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Figure 1f. Sporadic Creutzfeldt-Jakob disease in a 72-year-old woman with left hemiparesis. (a–c) MR images obtained 7 weeks after the onset of dementia and 2 weeks before the appearance of periodic synchronous discharges on the EEG and a negative result at testing for the 14-3-3 protein level in cerebrospinal fluid. Axial T1-weighted (a) and axial T2-weighted (b) images show no abnormalities. The diffusion-weighted image (c) reveals symmetric bilateral areas of abnormal high signal intensity in the frontotemporal region of the cerebral cortex, caudate nuclei, and putamen. (d–f) MR images obtained after disease progression to akinetic mutism, 27 weeks after the onset of symptoms. The T1-weighted image (d) shows marked cerebral atrophy. The T2-weighted image (e) shows periventricular white-matter signal hyperintensity and caudate nucleus atrophy. Asymmetric bilateral areas of high signal intensity are visible in the basal ganglia in e. The diffusion-weighted image (f) shows that the bilateral areas of high signal intensity in the basal ganglia have disappeared. High signal intensity is noticeable only in the insula and the thalamus in f. (g) Photomicrograph (original magnification, x20; hematoxylin and eosin stain) shows severe spongiform vacuolar degeneration and neuronal loss of the gray matter neuropil (ie, status spongiosus) in a specimen from the cerebral cortex.
