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

Crawford, Abbe H; Hornby, Natasha L; De La Fuente, Alerie G; Piercy, Richard J

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 ultra-structure (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 litter-mate 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 neu-rocognitive phenotype.

Citation

Crawford, A. H., Hornby, N. L., De La Fuente, A. G., & Piercy, R. J. (2023). Brain magnetic resonance imaging in the DE50-MD dog model of Duchenne muscular dystrophy reveals regional reductions in cerebral gray matter. BMC Neuroscience, https://doi.org/10.1186/s12868-023-00788-2

• Journal Article Type: Article
• Acceptance Date: Feb 24, 2023
• Online Publication Date: Mar 17, 2023
• Publication Date: Mar 17, 2023
• Deposit Date: Mar 25, 2023
• Publicly Available Date: Mar 27, 2023
• Publisher: BioMed Central
• Peer Reviewed: Peer Reviewed
• DOI: https://doi.org/10.1186/s12868-023-00788-2
• Keywords: Duchenne muscular dystrophy; Canine; Brain; Magnetic resonance imaging