MR of childhood-onset dentatorubral-pallidoluysian atrophy |
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Authors: | M Miyazaki T Kato T Hashimoto M Harada I Kondo Y Kuroda |
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Affiliation: | Department of Pediatrics, Tokushima University School of Medicine, Japan. |
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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. |
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