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AJNR - American Journal of Neuroradiology

Published ahead of print on December 7, 2007
doi: 10.3174/ajnr.A0857

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Selective Cerebral Volume Reduction in Rett Syndrome: A Multiple-Approach MR Imaging Study

J.C. Carter^a, D.C. Lanham^b, D. Pham^d, G. Bibat^c,f, S. Naidu^c,e,f and W.E. Kaufmann^a,d,e,f,g,h

^a Center for Genetic Disorders of Cognition and Behavior, Kennedy Krieger Institute, Baltimore, Md
^b Department of Developmental Cognitive Neurology, Kennedy Krieger Institute, Baltimore, Md
^c Neurogenetics Unit, Kennedy Krieger Institute, Baltimore, Md
^d Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, Md
^e Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Md
^f Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Md
^g Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Md
^h Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Md

View larger version (41K): [in this window] [in a new window]   Fig 1. Concordance of Talairach and voxel-based findings in the posterior dorsal parietal lobe in RTT versus control subjects. Talairach-derived data (top row) show preferential bilateral reduction of GM in the posterior dorsal parietal lobe (outlined; only left side shown), corresponding with Brodmann areas 5 and 7. Similarly, VBM-derived data (bottom row) show bilateral GM volume reduction in the posterior dorsal parietal region (outlined), roughly corresponding with Brodmann area 7 (left hemisphere differences are depicted here).

View larger version (20K): [in this window] [in a new window]   Fig 2. Concordance of Talairach and voxel-based findings in the prefrontal GM in more severe versus less severe RTT subjects. Girls with less severe clinical presentation, according to gait abnormality criterion, showed relative preservation of prefrontal GM volumes, corresponding roughly with Brodmann areas 10/46. A, Top left depicts Talairach-derived data, whereas bottom left shows VBM-derived data. B, Boxplots (percentiles) of Talairach-based prefrontal GM volumes illustrate that girls with less severe clinical presentation show volumes intermediate between control subjects and those with more severe phenotype.

View larger version (26K): [in this window] [in a new window]   Fig 3. Cerebral lobar GM volumes in monozygotic twins discordant for the RTT phenotype. Note that the unaffected twin showed frontal, parietal, and temporal GM volumes within the normal control range (depicted as mean +1 SD), whereas the affected twin had smaller volumes in these regions, with the exception of the occipital GM volume, which was at nearly identical levels in both twins and only slightly below the normal control range.