An investigation of potential neurosurgical transmission of Creutzfeldt-Jakob disease: challenges and lessons learned.

In 2001, New York State health officials were notified about 2 patients with Creutzfeldt-Jakob disease who had undergone neurosurgical procedures at the same hospital within 43 days of each other. One patient had Creutzfeldt-Jakob disease at the time of surgery; the other patient developed Creutzfeldt-Jakob disease 6.5 years later. This investigation highlights the difficulties in assessing possible transmission of Creutzfeldt-Jakob disease.     

Probable Creutzfeldt-Jakob disease after a cadaveric dural graft

A 26-year old female who had received a cervical cadaveric dural graft 7 years earlier developed psychotic behavior and generalized myoclonic jerks. Following rapid deterioration and appearance of high-charge sharp periodic waves on electroencephalography she died within two months. We believe that this patient is the first German who acquired Creutzfeldt-Jakob disease by dural implant.     

Geographic accumulation of Creutzfeldt-Jakob disease in Slovakia--environmental metal imbalance as a possible cofactor

Slovakia is characterised by an unusually high number of patients affected by genetic Creutzfeldt-Jakob disease (CJD) with E200K mutation at the PRNP gene. Penetrance of the mutation is incomplete (59%). Therefore, for the onset of the clinical manifestation, an influence of other endo- or exogenous factors could not be excluded. Experimental data suggest that copper and manganese levels may play an important role in the pathogenesis of prion diseases. The highest number of Slovak genetic CJD patients originates from Orava - the northern region of central Slovakia. Manganese is a dominant pollutant in Orava. The objective of this study was to clarify a possible exogenous influence of environmental Mn/Cu imbalance on the CJD clustering. Mn and Cu levels were analysed in the brain tissue of genetic CJD cases (from Orava and from control regions of Slovakia), as well as of sporadic CJD patients and controls. Analyses demonstrate i) significantly higher Mn level in focally accumulated, "clustering" genetic CJD cases in comparison to all other groups, ii) Cu status differences between compared groups were without statistical significance; decreased concentrations were found in genetic cases from extrafocal genetic CJD areas, iii) Mn/Cu ratios were increased in all CJD groups in comparison to controls. Metal ratios in clustering gCJD cases were significantly higher in comparison to sporadic cases and also to controls, but not to the extrafocal genetic CJD subgroup. These results indicate that more important than increasing Mn level in pathogenesis of CJD appears to be the role of the Mn/Cu imbalance in the CNS. The imbalance observed in the cluster of genetic CJD cases is probably a result of both: the excessive environmental Mn level and the disturbance of Mn/Cu ratios in the Orava region. Presented findings indicate an environmental Mn/Cu imbalance as a possible exogenous CJD risk co-factor which may, in coincidence with endogenous (genetic) CJD risk, contribute to the focal accumulation (cluster) of genetic CJD in Slovakia.     

A case of Creutzfeldt-Jakob disease associated with a dura mater graft in the United States.

We describe a case of Creutzfeldt-Jakob disease associated with a dura mater graft (Lyodura brand) in a 26-year-old man who underwent several neurosurgical procedures as a child. Clinicians and infection control personnel should be aware that recipients of Lyodura brand dura mater grafts processed before May 1987 may remain at increased risk for Creutzfeldt-Jakob disease throughout their lives.     

Disease latency in Creutzfeldt-Jakob disease via dural grafting: a case report

The case of a 57-year old man is reported who had been operated upon for cerebellar angioblastoma more than 19 years previously. Having received a lyophilized dural patch he developed Creutzfeldt–Jakob disease of which he died 19 months later. This is the longest latency with dura-associated Creutzfeldt–Jakob disease reported so far.     

神经外科术后患者医院感染调查分析

神经外科手术后医院感染的发生率很高,治疗上较为困难,也严重影响患者的预后.为了解其发生率及其相关因素,我们对本院2002年神经外科227例手术患者进行了调查分析.结果如下.     

A case of Creutzfeldt-Jakob disease related to familial retinitis pigmentosa patients

Similarities between serological alterations and retinal degeneration occurring in natural and experimental CJD and in some forms of human retinal degeneration have recently been reported. In the present paper a family from an areal focal accumulation of CJD in Central Slovakia with 1 histopathologically verified case of Creutzfeldt-Jakob disease and 2 case of retinitis pigmentosa is described. Neuropathological and epidemiological data obtained in investigated patients are discussed from the point of view of a possible relationship between the slow virus infections caused by unconventional agents and degenerative disease affecting the ocular system.     

