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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 20:324-326 (2 1999)
© 1999 American Society of Neuroradiology

ARTICLE

MR Imaging Findings in Children with Merosin-Deficient Congenital Muscular Dystrophy

Pilar A. Caro^a, Mena Scavina^,a, Eric Hoffman^a, Elena Pegoraro^a and Harold G. Marks^a

^a From the Division of Neuroimaging, Thomas Jefferson University Medical College, Philadelphia (P.A.C.); the Department of Medical Imaging (P.A.C.) and the Division of Neurology (M.S., H.G.M.), Alfred I. duPont Hospital for Children, Wilmington, DE; and the Departments of Molecular Genetics and Biochemistry, Human Genetics, and Pediatrics, University of Pittsburgh School of Medicine (E.H., E.P.).

BACKGROUND AND PURPOSE: Our purpose was to determine the brain MR imaging characteristics of merosin-deficient congenital muscular dystrophy in children.

METHODS: We reviewed the MR imaging findings of the brain in three children with known merosin-deficient congenital muscular dystrophy to determine the presence of any cerebral or cerebellar abnormalities of development or abnormalities of the white matter.

RESULTS: In all three patients, there was normal formation of the cerebrum, the cerebellum, and no evidence of neuronal migration anomalies. All three patients had abnormal white matter in the cerebrum, with sparing of the corpus callosum, internal capsule, cerebellum, and brain stem.

CONCLUSION: MR imaging of the brain in children with merosin-deficient congenital muscular dystrophy reveals a consistent pattern of white matter abnormality. We postulate that disruption of the blood-brain barrier associated with merosin deficiency leads to increased water content, resulting in abnormal white matter signal intensity.

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