Diffusion-weighted MR imaging of early-stage Creutzfeldt-Jakob disease: Typical and atypical manifestations

Creutzfeldt-Jakob disease causes progressive dementia and, eventually, death. The infectious agent is thought to be proteinaceous scrapie particles. Prompt diagnosis is essential to prevent human-to-human transmission. Progressive brain atrophy and areas of high signal intensity in the cerebral cortex and basal ganglia are well-known features of Creutzfeldt-Jakob disease depicted on T2-weighted magnetic resonance (MR) images. However, in the early stage of disease, the appearance of the brain on T2-weighted MR images often is normal, and it may be impossible on that basis to reach a diagnosis. Diffusion-weighted imaging therefore has gained attention as a useful modality for the early diagnosis of Creutzfeldt-Jakob disease. Even before the appearance of the characteristic periodic synchronous discharges on the electroencephalogram, diffusion-weighted images in most cases of Creutzfeldt-Jakob disease depict areas of abnormal signal hyperintensity in the cortex and in the basal ganglia or thalamus. These imaging abnormalities are accompanied by decreased apparent diffusion coefficient values suggestive of restricted diffusion within the tissue. However, if diffusion-weighted imaging findings of abnormal high signal intensity are restricted to the cerebral cortex, it may be necessary to differentiate between Creutzfeldt-Jakob disease and other conditions that may produce progressive dementia (eg, venous hypertensive encephalopathy; chronic herpes encephalitis; and the syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes).