Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918

This article has now been updated. Please use the final version.

Significance of Cortical Ribboning as a Biomarker in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease
Yasuhiro HamadaKazushi DeguchiKisaki TachiMakoto KitaWakako NonakaTadayuki TakataHideki KobaraTetsuo TougeKatsuya SatohTsutomu Masaki
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JOURNAL OPEN ACCESS Advance online publication

Article ID: 8354-21

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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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© 2022 by The Japanese Society of Internal Medicine
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