Maeda Kengo, Sugihara Yoshiko, Shiraishi Tomoyuki, Hirai Akinori, Satoh Katsuya
Department of Neurology, National Hospital Organization Higashi-Ohmi General Medical Center, Japan.
Department of Neurology, Nagahama Red Cross Hospital, Japan.
Intern Med. 2019 Mar 1;58(5):727-729. doi: 10.2169/internalmedicine.1155-18. Epub 2018 Oct 17.
We herein report a sporadic Creutzfeldt-Jakob disease (sCJD) patient followed from the presymptomatic phase to death. A 67-year-old woman had abnormal hyperintense cortical lesions on diffusion-weighted magnetic resonance imaging (MRI) one year before the onset. The levels of 14-3-3 protein and total tau protein, and findings from a real-time quaking-induced conversion test were normal at first but became abnormal after disease onset. Although there are four reports of presymptomatic sCJD identified by MRI, this is the first case report in which all three biomarkers had been assessed before and after the disease onset. MRI might be the most sensitive modality for detecting presymptomatic sCJD patients.
我们在此报告一例散发性克雅氏病（sCJD）患者，从症状前期一直随访至死亡。一名67岁女性在发病前一年的扩散加权磁共振成像（MRI）上出现异常的皮质高信号病变。14-3-3蛋白和总tau蛋白水平以及实时震颤诱导转化试验的结果起初正常，但在疾病发作后变得异常。尽管有4例通过MRI确诊的症状前sCJD的报告，但这是第一例在疾病发作前后对所有三种生物标志物进行评估的病例报告。MRI可能是检测症状前sCJD患者最敏感的方法。
