Out of nearly 100,000 people, about six to seven suffer from what is known as progressive supranuclear gaze palsy . The brain dysfunction – also known as PSP – can be compared to Parkinson’s disease. The causes of the disease are as yet unknown; there is no cure.
What is progressive supranuclear gaze palsy?
Progressive supranuclear gaze palsy, or PSP, describes a dysfunction of the human brain. Doctors Dr. John C. Steele, Dr. J. Olszewski, and Dr. J.C. Richardson discovered progressive supranuclear gaze palsy in 1963, which is why many medical professionals refer to the brain dysfunction as Steele-Richardson-Olszewski syndrome, also known by the acronym SRO. Progressive supranuclear gaze palsy occurs predominantly in the second half of life, with men more often affected than women. Out of approximately 100,000 people, about six to seven will develop progressive supranuclear gaze palsy during their lifetime.
Cause
The cause of progressive supranuclear gaze palsy is largely unknown. Many researchers argue that it develops because of interactions as well as environmental influences. Sometimes genetic changes may also be a reason. There are also theories of viral disease, which subsequently destroys the cells of the cranial nerves. There are also researchers who believe that pollutants that have been ingested for decades through the environment can sometimes trigger progressive supranuclear gaze palsy. For some time now, physicians have been aware that there are different courses of progressive supranuclear gaze palsy. Thus, the patient may suffer from Richardson’s syndrome (the classic progressive supranuclear gaze palsy, which was first documented in 1963) as well as from courses that are also comparable to Parkinson’s disease and involve a similar disease course (that course is also called “Pure Akinesia with Gait Freezing” or “PAGF”).
Symptoms, complaints, and signs
Symptoms vary. However, there are several signs that have been observed in many patients. These include sudden falls as well as enormous difficulties in locomotion or walking. Many patients also suffer from balance problems or complain of vision problems (blurred vision and double vision). Other symptoms that indicate progressive supranuclear palsy are swallowing and speech problems. In some cases, changes in mood as well as personality have also been 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 a paralysis of the eyes; therefore, those affected have problems with the movement of their eyes.
Diagnosis and course of the disease
If progressive supranuclear gaze palsy is suspected, the medical professional begins with a physical examination and orders magnetic resonance imaging (MRI). Using MRI, the physician can determine if there is an altered shape of the brainstem. Nuclear medicine procedures (PET) can also be used, which check the activity of dopamine. The physician then examines the CSF (cerebrospinal fluid) to rule out any other diseases that may have similar symptoms. Progressive supranuclear gaze palsy cannot be stopped. However, there are medications that sometimes alleviate the symptoms or slow down the course of the disease. This means that those affected can participate in social, “normal” life for longer. Especially when progressive supranuclear gaze palsy is diagnosed at an early stage, the drugs, which are also used for Parkinson’s disease, help to delay its progression. 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 because the brain cells die (due to the dysfunction) and thus absorption is no longer possible.
Complications
As a rule, there is no treatment and therefore no cure for this disease. The affected person must live with the symptoms for the rest of his or her life. First and foremost, this disease leads to significant movement restrictions. The affected person often suffers from sudden falls and can also be seriously injured. Balance and coordination problems 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 vision problems and possibly double vision. Speech difficulties or swallowing problems may also occur. In this case, there are problems with the intake of food and liquids, so that the affected person can suffer from underweight or various deficiency symptoms. Gaze paralysis also occurs, so that patients can no longer move their eyes at all or only to a limited extent. Further complications do not occur. With the help of medication, mental disorders can possibly be limited. However, complete treatment and cure does not occur with this disease.
When should you see a doctor?
