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dentatorubralpallidoluysian atrophy


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A syndrome (Haw river syndrome, Naito–Oyanagi disease) of myoclonic epilepsy, dementia, ataxia, and choreoathetosis as a result of degeneration of the dentatorubral and pallidoluysian systems caused by an expanded trinucleotide repeat (CAG repeat) in the gene encoding atrophin-1. As with other trinucleotide repeat disorders, the longer the expansion the earlier the onset and the more severe the effects.

Subjects: Medicine and Health.


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