IN 1964, Steele et al1 focused attention upon "a distinct clinicopathological syndrome" which they termed progressive supranuclear palsy. The disorder was characterized clinically by ophthalmoplegia, mainly affecting vertical, and particularly downward gaze, pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and upper trunk, and by other less constant cerebellar and pyramidal signs. It was emphasized that intellectual impairment was mild. The pathologic picture was one of neuronal loss, gliosis, neurofibrillary tangles, granulovacuolar degeneration, and demyelination which they believed to represent a degenerative disease, affecting chiefly the brain stem, basal ganglia, and cerebellum.
There have been subsequent reports of this disorder, some with minor variation from the complete picture described by Steele et al,1 but always with striking ophthalmoplegia and tonic rigidity as the outstanding clinical features.
The purpose of this report is to present the second case of a woman studied pathologically as well as clinically, and
Blumenthal H, Miller C. Motor Nuclear Involvement in Progressive Supranuclear Palsy. Arch Neurol. 1969;20(4):362–367. doi:10.1001/archneur.1969.00480100038005
Coronavirus Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.