PMID- 28981141 OWN - NLM STAT- MEDLINE DCOM- 20180730 LR - 20180730 IS - 1365-2362 (Electronic) IS - 0014-2972 (Linking) VI - 47 IP - 12 DP - 2017 Dec TI - Brain and heart magnetic resonance imaging/spectroscopy in duchenne muscular dystrophy. LID - 10.1111/eci.12842 [doi] AB - Duchenne muscular dystrophy (DMD) is an X-linked muscle disorder characterized by progressive and irreversible loss of muscular function. As muscular disease progresses, the repair mechanisms cannot compensate for cellular damage, leading inevitably to necrosis and progressive replacement by fibrous and fatty tissue. Cardiomyopathy and respiratory failure are the main causes of death in DMD. In addition to the well-described muscle and heart disease, cognitive dysfunction affects around 30% of DMD boys. Myocardial fibrosis, assessed by late gadolinium enhancement (LGE), using cardiovascular magnetic resonance imaging (CMR), is an early marker of heart involvement in both DMD patients and female carriers. In parallel, brain MRI identifies smaller total brain volume, smaller grey matter volume, lower white matter fractional anisotropy and higher white matter radial diffusivity in DMD patients. The in vivo brain evaluation of mdx mice, a surrogate animal model of DMD, showed an increased inorganic phosphate (P(i))/phosphocreatine (PCr) and pH. In this paper, we propose a holistic approach using techniques of magnetic resonance imaging, spectroscopy and diffusion tensor imaging as a tool to create a "heart and brain imaging map" in DMD patients that could potentially facilitate the patients' risk stratification and also future research studies in the field. CI - (c) 2017 Stichting European Society for Clinical Investigation Journal Foundation. FAU - Mavrogeni, Sophie AU - Mavrogeni S AUID- ORCID: 0000-0003-1089-7766 AD - Onassis Cardiac Surgery Center, Athens, Greece. FAU - Pons, Roser AU - Pons R AD - 1st Department of Pediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens, Greece. FAU - Nikas, Ioannis AU - Nikas I AD - 1st Department of Pediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens, Greece. FAU - Papadopoulos, George AU - Papadopoulos G AD - 1st Department of Pediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens, Greece. FAU - Verganelakis, Dimitrios A AU - Verganelakis DA AD - 1st Department of Pediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens, Greece. FAU - Kolovou, Genovefa AU - Kolovou G AD - Onassis Cardiac Surgery Center, Athens, Greece. FAU - Chrousos, George P AU - Chrousos GP AD - 1st Department of Pediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens, Greece. LA - eng PT - Journal Article PT - Review DEP - 20171026 PL - England TA - Eur J Clin Invest JT - European journal of clinical investigation JID - 0245331 RN - 0 (Phosphates) RN - 020IUV4N33 (Phosphocreatine) SB - IM MH - Animals MH - Anisotropy MH - Brain/*diagnostic imaging/metabolism/pathology MH - Cardiomyopathies/*diagnostic imaging/etiology MH - Cognitive Dysfunction/*diagnostic imaging/etiology/metabolism/pathology MH - Diffusion Tensor Imaging MH - Disease Models, Animal MH - Fibrosis MH - Gray Matter/diagnostic imaging/pathology MH - Heart/*diagnostic imaging MH - Heterozygote MH - Humans MH - Hydrogen-Ion Concentration MH - Magnetic Resonance Imaging MH - Magnetic Resonance Spectroscopy MH - Mice MH - Mice, Inbred mdx MH - Muscular Dystrophy, Duchenne/complications/*diagnostic imaging/metabolism MH - Myocardium/*pathology MH - Organ Size MH - Phosphates/metabolism MH - Phosphocreatine/metabolism MH - White Matter/diagnostic imaging/pathology OTO - NOTNLM OT - brain magnetic resonance imaging OT - brain magnetic resonance spectroscopy OT - cardiovascular magnetic resonance imaging OT - diffusion tensor imaging OT - duchenne muscular dystrophy EDAT- 2017/10/06 06:00 MHDA- 2018/07/31 06:00 CRDT- 2017/10/06 06:00 PHST- 2017/03/12 00:00 [received] PHST- 2017/10/02 00:00 [accepted] PHST- 2017/10/06 06:00 [pubmed] PHST- 2018/07/31 06:00 [medline] PHST- 2017/10/06 06:00 [entrez] AID - 10.1111/eci.12842 [doi] PST - ppublish SO - Eur J Clin Invest. 2017 Dec;47(12). doi: 10.1111/eci.12842. Epub 2017 Oct 26.