Significance of Cortical Ribboning as a Biomarker in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease |
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| Authors: | Yasuhiro Hamada Kazushi Deguchi Kisaki Tachi Makoto Kita Wakako Nonaka Tadayuki Takata Hideki Kobara Tetsuo Touge Katsuya Satoh Tsutomu Masaki |
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| Affiliation: | 1.Department of Gastroenterology and Neurology, Graduate School of Medicine and Faculty of Medicine, Kagawa University, Japan; 2.Department of Health Sciences, Graduate School of Biomedical Sciences and School of Medicine, Nagasaki University, Japan |
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| Abstract: | ![]()
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. |
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| Keywords: | Creutzfeldt-Jakob disease prion cortical ribboning orofacial dystonia prodromal phase |
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