Brain ketones detected by proton magnetic resonance spectroscopy in an infant with Ohtahara syndrome treated with ketogenic diet

Pediatr Radiol. 2015 Jan;45(1):133-7. doi: 10.1007/s00247-014-3032-y. Epub 2014 Jun 8.

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.

Publication types

  • Case Reports

MeSH terms

  • Aicardi Syndrome / diagnosis
  • Aicardi Syndrome / diet therapy*
  • Aicardi Syndrome / metabolism*
  • Brain / metabolism*
  • Diet, Ketogenic / methods*
  • Humans
  • Infant
  • Ketones / metabolism*
  • Male
  • Proton Magnetic Resonance Spectroscopy / methods*
  • Spasms, Infantile / diagnosis
  • Spasms, Infantile / diet therapy*
  • Spasms, Infantile / metabolism*
  • Treatment Outcome

Substances

  • Ketones

Supplementary concepts

  • Infantile Epileptic-Dyskinetic Encephalopathy