Immunohistochemical study of a case with progressive supranuclear palsy without ophthalmoplegia.

A case of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome; PSP) with parkinsonism and absence of gaze palsy or mental changes is reported. Neuropathological examination, apart from typical changes, showed, lack of midbrain tegmentum demyelination, marked loss of Purkinje cells and presence of hyalin-like bodies in individual neurons of the substantia nigra. Immunostaining against tau-1 protein revealed the prevalence of a diffuse reaction in locus coeruleus neurons; reflecting either different ability of these cells to accumulate straight filaments, or a various time sequence of neurofibrillary tangles formation. Ferritin immunohistochemistry demonstrated widespread microglial cell proliferation, confirming further the generalized character of CNS pathology in PSP.