Brain magnetic resonance imaging in the DE50-MD dog model of Duchenne muscular dystrophy reveals regional reductions in cerebral gray matter

Abbe H Crawford, Natasha L Hornby, Alerie G de la Fuente, Richard J Piercy*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

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Abstract

Background
Duchenne muscular dystrophy is a X-linked disease characterized by severe and progressive muscle weakness, alongside cognitive impairment and a range of neurobehavioral disorders secondary to brain dystrophin deficiency. Duchenne muscular dystrophy patients have reduced cerebral gray matter and altered white matter ultrastructure (detected by magnetic resonance imaging) compared to age-matched controls.

Methods
We studied the DE50-MD canine model of Duchenne muscular dystrophy, which is deficient in full length brain dystrophin (Dp427) isoforms and has a neurocognitive phenotype. Eight DE50-MD and 6 age-matched littermate wild type male dogs underwent serial brain magnetic resonance imaging from 14 to 33 months of age.

Results
Reduced regional gray matter was detected in DE50-MD dogs compared with wildtype, including the piriform lobe, hippocampus and cingulate gyrus. Lateral ventricle volume was larger in DE50-MD dogs. Differences did not progress over time. White matter volume did not differ between DE50-MD and wildtype dogs. There was no difference in brain nor cranial vault volume between DE50-MD and wildtype dogs.

Conclusion
Dystrophin deficiency in the canine brain results in structural changes that likely contribute to the neurocognitive phenotype.
Original languageEnglish
Article number21
JournalBMC Neuroscience
Volume24
Issue number1
DOIs
Publication statusPublished - 17 Mar 2023

Keywords

  • Brain
  • Canine
  • Duchenne muscular dystrophy
  • Magnetic Resonance Imaging
  • Animals
  • Dogs
  • Muscular Dystrophy, Duchenne
  • Dystrophin
  • Male
  • Gray Matter

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