title: Brain ketones detected by proton magnetic resonance spectroscopy in an infant with Ohtahara syndrome treated with ketogenic diet.
abstract: Atypical resonances on proton magnetic resonance spectroscopy (MRS) examinations are occasionally found in children undergoing a metabolic evaluation for neurological conditions. While a radiologist's first instinct is to suspect a pathological metabolite, usually the origin of the resonance arises from an exogenous source. We report the appearance of distinct resonances associated with a ketogenic diet in a male infant presenting with Ohtahara syndrome. These resonances can be confused in interpretation with lactate and glutamate. To confirm assignments, the basis set for quantification was supplemented with simulations of β-hydroxybutyrate, acetone and acetoacetate in LCModel spectroscopy processing software. We were able to quantitate the levels of end products of a ketogenic diet and illustrate how to distinguish these resonances.
authors.affiliation: Department of Radiology, Imaging Research Center, Cincinnati Children's Hospital Medical Center, MLC 5033, 3333 Burnet Ave., Cincinnati, OH, 45229, USA, kim.cecil@cchmc.org.
authors.forename: Kim M
authors.initials: KM
authors.lastname: Cecil
authors.forename: Sarah B
authors.initials: SB
authors.lastname: Mulkey
authors.forename: Xiawei
authors.initials: X
authors.lastname: Ou
authors.forename: Charles M
authors.initials: CM
authors.lastname: Glasier
mesh_terms: D058540:Aicardi Syndrome / Q000175:diagnosis / Q000178:diet therapy* / Q000378:metabolism*; D001921:Brain / Q000378:metabolism*; D055423:Diet, Ketogenic / Q000379:methods*; D006801:Humans; D007223:Infant; D007659:Ketones / Q000378:metabolism*; D008297:Male; D066244:Proton Magnetic Resonance Spectroscopy / Q000379:methods*; D013036:Spasms, Infantile / Q000175:diagnosis / Q000178:diet therapy* / Q000378:metabolism*; D016896:Treatment Outcome
chemical_list: D007659:Ketones