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Abstract #4016

Using Proton MR Spectroscopy and Quantitative Susceptibility Mapping (QSM) at 7 Tesla to Decipher Mitochondrial Membrane Protein–Associated Neurodegeneration (MPAN)

Ralf Mekle1, Florian Schubert1, Thoralf Niendorf2, Till Huelnhagen2, Antje Else2, Simon Daniel Robinson3, Bernd Ittermann1, Vince Madai4, Marta Skowronska5, Petr Dusek6,7, Jens Wuerfel8, and Susanne A. Schneider9

1Physikalisch-Technische Bundesanstalt (PTB), Braunschweig and Berlin, Germany, 2Berlin Ultrahigh Field Facility (B.U.F.F.), Max Delbrück Center for Molecular Medicine in the Helmholtz Association, Berlin, Germany, 3High Field MR Centre, Department of Biomedical Imaging and Image guided Therapy, Medical University of Vienna, Vienna, Austria, 4Department of Neurology and Center for Stroke Research Berlin, Charité Universitaetsmedizin, Berlin, Germany, 52nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland, 6Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine and General University Hospital in Prague, Charles University in Prague, Prague, Czech Republic, 7Institute of Neuroradiology, University Medicine Goettingen, Goettingen, Germany, 8Medical Image Analysis Center, Basel, Switzerland, 9Neurology Department, University of Kiel, Kiel, Germany

1H MR spectroscopy (MRS) in the white matter (WM) of the precentral region and quantitative susceptibility mapping (QSM) of basal ganglia acquired at 7 Tesla in patients (homozygotes), non-manifesting heterozygotes, and control subjects were used to investigate mitochondrial membrane protein-associated neurodegeneration (MPAN). MPAN is a rare, but severe disorder from the neurodegeneration with brain iron accumulation (NBIA) group. Comparison of results for the different subject groups showed no abnormalities in heterozygotes. In contrast, metabolic changes in patients detected by MRS, in particular an increase in glutamate, suggests an underlying mechanism for MPAN related to neurotransmission in corticospinal pathway.

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