Multiple System Atrophy

Multiple system atrophy is a neurological disease that belongs to the atypical parkinsonian syndromes. The atypical Parkinson’s syndromes often have symptoms similar to the relatively well-known Parkinson’s disease, but are often more rapidly progressive, already have a symmetrical distribution of symptoms at the onset of the disease, respond less well to the Parkinson’s drug L-dopa and also differ in other abnormalities from the “original” Parkinson. The distinction is often difficult and definitely a matter for a neurologist. Nevertheless, here is a small overview of the MSA.

Multiple System Atrophy

What is multiple system atrophy?

Multiple system atrophy (MSA) refers to a sporadically occurring neurodegenerative disease of middle adulthood, which is characterized by the combination of disorders of the autonomic nervous system (see below) with Parkinson’s symptoms or cerebellar ataxia.┬áSee foodezine for Everything about Anthrax.

The term MSA now summarizes some clinical pictures that have been known for a long time, which were originally considered independent diseases: Shy-Drager syndrome, sporadic olivo-ponto-cerebellar atrophy and striatonigral degeneration, which are the most common form of manifestation with pronounced autonomic disorders and extrapyramidal disorders.

About 4 out of 100,000 people get MSA. Parkinson’s is about 50 times more common.

Causes

The etiology, i.e. the mechanism by which multisystem atrophy develops, is unknown, and the field of research is very complex, even for experts. The atypical Parkinson’s syndromes are classified primarily according to neuropathological findings, i.e. according to certain criteria that can only be verified under the microscope (and thus often only after the death of the person concerned).

The characteristic finding in the case of MSA is the detection of alpha-synuclein-positive inclusion bodies in the oligodendrocytes of the brain. It is a protein that is deposited in these “supporting cells” of the brain without knowing the exact reason or origin. Like Parkinson’s disease and Lewy body dementia, MSA is therefore classified as a synucleinopathy.

One also observes a loss of nerve cells in various areas of the brain, which ultimately causes the symptoms and progresses rapidly. The degeneration in the substantia nigra is typical for Parkinson’s and therefore causes symptoms similar to Parkinson’s, but at the same time in MSA dopamine-receptor-containing tissue in the striatum is lost and causes the non-responsiveness to dopamine as a drug, which in turn is MSA different from Parkinson’s disease.

A death of nerve tissue in the cerebellum and pons is responsible for cerebellar ataxia. The death of nerve tissue in the spinal cord is meanwhile held responsible for the dysfunction of the autonomic nervous system.

Symptoms, Ailments & Signs

Multiple system atrophy is characterized by a variety of neurological and physical symptoms. Over time, the symptoms worsen. After about three to five years, the ability to walk is lost. Death usually occurs after eight to ten years. There are two possible courses of the disease.

In one, the so-called Parkinson’s symptoms predominate. The other course of the disease shows mainly cerebellar symptoms. In the context of Parkinsonism, the symptoms of tremor (shaking), muscle rigidity (rigor) and slowing of movements (bradykinesia) predominate. In the case of the cerebellar progression, coordination disorders of movement (stance and gait ataxia) with a constant risk of falling, pathological drowsiness (nystagmus) and disorders of the voluntary movement sequences (dysmetry) are observed.

These symptoms can also include swallowing disorders, speech disorders, constant fluctuations in blood pressure, low blood pressure, erectile dysfunction and urinary incontinence. Dementia often develops as well. In addition, pyramidal tract signs also appear, which are expressed by an increased readiness for reflexes or by a positive Babinski reflex. In the Babinski reflex, the big toe moves outwards when rubbing the sole of the foot, in contrast to the normal reflex.

However, not all symptoms have to be present with the disease. Symptoms are highly dependent on which systems are affected. Life expectancy is significantly reduced by complications such as aspiration pneumonias associated with swallowing disorders or severe respiratory disorders. Therapy is not possible, especially for cerebellar multiple system atrophy. Overall, multiple system atrophy cannot currently be cured.

