Frontal lobe-dominant cerebral blood flow reduction and atrophy can be progressive in Duchenne muscular dystrophy

Mori‐Yoshimura, Madoka; Aizawa, Kazutaka; Shigemoto, Yoko; Ishihara, Naoko; Minami, Narihiro; Nishino, Ichizo; Yoshida, Sumiko; Sato, Noriko; Takahashi, Yūji

doi:10.1016/j.nmd.2022.02.008

Cited by 5 publications

(2 citation statements)

References 15 publications

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“…Other imaging modalities, such as arterial spin labeling, resting-state functional MRI, and PET imaging, have demonstrated differences in cerebral blood flow, spontaneous cerebral activity, and glucose metabolism in DMD patients. [16][17][18][19] However, the exact relationship between dystrophin expression, brain structure and function, and behavioral and cognitive outcomes remains unclear. 13,20 Corticosteroids can cross the blood-brain barrier and bind to mineralocorticoid and glucocorticoid receptors to affect gene transcription in all major cell types in the brain.…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, these findings provide evidence of the involvement of the brain in DMD, as they suggest that DMD patients with mutations located more distally in the dystrophin gene, and thus lacking brain isoforms, exhibit more pronounced brain alterations. Other imaging modalities, such as arterial spin labeling, resting‐state functional MRI, and PET imaging, have demonstrated differences in cerebral blood flow, spontaneous cerebral activity, and glucose metabolism in DMD patients 16–19 . However, the exact relationship between dystrophin expression, brain structure and function, and behavioral and cognitive outcomes remains unclear 13,20 …”

Section: Introductionmentioning

confidence: 99%

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Comparison of two corticosteroid regimens on brain volumetrics in patients with Duchenne muscular dystrophy

Geuens,

Van Dessel,

Govaarts

et al. 2023

Ann Clin Transl Neurol

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ObjectiveDuchenne muscular dystrophy (DMD) is a neuromuscular disorder in which many patients also have neurobehavioral problems. Corticosteroids, the primary pharmacological treatment for DMD, have been shown to affect brain morphology in other conditions, but data in DMD are lacking. This study aimed to investigate the impact of two corticosteroid regimens on brain volumetrics in DMD using magnetic resonance imaging (MRI).MethodsIn a cross‐sectional, two‐center study, T1‐weighted MRI scans were obtained from three age‐matched groups (9–18 years): DMD patients treated daily with deflazacort (DMDd, n = 20, scan site: Leuven), DMD patients treated intermittently with prednisone (DMDi, n = 20, scan site: Leiden), and healthy controls (n = 40, both scan sites). FSL was used to perform voxel‐based morphometry analyses and to calculate intracranial, total brain, gray matter, white matter, and cerebrospinal fluid volumes. A MANCOVA was employed to compare global volumetrics between groups, with site as covariate.ResultsBoth patient groups displayed regional differences in gray matter volumes compared to the control group. The DMDd group showed a wider extent of brain regions affected and a greater difference overall. This was substantiated by the global volume quantification: the DMDd group, but not the DMDi group, showed significant differences in gray matter, white matter, and cerebrospinal fluid volumes compared to the control group, after correction for intracranial volume.InterpretationVolumetric differences in the brain are considered part of the DMD phenotype. This study suggests an additional impact of corticosteroid treatment showing a contrast between pronounced alterations seen in patients receiving daily corticosteroid treatment and more subtle differences in those treated intermittently.

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