Estimation of skeletal muscle energy metabolism in Machado‐Joseph disease using 31P‐MR spectroscopy |
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| Authors: | Ichiro Yabe MD PhD Khin K Tha MD PhD Takashi Yokota MD PhD Kazunori Sato MD Hiroyuki Soma MD PhD Asako Takei MD Satoshi Terae MD PhD Koichi Okita MD PhD Hidenao Sasaki MD PhD |
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| Institution: | 1. Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Japan;2. Department of Radiology, Hokkaido University Graduate School of Medicine, Sapporo, Japan;3. Department of Cardiovascular Medicine, Hokkaido University Graduate School of Medicine, Sapporo, Japan;4. Hokuyukai Neurology Hospital, Sapporo, Japan;5. Graduate School of Program in Lifelong Learning Studies, Hokusho University, Ebetsu, Japan |
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| Abstract: | The aim of this study was to determine if muscle energy metabolism, as measured by 31P‐magnetic resonance spectroscopy (MRS), is a metabolic marker for the efficacy of treatment of Machado‐Joseph disease (MJD). We obtained 31P‐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 (Vmax) (P < 0.01). In addition, Vmax 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 Vmax 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. © 2010 Movement Disorder Society |
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| Keywords: | Machado‐Joseph disease muscle energy metabolism SARA cerebellar ataxia surrogate marker 31P‐MRS |
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