糖原贮积症一例报告

1　临床资料　患者 ,男 ,14岁 ,因活动后四肢近端肌肉酸痛2年于 2 0 0 1- 10 - 18入上海长征医院神经内科。患者入院前 2年在一次剧烈活动后出现四肢近端肌肉酸痛 ,休息后缓解 ,无发热及无力。之后 ,每于剧烈活动后即出现四肢肌肉酸痛 ,伴有痉挛性疼痛 ,活动范围受限。当地医院查肌电图未见异常 ,未治疗。 4个月前 ,在持续半小时用力蹬车后出现双大腿剧痛 ,不能活动 ,对症药物治疗 1周后恢复。 1个月前在较长距离负重行走后再次出现双大腿部肌肉剧痛 ,伴无力 ,登楼梯及下蹲起立时明显 ,外院治疗无效 ,疼痛加重而入院。否认特殊个人史、家族…     

首发偏执型精神分裂症患者执行功能研究

目的 探讨偏执型精神分裂症患者执行功能障碍与精神症状、注意、记忆及预后的关系。方法 对80例首次发病的偏执型精神分裂症患者采用威斯康星卡片分类测验（WCST）评估执行功能，数字划销测验（CT）评估注意功能，修订韦氏成人记忆量表（WMS—RC）评估记忆功能，PANSS量表评估精神症状。分别将治疗前WCST成绩与精神症状、净分、失误率、记忆商做相关分析；将治疗前WCST成绩与治疗6周后精神症状的减分值做相关分析。结果 WCST的总测验次数、持续错误数、随机错误数均与阴性症状评分呈显著正相关（r分别为0．299，0．398，0．31；P〈0．01）。与数字划销测验的净分呈显著负相关（r分别为0．38，0．35，0．31；P〈0．01），正确数、分类完成数均与净分呈显著正相关（r分别为0．23，0．26；P〈0．05）；总测验次数、随机错误数均与记忆商呈显著负相关（r分别为0．29，0．28；P〈0．01或P〈0．05）．治疗前持续错误数与阴性症状的减分值呈显著负相关（r=-0．225，P〈0．05）。结论 偏执型精神分裂症执行功能障碍与阴性症状的严重程度及预后相联系．执行功能障碍可能是其他认知缺陷如注意、记忆及某些精神症状的产生机制。     

首发偏执型精神分裂症患者执行功能研究

目的探讨偏执型精神分裂症患者执行功能障碍与精神症状、注意、记忆及预后的关系.方法对80例首次发病的偏执型精神分裂症患者采用威斯康星卡片分类测验(WCST)评估执行功能,数字划销测验(CT)评估注意功能,修订韦氏成人记忆量表(WMS-RC)评估记忆功能,PANSS量表评估精神症状.分别将治疗前WCST成绩与精神症状、净分、失误率、记忆商做相关分析;将治疗前WCST成绩与治疗6周后精神症状的减分值做相关分析.结果WCST的总测验次数、持续错误数、随机错误数均与阴性症状评分呈显著正相关(r分别为0.299,0.398,0.31;P＜0.01).与数字划销测验的净分呈显著负相关(r分别为0.38,0.35,0.31;P＜0.01),正确数、分类完成数均与净分呈显著正相关(r分别为0.23,0.26;P＜0.05);总测验次数、随机错误数均与记忆商呈显著负相关(r分别为0.29,0.28;P＜0.01或P＜0.05),治疗前持续错误数与阴性症状的减分值呈显著负相关(r=-0.225,P＜0.05).结论偏执型精神分裂症执行功能障碍与阴性症状的严重程度及预后相联系,执行功能障碍可能是其他认知缺陷如注意、记忆及某些精神症状的产生机制.     

社区重性精神疾病患者个案管理效果分析

目的:探讨社区重性精神疾病患者实施个案管理与基础管理的效果差异。方法:选取40例重性精神疾病患者,随机分为个案组和对照组,每组各20例。个案组结合患者情况制定个性化方案,予以综合干预措施;对照组仅维持基础管理。采用阳性与阴性症状量表、日常生活能力量表、社会功能缺陷量表评定治疗效果。结果:入组前两组SDSS、ADL及PANSS评分差异比较均无统计学意义(P>0.05);经过一年的治疗后,两组的评分均低于治疗前,且SDSS和PANSS评分组间比较具有统计学意义(P<0.05)。结论:在社区开展个案管理能够有效地改善患者的精神症状,提高社会功能,促进病情的好转。     

A functional psychosis precipitated by quinidine

A 62-year-old man with a syndrome of gradually progressive cognitive deterioration accompanied by paranoid features is described. He had been taking quinidine since 1974 for a recurring supraventricular tachyarrhythmia. Examination revealed a suspicious man with widespread patchy cognitive deficits but no focal neurological signs. An exhaustive range of investigations gave unremarkable results. Within 24 hours of cessation of quinidine there was a dramatic improvement in his mental state and, after a further four days, he had returned essentially to normal with no demonstrable cognitive deficits. Several months later he suffered a recrudescence with prominent paranoid and depressive features which gradually settled after commencement of pimozide and dothiepin. It is likely that these events reflect a recurring functional psychosis which was precipitated or exacerbated in the first instance by quinidine. This represents a significant complication of quinidine therapy which has been largely unrecognized. Therapy with quinidine should be considered as a potential contributing factor in any patient with dementia or a functional psychosis who is also taking this drug.     

A case of Klinefelter’s syndrome with type 1 diabetes mellitus

Klinefelter’s syndrome (KS) is the most common sex chromosome disease in men. Classical features of the syndrome include a eunuchoidal body habitus, small testes and hypergonadotrophic hypogonadism. There has been an increased risk of diabetes mellitus and autoimmune disease for KS patients. This paper reports a case of KS in association with type 1 diabetes mellitus. The patient was a 21-year-old man, who has been confirmed by absolute insulin deficiency and positive IA-2 autoantibody. The hyperinsulinemic euglycemic clamp test indicated his insulin sensitivity in normal range, and his blood glucose was controlled well by the insulin therapy.

     

利培酮对首发偏执型分裂症患者血糖及血脂代谢的影响

目的探讨利培酮对首发偏执型精神分裂症患者糖脂代谢的影响。方法 2009年1月～2012年1月入院给予利培酮治疗的首发偏执型精神分裂症患者169例,口服利培酮单药治疗8周后测定血糖、胆固醇、三酰甘油浓度。结果经8周利培酮治疗后,①血糖水平没有变化（t=1.37,P=0.21）,男女之间血糖变化无差异（t=1.55,P=0.41）;②血胆固醇水平没有变化（t=1.02,P=0.39）,男女之间血糖变化无差异（t=1.64,P=0.11）;③患者总体上血三酰甘油变化无差异（t=1.28,P=0.27）,但进一步将男女性别分组后发现,利培酮对于男女患者三酰甘油水平的影响存在显著差异（t=3.07,P=0.02）,女性组患者三酰甘油水平无变化,男性患者组三酰甘油升高显著。结论利培酮急性治疗期对于首发偏执型分裂症患者空腹血糖水平及胆固醇无影响,但男性首发偏执型分裂症患者三酰甘油水平升高。