title: MR of childhood-onset dentatorubral-pallidoluysian atrophy.
abstract: MR findings in a 14-year-old boy with progressive myoclonic epilepsy, who was diagnosed as having dentatorubral-pallidoluysian atrophy by DNA analysis, were compared with those of his father, who had adult-onset dentatorubral-pallidoluysian atrophy. Besides showing severe brain atrophy, especially of the brain stem tegmentum and cerebellum, MR showed diffuse periventricular hyperintensity on T2-weighted images. As compared with the proband, the father had a mild case.
authors.affiliation: Department of Pediatrics, Tokushima University School of Medicine, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Miyazaki
authors.forename: T
authors.initials: T
authors.lastname: Kato
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
authors.forename: M
authors.initials: M
authors.lastname: Harada
authors.forename: I
authors.initials: I
authors.lastname: Kondo
authors.forename: Y
authors.initials: Y
authors.lastname: Kuroda
mesh_terms: D000293:Adolescent; D000328:Adult; D001284:Atrophy / Q000175:diagnosis / Q000235:genetics; D001921:Brain / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male