Peculiarities or abnormalities in mobility are worrisome clues and signs of impaired health. If falls, unsteadiness of gait or problems with locomotion occur, a doctor should be consulted. Disturbances in balance, dizziness and an increased risk of accidents should be investigated further. Decreased vision, blurred vision or perception of double vision should be investigated and treated. If there are irregularities in speech, impaired swallowing, or a refusal to eat, a physician is needed. If the symptoms persist over a long period of time or steadily increase in intensity, a physician should be consulted. If the eye movements are no longer under the arbitrary power of the affected person, a visit to the doctor is necessary. A fixed gaze as well as a paralysis in the eye should be presented to a doctor. If the affected person 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 physician. In the case of anxiety, depressive phases or sleep disturbances, clarification of the complaints is advisable. If daily responsibilities can no longer be performed without assistance or if there is occupational disability due to decreased vision, a physician is needed so that a treatment plan can be developed.
Treatment and therapy
One of the main problems is that progressive supranuclear gaze palsy is very difficult to diagnose. In the past, many medical professionals did not even know that the patient suffered from this functional disorder. For this reason, therapies as well as treatments were started relatively late. Today, however, it is possible that – with early treatment – the symptoms of progressive supranuclear gaze palsy can be alleviated, so that the course of the disease is delayed. However, there is no complete cure for progressive supranuclear palsy. Primarily, the medical profession is concerned with the administration of medications. The medications relieve symptoms and slow the progression of the disease. Medical practitioners predominantly use L-dopa. L-dopa ensures that the brain can convert the neurotransmitter dopamine. However, the effect of L-dopa wears off after about two to three years, as the brain cells – due to progressive supranuclear gaze palsy – die and it is no longer possible to absorb the active substance. The doctors also prescribe rasagalin and selegiline; both active ingredients ensure a reduced breakdown of dopamine in the brain. Other active substances that are administered in the context of progressive supranuclear gaze palsy are imipramine and amitriptyline. Both belong to the tricyclic antidepressants, which are mainly used for depression and depressed moods.Other medications administered as part of treatment include serotonin reuptake inhibitors and ceonzyme Q10.
Prevention
Because no causes are known to date, or physicians do not know which factors promote the development of progressive supranuclear gaze palsy, no preventive measures can be taken.
Follow-up
At the present time, only symptomatic follow-up is possible for progressive supranuclear gaze palsy. The purpose of this is to alleviate the symptoms that occur and, under certain circumstances, to slow the progression of the disease. For this purpose, L-Dopa drugs are used, which are also used for 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 for the absorption of the active substance. In addition, physiotherapy, occupational therapy and complementary speech therapy can help sufferers to delay the progression of the disease. Furthermore, psychological support helps patients maintain a more positive attitude despite the disease. In some cases, the use of antidepressants may also be necessary. After diagnosis with progressive supranuclear gaze palsy, it is important to see the doctor if there are signs of infection, breathing or swallowing difficulties. Furthermore, it should be observed whether a wheelchair may be necessary if the disease progresses. The prognosis for progressive supranuclear gaze palsy is unfortunately negative. Since it is a currently incurable disease, a completely symptom-free life is impossible. Quality of life is reduced, especially due to the significant limitation in movement, balance and coordination. After symptom onset, the average survival time is approximately six years.
What you can do yourself
Progressive supranuclear gaze palsy causes unsteadiness when walking and can cause balance problems in everyday life. It is not easy for those affected to specifically do something about the symptoms. Doctors can also only prescribe medication to a limited extent. That is why patients should seek physiotherapeutic help in addition to medication. Occupational therapy and complementary speech therapy can alleviate problems with speaking and swallowing. Memory training counteracts the dementia that often occurs. As the disease progresses, personality can change. Mood swings and depressive moods also often occur. The quality of life can be improved somewhat through individual medication as well as individual therapy. The physician prescribes the appropriate means to promote safe gait. On the one hand, patients should pay attention to taking the medication regularly, and on the other hand, they should participate in physiotherapy on a regular basis. In everyday life, a lot of patience is also required, both from those affected and from their relatives. In addition, the ill person should keep a close eye on his or her body and, if necessary, request a wheelchair. Especially in the case of infections, breathing and swallowing difficulties, a short-term visit to the doctor is advisable.
