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American Journal of Neuroradiology, Vol 19, Issue 4 672-674, Copyright © 1998 by American Society of Neuroradiology


ARTICLES

Proton MR spectroscopy in Lesch-Nyhan disease

P Davanzo, Y Ke, MA Thomas, C Anderson, B King, T Belin, J Levitt, J Curran and B Guze
Department of Psychiatry and Behavioral Sciences, University of California, Los Angeles 90024, USA.

In vivo proton spectra for four patients with Lesch-Nyhan disease and four control subjects matched for age and sex were acquired from voxels (1.5 x 1.5 x 1.5 cm3) placed in the prefrontal cortex and striatum. The patients with Lesch-Nyhan disease had decreased metabolites, especially N-acetylaspartate and glutamate/glutamine, only in the prefrontal cortex as compared with the control group. These findings suggest axonal loss in the prefrontal area of this population. The cortical glutamate/glutamine peak decrement does not confirm cytopathologic studies of Lesch-Nyhan disease and deserves further investigation.