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Quantitative magnetic resonance imaging in Rett syndrome
M F Casanova1, S Naidu, T E Goldberg
1Clinical Brain Disorders Branch, National Institute of Mental Health, St. Elizabeths Hospital, Washington, DC 20032, USA.
The Journal of Neuropsychiatry and Clinical Neurosciences
|January 1, 1991
Summary
Rett syndrome (RS) brain imaging reveals distinct structural abnormalities, particularly in the caudate nucleus, differing from autism. This highlights the striatal system's unique involvement in RS.
Area of Science:
- Neuroscience
- Neurology
- Developmental disorders
Background:
- Rett syndrome (RS) is a progressive neurological disorder affecting females.
- Key features include autistic behavior, ataxia, and characteristic handwringing movements.
Purpose of the Study:
- To investigate structural brain abnormalities in Rett syndrome patients using magnetic resonance imaging (MRI).
- To compare these abnormalities with those previously reported in autism spectrum disorder.
Main Methods:
- MRI scans were analyzed for eight female patients with RS and eight age- and sex-matched controls.
- Area and shape measurements were taken for brain hemispheres, corpus callosum, midbrain, pons, cerebellar lobules I-V and VI-VII, and the caudate nucleus head.
Main Results:
- Significant differences in whole brain hemisphere area were observed (p < 0.05).
- Significant differences were found in both the right and left caudate nucleus areas (p < 0.04).
Conclusions:
- Structural brain findings in Rett syndrome differ from those reported in autism.
- These results emphasize the significant involvement of the striatal system in Rett syndrome.