title: Magnetic resonance spectroscopy: use in monitoring MELAS treatment.
abstract: BACKGROUND
  Sodium dichloroacetate has been used to treat patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). Magnetic resonance spectroscopy (MRS) has been used to assess cerebral metabolism in MELAS, but to our knowledge, the findings of serial MRS studies performed after therapeutic intervention of strokelike episodes have not been reported.
  
  
  METHODS
  Proton MRS was serially used to measure brain metabolites in strokelike regions and in clinically uninvolved brain regions in a patient with MELAS.
  
  
  METHODS
  A patient with MELAS and a strokelike episode clinically improved after treatment with sodium dichloroacetate. An elevated lactate-creatine ratio in the "stroke" region decreased on MRS studies after treatment. After a second episode, the lactate-creatine ratio increased from baseline in a region of the brain that was normal on magnetic resonance imaging scans.
  
  
  CONCLUSIONS
  To our knowledge, this is the first study to assess the response to treatment of a MELAS strokelike episode and the first to show an increase in the lactate-creatine ratio in a brain region that was associated with a clinical abnormality, even though it appeared normal on magnetic resonance imaging. We conclude that MRS may help to monitor therapeutic efficacy in mitochondrial encephalomyopathies.
authors.affiliation: Department of Neurology, North Shore University Hospital, Manhasset, NY 11030, USA.
authors.forename: S G
authors.initials: SG
authors.lastname: Pavlakis
authors.forename: P B
authors.initials: PB
authors.lastname: Kingsley
authors.forename: G P
authors.initials: GP
authors.lastname: Kaplan
authors.forename: P W
authors.initials: PW
authors.lastname: Stacpoole
authors.forename: M
authors.initials: M
authors.lastname: O'Shea
authors.forename: D
authors.initials: D
authors.lastname: Lustbader
mesh_terms: D000328:Adult; D001921:Brain / Q000378:metabolism; D002561:Cerebrovascular Disorders / Q000175:diagnosis; D003401:Creatine / Q000032:analysis; D003999:Dichloroacetic Acid / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D019344:Lactic Acid / Q000032:analysis; D017241:MELAS Syndrome / Q000175:diagnosis* / Q000378:metabolism; D009682:Magnetic Resonance Spectroscopy; D016896:Treatment Outcome
chemical_list: D019344:Lactic Acid; D003999:Dichloroacetic Acid; D003401:Creatine