Reader response: Teaching NeuroImages: DWI and EEG findings in Creutzfeldt-Jakob disease

We read with interest the Resident & Fellow Teaching NeuroImage by Ganesh et al.¹ of a patient with Creutzfeldt-Jakob disease (CJD) presenting with rapidly progressive dementia, predominant clinical features, MRI findings of hyperintensity of the cortex and bilateral basal ganglia, and EEG (triphasic morphology). We agree with the diagnosis according to the updated clinical diagnostic criteria for sporadic CJD.² However, it is important to provide the data on whether this patient underwent biopsy for neuropathology or if 14-3-3 protein was detected in the CSF, in order to distinguish from familial, new variant, or iatrogenic CJD. The evidence-based guideline with Level B recommendations indicated that, for patients who have rapidly progressive dementia and are strongly suspected of having sporadic CJD (sCJD), the assays for CSF 14-3-3 protein are probably moderately accurate in diagnosing sCJD.³ Despite the unavailable biopsy, the detection of protease-resistant prion by neuropathology could be helpful to the diagnosis of definite sCJD. Further studies are needed to confirm the 2015 updated diagnostic criteria for CJD in clinical practice.⁴