• Monday May 25,2020

Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a nervous disorder that leads to gradual immobilization and paralysis of the muscles. The disease is progressive and incurable. However, supportive therapies can slow down the course and have a favorable influence.

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS) is a chronic disease of the nervous system, during which skeletal muscle is no longer sufficiently nourished and atrophied.

Amyotrophic lateral sclerosis (ALS) is a chronic disease of the nervous system, during which skeletal muscle is no longer sufficiently nourished and atrophied.

The skeletal muscles are used for voluntary movements, that is, for example, for the movement of arms and legs. Normally, nerve impulses are sent to the muscles from the brain via the spinal cord, triggering the movement. In amyotrophic lateral sclerosis, the nerve cells, the so-called motor neurons, are damaged in such a way that they can no longer transmit impulses to the muscles.

As a result, reflexes no longer function and gradually no movement is possible. The muscles atrophy (atrophy) and stiffen. Amyotrophic lateral sclerosis occurs mainly between the ages of 50 and 70 years. There are two forms of amyotrophic lateral sclerosis. The sporadic form, that is, it occurs only sporadically, and the familial form, which occurs frequently in families.

causes

The causes of amyotrophic lateral sclerosis are still unexplored. While it is known that there are genetic causes in the familial form, this form is much less common than sporadic amyotrophic lateral sclerosis.

The symptoms of the disease are caused by a degeneration of nerve cells and certain areas of the brain that are responsible for the movement of skeletal and facial muscles. However, why this degeneration takes place is still unclear. Research results from 2011 suggest that a disturbed protein metabolism is the trigger of amyotrophic lateral sclerosis. It is assumed that damaged proteins are stored in the nerve cells in the spinal cord and brain and cause them to die. Here one can see a parallel to Alzheimer's dementia, for which proteins are also the trigger.

The fact that familial amyotrophic lateral sclerosis has been found to mutate (alter) the gene responsible for the utilization of damaged proteins strengthens the assumption of protein involvement in the disease.

Symptoms, complaints & signs

The symptoms of amyotrophic lateral sclerosis (ALS) are caused by the increasing destruction of nerve cells, which are responsible for the muscle movements. However, both disease onset and disease course may have different forms. Sometimes the disease starts with minor motor failures, such as constant tripping or falling.

Often, there are problems with holding objects caused by muscle weakness or paralysis of the musculature. Less often speech or swallowing symptoms occur at the beginning of ALS. It is not possible to predict the exact course of the disease. This depends on the order in which certain nerve cells die off.

However, it is always an irreversible process that continues to progress and ultimately leads to death. ALS is characterized by painless paralysis of the musculature combined with increased muscle tension. Apart from the heart muscle, the eye muscles as well as the sphincter muscles of the intestine and bladder, otherwise all muscle groups can be affected by the failures.

Within the affected muscles there are involuntary, but painless muscle twitches. Furthermore, the affected areas are powerless. It comes to muscle cramps and muscle stiffening. Sooner or later, swallowing and speech disorders occur in many cases.

As part of the swallowing disorders, parts of the food can be inhaled and thus enter the respiratory system. Death occurs after a median time course of the disease of three to five years due to pneumonia due to food aspiration, suffocation, food denial or infectious diseases.

Diagnosis & History

The symptoms of amyotrophic lateral sclerosis are very different. It usually starts with various awkwardnesses that mainly affect the arms and legs. You run your toes while walking or stumble or have difficulty holding objects in your hand and performing coordinated movements.

Also, amyotrophic lateral sclerosis may begin to have difficulty speaking or swallowing, depending on whether the brain areas or motor neurons are damaged first. Also the further course shows different from person to person and is not predictable. Some patients suffer from painless paralysis, others are prone to spastic (convulsive) seizures and twitching in the muscles.

Due to the degeneration of the speech muscles, the language becomes more and more obscure. As amyotrophic lateral sclerosis progresses, more and more functions of the body diminish over time. Each of the symptoms can also be the sign of another disease and therefore a medical examination is absolutely necessary.

Diagnosis must be made via an exclusion procedure, as there are no clear screening methods for amyotrophic lateral sclerosis. As a rule, blood tests, magnetic resonance imaging (MRI), neurophysiological examinations and possibly a CSF examination (examination of the brain water) are carried out.

complications

Amyotrophic lateral sclerosis (ALS) causes a progressive destruction of motor neurons, so that the affected person is less and less able to move in the course of the disease and subsequently causes severe disability. The patients are dependent on the wheelchair and are in need of care, so the quality of life is very limited. This can lead to severe depression in those affected, but also in relatives.

Typically, the patient is affected by the nerves that are responsible for the swallowing muscles, so that a proper swallowing does not work properly. In addition, ALS patients swallow more easily and so food remains can get into the trachea. Since those affected can no longer cough enough, the person gets a strong shortness of breath and can choke in the worst case.

In addition, the foreign body can strongly irritate the bronchi and the lungs, so that in the course of pneumonia may arise. This can sometimes be fatal for the patient. In the case of ALS, weakening and subsequent paralysis of the respiratory muscles occur later, so that breathing is severely impaired and patients are dependent on ventilation.

In the late stages it comes to a paralysis of the patient and the end of the respiration, the death of the patient occurs. The average survival time of an ALS patient after diagnosis is three to five years.

