Proton MRS in Kennedy disease: absolute metabolite and macromolecular concentrations.

Abstract

PURPOSE:

To investigate whether glutamine and glutamate (Glx) were elevated in Kennedy Disease (KD), and whether pathological proteins were spectroscopically visible as altered macromolecular (MM) resonances.

MATERIALS AND METHODS:

Ten patients with KD and twelve healthy volunteers were investigated using a stimulated echo acquisition mode (STEAM) spectroscopy sequence with metabolite-nulling.

RESULTS:

The concentrations of Glx remained unchanged in KD. An increased myo-inositol (Ins), and elevated MM at 0.9 ppm were found within the motor area. The N-acetyl-aspartate (NAA)/creatine (Cr) ratio was negatively correlated to the number of cytosine adenosine guanine (CAG) repeats in the motor area.

CONCLUSION:

The elevated MM at 0.9 ppm may be attributed to a pathologically altered protein in KD. Additionally, the changes of Ins point to a clinically unexpected involvement of the motor cortex. The correlation of NAA/Cr with the number of CAG repeats indicates a link between metabolites and genetic failure.

• Authors: Irina Mader, Jochen Karitzky, Uwe Klose, Uwe Seeger, Anne Sperfeld, Thomas Naegele, Fritz Schick, Albert Ludolph, Wolfgang Grodd
• Journal: Journal of magnetic resonance imaging : JMRI (J Magn Reson Imaging) Vol. 16 Issue 2 Pg. 160-7 (Aug 2002) ISSN: 1053-1807 [Print] United States
• PMID: 12203763 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright 2002 Wiley-Liss, Inc.

Chemical References

• Glutamine
• Aspartic Acid
• Glutamic Acid
• Inositol
• N-acetylaspartate
• Creatine
• Choline

Topics

• Adult
• Aspartic Acid (analogs & derivatives, metabolism)
• Case-Control Studies
• Choline (metabolism)
• Creatine (metabolism)
• Female
• Glutamic Acid (metabolism)
• Glutamine (metabolism)
• Humans
• Inositol (metabolism)
• Magnetic Resonance Spectroscopy
• Male
• Middle Aged
• Motor Cortex (metabolism)
• Muscular Atrophy, Spinal (metabolism)
• Pons (metabolism)