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...

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Bibliographic Details
Published in:Movement disorders 2011-01, Vol.26 (1), p.165-168
Main Authors: Yabe, Ichiro, Tha, Khin K, Yokota, Takashi, Sato, Kazunori, Soma, Hiroyuki, Takei, Asako, Terae, Satoshi, Okita, Koichi, Sasaki, Hidenao
Format: Article
Language:English
Subjects:
Adult
Aged
Energy Metabolism - physiology
Female
Humans
Machado-Joseph Disease - diagnostic imaging
Machado-Joseph Disease - pathology
Machado-Joseph Disease - physiopathology
Magnetic Resonance Spectroscopy
Male
Middle Aged
Muscle, Skeletal - metabolism
Phosphorus Isotopes
Radionuclide Imaging
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Summary: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.
ISSN:1531-8257
DOI:10.1002/mds.23335