Progressive supranuclear palsy [Neurosurgery Wiki]

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====== 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.

AKA [[Steele-Richardson-Olszewski syndrome]]
((Kristensen MO. Progressive Supranuclear Palsy - 10 Years Later. Acta Neurol Scand. 1985; 71:177–189)).

===== 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.
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===== Triad =====

Progressive supranuclear ophthalmoplegia (chiefly vertical gaze): paresis of voluntary vertical eye movement, but still moves to vertical doll’s eyes maneuver

[[Pseudobulbar palsy]].

[[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.
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Similar presentation to [[NPH]]

===== Treatment =====

[[Progressive supranuclear palsy treatment]].