What is
PSP?
Progressive
Supranuclear Palsy (PSP) is also known as Steele Richardson
Olszewski Syndrome after the three Canadian doctors who
first described PSP as a separate condition in 1963. It
is a rare, degenerative neurological disease related to
Parkinson's disease.
It strikes middle aged adults (45+) and the elderly and
affects about 5 in 100,000. Its cause is currently unknown
and there is no available cure at this stage. Each case
of PSP can be different. Life expectancy from diagnosis
can vary between 3 to 10 years. Throughout the course
of the disease, the intellect of the sufferer remains
intact - they are still mentally alert but trapped in
a body that is slow or unable to react and follow the
brain's instructions. |
Dudley Moore, famous musician and actor, was diagnosed
with PSP.
| Progressive: |
Refers
to the progressive nature of the disease over time. |
| Supranuclear: |
The
area of the brainstem that controls up and down
eye movements. The nature of the eye problem in
PSP is a very important clinical feature for diagnosis. |
| Palsy: |
Refers
to weakness or paralysis in moving the eyes. |
| What
is the cause of PSP? |
 |
The cause is unknown. Extensive research is being carried
out throughout the world. Research recently has suggested
a genetic link may be present. Another theory is that
an environmental trigger factor (or several triggers)
may be needed to bring on the disease. Presently the main
suspicion for cause falls upon a slow toxin. For example,
a toxin called "MPTP", a containment in a drug
of abuse, causes a condition similar to Parkinsons.
There is no current proof that the disease is inherited.
| What
are the symptoms? |
|
Early signs of PSP include falling, difficulty walking,
imbalance and slow movement similar to Parkinson's Disease.
A classic symptom of PSP is the limitation of upward and
downward eye movements. People with PSP experience vision
problems such as double and blurred vision, as well as
difficulty with speech and swallowing. There may also
be changes in mood and behaviour. In its advanced stages,
persons with PSP are bedridden or in wheelchairs and require
full-time care.
| How
is PSP diagnosed? |
|
PSP is usually diagnosed by a neurologist. There are no
diagnostic tests other than a clinical examination. Typical
findings include features of Parkinson's with limb stiffness,
slowness, imbalance and trouble walking with limitation
of upward and downward eye movement. Given the rare nature
of PSP and similarities with Parkinson's disease, many
people are initially mis-diagnosed with Parkinsons.
| What
is the treatment? |
|
At present, there is no form of treatment that can reverse
the effects of this disease, nor is there a cure. A variety
of medications and other forms of therapy can help the
symptoms.
Physiotherapists can asses gait, balance and transfer
problems. They can arrange appropriate walking aids and
give advice to carers about safe practise to help assist
the person with PSP.
Speech pathologists can asess speech and swallowing problems
and provide advice to assist in management. In order to
reduce the risk of choking and chest infections, it is
critical for the person with PSP and swallowing problems
to see a speech pathologist.
Occupational Therapists (OT) offer assessment of problems
with managing daily activites such as dressing, showering,
toileting etc. OT's can provide appropriate aids and equipment
to allow the person to be safe and to enhance independence
in the home.
Counselling support for patients, carers and family members
can be critical.
| Can
people die from PSP? |
|
Yes, people with PSP die from complications of immobility
and the inability to swallow, including pneumonia and
aspiration of food into the lungs.
|
