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Abstract
Objectives Currently, no established biomarkers exist for presymptomatic sporadic Creutzfeldt-Jakob disease (sCJD). The purpose of this study was to raise awareness about sCJD cases showing abnormalities on brain MRI diffusion-weighted imaging (DWI) before symptom onset and demonstrate temporal changes in DWI abnormalities during the preclinical period.
Methods We described the clinical presentation including the results of MRI—performed multiple times in the preclinical period—and the diagnostic workup of a middle-aged man with sCJD.
Results MRI of the brain performed 27 months before symptom onset revealed an extremely localized lesion on DWI in the right occipital cortex. Follow-up MRI scans showed propagation of DWI abnormalities along the cortices without the appearance of neurologic symptoms/signs. After symptom onset, the patient's neuropsychiatric condition rapidly deteriorated. Elevated total tau protein levels and positive 14-3-3 protein were observed in the CSF, and periodic synchronous discharges using electroencephalography resulted in the diagnosis of sCJD.
Discussion CJD should be considered in differential diagnoses when localized DWI signal abnormalities propagate along the cortices over time, even in the absence of typical CJD symptoms. DWI signal abnormalities on brain MRI scans may be highly sensitive diagnostic markers for CJD, even in the preclinical stage.
Footnotes
Submitted and externally peer reviewed. The handling editor was Linda Hershey, MD, PhD, FAAN.
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
eReference is available at links.lww.com/WNL/C288.
- Received February 12, 2022.
- Accepted in final form July 26, 2022.
- © 2022 American Academy of Neurology
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