title: Neuroimaging study of myotonic dystrophy. I. Magnetic resonance imaging of the brain.
abstract: Magnetic resonance imaging scans of the brain were obtained in 13 patients with myotonic dystrophy, seven with congenital myotonic dystrophy and six with adult-type myotonic dystrophy. All seven patients with congenital myotonic dystrophy had ventriculomegaly and a low IQ (DQ). Cerebral white matter lesions were observed in six cases, a small corpus callosum in four cases, a small brainstem in two cases, and a cerebellar white matter lesion in one case. Cerebral white matter lesions were observed in five of the six cases with adult-type myotonic dystrophy of which one had ventriculomegaly. The IQ (DQ) was significantly lower in patients with congenital myotonic dystrophy than in those with adult-type myotonic dystrophy. The incidence of a small corpus callosum or ventricular enlargement was higher in congenital myotonic dystrophy than in adult-type myotonic dystrophy. These findings may be related to the presence of neurologic impairment in congenital myotonic dystrophy.
authors.affiliation: Department of Pediatrics, University of Tokushima School of Medicine, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
authors.forename: M
authors.initials: M
authors.lastname: Tayama
authors.forename: M
authors.initials: M
authors.lastname: Miyazaki
authors.forename: K
authors.initials: K
authors.lastname: Murakawa
authors.forename: H
authors.initials: H
authors.lastname: Kawai
authors.forename: H
authors.initials: H
authors.lastname: Nishitani
authors.forename: Y
authors.initials: Y
authors.lastname: Kuroda
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology*; D002552:Cerebral Ventricles / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003337:Corpus Callosum / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009223:Myotonic Dystrophy / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology