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American Journal of Neuroradiology, Vol 16, Issue 9 1834-1836, Copyright © 1995 by American Society of Neuroradiology

ARTICLES

MR of childhood-onset dentatorubral-pallidoluysian atrophy

M Miyazaki, T Kato, T Hashimoto, M Harada, I Kondo and Y Kuroda
Department of Pediatrics, Tokushima University School of Medicine, Japan.

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.


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Copyright © 2008 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X