PROTON MR SPECTROSCOPY OF CHILDHOOD ADRENOLEUKODYSTROPHY

PURPOSE: To determine the potential of proton MR spectroscopy to monit or patients with childhood-onset cerebral adrenoleukodystrophy (COCALD ). METHODS: Single-voxel MR spectroscopy was performed in 16 children with COCALD (24 examinations) who had had no treatment and in 7 childr en (13 examinations) who had had bone marrow transplantation. RESULTS: In the untreated children with clinically active COCALD, the metaboli te ratios N-acetyl-aspartate (NAA)/creatine (Cr) and NAA/choline (Ch) were decreased while Ch/Cr was increased. This trend agrees well with those reported by other researchers, although different experimental s equences and parameters were used in our study. Comparison of these ra tios with those from a control group yielded significant differences i n the occipital region. In the children who were clinically stable aft er bone marrow transplantation, the mean levels of the three ratios we re between those of the control subjects and the patients with untreat ed COCALD: the differences in these ratios approached significance. In patients who had been monitored periodically, MR spectroscopy metabol ite ratios correlated well with the dementia rating score, reflecting clinical status. CONCLUSION: There is good correlation between MR spec troscopy metabolite ratios and a patient's clinical status. MR spectro scopy appears to be a useful, noninvasive tool to monitor patients wit h adrenoleukodystrophy, and it increases the overall sensitivity of MR techniques in clinical applications.