Neuropathology in cerebral lactic acidosis

The neuropathology in two young infants with cerebral lactic acidosis is presented. Both cases showed microcephaly, ventricular dilatation, absent corpus callosum, absent or extremely hypoplastic pyramids, heterotopic inferior olives, focal neuroglial overgrowth into meninges, focal cystic change with gliosis and recent degenerative changes in the germinal matrix. The cerebellum, basal ganglia, Ammon's horn, inferior colliculi and layering of the cortex were well preserved. The features suggest a continuous damage with very old, relatively old and recent lesions. This striking combination of abnormalities should raise the possibility of a disturbance of lactate/pyruvate metabolism.