伴精神分裂样或类躁狂抑郁症状的Turner综合征

Clinical analysis of Turner syndrome with schizophrenform or manic depressive symptoms.

为较全面地了解Ｔｕｒｎｅｒ综合征伴精神障碍的临床特征，对１７例患者进行临床分析。躯体表现主要为缺乏女性特征、原发性闭经、乳房发育不良、外阴幼稚型、多伴身材矮小和其它躯体外形异常。肛诊和Ｂ超显示子宫和卵巢发育不良；ＩＱ平均６８．５，其中操作ＩＱ明显为低（平均５７．８），多呈边缘性至中度智力障碍；皮纹学改变主要是猿线出现率增高，ａｔｄ角增大，总指纹嵴数和ａ－ｂ嵴纹明显增加；染色体核型：４５，Ｘ／４６，ＸＸ８例，４６，Ｘｉ（Ｘｑ）和４５，Ｘ／４６，Ｘｉ（Ｘｑ）各２例，４５，Ｘ／４６，ＸＹ１例；仅４例为纯合子４５，Ｘ。１４例呈精神分裂样改变，３例类似躁狂抑郁症状群。精神障碍发生的原因除有其生物学和心理社会学的因素外，智力水平低下可作为促发因素，并对精神障碍的类型和治疗略加讨论，指出注意躯体外型特征和进行遗传学检验有助于鉴别诊断。

In order to know the clinical characterisics of Turner's syndrome with mental disorder overall, we analysed 17 patients we knew. The result of rectoabdominal examination and B type ultrasonic detection showed uterus and overian dysgenesis. The mean score of IQ was 68.5. Performance IQ (average 57.8) was evidently lower than normal. Most of them presented border to moderate intelligence disorder. Dermatoglyphic alteration was mainly promoted frequency of simian line, increased calue of atdangle and increased figure of total finger ridge count and a bridge count. Chromosome karyotype: 45, X/46, XX 8 cases, 46, Xi(Xq) and 45, X/46,Xi(Xq) 2 cases respectively, 45, X/46, XY 1 case, only 4 cases homozygote 46, XX. Mental symptoms in 14 cases were schizophrenform altenation, another 3 cases presented manic depressive symptoms. The mental disorder might be caused by biologic and psychosocialogical factors, and low intelligence might be accelerative factor. The paper has been discussed the type and treatment of mental disorder slightly. It suggests that noting characteristics of body appearance and genetic tests may help the differential diagnosis.