Abstract |
Magnetic resonance imaging (MRI) abnormalities in the cerebral white matter are a consistent feature of merosin-deficient human congenital muscular dystrophy, a disease caused by a primary defect in the expression of the laminin alpha2 chain of merosin. To investigate the relationship between imaging changes and merosin deficiency we undertook a MRI study in the dy/dy mouse, an animal model for this form of human congenital muscular dystrophy. High resolution in vivo imaging was performed on anaesthetized animals (two homozygous dy/dy mutants and two heterozygous dy/DY controls, aged 2.5 months) in a dedicated 11.7T magnetic resonance imaging scanner. T(1) and T(2) weighted images were normal in all mice and white matter changes were not seen at a stage of maturity when MRI changes are already very striking in human patients. Cerebral MRI abnormalities do not appear to be a feature of dy/dy mice, despite the virtual absence of merosin expression in the dy/dy mouse brain. Possible causes for this absence of MRI changes, and implications for the pathogenesis of the MRI changes in humans are reviewed.
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Authors | D J Dubowitz, J M Tyszka, C A Sewry, R A Moats, M Scadeng, V Dubowitz |
Journal | Neuromuscular disorders : NMD
(Neuromuscul Disord)
Vol. 10
Issue 4-5
Pg. 292-8
(Jun 2000)
ISSN: 0960-8966 [Print] England |
PMID | 10838257
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
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Topics |
- Animals
- Brain
(pathology, physiopathology)
- Disease Models, Animal
- Female
- Humans
- Infant
- Laminin
(deficiency)
- Magnetic Resonance Imaging
- Mice
- Mice, Inbred C57BL
- Mice, Mutant Strains
- Muscular Dystrophies
(genetics, pathology, physiopathology)
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