Atypical form of dural graft associated Creutzfeldt-Jakob disease: report of a postmortem case with review of the literature

被引:25
作者
Kimura, K
Nonaka, A
Tashiro, H
Yaginuma, M
Shimokawa, R
Okeda, R
Yamada, M
机构
[1] Kanazawa Univ, Sch Med, Dept Neurol, Kanazawa, Ishikawa 9208640, Japan
[2] Tokyo Metropolitan Bokutoh Gen Hosp, Dept Infect Dis, Tokyo, Japan
[3] Tokyo Med & Dent Univ, Dept Internal Med, Tokyo 113, Japan
[4] Tokyo Med & Dent Univ, Dept Neuropathol, Med Res Inst, Tokyo 113, Japan
关键词
cadaveric dura mater graft; Creutzfeldt-Jakob disease; prion protein; plaque;
D O I
10.1136/jnnp.70.5.696
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
A postmortem case of an atypical form of dural graft associated Creutzfeldt-Jakob disease (CJD) is described. A 42 gear old man developed progressive spastic paresis 163 months after a cadaveric dura mater graft. He presented with no myroclonus and very late occurrence of periodic synchronous discharges on EEG. The prion protein (PrP) gene was homozygous for methionine at the polymorphic codon 129. Neuropathological examination disclosed plaque-like PrP deposits with atypical distribution elf synaptic PrP accumulations in the brain. This patient represents an atypical form of dural graft associated CJD characterised by unusual clinicopathological features.
引用
收藏
页码:696 / 699
页数:4
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