title: Brain energy metabolism in early MSA-P: A phosphorus and proton magnetic resonance spectroscopy study.
abstract: BACKGROUND
  Recently, mutations in the COQ2 gene, encoding for an enzyme involved in coenzyme Q10 biosynthesis, have been suggested to confer susceptibility risk for multiple system atrophy (MSA). Thus, the possible role of mitochondrial dysfunction in the pathophysiology of MSA has emerged. Here, we studied brain energy metabolism in vivo in early MSA-parkinsonism (MSA-P) patients and compared to healthy controls.
  
  
  METHODS
  We have used combined phosphorus and proton magnetic resonance spectroscopy to measure high- and low-energy phosphates in the basal ganglia of early (Hoehn and Yahr stage I-III), probable MSA-P patients (N = 9) compared to healthy controls (N = 9).
  
  
  RESULTS
  No significant changes in the high energy phosphates and other parameters reflecting the energy status of the cells were found in the basal ganglia of MSA-P patients compared to healthy controls. N-acetylaspartate was significantly reduced in MSA-P compared to healthy controls and correlated with the Unified Multiple System Atrophy Rating Scale.
  
  
  CONCLUSIONS
  Brain energy metabolism in early MSA-P is not impaired, despite the presence of impaired neuronal integrity. This may imply that mitochondrial dysfunction may not play a primary role in the pathophysiology of MSA, at least in European populations.
authors: Department of Neurology, Philipps University Marburg, Germany; Second Department of Neurology, University of Athens, Greece; Movement Disorders Department, Hygeia Hospital, Athens, Greece. Electronic address: mariastamelou@gmail.com.
authors: M
authors: M
authors: Stamelou
authors: Institute of Neuroradiology, Goethe-University Frankfurt, Frankfurt, Germany.
authors: U
authors: U
authors: Pilatus
authors: Center for Clinical Trials, Philipps University, Marburg, Germany.
authors: A
authors: A
authors: Reuss
authors: Department of Neurology, Philipps University Marburg, Germany; Department of Translational Neurodegeneration, German Centre for Neurodegenerative Diseases (DZNE), Munich, Germany; Department of Neurology, Technische Universität München, Munich, Germany.
authors: G
authors: G
authors: Respondek
authors: Department of Neurology, Philipps University Marburg, Germany.
authors: S
authors: S
authors: Knake
authors: Department of Neurology, Philipps University Marburg, Germany.
authors: W H
authors: WH
authors: Oertel
authors: Department of Neurology, Philipps University Marburg, Germany; Department of Translational Neurodegeneration, German Centre for Neurodegenerative Diseases (DZNE), Munich, Germany; Department of Neurology, Technische Universität München, Munich, Germany.
authors: G U
authors: GU
authors: Höglinger
mesh_terms: D000368:Aged; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D042241:Early Diagnosis; D004734:Energy Metabolism / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000175:diagnosis* / Q000378:metabolism*; D010759:Phosphorus Isotopes; D010865:Pilot Projects; D066244:Proton Magnetic Resonance Spectroscopy / Q000379:methods* / Q000592:standards
chemical_list: D010759:Phosphorus Isotopes
keywords: COQ2 gene mutations; Energy metabolism; Mitochondrial dysfunction; Multiple system atrophy; Phosphorus spectroscopy