Creutzfeldt-Jakob disease: Which diffusion-weighted imaging abnormality is associated with periodic EEG complexes?

摘要

BACKGROUND : Clinical diagnosis of CJD remains important due to lack of access to a genetic or histopathological diagnosis. Using current WHO criteria, diagnostic certainty can be increased from "possible" to "probable" CJD if periodic complexes are recorded on EEG. OBJECTIVE : To study the correlation between patterns of MRI-DWI hyperintensity and typical EEG findings among patients with CJD. METHODS : Demographics, clinical findings, MRI-DWI and EEG findings of CJD patients were retrospectively reviewed. RESULTS : A total of 14 patients ranging in age from 35 to 81 years were identified. All had dementia and cerebellar ataxia. Psychiatric manifestations were seen in 5 patients. Seven patients had both cortical and striatal DWI changes, five had isolated cortical DWI changes and two had isolated striatal DWI changes. All twelve patients with cortical DWI changes also had periodic EEG changes. In ten, periodic EEG was recorded within seven days of the DWI. The two patients with isolated striatal DWI changes did not develop periodic EEG complexes despite serial EEG recordings, 40 and 88 days from their respective DWI scans. CONCLUSIONS : Serial EEGs are not useful for patients with isolated striatal DWI hyperintensity but will increase diagnostic certainty from "possible" to probable" CJD for patients with cortical DWI hyperintensity.