A 60 year old man presents to a physician because of difficulty in reading and coming down stairs, which he attributes to an inability to "look down." Physical examination reveals that the patient looks around by moving his head rather than his eyes and also shows a distinctive axial rigidity of neck, trunk, and proximal limb muscles. He shows povey of movement and dysahric speech. Mentally, the patient responds very slowly but has better memory and intellect than are initially apparent. Which of the following pathologic findings of the brain would most likely be present?
The disease is progressive supranuclear palsy, a degenerative disorder characterized by ophthalmoplegia, pseudobulbar palsy, axial dystonia, and bradykinesia. The presentation described in the question is typical. The pathologic changes consist of widespread neuronal loss and gliosis in subcoical sites with sparing of the cerebral and cerebellar coices. Pigmented neurons in the substantia nigra (pars compacta) and locus ceruleus selectively degenerate in Parkinson's disease. In Alzheimer's disease, there is diffuse coical atrophy, especially over the association coex of frontal, temporal, and parietal lobes, with relative sparing of primary sensory and motor areas.Selective frontal and temporal lobe atrophy is characteristic of Pick's disease. Ref: Wyatt C., Butterwoh IV J.F., Moos P.J., Mackey D.C., Brown T.G. (2008). Chapter 11. Neuropathology. In C. Wyatt, J.F. Butterwoh IV, P.J. Moos, D.C. Mackey, T.G. Brown (Eds), Pathology: The Big Picture.
Depigmentation of the substantia nigra and locus ceruleus
Diffuse coical atrophy with relative sparing of primary motor and sensory areas
Selective frontal and temporal lobe atrophy
Widespread neuronal loss and gliosis in subcoical sites