Age-dependence and Clinical Correlates of Brain Metabolism in Rett Syndrome
Farage L, Izbudak I, Nagae-Poetscher L, Bibat G, Kaufmann W, Barker P, Naidu S, Horska A
Jonhs Hopkins Medical Institutions
Thirty-five girls with a neurodevelopmental disorder of Rett Syndrome (RTT) and eight healthy girls were examined with single voxel proton MR spectroscopy at short TE. Spectra were acquired from the left frontal white matter. Compared to controls, a 10% lower NAA/Cr and a 174% higher mI/Cr was detected in RTT. NAA/Cr decreased and mI/Cr increased with age in RTT while both ratios were stable in controls. Age-related changes of NAA/Cr and mI/Cr in RTT are suggestive of progressive axonal damage with accompanying gliosis.