Fig. 2

Cranial imaging at different time points from a 48-year-old man with acute hyperammonemia and hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Computed tomography of the brain 4 days after admission shows a considerable general cerebral edema (a). Magnetic resonance imaging at day 5 shows a widespread cytotoxic edema in insula, gyrus cingula, and the temporal and frontal lobes (b). Magnetic resonance imaging at day 31 demonstrates regression of edema, widespread gliosis in the frontotemporal lobes bilaterally, and hemorrhagic necrosis/malacia in frontotemporal cortex and basal ganglia bilaterally (c). Magnetic resonance imaging 7 months after admission shows pronounced progress of gliosis development, widespread malacia in the tips of the temporal lobes with greatly widened temporal horns of the lateral ventricles (d)