Unusual paired helical filaments in progressive supranuclear palsy

Unusual paired helical filaments (PHF) coexisting with single filaments were observed in neurofibrillary degeneration (NFD) in the globus pallidus, subthalamic nucleus, substantia nigra, and pontine tegmentum of a typical case of progressive supra-nuclear palsy (PSP). Each filament had a diameter of 10-12 nm and showed central low density and a smooth contour. The thickest portion of a pair was 22-24 nm in diameter. The periodicity of twist varied from 150 nm to 300 nm, but each PHF had regular periodicity. The present ultrastructural finding is unusual in the neurofibrillary pathology of PSP, and is also different from the PHF found in Alzheimer type NFD.