P.63 Comparison of brain volume reduction in boys with Duchenne muscular dystrophy treated with different corticosteroid regimes

Abstract

Recent studies show lower gray matter volumes and altered white matter networks in boys with Duchenne Muscular Dystrophy (DMD) compared with healthy controls, suggesting a lack of dystrophin impacts brain structures. However, the majority of boys with DMD are chronically treated with corticosteroids (CS), which are known to cross the blood-brain barrier and cause brain atrophy. Unraveling the impact of CS treatment on the brain of boys with DMD is key to understand brain involvement in this disease. This study compared two groups of DMD boys treated with a different CS regimen with 2 healthy control groups. 3D T1-weighted images were obtained on a 3T Philips system at two different sites. Scans from 20 DMD patients (13.0±3.1y) on a 10 days on/10 days off prednisolone schedule and 20 age-matched controls (13.2±1.8y) were obtained at the LUMC Leiden, the Netherlands. Additionally scans of 20 age-matched DMD patients (12.8±3.0y) on a daily deflazacort regime and 20 age-matched controls (13.1±3.2y) were acquired at the UZ Leuven, Belgium. All 80 scans were processed using FSL software to calculate intracranial (ICV), total brain (TBV), grey matter (GMV), white matter (WMV) and CSF volumes. In addition, volumes of the hippocampus, amygdala, thalamus, nucleus accumbens, caudate nucleus, globus pallidus and putamen were calculated. To control for intracranial volume differences, relative volumes were compared between controls and DMD per center using univariate analyses of variances in SPSS and considered significant at p<0.05. Both DMD groups showed a significant lower relative GMV, and higher relative CSF volume compared to their control groups. Interestingly, a significantly lower relative WMV (33.81% vs 35.45%, p=.002) and relative volume of the globus pallidus (0.25% vs 0.22%, p<.001) was found in the deflazacort group compared with their control group, but not in the prednisolone group. This study suggest that different CS regimes lead to different regional brain volume reductions in patients with DMD.