Progressive supranuclear palsy
) (or the Steele-Richardson-Olszewski syndrome
, after the Canadian physicians who described it in 1963) is a rare degenerative disease
involving the gradual deterioration and death of specific areas of the brain
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
Symptoms and signs
The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls.
Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms.
Later symptoms and signs are dementia
(typically including loss of inhibition and ability to organize information), slurring of speech
, difficulty swallowing
, and difficulty moving the eyes
, particularly in the vertical direction. The latter accounts for some of the falls experienced by these patients as they are unable to look up or down.
Some of the other signs are poor eyelid
of the facial muscles
, a backward tilt of the head with stiffening of the neck muscles
, sleep disruption
, urinary incontinence
The visual symptoms are of particular importance in the diagnosis of this disorder. Notably, the......