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What is  Progressive Supranuclear Palsy (PSP)?


Three Canadian scientists first described progressive supranuclear palsy (PSP) in 1963, and lent their names to its alternative title - Steele Richardson Olszewski syndrome. Although in its early stages, PSP can mimic Parkinson's disease, it's less common, and has its own distinct diagnosis, prognosis and treatment.

PSP is a degenerative disease affecting the brain.

Progressive means it starts slowly and continues to get worse, supranuclear refers to the affected part of the brain - pea-sized structures known as nuclei - and palsy describes the outcome - paralysis.

It can cause permanent and serious problems with a patient's vision and ability to move.

Symptoms of PSP


The most obvious and characteristic symptom is the effect on vision. Patients suffer blurring as lesions in the brain prevent the eyes from moving properly. This is most characteristically seen as downward gaze palsy - the inability to look down. Other key symptoms include falls - usually backwards - and the inability to control movement paired with problems breathing, speaking and swallowing.

As the disease progresses, the patient may suffer depression, sleeplessness, symptoms similar to Parkinson’s disease, such as limb rigidity and those in mild dementia, such as memory loss and difficulty thinking.

Causes


Recent research has contradicted the view that men develop PSP more than women, showing that it is equally common in both sexes. Most cases start between the ages 50 and 60, although the disease can set in at any time from the late thirties to the late eighties. It is thought to affect about six people in every 100,000.

The cause is unknown, but scientists suggest several theories as to why the brain would degenerate in such a way. The first is that, like Creutzfeldt-Jakob Disease, PSP may occur as the result of a virus-like particle that enters the body and then takes many years to produce obvious effects. Secondly, it could be the result of a rare genetic mutation. Thirdly is the possibility that a mystery particle in the air or water might trigger it - a theory based on the phenomenon of Guam Island in the Pacific. A surprisingly high proportion of the residents there in the 1950's suffered from a disease known as Lytico Bodig, which has symptoms remarkably similar to PSP.


 

Progressive Supranuclear Palsy (PSP)

 

 

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Treatment

 

There is currently no effective treatment or cure for PSP.

Drug treatments used in Parkinson's, such as L-dopa, are rarely of much benefit, although they can sometimes be of temporary benefit in movement disorder symptoms. Anti-depressants such as Prozac have had some impact, but the effect is not thought to be associated to the drugs ability to alleviate depression. Dementia treatments such as donezepil are ineffective in PSP.

Experimental procedures being tested in Parkinson's - such as the implantation of foetal brain tissue - have proved fruitless in PSP. Treatment instead focuses on alleviating the symptoms, so patients may need a tube inserted into their stomach to overcome problems swallowing, a wheelchair to prevent injury from falls or a weighted walking frame to prevent falling over backwards, and special glasses to correct visual problems.

Although PSP in itself is not fatal, the disease does get progressively worse, and patients often die from complications such as pneumonia and choking on food.