Herbert Budka

TL;DR: The results suggest that the ubiquitin-proteasome system takes part in the pathogenesis of neurodegeneration and possibly plays a role in protecting certain neuronal populations in CJD and AD.

TL;DR: A patient with VPSPr is presented who received doxycycline and survived for an extended period of time in an akinetic and mute state and is linked to human prion diseases, which are uniformly fatal disorders.

TL;DR: Although the central nervous system is the major site of PrP(d) accumulation, it may be observed in peripheral nerves as adaxonal deposits; in skeletal muscle as granular immunoreactivity in particular in abundance in a unique instance of concomitant Creutzfeldt-Jakob disease and inclusion body myositis; as well as associated with dendritic cells and macrophages in vessel walls.

TL;DR: In this article, 6 autopsy cases of primary leptomeningeal sarcomatosis are presented as a distinct nosological entity with a variable clinical picture and morphology in 5 males and 1 female.

TL;DR: There is an inverse correlation between the proportion of neurons with INIR and the intensity of disease-associated PrP immunoreactivity and severity of lesions, and large intracytoplasmic inclusion-like bodies in ballooned neurons in PrD cases are found.