PMC4308028_01
EEG displayed in bipolar montage using the 10-20 system. Interictal generalized periodic 1-Hz triphasic sharp waves.
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Because the patient had unexplained altered mental status, he underwent long-term EEG monitoring at the start of admission which revealed sharp generalized periodic discharges with a triphasic morphology at a frequency of 1 Hz with greater amplitude on the left (Fig. 1).
a. DWI and ADC images of MRI with DWI demonstrating diffusion restriction in the cortical ribbon diffusely within the left hemisphere. b. DWI and ADC images of MRI with DWI demonstrating diffusion restriction in the bilateral caudate nuclei and the cortical ribbon diffusely within the left hemisphere.
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Brain MRI demonstrated prominent diffusion restriction in the cortical ribbon of the left hemisphere and in the caudate nuclei bilaterally (Fig. 2).
a. Onset of electrographic seizure with generalized spike-wave complexes evolving out of quiescent background and increasing in frequency from 0.5 Hz to 1 Hz. b. In the midst of the same electrographic seizure with generalized spike-wave complexes at a frequency of 2 Hz. c. End of the same electrographic seizure with termination of generalized spike-wave complexes and resumption of background activity.
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With worsening mental status, a repeat EEG disclosed recurrent runs lasting up to 60 s of spike and wave activity arising from a quiescent background and evolving in frequency from 0.5 Hz to a maximum of 2 Hz before terminating abruptly (Fig. 3).
a. Frontal cortex (LH&E stain) showing marked spongiform change at 10 x magnification. b. Frontal cortex (LH&E stain) showing marked spongiform change, vacuoles, and reactive gliosis at 40 x magnification.
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Autopsy confirmed the diagnosis of sporadic CJD (Fig. 4).