临床诊断的克罗伊茨费尔特-雅各布病

克罗伊茨费尔特 雅各布病 (Creutzfeldt Jakobdisease,CJD)是最常见的人类海绵状脑病 ,年发病率约为 1/ 10 0万 ,由于该病的确诊需要脑病理资料 ,普遍认为其确切的发病率要高于目前的报道。 1995年以来 ,疯牛病和新人类海绵状脑病的不断报道 ,再次引起人们对CJD的关注 ,并掀起     

影像表现异常的人生长激素相关医源性克罗伊茨费尔特-雅各布病

背景：尽管证实有超过160例源于人生长激素（hGH）治疗的医源性克罗伊茨费尔特-雅各布病（iCJD），但据悉磁共振成像（MRI）检查异常的小脑病变尚无报道。目的：报道l例液体衰减反转恢复（FLAIR）和弥散加权MRI（DWI）检查显示异常小脑MRI表现的hGH相关iCJD患者。设计：病例报道。机构：1所大学医疗中心的神经科门诊患者。患者：1例33岁的亚急性步态共济失调和视物模糊男性患者。结果：患者19年前接受了至少5年的从尸体垂体提取的hGH治疗。MRI显示FLAIR和DWI表现异常，尤其小脑的表现异常。该患者在发病7个月后死于尸检证实的iCJD，患者的病理改变在很大程度上与MRI表现相符。结论：据知，这是第1例伴有小脑FLAIR和DWI检查异常的hGH源性iCJD的病例报道。在iCJD，小脑引发的症状发生较早，因而提示上述MRI改变有助于早期诊断该类朊蛋白病。     

Long-term efficacy of BCG vaccine in American Indians and Alaska Natives: A 60-year follow-up study

Context  The duration of protection from tuberculosis of BCG vaccines is not known. Objective  To determine the long-term duration of protection of a BCG vaccine that was previously found to be efficacious. Design  Retrospective record review using Indian Health Service records, tuberculosis registries, death certificates, and supplemental interviews with trial participants. Setting and Participants  Follow-up for the period 1948-1998 among American Indians and Alaska Natives who participated in a placebo-controlled BCG vaccine trial during 1935-1938 and who were still at risk of developing tuberculosis. Data from 1483 participants in the BCG vaccine group and 1309 in the placebo group were analyzed. Main Outcome Measures  Efficacy of BCG vaccine, calculated for each 10-year interval using a Cox regression model with time-dependent variables based on tuberculosis events occurring after December 31, 1947 (end of prospective case finding). Results  The overall incidence of tuberculosis was 66 and 138 cases per 100 000 person-years in the BCG vaccine and placebo groups, respectively, for an estimate of vaccine efficacy of 52% (95% confidence interval, 27%-69%). Adjustments for age at vaccination, tribe, subsequent BCG vaccination, chronic medical illness, isoniazid use, and bacille Calmette-Guérin strain did not substantially affect vaccine efficacy. There was slight but not statistically significant waning of the efficacy of BCG vaccination over time, greater among men than women. Conclusion  In this trial, BCG vaccine efficacy persisted for 50 to 60 years, suggesting that a single dose of an effective BCG vaccine can have a long duration of protection.      

Creutzfeldt-Jakob disease in pituitary growth hormone recipients in the United States

To assess the magnitude of Creutzfeldt-Jakob disease (CJD) occurrence among recipients of pituitary-derived human growth hormone (HGH), we conducted an epidemiologic follow-up of 6284 recipients of HGH distributed through the National Hormone and Pituitary Program. Seven neuropathologically confirmed cases of CJD have occurred in this population to date: six patients with clinical CJD presented with ataxia and imbalance, rather than with altered mentation, which is the most common initial manifestation in sporadic CJD, and one patient died in the preclinical incubation state of the disease. All seven cases occurred among the nearly 700 HGH recipients who started therapy before 1970. Since only 10% of the cohort has been followed up for the 15-year average incubation interval from midpoint of HGH treatment to onset of symptoms, the great majority of potentially exposed patients have not yet attained the requisite incubation period for expression of CJD. The median duration of HGH therapy of 100 months in the CJD cases was significantly longer than 41 months for all patients starting treatment before 1970; thus, the duration of pituitary HGH therapy is a major risk factor for CJD.