Phosphorus-31 magnetic resonance spectroscopy of skeletal muscle in maternally inherited diabetes and deafness A3243G mitochondrial mutation carriers

✍ Scribed by Saskia G.C. van Elderen; Joost Doornbos; Einar H.R. van Essen; Herman H.P.J. Lemkes; J. Antonie Maassen; Jan W.A. Smit; Albert de Roos

• Publisher: John Wiley and Sons
• Year: 2009
• Tongue: English
• Weight: 136 KB
• Volume: 29
• Category: Article
• ISSN: 1053-1807
• DOI: 10.1002/jmri.21620

✦ Synopsis

Abstract

Purpose

To investigate high‐energy phosphate metabolism in striated skeletal muscle of patients with Maternally Inherited Diabetes and Deafness (MIDD) syndrome.

Materials and Methods

In 11 patients with the MIDD mutation (six with diabetes mellitus [DM] and five non‐DM) and eight healthy subjects, phosphocreatine (PCr) and inorganic phosphate (Pi) in the vastus medialis muscle was measured immediately after exercise using ^31^P‐magnetic resonance spectroscopy (MRS). The half‐time of recovery (t1/2) of monoexponentially fitted (PCr+Pi)/PCr was calculated from spectra obtained every 4 seconds after cessation of exercise. A multiple linear regression model was used for statistical analysis.

Results

Patients with the MIDD mutation showed a significantly prolonged t1/2 (PCr+Pi)/PCr after exercise as compared to controls (13.6±3.0 vs. 8.7±1.3 sec, P = 0.01). No association between the presence of DM and t1/2 (PCr + Pi)/PCr was found (P = 0.382).

Conclusion

MIDD patients showed impaired mitochondrial oxidative phosphorylation in skeletal muscle shortly after exercise, irrespective of the presence of DM. J. Magn. Reson. Imaging 2009;29:127–131. © 2008 Wiley‐Liss, Inc.