Zhao Xiongfei, Yu Yingxin, Zhao Zhiru, Xu Jiaping
Department of Neurology, Yan An University Xianyang Hospital, Wenlin Road, Xianyang, 712000, China.
Department of Neurology, Navy General Hospital, Beijing, 100048, China.
Cell Biochem Biophys. 2015 Jun;72(2):311-5. doi: 10.1007/s12013-014-0458-7.
Definite accurate diagnosis for Creutzfeldt-Jakob disease (CJD) depends on neuropathologic examination of brain biopsy or autopsy. However, transmissible nature makes the invasive examination dangerous. This study was set to determine that the clinical features are for the diagnosis of CJD through a comparison study. We compared clinical features of two cases with initial diagnosis of sporadic CJD. One case was finally diagnosed as definite sporadic CJD. According to World Health Organization diagnosis criteria, the other one, which had been diagnosed as probable sporadic CJD, was confirmed as limbic encephalitis after long-term follow-up. Compared with the case of definite sporadic CJD, the misdiagnosed case did not present typical electroencephalogram (EEG) and diffusion-weighted in magnetic resonance images (DWI) of CJD. However, cerebrospinal fluid in the misdiagnosed patient showed 14-3-3 protein positivity. The patient conditions improved after treatment. Through this case comparison, we conclude that EEG and DWI are necessary for accurate diagnosis of sporadic CJD. Further, long-term follow-up is crucial to diagnosis and treatment of CJD.
克雅氏病(CJD)的确切准确诊断依赖于脑活检或尸检的神经病理学检查。然而,其可传播性使得这种侵入性检查具有危险性。本研究旨在通过比较研究确定临床特征对CJD诊断的作用。我们比较了最初诊断为散发性CJD的两例患者的临床特征。其中一例最终被确诊为明确的散发性CJD。根据世界卫生组织的诊断标准,另一例最初被诊断为可能的散发性CJD,经过长期随访后被确认为边缘性脑炎。与明确的散发性CJD病例相比,误诊病例未表现出典型的克雅氏病脑电图(EEG)和磁共振成像扩散加权成像(DWI)表现。然而,误诊患者的脑脊液显示14-3-3蛋白阳性。患者经治疗后病情有所改善。通过该病例比较,我们得出结论,EEG和DWI对于散发性CJD的准确诊断是必要的。此外,长期随访对CJD的诊断和治疗至关重要。