儿童期局限于纹状体的变性是罕见的,它构成了一个诊断问题。少年期Huntington舞蹈病的患儿证实有纹状体变性,然而损害通常并不局限此部位。纹状体坏死的病例也有报导。本文报导3例非亲缘关系的儿童患进行性纹状体变性。病例报导病例1:女孩,第一胎,双亲白种人,身体健康,无亲缘关系。妊娠分娩均正常,足月顺产,出生体重3.02kg。无神经疾病家族史,其父亲系养子,不知其详细家族史。父亲以往身体健康,约在患儿死后1年,逐渐发生舞蹈病 Degeneration of the striatum in childhood is rare and constitutes a diagnostic problem. Children with juvenile Huntington’s disease have evidence of striatal degeneration, although damage is usually not limited to this site. There are also reports of striatal necrosis. This article reports on 3 unrelated children with progressive striatum degeneration. Case Report Case 1: Girl, First Child, Parents White, Healthy, Unrelated. Pregnancy and childbirth were normal, term full-term birth, birth weight 3.02kg. No family history of neurological diseases, his father’s Department of Fetus, I do not know their detailed family history. In the past, my father was in good health. About 1 year after his death, he gradually developed chorea