MRI in medium-chain acyl-coenzyme a dehydrogenase deficiency: neuroimaging during the first month

J Pediatr Endocrinol Metab. 2017 Aug 28;30(8):905-908. doi: 10.1515/jpem-2017-0045.

Abstract

Background: Medium-chain acyl-coenzyme A dehydrogenase (MCAD) is the most common genetic disorder of fatty acid oxidation, which presents before the age of 2 with the onset of acute hypoketotic hypoglycemia, and is typically precipitated by stress.

Case presentation: We report serial brain magnetic resonance imaging (MRI) changes, including MR spectroscopy (MRS) and diffusion weighted imaging (DWI), in a patient with a classical MCAD presentation, compared with five healthy controls.

Conclusions: Through this unique case we analyze the evolution of radiological findings during the first month of illness and we highlight the pivotal role of MRI, especially DWI, in the early diagnosis of the decompensated state of the disease.

Keywords: DWI; MCAD; MRI; MRS; metabolic diseases; neuroradiology.

Publication types

  • Case Reports

MeSH terms

  • Acyl-CoA Dehydrogenase / deficiency*
  • Brain / diagnostic imaging*
  • Female
  • Humans
  • Infant
  • Lipid Metabolism, Inborn Errors / diagnostic imaging*
  • Magnetic Resonance Imaging*
  • Neuroimaging

Substances

  • Acyl-CoA Dehydrogenase

Supplementary concepts

  • Medium chain acyl CoA dehydrogenase deficiency