1例28岁医源性克罗伊茨费尔特-雅各布病患者脑膜移植后的阿尔茨海默型神经病理学研究  

Alzheimer-type neuropathology in a 28 year old patient with iatrogenic Creutzfeldt-Jakob disease after dural grafting

作　　者：Preusser M. Strbel T. Gelpi E. H. Budka 王鹏 

机构地区：[1]Institute of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 1097 Vienna, Austria Dr.

出　　处：《世界核心医学期刊文摘（神经病学分册）》2006年第6期46-47,共2页Digest of the World Core Medical Journals:Clinical Neurology

摘　　要：We report the case of a 28 year old man who had received a cadaverous dura mater graft after a traumatic open skull fracture with tearing of the dura at the age of 5 years. A clinical suspicion of Creutzfeldt-Jakob disease (CJD) was confirmed by a brain biopsy 5 months prior to death and by autopsy, thus warranting the diagnosis of iatrogenic CJD (iCJD) according to WHO criteria. Immunohistochemistry showed widespread cortical depositions of disease associated prion protei n (PrPSC)in a synoptic pattern, and western blot analysis identified PrPSC of type 2A according to Parchi et al. Surprisingly, we found Alzheimer-type senile plaques and cerebral amyloid angiopathy in widespread areas of the brain. Plaque-type and vascular amyloid was immunohistochemically identified as deposits of beta-A4 peptide. CERAD criteria for diagnosis of definite Alzheimer’s disease (AD) were met in the absence of neurofibrillar tangles or alpha-synuclein immunoreactive inclusions. There was no family history of AD, CJD, or any other neurological disease, and genetic analysis showed no disease specific mutations of the prion protein, presenilin 1 and 2, or amyloid precursor protein genes. This case represents (a) the iCJD case with the longest incubation time after durai grafting reported so far, (b) the youngest documented patient with concomitant CJD and Alzheimer-type neuropathology to date, (c) the first description of Alzheimer-type changes in iCJD, and (d) the second case of iCJD in Austria. Despite the young patient age, the Alzheimer-type changes may be an incidental finding, possibly related to the childhood trauma.We report the case of a 28 year old man who had received a cadaverous dura mater graft after a traumatic open skull fracture with tearing of the dura at the age of 5 years. A clinical suspicion of Creutzfeldt-Jakob disease （CJD） was confirmed by a brain biopsy 5 months prior to death and by autopsy, thus warranting the diagnosis of iatrogenic CJD （iCJD） according to WHO criteria. Immunohistochemistry showed widespread cortical depositions of disease associated prion protein （PrP^sc） in a synoptic pattern, and western blot analysis identified PrP^sc of type 2A according to Parchi et al. Surprisingly, we found Alzheimer-type senile plaques and cerebral amyloid angiopathy in widespread areas of the brain. Plaque-type and vascular amyloid was immunohistochemically identified as deposits of beta-A4 peptide. CERAD criteria for diagnosis of definite Alzheimer＇ s neurofibrillar disease tangles （AD） were met in the absence of or alpha-synuclein immunoreactive inclusions. There was no family history of AD, CJD, or any other neurological disease, and genetic analysis showed no disease specific mutations of the prion protein, presenilin 1 and 2, or amyloid precursor protein genes. This case represents （a） the iCJD case with the longest incubation time after durai grafting reported so far, （b） the youngest documented patient with concomitant CJD and Alzheimer-type neuropathology to date, （c） the first description of Alzheimer-type changes in iCJD, and （d） the second case of iCJD in Austria. Despite the young patient age, the Alzheimer-type changes may be an incidental finding, possibly related to the childhood trauma.

关 键 词：克罗伊茨费尔特-雅各布病 阿尔茨海默病 硬脑膜移植 神经纤维缠结 男性患者 移植后 医源性 病理学研究 WHO诊断标准 神经病理学改变 

分 类 号：R51[医药卫生—内科学] R749.16[医药卫生—临床医学]