Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease
Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease
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DOI：
10.1136/jnnp.2003.035154
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发表时间：
2005-03-01
影响因子：
11
通讯作者：
Yamada, M
中科院分区：
文献类型：
--
作者：
Ishida, C;Okino, S;Yamada, M
目的：目的：探讨朊蛋白（PrP）在人朊病毒病外周神经系统中的异常沉积。3例为散发性克雅氏病（sCJD），2例为硬脑膜移植相关性克雅氏病（dCJD），1例为Gerstmann-Straussler-Sckeinker病（GSS）伴PrP P102 L突变（GSS 102），2例为P105 L突变（GSS 105）。一个不典型的病例sCJD与PrP斑块在大脑临床表现为周围神经病变，并显示脱髓鞘的腓肠神经的戏弄纤维的12%。结果：1例sCJD和2例dCJD患者的背根神经节部分神经元、周围神经和脊神经后根的少数纤维中可见颗粒状PrP沉积，而GSS 102和GSS 105患者中未见颗粒状PrP沉积。伴周围神经病变的不典型病例在神经内未见明显的PrP沉积。dCJD患者PNS的Western blotting分析显示，在背根神经节和周围nerves.Conclusions：异常的PrP沉积发生在背根神经节和周围神经sCJD和dCJD患者的少量蛋白酶K抵抗PrP。PNS中PrP沉积与CJD周围神经病变的临床表现无关。
Objective: To investigate abnormal prion protein (PrP) deposition in the peripheral nervous system INS) in human prion diseases.Methods: Eight patients with prion diseases were examined: three with sporadic Creutzfeldt-Jakob disease (sCJD), two with dural graft associated CJD (dCJD), one with Gerstmann-Straussler-Sckeinker disease (GSS) with a PrP P102L mutation (GSS102), and two with a P105L mutation (GSS105). An atypical case of sCJD with PrP plaques in the brain presented clinically with peripheral neuropothy, and showed demyelination in 12% of the teased fibres of the sural nerve. The PNS was investigated by immunohistochemical and western blotting analyses of PrP.Results: In immunohistochemical studies, granular PrP deposits were detected in some neurones of dorsal root ganglia and a few fibres of peripheral nerves and spinal posterior roots in one sCJD and two dCJD patients, but not in GSS102 or GSS 105 patients. The atypical case of sCJD with peripheral neuropathy showed no obvious PrP deposition in the nerves. Western blotting analysis of the PNS from the dCJD patients revealed a small amount of protease K resistant PrP in the dorsal root ganglia and peripheral nerves.Conclusions: Abnormal PrP deposition occurs in the dorsal root ganglia and peripheral nerves in sCJD and dCJD. The PrP deposits in the PNS are not correlated with clinical manifestation of peripheral neuropathy in CJD.