The physician describes an extremely rare disease of the vegetative or autonomic nervous system as Shy-Drager syndrome. Thus, Shy-Drager syndrome falls into the category of neurological diseases. However, the term Shy-Drager syndrome is rarely used today; Doctors refer to the syndrome primarily as multiple system atrophy ( MSA for short ).
What is Shy Drager Syndrome?
Multiple system atrophy ( MSA ), also known as Shy-Drager syndrome, occurs primarily in middle adulthood. The term MSA summarizes several clinical pictures, which were considered a separate disease a few years ago. For meckel-gruber syndrome in dictionary, please visit dictionaryforall.com.
For this reason, the terms Shy-Drager syndrome or striatonigral degeneration or sporadic olivo-ponto-cerebellar atrophy are terms of the past. About 4 out of 100,000 people develop multiple system atrophy every year.
Causes
So far it is not known why and why multisystem atrophy develops. Experts mainly subdivide the atypical Parkinson’s syndrome according to the neuropathological findings, so that certain criteria are sought that could sometimes have been the trigger for multisystem atrophy.
As a rule, however, these criteria can only be established after the death of the patient; Here, however, the physicians are still researching in an unknown area, but are trying to use a microscope to find an answer as to why the so-called multiple system atrophy has broken out.
Symptoms, Ailments & Signs
The multiple system atrophy manifests itself through coordination disorders or the patient complains about motor disorders; Doctors also speak again and again of the so-called “penguin gait”. Furthermore, those affected complain about sensitivity and emotional disorders and paralysis, swallowing difficulties and speech problems.
Other symptoms include personality changes, severe tremors, muscle weakness, as well as fatigue and tension. As a result, spasticity and nystagmus (trembling of the eyes) can occur. Those affected are relatively often tired, suffer from depression and inner restlessness.
Diagnosis & course of disease
In the course of the diagnosis, the physician primarily pays attention to the anamnesis or. He then carries out a clinical examination. The description of the symptoms as well as the observation of the doctor are usually reasons for the doctor relatively quickly suspecting that it could be a case of multiple system atrophy.
Above all, the combinations of autonomic disorders, which are usually reminiscent of Parkinson’s symptoms, are a clear indication that the patient is suffering from multiple system atrophy. However, it should be noted that the multiple system atrophy – due to the similarity with the Parkinson’s syndromes – is relatively difficult to identify. Specialists, too, often have difficulties in committing themselves to multiple system atrophy.
Obligatory are primarily the autonomic regulation disorders of the patient. Many symptoms may or may not occur, further complicating diagnosis. If there are actually difficult cases that make it impossible for the doctor to be sure that there is multiple system atrophy, imaging procedures can help. The doctor relies on computed tomography, magnetic resonance imaging or IBZM-SPECT.
Using CT or MRI, it is possible to examine the atrophied brain regions. The IBZM-SPECT makes it possible to determine whether any dopamine receptors are missing. If the physician is unsure, these imaging procedures should provide evidence that multiple system atrophy is present.
According to the physicians, autonomic disorders of the autonomic nervous system include physical processes that humans do not knowingly regulate. This includes, for example, blood pressure regulation, emptying the bladder as well as sleeping, swallowing and speaking processes. Those areas can cause problems due to multiple system atrophy. The main problem of the disease is that sometimes misdirection leads to food residues getting into the lungs.
As a further consequence, pneumonia develops or aspiration pneumonia can also trigger a potentially life-threatening condition. Many patients who suffer from multiple system atrophy have already died as a result of that aspiration pneumonia. The disease course of a multiple system atrophy is usually negative; Especially in the advanced stage there is no chance of healing or surviving the multiple system atrophy.
Complications
Shy-Drager syndrome primarily leads to severe coordination and concentration disorders. These complaints have a very negative effect on the quality of life of those affected and can lead to significant limitations in everyday life. Paralysis or sensitivity disorders can also occur and are often accompanied by speech or swallowing difficulties.
As a result, it is no longer possible for the patient to take food and liquids as usual. Shy-Drager Syndrome can also cause changes in consciousness and personality, with sufferers even losing consciousness completely in severe cases. It also leads to permanent tiredness and exhaustion.
