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Magnetic resonance spectroscopy (MRS) in five patients with treated propionic acidemia

A P Chemelli1, M Schocke, W Sperl

  • 1Department of Radiology, University of Innsbruck, 6020 Innsbruck, Austria.

Journal of Magnetic Resonance Imaging : JMRI
|June 22, 2000
PubMed

Insights

Propionic acidemia, a metabolic disorder, can show brain changes like cerebral atrophy and elevated lactate peaks, detectable by magnetic resonance spectroscopy (MRS) even during stable conditions.

Area of Science:

  • Neurology
  • Metabolic Disorders
  • Medical Imaging

Background:

  • Propionic acidemia is an inherited metabolic disorder resulting from propionyl CoA carboxylase deficiency.
  • Untreated, it can cause severe metabolic decompensation and toxic encephalopathy.
  • Early diagnosis and treatment are crucial for managing this condition.

Purpose of the Study:

  • To investigate the utility of magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) in evaluating brain alterations in treated children with propionic acidemia.
  • To assess if MRS can detect metabolic changes during clinically stable phases of the disorder.

Main Methods:

  • Five children with propionic acidemia, undergoing protein restriction and carnitine supplementation, were examined.
  • Conventional MRI and MRS (long echo time single-voxel spectroscopy and chemical shift imaging) were performed using a 1.5 T scanner.
  • Data were collected between 1992 and 1996.

Main Results:

  • Cerebral atrophy was observed in two children with delayed treatment onset.
  • Elevated lactate peaks were detected by MRS in four of the five children.
  • MRS identified metabolic alterations in the brain, even during metabolically stable periods.

Conclusions:

  • Magnetic resonance spectroscopy is a valuable tool for detecting subclinical metabolic brain alterations in propionic acidemia.
  • Elevated lactate may indicate impaired aerobic oxidation within the citrate cycle due to propionic acid metabolites.
  • These findings highlight the potential of advanced neuroimaging in monitoring treated metabolic disorders.

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