论文部分内容阅读
Huntington’s病是以进行性痴呆和舞蹈为特征的常染色体显性遗传性疾病。发病年龄差别很大,通常好发于21～50岁(平均41岁),平均死亡年龄为54岁。Huntington′s病的表现受双亲中患病者性别影响。在21岁之前发病者,由父亲遗传的占优势,而在晚发的病人中,大部份是由母亲遗传。早发的病例呈强直型,有人报道70例强直型Huntinton’s病平均发病年龄为22.2岁。少年型病人,发病年龄4～10岁(占总数的2%),临床上以强直、运动减少、癫痫和精神衰退为特征,病程较中年发病者轻。青年型(10～20岁发病,占4%)比较接近中年型。50岁以后发病者为晚发型。本文作者对25例平均发病年龄为57.5岁的晚发型病例进行了分析,男12例,女13例,明确诊断时平均为63.1岁。25例均表现有舞蹈、肌张力低下和
Huntington’s disease is an autosomal dominant genetic disorder characterized by progressive dementia and dance. Age varies greatly, usually occurs in 21 to 50 years (mean 41 years), the average age of death was 54 years. The performance of Huntington’s disease is influenced by the gender of the patient in the parent. The onset of the disease before the age of 21 is dominated by the father, while in the late-onset patients, the majority are inherited by the mother. Early cases of ankylosis, it was reported 70 cases of tetanus Huntinton’s disease, the average age of onset was 22.2 years old. Juvenile patients, the age of onset of 4 to 10 years old (2% of the total), clinically characterized by rigidity, decreased exercise, epilepsy and mental decline, the disease course than the incidence of middle age. Youth type (10 to 20 years of age, accounting for 4%) is relatively close to middle-aged. 50-year-old onset of late-onset type. The authors analyzed 25 cases of late-onset cases with an average age of onset of 57.5 years. There were 12 males and 13 females, with an average of 63.1 years of age at the time of diagnosis. 25 cases showed performance of dance, muscle tension and low