A clinical and pathological study of progressive supranuclear palsy

The clinical features of 26 patients diagnosed as progressive supranuclear palsy are reviewed. The atypical findings were the relatively low frequency of visual complaints (23%) and of significant dementia (20%). As well, the characteristic eye signs, supranuclear ophthalmoplegia of vertical gaze, occurred some years after the onset of the initial symptoms in a small but substantial number (31%), which significantly delayed the diagnosis, in one case by as long as 18 years. The pathological studies on 2 of the clinical cases and an additional 6 cases showed the characteristic pattern of involvement of particular subcortical and brainstem nuclei. In particular, significant degeneration of the pedunculopontine tegmental nucleus was confirmed. Degeneration of the pedunculopontine tegmental nucleus may well play an important role in the motor disability in progressive supranuclear palsy.