Probable variant Creutzfeldt-Jakob disease in a U.S. resident--Florida, 2002

On April 18, 2002, the Florida Department of Health and CDC announced the occurrence of a likely case of variant Creutzfeldt-Jakob disease (vCJD) in a Florida resident aged 22 years. This report documents the investigation of this case and underscores the importance of physicians increasing their suspicion for vCJD in patients presenting with clinical features described in this report who have spent time in areas in which bovine spongiform encephalopathy (BSE) is endemic.     

Computed tomography of Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD), one of the transmissible spongiform encephalopathy, affects gray matter primarily. Degeneration of the white matter is uncommon and when it occurs is usually mild, and limited in distribution. Mizutani separated the "panencephalopathic type" of CJD from the other type, because this panencephalopathic type has extensive white matter changes, that could not be explained simply as secondary to cortical degeneration. This communication discusses six necropsy cases of CJD. In four cases of panencephalopathic type, out of our cases, there were extensive changes of cerebral white matter in addition to severe loss of neurons and hypertrophic astrogliosis in cortex and basal ganglia. Computed tomography demonstrated diffuse low attenuation areas in the white matter with marked atrophy of the cortex, brainstem and cerebellum.     

一起疑似输血传播HIV的分子流行病学研究

目的 采用基于准种分析的HIV暴露后感染溯源检测技术调查一起疑似输血传播HIV案件.方法 采集可疑传播链上3例HIV感染者(编号T1～T3)及作为对照的13例已知HIV感染者或艾滋病患者(编号C1～C13)的血样,共计16份血样.提取血浆RNA后,进行逆转录套式PCR扩增,对扩增成功的PCR产物进行克隆、测序.利用BioEdit 6.0.7和MEGA 4.0软件分析基因序列,计算基因离散率、构建系统进化树.结果 成功获得了13份样品的核酸序列,T1、T2和T3样品中毒株为CRF07_BC重组亚型,与T2和T3来自同一城市的6份对照样品中有5份毒株为CRF07_BC重组亚型,而与T1来自同一城市的4份对照样品中毒株全部为CRF01_AE重组亚型.T1与T2间的平均基因离散率最小(2.0％),随后依次为C12(2.8％)和T3(2.9％)等.系统进化树分析显示,T1、T2、T3和C12的所有克隆聚为一簇,并提示HIV的传播方向是从T3经T2到T1.结论 实验结果支持HIV从T3经T2传播到T1的流行病学调查线索,显示分子流行病学技术可以为HIV暴露后感染溯源调查提供更直接的证据.     

The epidemiology of Creutzfeldt-Jakob disease in Canada: a review of mortality data.

Creutzfeldt-Jakob disease (CJD), and particularly its transmissibility through blood and blood products, has become a focus of concern in Canada. The recent identification of new variant CJD led to a review of the Canadian mortality database to identify any clustering of CJD by age, sex, or geographic location.     

1例疑似职业病的调查与思考

目的调查分析1例疑似职业病不能诊断为职业病的原因。方法对该例疑似职业病发生经过和诊断进行调查分析。结果由于接触苯的时间不够1年,患者所患的"急性早幼粒细胞白血病"不能诊断为职业病,但用人单位有多项违法行为,应承担法律责任。结论作为职业病诊断医生,首先要熟练掌握职业病诊断标准,然后结合其他证据资料,客观、科学地作出职业病诊断。作为用人单位,应贯彻执行《中华人民共和国职业病防治法》,重视做好职业卫生工作。这是保护劳动者身体健康及其相关权益的关键,也是保护用人单位自身权益的关键。     

A case-control study of Creutzfeldt-Jakob disease. Dietary risk factors

The mode of natural transmission of Creutzfeldt-Jakob disease remains unknown. In a case-control study conducted in 1981-1983 to evaluate possible dietary and other sources of the disease, 26 cases were ascertained in the mid-Atlantic region of the United States, 23 of which were obtained from accumulated records of the Laboratory of Central Nervous System Studies of the National Institutes of Health. Controls included 18 family members and 22 hospital-matched individuals (total sample size, 66). An increased consumption among patients was found for roast pork, ham, hot dogs (p less than 0.05), roast lamb, pork chops, smoked pork, and scrapple (p less than 0.1). An excess consumption of rare meat (p less than 0.01) and raw oysters/clams (p less than 0.1) was also reported among the patients. Liver consumption, among organ foods, was greater (p less than 0.1) among the cases. If Creutzfeldt-Jakob disease is acquired through ingestion of foods containing the agent, then the food items identified may be among those which need to be evaluated more intensively. Larger case-control studies with more focused dietary questions are warranted.