Diagnosis & History

Multisystem atrophy is clinically noticeable through the combination of autonomic disorders with Parkinson’s symptoms or cerebellar ataxia.

Autonomic disorders include those of the autonomic nervous system, i.e. that part of our nervous system that regulates physical processes of daily life without our willing control and influence: disorders of blood pressure regulation, bladder emptying disorders, sleeping, speaking and swallowing disorders can occur in this context. Difficulty swallowing in particular can quickly become dangerous if leftover food gets into the lower respiratory tract and triggers pneumonia there – such aspiration pneumonia is a frequent cause of death in MSA patients.

Medicine understands Parkinson’s symptoms as a combination of rigidity, tremor and akinesia. The symptom rigor describes an increase in muscular tone up to and including muscle rigidity that cannot be broken through at will, a tremor is a fine muscle tremor (e.g. of the hands), akinesia (or somewhat less pronounced: hypokinesia) describes a lack of movement, which is caused by a lack of drive in the brain . Overall, there is a impoverishment of facial expressions and gestures, a stooped posture, a slower reaction and therefore a tendency to fall forward as well as the typical small-step walk of Parkinson’s patients.

Cerebellar ataxia can also occur with MSA and describes a disturbance in the coordination of movement and posture, which has its origin in the cerebellum. For example, there may be a tendency to fall while walking or standing.

The cornerstones of diagnostics are anamnesis and clinical examination. Here, based on the rough description and the appearance, which is also visible to the doctor, a Parkinson’s syndrome can be quickly concluded. However, the exact diagnosis within the Parkinson’s syndrome is often difficult, even for the specialist.

The autonomic regulatory disorders are always obligatory for MSA, everything else can be added, but does not always have to be. Some characteristics such as swallowing and speech disorders, an early tendency to fall and rapid disease progression make one think of MSA – but in particular the non-response to L-Dopa as a drug often only helps the doctor to differentiate it from “normal” Parkinson’s disease.

In difficult cases, imaging using CT, MRI or IBZM-SPECT can help and demonstrate atrophied brain regions (CT, MRI) or the absence of dopamine receptors (SPECT).

Complications

In most cases, multiple system atrophy causes symptoms that are similar to Parkinson’s disease. These complaints can lead to significant restrictions and complications in the patient’s everyday life, so that he is dependent on the help of other people. This leads to paralysis and sensory disturbances, which can also lead to restricted mobility. The patients suffer from disturbances of concentration and also from motor disorders.

Furthermore, patients often find it difficult to speak and have difficulty swallowing. These can lead to problems with the intake of liquids and food, so that people suffer from nutritional deficiencies or dehydration. There is also tension and muscle weakness. Those affected often tremble and suffer from inner restlessness or depression.

The quality of life of the patient is significantly reduced and restricted by the multi-system atrophy. It is also not uncommon for inflammation to occur in the lungs, which in the worst case can even lead to the death of the patient. A direct and causal treatment of this disease is usually not possible.

However, the symptoms can be reduced by various therapies and with the help of medication. In most cases, multiple system atrophy reduces the patient’s life expectancy.

When should you go to the doctor?

Unsteady gait and mobility disorders are signs of a health impairment that must be examined by a doctor. A doctor is needed if there is a weakness in physical performance, low resilience and a decrease in mobility. If the person concerned suffers from trembling limbs, inner restlessness or disturbances in voluntary movement, a doctor must be consulted. Patients with multiple system atrophy are often no longer able to safely and stably reach their intended goals when moving.

If everyday obligations can no longer be met, the general risk of accidents and falls increases and injuries are frequent, a doctor must be consulted. If the usual sporting activities can no longer be carried out, if there is a restriction in the daily routine or if vegetative complaints arise, the person concerned needs help and support. In the case of incontinence, changes in memory, disturbances in the act of swallowing and problems with vocalization, a doctor should be consulted.

