3975 These metabolically active astrocytes have swollen nuclei with chromatin pushed against the nuclear membrane. The cytoplasm contains rER and mitochondria. There is a defect in copper metabolism in which there is abnormal binding of copper to ceruloplasmin. This results in Cu deposition in certain tissues. Reye's Syndrome A childhood disease characterized by abrupt onset of encephalopathy with seizures, microvesicular fatty liver, and widespread mitochondrial injury.
Many cases are fatal and preceded by a viral upper respiratory infection.
It may to be related to aspirin use. There is micro-vesicular fatty change with mitochondrial injury in the liver, and there is elevated NH3, bilirubin, and aminotransferase levels. Treatment is plasmaphoresis and exchange transfusion for liver failure, and mannitol infusion to decrease intracranial pressure. This is an Axonal Neuropathy due to degeneration of distal axons and probably results from disordered glucose metabolism. Diabetes also produces a neuropathy due to microangiopathy.