硬脑膜移植相关克-雅病等朊蛋白病的周围神经受累

Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease

Objective: To investigate abnormal prion protein (PrP) deposition in the perip heral nervous system (PNS) in human prion diseases. Methods: Eight patients with prion diseases were examined:three with sporadic Creutzfeld-Ja- kob disease (sCJD),two with dural graft associated CJD (dCJD), one with Gerstm ann-Strussler-Scheinker disease (GSS) with a PrP P102L mutation (GSS102), an d two with a P105L mutation (GSS105).An atypical case of sCJD with PrP plaques i n the brain presented clinically with peripheral neuropathy, and showed demyelin ation in 12%of the teased fibres of the sural nerve. The PNS was investigated b y immunohistochemical and western blotting analyses of PrP. Results: In immunohi stochemical studies,granular PrP deposits were detected in some neurones of dors al 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 GSS105 patients. The at ypical case of sCJD with peripheral neuropathy showed no obvious PrP deposition in the nerves.Western blotting analysis of the PNS from the dCJD patients reveal ed a small amount of protease K resistant PrPin the dorsal root ganglia and peri pheral nerves. Conclusions:Abnormal PrP deposition occurs in the dorsal root gan glia and peripheral nerves in sCJD and dCJD. The PrP deposits in the PNS are not correlated with clinical manifestation of peripheral neuropathy in CJD.