About six to seven out of almost 100,000 people suffer from the so-called progressive supranuclear gaze palsy. The brain dysfunction – also known as PSP – can be compared to Parkinson’s disease. The causes of the disease are not yet known; there is no cure.
What is progressive supranuclear palsy?
The progressive supranuclear palsy or PSP describes a functional disorder of the human brain. The doctors dr. John C Steele, Dr. J. Olszewski and Dr. JC Richardson discovered progressive supranuclear palsy in 1963. For this reason, many medical professionals also refer to the brain dysfunction as the so-called Steele-Richardson-Olszewski syndrome, which is also known by the abbreviation SRO. See gradphysics for Baller-Gerold Syndrome in English.
Progressive supranuclear palsy occurs predominantly in the second half of life, with men being affected more often than women. Around six to seven out of around 100,000 people will develop progressive supranuclear palsy in their lifetime.
The cause of the development of progressive supranuclear palsy is largely unknown. Many researchers believe that it arises due to interactions and environmental influences. Sometimes genetic changes can also be a reason. There are also theories of the viral disease, which subsequently destroys the cells of the cranial nerves.
There are also researchers who believe that pollutants that have been absorbed through the environment for decades can sometimes trigger progressive supranuclear palsy. Physicians have been aware for some time that there are different forms of progressive supranuclear palsy.
The patient can suffer from Richardson syndrome (the classic progressive supranuclear gaze palsy, which was first documented in 1963) as well as from progressive forms which are also comparable to Parkinson’s disease and bring with them a similar course of the disease (that progressive form 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 observed in many patients. These include sudden falls and enormous difficulties in moving or walking. Many patients also suffer from balance problems or complain about vision problems (those affected suffer from blurred vision and double vision).
Other symptoms that indicate progressive supranuclear palsy include 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. Finally, gaze palsy means nothing more than gaze palsy; those affected therefore have problems with the movement of their eyes.
Diagnosis & course of disease
If progressive supranuclear palsy is suspected, the doctor will start with a physical examination and order magnetic resonance therapy (MRI). Using MRI, the doctor can determine whether there is a changed shape of the brainstem. Nuclear medicine (PET) procedures can also be used to check dopamine activity. The doctor then examines the liquor (cerebrospinal fluid) so that any other diseases that have similar symptoms can be ruled out.
Progressive supranuclear palsy cannot be stopped. However, there are medications that sometimes alleviate the symptoms that occur 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 detected in the early stages, the drugs that are also used in Parkinson’s disease help to delay the progression.
The problem with the drugs, however, is that they do not last for a long time – as with Parkinson’s – but become ineffective after a certain time, since the brain cells die (due to the functional disorder) and absorption is therefore no longer possible.
There is usually no treatment for this disease and therefore no cure. The person affected has to live with the symptoms for the rest of their life. In the first place, this disease leads to significant limitations in movement. Those affected very often suffer from sudden falls and can also seriously injure themselves.
Balance and coordination problems also occur and significantly reduce the patient’s quality of life. Furthermore, the symptoms can also depend on other people in their lives. The disease also causes vision 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 various deficiency symptoms.
Gaze paralysis also occurs, so that the patients can no longer move their eyes at all or only to a limited extent. There are no other complications. Mental disorders can be curbed with the help of medication. However, complete treatment and healing does not occur with this disease.
When should you go to the doctor?
Peculiarities or abnormalities of mobility are worrying indications and signs of health impairment. If you fall, have an unsteady gait or have problems moving, you should see a doctor. Balance disorders, dizziness and an increased risk of accidents must be examined more closely. Decreased vision, blurred vision, or seeing double vision should be evaluated and treated.
A doctor is needed in the event of irregularities in speech, impairment of the act of swallowing or a refusal to eat. If the symptoms persist for a long time or if they steadily increase in intensity, a doctor should be consulted. If the eye movements are no longer subject to the patient’s volition, a doctor’s visit is necessary. A stare as well as a sign of 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 worrisome. Mood swings, withdrawal from social life, or aggressive tendencies should be discussed with a doctor. In the case of anxiety, depressive phases or sleep disorders, it is advisable to clarify the symptoms. If everyday obligations can no longer be fulfilled without outside help, or if the reduced vision means that you are unable to work, a doctor is needed 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. In the past, many medical professionals did not even know 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 to that extent. However, there is no complete cure for progressive supranuclear palsy.
The doctor mainly deals with the allocation of medicinal products. The medication relieves symptoms and slows the progression of the disease. The physicians mainly use L-Dopa. L-Dopa ensures that the brain can convert the messenger substance dopamine. However, the effect of L-Dopa wears off after around two to three years, since the brain cells – due to the progressive supranuclear gaze palsy – die off and the active ingredient can no longer be absorbed.
The doctors also prescribe rasagaline and selegiline; both active ingredients ensure a reduced breakdown of dopamine in the brain. Other drugs that are administered in the context of progressive supranuclear palsy are imipramine and amitriptyline. Both are among the tricyclic antidepressants, which are used primarily for depression or depressive moods. Other drugs that are administered as part of the treatment are serotonin reuptake inhibitors and ceonzyme Q10.
Since no causes are known to date and doctors do not know which factors favor the development of progressive supranuclear palsy, no preventive measures can be taken.
In the case of progressive supranuclear gaze palsy, only symptomatic aftercare is currently possible. The purpose of this is to alleviate the symptoms that occur and, under certain circumstances, 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 ingestion, 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, physical therapy, occupational therapy and supplementary speech therapy can help the sufferer to delay the progression of the disease. Furthermore, psychological support helps patients maintain a more positive attitude despite the illness. In some cases, antidepressants may also be necessary.
After being diagnosed with progressive supranuclear palsy, it is important to seek medical attention if there are any signs of infection, difficulty breathing or swallowing. Furthermore, it should be observed whether a wheelchair may be necessary as the disease progresses. Unfortunately, the prognosis for progressive supranuclear palsy is negative.
Since this is a currently incurable disease, a completely symptom-free life is impossible. The quality of life is reduced primarily 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
Progressive supranuclear palsy leads to unsteadiness when walking and can cause balance problems in everyday life. It is not easy for those affected to do something about the complaints in a targeted manner. Doctors can also only prescribe a limited number of drugs.
For this reason, patients should also seek physiotherapeutic help in addition to drug treatment. Problems with speaking and swallowing can be alleviated by occupational therapy and supplementary speech therapy . Memory training counteracts the frequently occurring dementia. Personality can change as the disease progresses. 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. Patients should, on the one hand, ensure that they take their medication regularly and, on the other hand, regularly participate in theattend physiotherapy.
A lot of patience is also required in everyday life, both from those affected and from their relatives. In addition, the sick person should observe their body carefully and apply for a wheelchair if necessary. A short-term visit to the doctor is advisable, especially in the case of infections, breathing and swallowing difficulties.