MR of childhood-onset dentatorubral-pallidoluysian atrophy.
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.
Miyazaki
M
M
Department of Pediatrics, Tokushima University School of Medicine, Japan.
Kato
T
T
Hashimoto
T
T
Harada
M
M
Kondo
I
I
Kuroda
Y
Y
D000293:Adolescent; D000328:Adult; D001284:Atrophy / Q000175:diagnosis / Q000235:genetics; D001921:Brain / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male