Brain Developmental Abnormalities in Prader-Willi Syndrome Detected by Diffusion Tensor Imaging
- 1 August 2006
- journal article
- Published by American Academy of Pediatrics (AAP) in Pediatrics
- Vol. 118 (2), e442-e448
- https://doi.org/10.1542/peds.2006-0637
Abstract
OBJECTIVE. The purpose of this work was to detect brain developmental abnormalities in Prader-Willi syndrome by using diffusion tensor imaging based on a high-field MRI system.METHODS. Eight patients with Prader-Willi syndrome and 8 age- and gender-matched normal control subjects were examined using a high-field (3.0 T) MRI system. Trace value and fractional anisotropy were assessed simultaneously in multiple representative brain regions: the deep gray matter (putamen, caudate head, and dorsomedial thalamus) and the white matter structures (frontal and parietal white matter, posterior limb of internal capsule, and corpus callosum).RESULTS. In Prader-Willi syndrome patients, trace value was found to be significantly higher in the left frontal white matter and the left dorsomedial thalamus, whereas fractional anisotropy was significantly reduced in the posterior limb of the internal capsule bilaterally, the right frontal white matter, and the splenium of the corpus callosum. The observed diffusivity characteristics indicate developmental abnormalities in these areas, which are highly consistent with the clinical features of Prader-Willi syndrome.CONCLUSIONS. The study provides the first objective evidence that Prader-Willi syndrome patients indeed have developmental abnormalities in specific areas of the brain, providing a new window toward understanding the pathophysiology of Prader-Willi syndrome.Keywords
This publication has 39 references indexed in Scilit:
- Less white matter concentration in autism: 2D voxel-based morphometryNeuroImage, 2004
- Diffusion tensor MRI of early upper motor neuron involvement in amyotrophic lateral sclerosisBrain, 2004
- Population prevalence and estimated birth incidence and mortality rate for people with Prader-Willi syndrome in one UK Health RegionJournal of Medical Genetics, 2001
- Clinical application of three-dimensional anisotropy contrast magnetic resonance axonographyJournal of Neurosurgery, 1999
- Growth Hormone Secretion and Effects of Growth Hormone Therapy on Growth Velocity and Weight Gain in Children with Prader-Willi SyndromeJournal of Pediatric Endocrinology and Metabolism, 1996
- PRADER‐WILLI SYNDROME IN A SWEDISH RURAL COUNTY: EPIDEMIOLOGICAL ASPECTSDevelopmental Medicine and Child Neurology, 1991
- Genetic imprinting suggested by maternal heterodisomy in non-deletion Prader-Willi syndromeNature, 1989
- The Prader-Willi SyndromeMedicine, 1983
- Deletions of Chromosome 15 as a Cause of the Prader–Willi SyndromeNew England Journal of Medicine, 1981
- FLUID COMPARTMENTATION AND ELECTROLYTES OF CAT CEREBRAL CORTEX IN VITRO?III ONTOGENETIC AND COMPARATIVE ASPECTSJournal of Neurochemistry, 1966