Rett 氏综合征(RS)极为罕见,我科收院1例,报告如下:患儿女,6岁。出生后4个月不能抬头及转颈,6个月不会翻身,8个月不能独坐。1岁多尚不能独站,扶起时双足稍内翻。不会讲话,不认亲人,双手不能持物,每遇到寒冷时右足发紫、发凉。饮水时呛咳。2岁时出现婴儿痉挛症样发作(点头)。5岁自动消失。每当发热(>38℃)伴 Rett’s syndrome (RS) is extremely rare, our hospital admission in 1 case, the report is as follows: Children, 6 years old. 4 months after birth can not rise and neck, 6 months will not stand up, 8 months can not sit alone. 1 year old still can not stand alone, raised his feet slightly varus. Will not speak, do not recognize their loved ones, hands can not hold things, each encounter a cold right foot purple, cold. Choke when drinking water. Infantile spasm onset at age 2 (nodding). 5 years old disappear automatically. Whenever fever (> 38 ℃) with