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 Dusek^6,7, 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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