[Prionic neuroinvasion and cerebellar lesion in Creutzfeldt-Jakob disease]

Examining the histological brain sections of patients with Creutzfeldt-Jakob disease (CJD) showed the accumulation of PrP(CJD) in the cerebellar cortical layers. In nCJD, the prion accumulated mainly in the granular layer in the area of synapses of moss fibers with granular cell dendrites in the cerebellar glomeruli and in the molecular layer in the area of the synapses formed by granular cell axons and great stellate neurons of the granular layer with the dendrites of Purkinje's piriform cells of busket cells and Golgi's cells. PrP(CJD) amyloid plaques were formed in these regions. In sporadic CJD, PrP(CJD) accumulated only in the cerebellar molecular layer in the area of the synapses formed by climbing fibers with the dendrites of Purkinje cells. The findings lead to the conclusion that prion spreads along different nerve fibers through the synapses. The preserved Purkinje cells contacting the plaques suggest that prion is not itself highly neurotoxic.