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What is Progressive Supranuclear Palsy?
Firstly, let's break the term down. Progressive meaning over a period of time. Supranuclear meaning above the level of the motor neurons on the spinal nerves. Palsy meaning weakness. Therefore it is a weakness above the level of the motor neurons on the spinal nerves which builds up over a period of time.
Symptoms typically begin in one's 60's but it may start as early as 40. Those affected usually survive between six and ten years after the initial symptoms occur. The causes of PSP are not yet known however, it is at least partly genetic. A brain protein called tau gathers in abnormal clumps in certain brain cells in people with PSP. Unidentified chemicals in foods or the environment may also contribute to the failure of brain cells and the accumulation of tau.
What is the diagnosis?
Progressive supranuclear palsy is often identified by the slowing of movement and reduced control of walking, balance, swallowing, speaking, and eye movement. People with PSP eventually become bound or bedridden.
Loss of balance while walking is a very common complaint. Weakness of the eye is another problem that occurs. Patients have trouble looking down and they develop involuntary eye closing. Emotional and personality changes such as increased irritability, violent outburst or even sudden bouts of crying or laughter are just come of the psychological implications. Like Alzheimer's disease, patients develop slurred speech and mental slowing including forgetfulness.
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Is there any treatment?
At the moment there is no effective medication against PSP. Some drugs such as Levodopa (Sinemet), which we learned about in issue 5 on Parkinson's Disease, amantidine, and amitriptyline may have a temporary or modest benefit on certain Parkinson-like symptoms. Injections can be given to help reduce involuntary eye closure. Eye drops can also be prescribed to prevent the eyes drying out due to decreased rates of blinking. Backward falls can also be helped by using a weighted walker. To aid patients to see downwards, glasses with special prisms incorporated.
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