title: Cerebral energy metabolism in isovaleric acidaemia.
abstract: A newborn infant with an acute metabolic encephalopathy caused by isovaleric acidaemia had severe impairment of cerebral energy metabolism. This was detected by phosphorus and proton magnetic resonance spectroscopy. After treatment she made excellent clinical recovery, her spectroscopic abnormalities resolved, and she was neurologically normal at the age of 1 year.
authors.affiliation: Department of Paediatrics, University College London School of Medicine.
authors.forename: A K
authors.initials: AK
authors.lastname: Lorek
authors.forename: J M
authors.initials: JM
authors.lastname: Penrice
authors.forename: E B
authors.initials: EB
authors.lastname: Cady
authors.forename: J V
authors.initials: JV
authors.lastname: Leonard
authors.forename: J S
authors.initials: JS
authors.lastname: Wyatt
authors.forename: R A
authors.initials: RA
authors.lastname: Iles
authors.forename: S P
authors.initials: SP
authors.lastname: Burns
authors.forename: E O
authors.initials: EO
authors.lastname: Reynolds
mesh_terms: D001921:Brain / Q000378:metabolism*; D001927:Brain Diseases / Q000209:etiology / Q000378:metabolism*; D004734:Energy Metabolism; D005260:Female; D006801:Humans; D006859:Hydrogen; D007223:Infant; D007231:Infant, Newborn; D050769:Isovaleryl-CoA Dehydrogenase; D009682:Magnetic Resonance Spectroscopy; D008661:Metabolism, Inborn Errors / Q000150:complications / Q000378:metabolism* / Q000628:therapy; D010088:Oxidoreductases / Q000172:deficiency*; D044925:Oxidoreductases Acting on CH-CH Group Donors; D010759:Phosphorus Isotopes; D016896:Treatment Outcome
chemical_list: D010759:Phosphorus Isotopes; D006859:Hydrogen; D010088:Oxidoreductases; D044925:Oxidoreductases Acting on CH-CH Group Donors; D050769:Isovaleryl-CoA Dehydrogenase