cases with different concomitant neurodegenerative diseases have been reported. This retrospective study examined correlations of clinical, neuropathological, molecular-genetic, immunological, and neuroimaging biomarkers in pure and comorbid CJD. A total of 215 patients have been diagnosed with CJD during the last ten years by the Czech National Center for Prion Disorder Surveillance. Data were collected from all patients with respect to diagnostic criteria for probable CJD, including clinical description, EEG, MRI, and CSF findings. A detailed neuropathological analysis uncovered that only 11.16% were “pure” CJD, while 62.79% had comorbid tauopathy, 20.47% had Alzheimer’s disease, 3.26% had frontotemporal lobar degeneration, and 2.33% had synucleinopathy. The comorbid subgroup analysis revealed that tauopathy was linked to putaminal hyperintensity on MRIs, and AD mainly impacted the age of onset, hippocampal atrophy on MRIs, and beta-amyloid levels in the CSF. The retrospective data analysis found a surprisingly high proportion of comorbid neuropathologies; only 11% of cases were verified as “pure” CJD, i.e., lacking hallmarks of other neurodegenerations. Comorbid neuropathologies can impact disease manifestation and can complicate the clinical diagnosis of CJD.
科研通智能强力驱动
Strongly Powered by AbleSci AI