title: Metabolic disorders of the brain: part II.
abstract: Metabolic disorders of the brain are extensive and to encompass these disorders in two articles is impossible. The aim of this article to provide a brief overview of the non-organelle based metabolic disorders affecting the brain. Due to its complexity, classification of the metabolic disorders of brain is not easy. In this article we have discussed the non-organelle based metabolic disorders under following headings: aminoaciduria, organic academia, nuclear DNA repair defects, a defect in the gene encoding myelin proteins and various miscellaneous defects. Even though the neuroimaging findings overlay and may be non-specific for many of the metabolic disorders, some of the imaging features enable the radiologist to provide the physician with a logical approach by providing a road map and/or limiting the differential diagnosis. Understanding the basic biochemical defects is important to recognize these disorders. The relevant biochemistry and genetic defects are also discussed.
authors: Department of Radiology, Penn State Milton S. Hershey Medical Center and College of Medicine, Hershey, PA 17033, USA. skanekar@hmc.psu.edu
authors: Sangam
authors: S
authors: Kanekar
authors: Joel
authors: J
authors: Verbrugge
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D001928:Brain Diseases, Metabolic / Q000175:diagnosis* / Q000378:metabolism*; D003952:Diagnostic Imaging / Q000379:methods*; D006801:Humans; D011859:Radiography