Progressive supranuclear palsy
Progressive supranuclear palsy (PSP; or the Steele-Richardson-Olszewski syndrome, after the doctors who described it in 1963) is a degenerative disease involving the gradual deterioration and death of specific volumes of the brain.
Epidemiology
Males and females are affected approximately equally and there is no racial, geographical or occupational predilection. Approximately 6 people per 100,000 population have PSP. It has been described as a tauopathy.
Triad
Progressive supranuclear ophthalmoplegia (chiefly vertical gaze): paresis of voluntary vertical eye movement, but still moves to vertical doll’s eyes maneuver
Axial dystonia (especially of the neck and upper trunk)
Associated findings: subcortical dementia (inconstant), motor findings of pyramidal, extrapyramidal and cerebellar systems. Average age of onset: 60 yrs. Males comprise 60%. Response to anti-parkin- son drugs is usually very short lived. Average survival after diagnosis: 5.7 yrs.
Differential diagnosis
Differentiating from Parkinson’s disease (IPA):
Patients with PSNP have a pseudo-parkinsonism. They have mask facies but do not walk bent for- ward (they walk erect), and they do not have a tremor. They tend to fall backward.
Similar presentation to NPH