title: In vivo functional brain imaging and a therapeutic trial of L-arginine in MELAS patients.
abstract: BACKGROUND
  Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is the most common type of mitochondrial disease and is characterized by stroke-like episodes (SEs), myopathy, lactic acidosis, diabetes mellitus, hearing-loss and cardiomyopathy. The causal hypotheses for SEs in MELAS presented to date are angiopathy, cytopathy and neuronal hyperexcitability. L-arginine (Arg) has been applied for the therapy in MELAS patients.
  
  
  METHODS
  We will introduce novel in vivo functional brain imaging techniques such as MRI and PET, and discuss the pathogenesis of SEs in MELAS patients. We will further describe here our clinical experience with L-arg therapy and discuss the dual pharmaceutical effects of this drug on MELAS.
  
  
  CONCLUSIONS
  Administration of L-arg to MELAS patients has been successful in reducing neurological symptoms due to acute strokes and preventing recurrences of SEs in the chronic phase. L-Arg has dual pharmaceutical effects on both angiopathy and cytopathy in MELAS.
  
  
  CONCLUSIONS
  In vivo functional brain imaging promotes a better understanding of the pathogenesis and potential therapies for MELAS patients. This article is part of a Special Issue entitled Biochemistry of Mitochondria, Life and Intervention 2010.
authors: Department of Neurology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan. myoneda@u-fukui.ac.jp
authors: Makoto
authors: M
authors: Yoneda
authors: Masamichi
authors: M
authors: Ikawa
authors: Kenichiro
authors: K
authors: Arakawa
authors: Takashi
authors: T
authors: Kudo
authors: Hirohiko
authors: H
authors: Kimura
authors: Yasuhisa
authors: Y
authors: Fujibayashi
authors: Hidehiko
authors: H
authors: Okazawa