Out of almost 100,000 people, around six to seven people get the so-called progressive supranuclear palsy . The - also as PSP known - brain dysfunction can be compared to Parkinson's disease. The causes of the disease are so far unknown; there is no cure.
What is progressive supranuclear palsy?
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The progressive supranuclear palsy respectively PSP describes a dysfunction of the human brain. The doctors Dr. John C. Steele, Dr. J. Olszewski and Dr. J.C. Richardson discovered progressive supranuclear palsy in 1963. For this reason, many medical professionals also refer to the dysfunction of the brain as so-called Steele-Richardson-Olszewski syndrome, which is also known by the abbreviation SRO.
Progressive supranuclear palsy predominantly occurs in the second half of life, with men getting the disease more often than women. Of around 100,000 people, around six to seven people will develop progressive supranuclear palsy in the course of their lives.
root cause
The cause of progressive supranuclear palsy is largely unknown. Many researchers are of the opinion that it arises as a result of interactions and environmental influences. Sometimes genetic changes can also be a reason. There are also theories of viral disease, which subsequently destroys the cells of the brain nerves.
There are also researchers who are of the opinion that pollutants that have been ingested through the environment for decades can sometimes trigger progressive supranuclear palsy. For some time now, medical professionals have been aware that there are different forms of progressive supranuclear palsy.
For example, the patient can suffer from Richardson's syndrome (the classic progressive supranuclear palsy, which was documented for the first time in 1963) and also develop forms that are comparable to Parkinson's disease and bring about a similar course of the disease (that form is also known as “Pure Akinesia with Gait Freezing” or “PAGF”).
Symptoms, ailments & signs
The symptoms are different. However, there are several signs that could be seen in many patients. These include, for example, sudden falls as well as enormous difficulties in locomotion or walking. Many patients also suffer from balance problems or complain of vision problems (those affected suffer from blurred vision and double vision).
Other symptoms that indicate progressive supranuclear palsy are swallowing and speech problems. In some cases changes in mood and personality were also noted. Another classic symptom, which is sometimes responsible for the naming of the disease, is the problem of eye movement. Ultimately, gaze palsy means nothing more than gaze paralysis; therefore, those affected have problems with the movement of their eyes.
Diagnosis & course of disease
If progressive supranuclear palsy is suspected, the doctor begins with a physical examination and orders magnetic resonance therapy (MRI). Using an MRI, the doctor can determine whether the shape of the brain stem has changed. Nuclear medicine procedures (PET) can also be used to check the activity of dopamine. The doctor then examines the liquor (nerve water) so that any other diseases that have similar symptoms can be excluded.
Progressive supranuclear palsy cannot be stopped. However, there are drugs that sometimes alleviate the symptoms or slow down the course of the disease. This means that those affected can participate in "normal" social life for longer. Especially when the progressive supranuclear palsy is recognized in the early stages, the drugs, which are also used in Parkinson's disease, help that the course can be delayed.
The problem with the drugs, however, is that they do not last for a long time - as is the case with Parkinson's - but become ineffective after a certain time, as the brain cells die (due to the dysfunction) and absorption is no longer possible.
Complications
As a rule, there is no treatment and therefore no cure for this disease. The affected person has to live with the symptoms his entire life. First and foremost, this disease results in significant mobility restrictions. Those affected very often suffer from sudden falls and can seriously injure themselves.
Problems of balance and coordination also occur and significantly reduce the patient's quality of life. Furthermore, the complaints can also depend on other people in their lives. The disease also causes visual problems and possibly double vision. Difficulty speaking or swallowing can also occur. This leads to problems with the intake of food and liquids, so that those affected can suffer from underweight or from various deficiency symptoms.
Eye paralysis also occurs, so that the patients can no longer move their eyes at all or only to a limited extent. There are no further complications. With the help of medication, mental disorders can possibly be limited. A complete treatment and cure does not occur in this disease.
When should you go to the doctor?
Particularities or abnormalities of mobility are worrying indications and signs of a health impairment. If you fall, walk unsteadily or have problems moving, you should consult a doctor. Problems with balance, dizziness and an increased risk of accidents must be examined more closely. Reduced vision, blurred vision or the perception of double vision should be examined and treated.
