Creutzfeldt-Jakob disease with florid plaques after cadaveric dura mater graft
Creutzfeldt-Jakob disease with florid plaques after cadaveric dura mater graft
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DOI：
10.1046/j.1440-1789.2003.00489.x
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发表时间：
2003-06-01
期刊：
影响因子：
2.3
通讯作者：
Kitamoto, T
中科院分区：
文献类型：
--
作者：
Mochizuki, Y;Mizutani, M;Kitamoto, T
本文报告一例硬脑膜相关性克雅氏病（D-CJD）患者，在硬脑膜移植后约15年发生。朊蛋白基因分析未发现突变。D-CJD在以下方面是非典型的：（i）共济失调发作和痴呆发生之间的长时间间隔;（ii）短暂性肌阵挛的存在;和（iii）大脑中的花斑的存在。电镜下可见成束的淀粉样纤维从斑块中心放射状分布，神经突起不变性。这例D-CJD可能属于D-CJD的一个新亚型。
A patient with dura-associated Creutzfeldt-Jakob disease (D-CJD) which occurred about 15 years after a dura mater graft is reported in the present study. The prion protein gene analysis disclosed no mutation. The D-CJD was atypical in: (i), the long interval between the onset of ataxia and the occurrence of dementia; (ii), the presence of transient myoclonus; and (iii), the presence of florid plaques in the brain. The electron-microscopic findings showed bundles of amyloid filaments which radiated from the center of the plaques without degenerating neurites. This case of D-CJD may belong to a new subtype of D-CJD.