caa a22 4500 445808713 CHVBK 20180317145204.0 cr unu---uuuuu 170323e20111201xx s 000 0 eng 10.1007/s00415-011-6099-5 doi (NATIONALLICENCE)springer-10.1007/s00415-011-6099-5 Exploratory 7-Tesla magnetic resonance spectroscopy in Huntington's disease provides in vivo evidence for impaired energy metabolism [Elektronische Daten] [Simon van den Bogaard, Eve Dumas, Wouter Teeuwisse, Hermien Kan, Andrew Webb, Raymund Roos, Jeroen van der Grond] Huntington's disease (HD) is a neurodegenerative genetic disorder that affects the brain. Atrophy of deep grey matter structures has been reported and it is likely that underlying pathologic processes occur before, or in concurrence with, volumetric changes. Measurement of metabolite concentrations in these brain structures has the potential to provide insight into pathological processes. We aim to gain understanding of metabolite changes with respect to the disease stage and pathophysiological changes. We studied five brain regions using magnetic resonance spectroscopy (MRS) using a 7-Tesla MRI scanner. Localized proton spectra were acquired to obtain six metabolite concentrations. MRS was performed in the caudate nucleus, putamen, thalamus, hypothalamus, and frontal lobe in 44 control subjects, premanifest gene carriers and manifest HD. In the caudate nucleus, HD patients display lower NAA (p=0.009) and lower creatine concentration (p=0.001) as compared to controls. In the putamen, manifest HD patients show lower NAA (p=0.024), lower creatine concentration (p=0.027), and lower glutamate (p=0.013). Although absolute values of NAA, creatine, and glutamate were lower, no significant differences to controls were found in the premanifest gene carriers. The lower concentrations of NAA and creatine in the caudate nucleus and putamen of early manifest HD suggest deficits in neuronal integrity and energy metabolism. The changes in glutamate could support the excitotoxicity theory. These findings not only give insight into neuropathological changes in HD but also indicate that MRS can possibly be applied in future clinical trails to evaluate medication targeted at specific metabolic processes. The Author(s), 2011 Huntington's disease nationallicence MRI nationallicence MRS nationallicence Energy metabolism nationallicence AU : Arbitrary units nationallicence BDI-II : Beck Depression Inventory 2nd version nationallicence CAG : Cytosine-adenine-guanine nationallicence DART : Dutch Adult Reading Test nationallicence FWHM : Full width half maximum nationallicence Glx : Glutamate+glutamine nationallicence HD : Huntington's disease nationallicence MMSE : Mini Mental State Exam nationallicence NAA : N-acetylaspartate nationallicence PMGC : Premanifest gene carrier nationallicence STEAM : Stimulated echo acquisition mode nationallicence TFC : Total functional capacity nationallicence TMS : Total motor score nationallicence UHDRS : Unified Huntington's Disease Rating Scale nationallicence van den Bogaard Simon Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, The Netherlands aut Dumas Eve Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, The Netherlands aut Teeuwisse Wouter C.J. Gorter Center for High Field MRI, Leiden University Medical Centre, Leiden, The Netherlands aut Kan Hermien C.J. Gorter Center for High Field MRI, Leiden University Medical Centre, Leiden, The Netherlands aut Webb Andrew C.J. Gorter Center for High Field MRI, Leiden University Medical Centre, Leiden, The Netherlands aut Roos Raymund Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, The Netherlands aut van der Grond Jeroen Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands aut Journal of Neurology Springer-Verlag 258/12(2011-12-01), 2230-2239 0340-5354 258:12<2230 2011 258 415 https://doi.org/10.1007/s00415-011-6099-5 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-011-6099-5 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- van den Bogaard Simon Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, The Netherlands aut NATIONALLICENCE 700 1- Dumas Eve Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, The Netherlands aut NATIONALLICENCE 700 1- Teeuwisse Wouter C.J. Gorter Center for High Field MRI, Leiden University Medical Centre, Leiden, The Netherlands aut NATIONALLICENCE 700 1- Kan Hermien C.J. Gorter Center for High Field MRI, Leiden University Medical Centre, Leiden, The Netherlands aut NATIONALLICENCE 700 1- Webb Andrew C.J. Gorter Center for High Field MRI, Leiden University Medical Centre, Leiden, The Netherlands aut NATIONALLICENCE 700 1- Roos Raymund Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2300 RC, Leiden, The Netherlands aut NATIONALLICENCE 700 1- van der Grond Jeroen Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/12(2011-12-01), 2230-2239 0340-5354 258:12<2230 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer