Decreased cerebral perfusion in Duchenne muscular dystrophy patients

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Decreased cerebral perfusion in Duchenne muscular dystrophy patients Nathalie Doorenweerd, Eve M. Dumas, Eidrees Ghariq, Sophie Schmid, Chiara S.M. Straathof, Arno A.W. Roest, Beatrijs H. Wokke, Erik W. van Zwet, Andrew G. Webb, Jos G.M. Hendriksen, Mark A. van Buchem, Jan J.G.M. Verschuuren, Iris Asllani, Erik H. Niks, Matthias J.P. van Osch, Hermien E. Kan Neuromuscular Disorders Volume 27, Issue 1, Pages 29-37 (January 2017) DOI: 10.1016/j.nmd.2016.10.005 Copyright © 2016 Elsevier B.V. Terms and Conditions

Fig. 1 Representative cerebral blood flow (CBF) maps. Three transverse slices of the same individual in native space. The left two columns show a nine year old participant with DMD with average CBF for the DMD group. The first column shows the net CBF and the second column shows the partial volume corrected (PVC) grey matter CBF. The right two columns show a nine year old control with average CBF for the control group, again with the first column showing net CBF and the second column showing the PVC CBF. The grey scale shows the perfusion in mL/100 g/min. Neuromuscular Disorders 2017 27, 29-37DOI: (10.1016/j.nmd.2016.10.005) Copyright © 2016 Elsevier B.V. Terms and Conditions

Fig. 2 Quantified cerebral blood flow (CBF) with partial volume correction (PVC CBF) and without (Net CBF). The quantified CBF in milliliter per 100 gram brain tissue per minute is shown. (A) depicts control and the whole patient group with a significantly lower CBF in patients. (B) shows control versus DMD_Dp140+ and DMD_Dp140− with a significantly lower CBF in both patient groups compared to control (*p < 0.05, **p < 0.01, ***p < 0.001). Neuromuscular Disorders 2017 27, 29-37DOI: (10.1016/j.nmd.2016.10.005) Copyright © 2016 Elsevier B.V. Terms and Conditions

Fig. 3 Grey matter volume (GMV) versus cerebral blood flow (CBF). No significant correlations were found within the DMD or control groups between CBF and GMV. Neuromuscular Disorders 2017 27, 29-37DOI: (10.1016/j.nmd.2016.10.005) Copyright © 2016 Elsevier B.V. Terms and Conditions

Fig. 4 Localization of significant differences within the grey matter of the brain. Statistical maps, overlaid on MNI_152_2 mm_brain from FSL, localizing regions that differ significantly between groups. Image dimension are shown in the radiological convention with the right hemisphere on the left side of the image and with the z-values indicating the cross-section positioning. There were no regions in which controls showed lower CBF than patients. (A) depicts the results for control versus the whole patient group showing widespread reduced CBF. (B) shows the results for controls versus patients who have isoform Dp140 where the regions with significantly reduced CBF are slightly smaller and more diffuse. (C) depicts the results for controls versus patients missing Dp140 showing widespread reduced CBF. Neuromuscular Disorders 2017 27, 29-37DOI: (10.1016/j.nmd.2016.10.005) Copyright © 2016 Elsevier B.V. Terms and Conditions

Fig. 5 Age versus partial volume corrected cerebral blood flow (PVC CBF). No significant correlations were found within the DMD or control groups between PVC CBF and age. Neuromuscular Disorders 2017 27, 29-37DOI: (10.1016/j.nmd.2016.10.005) Copyright © 2016 Elsevier B.V. Terms and Conditions

Fig. 6 Neuropsychological composite scores versus CBF. No significant correlations were found between information processing (A), word reading (B) or behavioural problems (C) composite scores obtained from the neuropsychological test battery to PVC CBF in controls and DMD patients. Neuromuscular Disorders 2017 27, 29-37DOI: (10.1016/j.nmd.2016.10.005) Copyright © 2016 Elsevier B.V. Terms and Conditions