Progressive supranuclear palsy (PSP)
What is it?
Progressive supranuclear palsy is a relatively rare neurodegenerative disease. PNP occurs due to the progressive and selective loss of nerve cells (neurons) in certain areas of the brain. Participating neurons are especially responsible for controlling eye movements, balance, speech, and swallowing. The first symptoms usually include loss of balance and sudden falls (usually backwards), neck stiffness, and vision problems. The latter concerns the inability to look primarily downward. Other initial symptoms are often vague and may include depression, lethargy, and loss of judgment. As the disease progresses, difficulty in speaking and swallowing increases. Walking deteriorates and it becomes necessary to use aids to maintain balance, and, as a result, a wheelchair. Despite the progression of the disability, intellectual ability usually does not decline. Patients often suffer from inability to communicate due to physical problems. There may be slight urinary incontinence. Other behavioral effects may also occur, such as emotional lability (laughing and / or crying for no apparent reason), depression, and an inability to solve difficult problems.
How is it diagnosed?
The diagnosis is predominantly clinical. PNP is part of the Parkinson's group, but it is actually a different disease from classic Parkinson's disease with different symptoms. Parkinson's disease is much more common than PNP. The former is often considered less severe and, in fact, the average survival rate of a patient with Parkinson's disease after treatment is not very different from the general population. Unfortunately, this is not the case for PNP, where the average patient's survival is significantly reduced. Patients with PNP are usually around 60 years of age; Parkinson's patients can be much younger. Although they manifest and progress in different ways, it can sometimes be difficult to distinguish them clinically, especially in the early stages. At first, PNP patients have stiffness in the body, especially in the neck; walking is careful; the fall predominantly occurs backward. On the other hand, postural instability and falls are late signs of Parkinson's disease. In Parkinson's disease, patients have tremors in the upper extremities, which is rare with PNP. MRI is the only diagnostic tool that is useful for confirming the diagnosis in the most advanced stages; at the onset of the disease, nothing may be detected.
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How is it treated?
There is currently no definitive cure that can stop the progression of the disease. Although various therapies have been tried, none have so far brought significant benefits. This is perhaps due to the fact that the first studies were done on a limited number of patients. Many of the symptoms of PSP can be treated and there are a number of measures that can help the patient and caregiver cope with the progress of the disease. Unfortunately, the treatment that is most effective in Parkinson's disease is usually of no use, and sometimes even counterproductive in PSP due to the side effects. Amantadine seems to have a moderate beneficial effect in helping mobility and some antidepressants may also be beneficial. The latter seem to work not only against depression, but also against the stiffness associated with the disease which can cause pain in the neck and limbs. Injections of botulinum toxin can be useful for relaxing the muscles. Speech therapists and physiotherapists can give you valuable advice to deal with the different symptoms.
Where do we treat it?
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