A doctor is required in the event of speech irregularities, impaired swallowing or refusal of food intake. If the symptoms persist over a long period of time or if they steadily increase in intensity, a doctor should be consulted. If the eye movements are no longer subject to the will of the person concerned, a visit to the doctor is necessary. A fixed stare and a paralysis in the eye should be presented to a doctor.
If the person concerned also suffers from emotional abnormalities, there is also a need for action. Changes in behavior or personality are worrying. Mood swings, withdrawal from social life, or aggressive tendencies should be discussed with a doctor. In the case of anxiety, phases of depression or sleep disorders, clarification of the symptoms is advisable. If the daily obligations can no longer be fulfilled without outside help or if there is an occupational disability due to reduced vision, a doctor is required so that a treatment plan can be drawn up.
Treatment & Therapy
One of the main problems is the fact that progressive supranuclear palsy is very difficult to diagnose. Many medical professionals did not even know earlier that the patient was suffering from this dysfunction. For this reason, therapies and treatments were started relatively late. Today, however, it is possible that - with early treatment - the symptoms of progressive supranuclear palsy can be alleviated, so that the course of the disease is delayed. However, there is no complete cure for progressive supranuclear palsy.
The physician is primarily concerned with the allocation of medicinal products. The drugs relieve symptoms and slow the course of the disease. Doctors mainly use L-Dopa. L-Dopa ensures that the brain can convert the neurotransmitter dopamine. However, the effect of L-Dopa diminishes after around two to three years, as the brain cells - due to the progressive supranuclear palsy - die and absorption of the active ingredient is no longer possible.
Doctors also prescribe rasagalin and selegiline; both active ingredients reduce the breakdown of dopamine in the brain. Other active ingredients that are administered in the course of progressive supranuclear palsy are imipramine and amitriptyline. Both are among the tricyclic antidepressants, which are mainly used for depression or depressed moods.Other drugs that are administered as part of the treatment are serotonin reuptake inhibitors and Ceonzym Q10.
You can find your medication here
➔ Medicines for eye infectionsprevention
Since no causes are known so far or doctors do not know which factors favor the development of progressive supranuclear palsy, no preventive measures can be taken.
Aftercare
In the case of progressive supranuclear palsy, only symptomatic follow-up is currently possible. The purpose of this is to alleviate the symptoms and, if necessary, to slow down the course of the disease. For this purpose, L-Dopa drugs are used, which are also used in Parkinson's disease. Unfortunately, after a certain period of use, these drugs are no longer effective.
The reason for this is the death of brain cells that are necessary to absorb the active ingredient. In addition, physiotherapy, occupational therapy and supplementary speech therapy can help the sick to delay the progression of the disease. Furthermore, psychological support helps the patient to maintain a more positive attitude despite the illness. In some cases it may also be necessary to take antidepressants.
After a diagnosis of progressive supranuclear palsy, it is important to see a doctor if there are signs of infection, or difficulty breathing, or swallowing. Furthermore, it should be observed whether a wheelchair may be necessary as the disease progresses. The prognosis for progressive supranuclear palsy is unfortunately negative.
Since this is a currently incurable disease, a completely symptom-free life is impossible. The quality of life is reduced, especially due to the considerable restriction in movement, balance and coordination. After the onset of symptoms, the average survival time is about six years.
You can do that yourself
The progressive supranuclear palsy leads to insecurity when walking and can cause balance disorders in everyday life. It is not easy for those affected to take targeted action against the symptoms. Doctors can only prescribe medication to a limited extent.
Therefore, in addition to drug treatment, patients should also seek physiotherapeutic help. Problems with speaking and swallowing can be alleviated through occupational therapy and complementary speech therapy. Memory training counteracts the common dementia. As the disease progresses, personality can change. Mood swings and depressive moods are also common. The quality of life can be improved somewhat through individual medication and individual therapy. The doctor prescribes the appropriate means to promote safe walking. On the one hand, patients should ensure that they take the medication regularly and, on the other hand, regularly take part in physiotherapy.
A lot of patience is also required in everyday life, both from those affected and from their relatives. In addition, the sick people should watch their bodies carefully and request a wheelchair if necessary. A short-term visit to a doctor is advisable, especially if you have infections, or if you have difficulty breathing or swallowing.
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