CT findings in a case of deficiency of 3-hydroxy-3-methylglutaryl-CoA-lyase

  title={CT findings in a case of deficiency of 3-hydroxy-3-methylglutaryl-CoA-lyase},
  author={G. Lisson and Dorothea Leupold and Doris Bechinger and Claus Werner Wallesch},
Two CT scans have been performed on a child with a biochemically confirmed 3-HMG-CoA-lyase deficiency. Macrocephalus, widespread hypodensity of the white matter with cystic alterations and progressive dilatation of the ventricles were found. The clinical features and CT findings are surprisingly similar to findings in patients with spongy degeneration (Canavan).