Proton Magnetic Resonance Spectroscopy Indicates Preserved Cerebral Biochemical Composition in Duchenne Muscular Dystrophy Patients

Nathalie Doorenweerd, Melissa Hooijmans, Stephanie A. Schubert, Andrew G. Webb, Chiara S.M. Straathof, Erik W. Van Zwet, Mark A. Van Buchem, Jan J.G.M. Verschuuren, Jos G.M. Hendriksen, Erik H. Niks, Hermien E. Kan

Research output: Contribution to journalArticleAcademicpeer-review

5 Citations (Scopus)

Abstract

Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin. DMD is associated with specific learning and behavioural disabilities. In the brain, dystrophin is associated with GABAA receptors and aquaporin-4 in neurons and astrocytes, respectively, but little is known about its function. Objective and Methods: In this study we aimed to compare the biochemical composition between patients and healthy controls in brain regions that are naturally rich in dystrophin using magnetic resonance spectroscopy. Given previous conflicting results obtained at clinical field strengths, we obtained data using a 7 Tesla system with associated higher signal-to-noise ratio and spectral resolution. Results: Results indicated unchanged biochemical composition in all regions investigated, and increased variance in glutamate in the frontal cortex.
Original languageEnglish
Pages (from-to)53-58
Number of pages6
JournalJournal of Neuromuscular Diseases
Volume4
Issue number1
DOIs
Publication statusPublished - 2017
Externally publishedYes

Keywords

  • Adolescent
  • Aquaporin 4/metabolism
  • Aspartic Acid/analogs & derivatives
  • Brain/diagnostic imaging
  • Case-Control Studies
  • Cerebellum/diagnostic imaging
  • Child
  • Choline/metabolism
  • Creatine/metabolism
  • Frontal Lobe/diagnostic imaging
  • Glutamic Acid/metabolism
  • Glutamine/metabolism
  • Hippocampus/diagnostic imaging
  • Humans
  • Inositol/metabolism
  • Male
  • Muscular Dystrophy, Duchenne/diagnostic imaging
  • Proton Magnetic Resonance Spectroscopy
  • Receptors, GABA-A/metabolism

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