Department of Gastroenterology and Neurology, Graduate School of Medicine and Faculty of Medicine, Kagawa University, Japan.
Department of Health Sciences, Graduate School of Biomedical Sciences and School of Medicine, Nagasaki University, Japan.
Intern Med. 2022 Sep 1;61(17):2667-2670. doi: 10.2169/internalmedicine.8354-21. Epub 2022 Feb 19.
A 63-year-old woman who presented for orofacial dystonia showed cortical ribboning, a typical MRI finding in sporadic Creutzfeldt-Jakob disease (sCJD). However, real-time quaking-induced conversion (RT-QuIC), the most sensitive method for an early diagnosis of sCJD, was negative. She developed sCJD six months later, at which time RT-QuIC became positive. The cerebral blood flow showed a decrease in the cerebral cortex (especially in the supramarginal gyrus) consistent with cortical ribboning, but an increase in the basal ganglia, probably involved in orofacial dystonia. Cortical ribboning on MRI might be a better biomarker than RT-QuIC in the prodromal phase of sCJD.
一位 63 岁的女性因口面肌张力障碍就诊，其 MRI 显示皮质带状征，这是散发性克雅氏病（sCJD）的典型 MRI 表现。然而，实时震颤诱导转化（RT-QuIC）是 sCJD 早期诊断最敏感的方法，其结果为阴性。6 个月后，该患者发展为 sCJD，此时 RT-QuIC 转为阳性。脑血流显示皮质（尤其是缘上回）血流量减少，与皮质带状征一致，但基底节区血流量增加，可能与口面肌张力障碍有关。在 sCJD 的前驱期，MRI 上的皮质带状征可能比 RT-QuIC 更能作为生物标志物。
