PROTON MAGNETIC-RESONANCE SPECTROSCOPY OF THE BRAIN IN PATIENTS WITH PRADER-WILLI-SYNDROME

Five patients with Prader-Willi syndrome underwent magnetic resonance imaging and proton magnetic resonance spectroscopy. Magnetic resonance images revealed mild abnormalities, including slight ventrifulomegaly , cortical atrophy, and a small brainstem, ill all cases, The N-acetyl aspartate/Choline (NAA/Cho) and N-acetylaspartate/Creatine (NAA/Cr) ra tios were decreased in one (Case 1) and two (Cases 1 and 4) patients, respectively. The Choline/Creatine (Cho/Cr) ratio did not differ from those in control subjects, Thus, in patients with Prader-Willi syndrom e, it is thought that there may be neuron loss or a neuron dysfunction caused by a chromosome abnormality. Statistically significant relatio nships were observed between IQ (DQ and the NAA/Cho and Cho/Cr ratios: (P = 0.895 (r < .05, NAA/Cho ratio and r = -0.898 (P < .05, Cho/Cr ra tio), This suggests that the parietal lobe pathology detected on H-1-m agnetic resonance spectroscopy may be associated with more global brai n damage and with loss of cognitive functions. (C) 1998 by Elsevier Sc ience Inc. All rights reserved.