Progressive supranuclear palsy presenting as primary lateral sclerosis

Abstract

We would like to thank Dr. King and his colleagues for their interest in our previously reported atypical progressive supranuclear palsy (PSP) case [1]. They also reported a similar autopsy case of PSP showing an atypical clinical presentation. Their case presented as primary lateral sclerosis (PLS) but lacked gaze palsy, parkinsonism, or dementia. As noted by the authors, the clinical presentation was consistent with that of our atypical PSP case. The pathological features in our case were: (i) evident corticospinal tract degeneration with minimal neuronal loss in the motor cortex, basal ganglia, and substantia nigra, (ii) tau pathology prominent in the posterior portion of the frontal convexity, i.e., the motor cortex, and (iii) relatively mild tau accumulation in the basal ganglia and brain stem nuclei.