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Huntington氏病(HD)的发病年龄为30~50岁左右,10岁以前发病者约占1~3%。现报告一对5岁前起病的单卵双生兄弟。 患者兄弟7岁,为第三胎足月顺产,出生时为一个胎盘。4岁以前生长发育正常。4岁后两兄弟同时起病,病情基本相同,最初表现为动作变慢,步态笨拙,行走时常向后跌倒,呈进行性加重。全身有肌肉抽动,持物易失落。近1年出现发作性全身抽搐,言语不清,吞咽缓慢,流涎。由于频繁地跌倒摔伤,被限制活动达8个月之后,皆丧失了自主活动能力。
The age of onset of Huntington’s disease (HD) is about 30 to 50 years old, and the incidence of Huntington’s disease is about 1 to 3% before the age of 10. Now report a pair of monozygotic twins who became ill before the age of 5. The patient’s brother, 7 years old, is full-term for the third birth and is a placenta at birth. 4 years old before normal growth and development. After the age of 4, the two brothers started their illness at the same time and their condition was basically the same. The initial performance was slow movement, gait clumsiness, and backward walking during walking, showing a progressive increase. The whole body has muscle twitching, holding things easy to lose. Nearly 1 years of paroxysmal generalized convulsions, vague words, swallow slowly, salivation. Due to frequent fall falls, were limited to activities up to 8 months after the loss of autonomy.