When should you go to the doctor?

The problem with amyotrophic lateral sclerosis is that it begins with debilitating but relatively nonspecific symptoms. However, these are so massive in their impact that those affected should go to the doctor immediately. The cause of gripping disorders or paralysis, muscle tremors or muscle wasting, dysphagia and cramps should be clarified. The visit to a neurologist is necessary.

The diagnosis at the neurologist is extensive. First, other neurological disorders such as Parkinson's disease, polyneuropathies, cerebral hemorrhages, inflammatory nerve diseases and the like must be excluded. A concrete proof that an expert physician could diagnose ALS is missing so far. It is an exclusion diagnosis.

If it is established that it is indeed Amyotrophic Lateral Sclerosis or ALS, it will be difficult for the attending physician. For a therapy that could cure, there is not today. The physicians can treat amyotrophic lateral sclerosis only as an adjunct or delay the course of the disease for some time.

The consulted physicians strive to relieve the symptoms of ALS patients for as long as possible. In addition to specialists also speech therapists, physiotherapists or occupational therapists can be consulted. Medicines ensure that overly burdensome sequelae of amyotrophic lateral sclerosis are kept in check or mitigated. Since the course of the ALS can be different in each case, the therapeutic measures are also different.

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Treatment & Therapy

The cure of amyotrophic lateral sclerosis is still not possible. But there are various therapies available that can influence and slow down the course of the disease. On the one hand, a drug treatment is used which acts on the nerve cells and protects them.

Protecting the nerve cells causes them to work longer and not die off so quickly. However, to date there is only one drug that is successfully used in amyotrophic lateral sclerosis. Supportive is treated symptomatically, that is the complaints are alleviated.

Symptomatic therapy mainly includes physiotherapy and occupational therapy. Splints and walking aids support the muscles, you train weaker muscle groups and treat swallowing and speech disorders. As the respiratory muscles become weaker in the advanced stage of amyotrophic lateral sclerosis, respiratory functions are also supported.

Outlook & Forecast

Through this disease sufferers suffer from a significant reduction in quality of life and also in other everyday life. In some cases, those affected are then dependent on the help of other people and can no longer master everyday life alone.

The patients suffer mainly from severe paralysis and other emotional disorders. These can occur in all regions of the body and thus limit everyday life. The affected people seem awkward and continue to suffer from speech disorders or dysphagia. These disorders can also make the intake of food and liquids significantly more difficult, resulting in malnutrition or deficiency symptoms.

In many cases, the disease can not be diagnosed early because the symptoms are slow and insidious. The communication with other people can be significantly limited by the disease. Patients continue to suffer from severe fatigue or headaches. The tiredness can not be compensated by sleep as a rule. Disorders of concentration and coordination also occur.

It usually can not be predicted whether direct treatment is possible. Some complaints can be restricted. Complete healing is usually not possible.

prevention

Prevention of amyotrophic lateral sclerosis is not possible. However, treatment at an early stage of the disease has a very positive effect on the progression and slows the progression of the symptoms.

aftercare

Since amyotrophic lateral sclerosis (ALS) progresses and is always fatal at the end, the term aftercare is actually incorrect. Rather, the goal is to make sustained treatment more bearable and bearable for the patient. The condition of this neurodegenerative disease is complex. The medical care during the entire time that the ALS patient is still facing is correspondingly intensive.

Instead of follow-up, which conceptually assumes a later cure or recovery, amyotrophic lateral sclerosis may be indicated only by co-treatment by physiotherapists, psychologists, or other agencies. The affected persons are dependent on extensive assistance and support measures - also technical kind - during the entire disease duration and in the advanced stage of the illness. This can give them the best possible quality of life. For example, ALS sufferers need a special diet in the advanced stage.

With ventilation and the help of voice computers or electric wheelchairs, the ALS patient can ensure his or her mobility and participation in life to at least a reasonable extent. However, whether all ALS patients all therapeutic options are available, may be doubted. With physiotherapy or occupational therapy, however, a lot can be done for patients with amyotrophic lateral sclerosis. Symptomatic treatment and care is possible, but not more.

You can do that yourself

The diagnosis "Amyotrophic Lateral Sclerosis" is a great burden for those affected. A psychotherapeutic consultation or the exchange in a self-help group can help to adapt to the new situation.

In order to master everyday life as long as possible without help, the maintenance of mobility is extremely important. Regular physiotherapy and occupational therapy helps to learn movements adapted to the disease and to compensate for any limitations that may occur with the use of aids. By adjusting the home environment early, habitual daily routines can often be maintained for a longer period of time.

To help with household activities, gripping aids, special cutlery and household electrical appliances are available that compensate for missing muscle power to a certain extent. Also the body care is facilitated by the use of aids. Mobility can be maintained with walking aids or - in the advanced stage - an electric wheelchair.

A logopedic therapy can counteract speech disorders, with increasing muscle weakness helps the speech therapist in the selection of a suitable means of communication. If swallowing causes problems, swallowing therapy and food preparation tailored to individual needs can bring relief. Physiotherapeutic measures, which in many cases are combined with the use of an aspirator and the assistance of a caregiver, help with breathing and exhaling secretions.


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