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Progressive supranuclear palsy (PSP) (or the Steele-Richardson-Olszewski syndrome, after the Canadian physicians who described it in 1963) is a rare degenerative disorder involving the gradual deterioration and death of selected areas of the brain. The sexes are affected approximately equally and there is no racial, geographical or occupational predilection. Approximately 6 people per 100,000 population have PSP.
The initial symptom in two-thirds of cases is loss of balance 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. Often, sufferers will need some 'supranuclear palsy swallowing therapy'.
Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles, sleep disruption, urinary incontinence and constipation.
The visual symptoms are of particular importance in the diagnosis of this disorder. Notably, the ophthalmoplegia experienced by these patients mainly concerns voluntary eye movement. Involuntary eye movement, as elicited by Bell's phenomenon, for instance, may be closer to normal. On close inspection, eye movements called "square wave jerks" may be visible when the patient fixes at distance. These are fine movements, similar to nystagmus, except that they are not rhythmic in nature. Difficulties with convergence, where the eyes come closer together while focusing on something near, like the pages of a book, is typical. Because the eyes have trouble coming together to focus on things at near, the patient may complain of double vision when reading. 'supranuclear palsy swallowing therapy' comes in to play when the muscles involved with swallowing become affected.
Cardinal Manifestations:
Supranuclear ophthalmoplegia
Neck dystonia
Parkinsonism
Pseudobulbar palsy
Behavioral and Cognitive impairment
Imbalance and Difficulties walking
Frequent Falls.
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