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Estimation of skeletal muscle energy metabolism in Machado‐Joseph disease using 31 P‐MR spectroscopy
Author(s) -
Yabe Ichiro,
Tha Khin K.,
Yokota Takashi,
Sato Kazunori,
Soma Hiroyuki,
Takei Asako,
Terae Satoshi,
Okita Koichi,
Sasaki Hidenao
Publication year - 2011
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.23335
Subject(s) - supine position , machado–joseph disease , energy metabolism , medicine , ataxia , skeletal muscle , endocrinology , disease , spinocerebellar ataxia , psychiatry
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. © 2010 Movement Disorder Society