Estimation of skeletal muscle energy metabolism in Machado-Joseph disease using (31)P-MR spectroscopy.
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.
Yabe
Ichiro
I
Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Tha
Khin K
KK
Yokota
Takashi
T
Sato
Kazunori
K
Soma
Hiroyuki
H
Takei
Asako
A
Terae
Satoshi
S
Okita
Koichi
K
Sasaki
Hidenao
H
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
D010759:Phosphorus Isotopes