J Neuropsychiatry Clin Neurosci 1991; 3:66-72
Copyright © 1991 by American Neuropsychiatric Association
Quantitative magnetic resonance imaging in Rett syndrome
MF Casanova, S Naidu, TE Goldberg, HW Moser, S Khoromi, A Kumar, JE Kleinman and DR Weinberger
Clinical Brain Disorders Branch, National Institute of Mental Health, St. Elizabeths Hospital, Washington, DC 20032, USA.
Rett syndrome (RS) is a progressive neurological disorder of females,
characterized by the early onset of autistic behavior, ataxia, and
"handwringing" movements. The present magnetic resonance imaging study was
undertaken with the purpose of investigating whether structural brain
abnormalities of RS patients are similar to those recently reported in
autism. The subject population consisted of eight patients and an equal
number of age- and sex-matched controls. Area and shape measurements were
taken at selected anatomical levels for the following structures: brain
hemisphere, corpus callosum, midbrain, pons, lobules I-V and VI-VII of the
cerebellum, and head of the caudate. Results revealed significant
differences in area for the whole brain hemisphere (p < 0.05) and in
both right and left caudate (p < 0.04). These morphological findings are
different from those recently reported in autism and emphasize the
involvement of the striatal system in RS.
