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Congenital Muscular Dystrophy:Use of Brain MR Imaging to Predict Merosin Deficiency
Radiology 206:811-816, Lamer,S.,et al, 1998
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Article Abstract
Nine patients had merosin deficiency.All patients had diffuse white matter alterations similar to those seen in cases of leukodystrophy. Periventricular and subcortical white matter were involved.The corpus callosum and internal capsule were spared.Follow-up MR images were available in two patients;changes were nonprogressive.White matter signal intensity was normal in the six patients with normal uniform labeling against merosin.Ventricular dilatation and cortical atrophy were observed in both groups.Diffuse white matter changes resembling those seen with leukodystrophy may be a valuable criterion for diagnosis of merosin deficiency in patients with classic congenital muscular dystrophy.
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cerebral cortical atrophy
hydrocephalus
leukodystrophy
merosin
MRI
MRI,abnormal
muscle biopsy
muscular dystrophy
muscular dystrophy,congenital
white matter disease
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