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Renee,

The article on PSP below was cut and pasted from an article in BBC News.  PSP
is a form of Parkinsonism but does not respond well to PD meds.  Dudley
Moore, the actor, has it.

Rees Jenkins


Three Canadian scientists first described progressive supranuclear palsy
(PSP) in 1963, and lent their names to its alternative title - Steele
Richardson Olszewski syndrome. It is very similar to Parkinson's disease
although much rarer, and, although it has its own distinct diagnosis and
treatment, is classed as a form of Parkinsonism.

What is it?

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.


What are the symptoms?

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 and the inability to control movement paired
with problems breathing, speaking and swallowing.

As the disease progresses, the patient may suffer depression, sleeplessness,
memory loss and difficulty thinking.


Who gets it?

Men develop PSP more than women, with most cases starting between 50 and 60,
although the disease can set in at any time from the early forties to late
eighties.

It is thought to affect about 1.4 people in every 100,000, compared to
Parkinson's which affects 200 people per 100,000.


What causes it?

The cause is unknown, but scientists say there are several reasons why the
brain would degenerate in such a way.

The first is that, like Creutzfeldt-Jakob Disease, PSP occurs as the result
of a virus-like particle that enters the body and then takes many years to
produce obvious effects.

Second, it could be the result of a rare genetic mutation.

Third is 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 high proportion of the residents there suffer a condition with symptoms of
Parkinson's, motor neurone disease and Alzheimer's - although it cannot be
identified with any single one.

As it has been seen nowhere else, scientists believe it could be caused by a
substance only found on Guam.


What treatment is available?

There is no cure, and drug therapies used in Parkinson's have been of limited
and temporary benefit, and only for the movement disorders.

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.

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.


What's the outlook?

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.


Is there help?

In the UK, the PSP Europe Association can be contacted at The Old Rectory,
Wappenham, Towcester, Northants NN12 8SQ.

In the US, the Society for Progressive Supranuclear Palsy is at Johns Hopkins
Hospital Outpatient Center 601 North Caroline Street, Suite 5065 Baltimore,
MD 21287.

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