Irregular heart rhythms, confusion, orientation problems, behavioral problems and a reduced sense of well-being are complaints that should be presented to a doctor. A diffuse feeling of illness, social withdrawal or visual changes due to weight fluctuations are signs of the organism that should be clarified. Since the disease has a progressive course, a doctor should be consulted as soon as the first discrepancies arise.

Treatment & Therapy

The therapy of multiple system atrophy is always multi-pronged and usually difficult. Only a third of the patients respond to the L-Dopa therapy, which is otherwise common in Parkinson’s disease, and usually only for a limited period of time. Amantadine is a drug that can alternatively help against movement disorders.

The autonomic disorders must be treated symptomatically with copious fluid intake or blood pressure medication to counteract the blood pressure, which is usually too low. Bladder emptying disorders are treated either with pads or with single-use catheterization. In more severe cases of incontinence, medication can also be tried. Accompanying depression should be treated with medication as early as possible.

To alleviate suffering (palliation) in advanced stages, the insertion of a gastric tube or ventilation with a mask is often helpful and necessary. A specific therapy using immunoglobulins is currently being researched.

Outlook & Forecast

The prognosis for multiple system atrophy is very poor. The same applies to both quality of life and life expectancy. The disease progresses quickly. Three to five years after a diagnosis, patients suffer from severe balance disorders. The ability to walk is limited or no longer available. The first sufferers die regularly from the eighth year of symptoms. Science has not yet produced an effective means to effectively combat multiple system atrophy. Only the symptoms can be alleviated so that sufferers do not have to suffer any pain.

The greatest risk of developing multiple system atrophy is in people who are advanced in life. Most patients are in their 60s when they are diagnosed. Statistically, ten diseases per 100,000 inhabitants are documented. According to the current status, men and women face the same risk.

A normal everyday life is no longer possible due to the typical signs. Affected people increasingly need help in everyday life. Last but not least, the diagnosis also leads to retirement. Many patients and their relatives complain about mental stress resulting from the untreatable multiple system atrophy.

Prevention

According to the current state of science, it is not possible to prevent multiple system atrophy.

Aftercare

In the case of multiple system atrophy, in most cases the affected person has only very few and sometimes very limited follow-up measures available. For this reason, the affected person should consult a doctor at an early stage in order to prevent the occurrence of other complications or symptoms. Self-healing cannot occur, so early diagnosis of the disease is paramount.

As a rule, those affected by multiple system atrophy are dependent on taking various drugs that can alleviate the symptoms. The person concerned should always follow all the doctor’s instructions and also pay attention to the correct dosage and regular intake of the medication. If anything is unclear or if you have any questions, it is advisable to consult a doctor first.

Likewise, many of the patients are dependent on the help and care of other people in their everyday life due to multiple system atrophy, whereby care by their own family in particular can have a positive effect on the course of the disease. Further aftercare measures are usually not available to the person concerned. The further course of the disease depends heavily on the cause, so that no general prediction can be given.

You can do that yourself

Everyday and self-help for patients with multiple system atrophy is primarily based on the symptoms and depends on the exact symptoms that are present.

If your blood pressure is too low, support stockings and a resting position with your head slightly elevated are suitable. In this way, the blood circulation is relieved. In addition, an increased fluid intake and a slightly higher salt diet are recommended. If there are problems with the swallowing muscles, the diet should be adjusted accordingly. Dry food that requires intensive chewing should be avoided.

In order to delay the loss of motor skills as much as possible, regular physiotherapy exercises are an approach worth mentioning. Both fine and gross motor skills should be taken into account. A positive side effect of these exercises also affects blood pressure. The same is also recommended for the speech muscles. Regular use can prevent a rapid loss of language ability.

Patients should also be careful not to isolate themselves from their environment despite the disease and the associated symptoms. On the one hand, the exercises already mentioned, especially the language ones, can be better tried out in everyday life, on the other hand, possible psychological effects are weakened.