Proton MR spectroscopy in Lesch-Nyhan disease.
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.
Davanzo
P
P
Department of Psychiatry and Behavioral Sciences, University of California, Los Angeles 90024, USA.
Ke
Y
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Thomas
M A
MA
Anderson
C
C
King
B
B
Belin
T
T
Levitt
J
J
Curran
J
J
Guze
B
B
D000293:Adolescent; D000328:Adult; D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000378:metabolism; D002648:Child; D002794:Choline / Q000378:metabolism; D003342:Corpus Striatum / Q000378:metabolism; D018698:Glutamic Acid / Q000378:metabolism; D005973:Glutamine / Q000378:metabolism; D006801:Humans; D007294:Inositol / Q000378:metabolism; D007926:Lesch-Nyhan Syndrome / Q000175:diagnosis* / Q000378:metabolism*; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D017397:Prefrontal Cortex / Q000378:metabolism; D011522:Protons
D011522:Protons; D005973:Glutamine; D001224:Aspartic Acid; D018698:Glutamic Acid; D007294:Inositol; C000179:N-acetylaspartate; D002794:Choline