Most of those affected appear tense and suffer from tremors and inner restlessness. Mental health problems or depression can also occur. Shy-Drager syndrome is most often treated with medication.
Usually there are no complications. If normal nutrition is no longer possible, it must be taken through a stomach tube. In some cases, however, Shy-Drager syndrome can also lead to the death of the affected person.
When should you go to the doctor?
Medical treatment is always necessary for Shy-Drager syndrome. As a rule, self-healing cannot occur here, so that the person concerned is dependent on treatment by a doctor so that life expectancy is not reduced. A doctor should be consulted if the patient suffers from severe emotional disturbances. The sensitivity in the organs drops significantly, so that paralysis or speech problems can also occur.
Difficulty swallowing can also indicate Shy-Drager syndrome and should definitely be examined and treated by a doctor. Many of those affected appear tired and depressed as a result of the disease and suffer from muscle weakness or severe tremors. Depression or inner restlessness can also indicate Shy-Drager syndrome and should also be examined and treated by a doctor.
The first diagnosis of Shy-Drager syndrome can be made by a general practitioner. For further treatment, however, a visit to a specialist is necessary. It cannot be universally predicted whether this will lead to complete healing.
Treatment & Therapy
The therapy is multi-pronged, is individually adapted and proves – since the multi-system atrophy can vary – to be quite difficult. About a third of those affected actually respond to therapy. The physicians mainly resort to L-Dopa therapy, which is also used in Parkinson’s patients. However, that therapy only helps for a certain amount of time. Amantadine is also often prescribed; This is a drug intended to help patients with movement disorders.
The physician must treat all known autonomic disorders symptomatically. It is important that the patient drinks enough fluids or receives blood pressure medication, as those affected not only suffer from bladder emptying disorders, but sometimes also from very low blood pressure. If the doctor already diagnoses depression that has occurred in the context of multiple system atrophy, it must also be treated.
In many cases incontinence also occurs; that too is treated symptomatically. In order to alleviate the patient’s suffering, if the disease has already reached an advanced stage, a gastric tube is inserted or the patient is ventilated – using a breathing mask. So far there has been no fight against the causes; Currently, however, a therapy is being researched that mainly deals with the administration of immunoglobulins. Therapy is based on relieving the symptoms, not addressing the cause.
Prevention
Since no cause is known so far, there is currently no possibility of preventive measures.
Aftercare
In most cases, those affected with Shy-Drager syndrome have very few measures and options for aftercare. Therefore, the affected person should consult a doctor at an early stage, so that there are no further complaints or complications that could have a negative impact on the affected person’s quality of life.
Since it is a genetic disease, it cannot be completely cured. However, if the affected person wishes to have children, they should primarily have a genetic examination and counseling carried out in order to prevent the recurrence of Shy-Drager syndrome in their descendants. Many of the symptoms can be alleviated and limited relatively well by taking various medications.
Regular intake and the right dosage must always be observed in order to limit the symptoms properly. If you have any questions or if you experience side effects, you should always consult a doctor first. Furthermore, regular checks are very important throughout the life of the affected person in order to identify other damage to the body at an early stage. In some cases, Shy-Drager syndrome also reduces the life expectancy of the sufferer.
You can do that yourself
Unfortunately, the causes of Shy-Drager Syndrome are still unknown. Therefore, curing or slowing down the disease is currently not possible. Living with Shy-Drager Syndrome means allowing and using adapted help with increasing, corresponding restrictions. Physical coordination disorders, tremors and muscle weakness should be improved and accompanied with physiotherapeutic treatments.
Nursing aids that are used for locomotion can enable and promote participation in social life over a certain period of time. Increasing language limitations should be improved or kept stable as far as possible by accompanying logopedic treatments. This means that communication with restrictions will remain possible for a while.
With regard to restlessness and depression, exercise and light sporting activities are of course recommended within the scope of the patient’s possibilities. In this context, occupational therapy and occupational therapy measures such as music therapy, scent therapy, aromatherapy and Snoezelen should also be used.
Since it is no longer possible for the patient to take in food and liquids in the later stages, everyday help with handouts is necessary. The changes in consciousness and personality as a whole should be discussed with those close to the patient. This is the only way to react adequately to behavior that causes a lack of understanding.
