title: Estimation of skeletal muscle energy metabolism in Machado-Joseph disease using (31)P-MR spectroscopy.
abstract: The aim of this study was to determine if muscle energy metabolism, as measured by (31)P-magnetic resonance spectroscopy (MRS), is a metabolic marker for the efficacy of treatment of Machado-Joseph disease (MJD). We obtained (31)P-MRS in the calf muscle of 8 male patients with MJD and 11 healthy men before, during, and after a 4 minute plantar flexion exercise in a supine position. The data showed that there was a significant difference between the groups in terms of the PCr/(Pi + PCr) ratio at rest (P = 0.03) and the maximum rate of mitochondrial ATP production (V(max)) (P < 0.01). In addition, V(max) was inversely correlated with the scale for the assessment and rating of ataxia score (r = -0.34, P = 0.04). The MJD group also showed a reduction in V(max) over the course of 2 years (P < 0.05). These data suggest that this noninvasive measurement of muscle energy metabolism may represent a surrogate marker for MJD.
authors.affiliation: Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Yabe
authors.forename: Khin K
authors.initials: KK
authors.lastname: Tha
authors.forename: Takashi
authors.initials: T
authors.lastname: Yokota
authors.forename: Kazunori
authors.initials: K
authors.lastname: Sato
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Soma
authors.forename: Asako
authors.initials: A
authors.lastname: Takei
authors.forename: Satoshi
authors.initials: S
authors.lastname: Terae
authors.forename: Koichi
authors.initials: K
authors.lastname: Okita
authors.forename: Hidenao
authors.initials: H
authors.lastname: Sasaki
mesh_terms: D000328:Adult; D000368:Aged; D004734:Energy Metabolism / Q000502:physiology*; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000378:metabolism; D010759:Phosphorus Isotopes; D011877:Radionuclide Imaging
chemical_list: D010759:Phosphorus Isotopes