Significance of Cortical Ribboning as a Biomarker in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease

A 63-year-old woman who presented for orofacial dystonia showed cortical ribboning, a typical MRI find-ing 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.

Automated summary

A case study of a 63-year-old woman with orofacial dystonia showed cortical ribboning on MRI, a typical presentation of sporadic Creutzfeldt-Jakob disease (sCJD). However, the real-time quaking-induced conversion (RT-QuIC) test, the most sensitive method for early diagnosis of sCJD, initially came out negative. Six months later, when the patient was diagnosed with sCJD, the RT-QuIC test became positive. The cerebral blood flow showed a decrease in the cerebral cortex, consistent with cortical ribboning, but an increase in the basal ganglia, possibly related to orofacial dystonia. The study suggests that cortical ribboning on MRI may be a more reliable biomarker than RT-QuIC in the prodromal phase of sCJD.