Serial imaging of a child with a clinical complex of bilateral facial PWS, early-onset severe seizures, and fatally progressive encephalopathy. Serial.

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Presentation transcript:

Serial imaging of a child with a clinical complex of bilateral facial PWS, early-onset severe seizures, and fatally progressive encephalopathy. Serial imaging of a child with a clinical complex of bilateral facial PWS, early-onset severe seizures, and fatally progressive encephalopathy. Imaging demonstrates progressive cerebral atrophy and calcification without evidence of pial enhancement. A, Contrast-enhanced CT scan at 1 month of age demonstrates mild cerebral atrophy and bilateral choroid plexus hypertrophy (black arrow). B, Axial T1WI postgadolinium image at 12 months of age shows further cerebral volume loss but no evidence of abnormal pial enhancement. C, CT scan at 15 months of age demonstrates progressive symmetric cerebral volume loss with calcification of the cortex and subcortical white matter, most marked within the frontal lobes bilaterally (white arrows). M.E. Adams et al. AJNR Am J Neuroradiol 2009;30:276-281 ©2009 by American Society of Neuroradiology