The amyotrophic lateral sclerosis (ALS) is a nerve disease that causes gradual immobilization and paralysis of muscles. The disease is progressive and incurable. However, supportive therapies can slow down the course and influence it favorably.
What is amyotrophic lateral sclerosis?
Amyotrophic lateral sclerosis (ALS) is a chronic disease of the nervous system in the course of which the skeletal muscles are no longer adequately nourished and atrophy.
The skeletal muscles are used for voluntary movements, for example for the movement of arms and legs. Normally, nerve impulses are sent from the brain via the spinal cord to the muscles, thereby triggering movement. In amyotrophic lateral sclerosis, the nerve cells, the so-called motor neurons, are damaged to such an extent that they can no longer transmit impulses to the muscles.
This leads to the fact that reflexes no longer work and gradually no more movements are possible. The muscles atrophy (atrophy) and stiffen. Amyotrophic lateral sclerosis mainly occurs between the ages of 50 and 70. There are two forms of amyotrophic lateral sclerosis. The sporadic form, that is, it occurs only sporadically, and the family form, which occurs frequently in families.
causes
The causes of amyotrophic lateral sclerosis have not yet been researched. It is known that there are genetic causes in the familial form, but this form is much less common than sporadic amyotrophic lateral sclerosis.
The symptoms of the disease result from a stunted nerve cells and certain brain areas that are responsible for the movement of the 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 for amyotrophic lateral sclerosis. It is believed that damaged proteins are stored in the nerve cells in the spinal cord and brain, causing them to die. Here you can see a parallel to Alzheimer's dementia, for which proteins are also the trigger.
The fact that a mutation (change) in the gene that is responsible for the utilization of damaged proteins has been found in those affected by familial amyotrophic lateral sclerosis strengthens the assumption that proteins are involved in the disease.
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➔ Medicines for muscle weaknessSymptoms, ailments & signs
The symptoms of amyotrophic lateral sclerosis (ALS) are caused by the increasing destruction of nerve cells that are responsible for muscle movements. However, both onset and course of the disease can have different forms. Sometimes the disease begins with small motor failures such as constant stumbling or falling.
Often there are problems holding objects caused by muscle weakness or paralysis of the muscles. Speech or swallowing problems occur less often at the onset 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.
However, it is always an irreversible process that constantly progresses and ultimately leads to death. Typical for ALS are painless paralysis of the muscles combined with increased muscle tension. With the exception of the heart muscle, the eye muscles and the sphincter muscles of the intestine and bladder, all muscle groups can otherwise be affected by the deficits.
Involuntary but painless muscle twitching occurs within the affected muscles. Furthermore, the affected areas are powerless. Muscle cramps and stiffening occur there. Sooner or later, swallowing and speech disorders occur in many cases.
In the context of swallowing disorders, parts of the food can be inhaled and thus enter the respiratory organs. Death occurs after a mean course of the disease of three to five years from pneumonia due to food aspiration, suffocation, refusal to eat, or infectious diseases.
Diagnosis & course
The symptoms of amyotrophic lateral sclerosis are very diverse. It usually starts with various awkwardnesses that mainly affect arms and legs. You bump your toes or stumble while running or have difficulty holding objects in your hand and performing coordinated movements.
Amyotrophic lateral sclerosis can also begin with problems speaking or swallowing, depending on whether the brain areas or the motor neurons are damaged first. The further course also differs from person to person and is hardly predictable. Some patients suffer from painless paralysis, others tend to have spastic (cramping) fits and twitching in the muscles.
Due to the degeneration of the speech muscles, speech becomes more and more indistinct. As amyotrophic lateral sclerosis is progressive, more and more of the body's functions decrease over time. However, every single one of the symptoms can also be the sign of a different illness and therefore a medical examination is absolutely necessary.
The diagnosis must take place via a process of elimination, as there are no clear examination methods for amyotrophic lateral sclerosis. As a rule, blood tests, magnetic resonance imaging (MRI), neurophysiological tests and possibly a CSF test (examination of the brain water) are carried out.
Complications
In amyotrophic lateral sclerosis (ALS for short) there is a progressive destruction of the motor neurons, so that the affected person can move less and less arbitrarily and then causes severe disability. The patients are dependent on wheelchairs and require care, so the quality of life is very limited. This can lead to severe depression in the person concerned, but also in the relatives.
Typically, the patient's nerves that are responsible for the swallowing muscles are affected, so that proper swallowing no longer works properly. In addition, ALS patients choke more easily and so food residues can get into the windpipe. Since those affected can no longer cough hard enough, the person gets severe shortness of breath and, in the worst case, can suffocate.
In addition, the foreign body can strongly irritate the bronchi and lungs, so that pneumonia can develop over time. This can sometimes be fatal for the patient. With ALS, the respiratory muscles are weakened and subsequently paralyzed, so that breathing is severely impaired and the patient is dependent on ventilation.
In the later stages, the patient becomes paralyzed and breathing stops, and the affected person dies. The average survival time for an ALS patient after the diagnosis is made 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 non-specific symptoms. However, their effects are so massive that those affected should see a doctor immediately. The cause of grasping disorders or paralysis, muscle tremors or muscle wasting, swallowing disorders and cramps should be clarified. A visit to a neurologist is necessary.
The diagnostics at the neurologist is extensive. First of all, other neurological diseases such as Parkinson's, polyneuropathies, cerebral haemorrhage, inflammatory nerve diseases and the like must be excluded. So far, there is no concrete evidence that an expert doctor could use to diagnose ALS. It is a diagnosis of exclusion.
If it is established that it is actually amyotrophic lateral sclerosis or ALS, it becomes difficult for the attending physician. Because there is still no therapy that could heal. Doctors can only treat amyotrophic lateral sclerosis as a supplement or delay the course of the disease for some time.
The doctors consulted try to ease the symptoms of ALS patients for as long as possible. In addition to specialists, speech therapists, physiotherapists or occupational therapists can also be consulted. Medicines ensure that overly stressful sequelae of amyotrophic lateral sclerosis are kept in check or alleviated. Since the course of ALS can be individually different, the therapeutic measures are also different.
Doctors & therapists in your area
Treatment & Therapy
The cure of amyotrophic lateral sclerosis is still not possible. However, there are various therapies available that can favorably influence and slow down the course of the disease. On the one hand, a drug treatment is used, which works on the nerve cells and protects them.
The protection of the nerve cells means that they function longer and do not die as quickly. However, to date there is only one drug that has been successfully used in amyotrophic lateral sclerosis. Supportive treatment is given symptomatically, i.e. the symptoms are alleviated.
Symptomatic therapy mainly includes physiotherapy and occupational therapy. Splints and walking aids support the muscles, weakening muscle groups are trained and swallowing and speech disorders are treated. Since the respiratory muscles also become weaker in the advanced stage of amyotrophic lateral sclerosis, the respiratory functions are also supported.
Outlook & forecast
As a result of this disease, those affected suffer from a significant reduction in quality of life and also from further restrictions in everyday life. In some cases, those affected are then dependent on the help of other people and can no longer cope with everyday life on their own.
The patients suffer mainly from severe paralysis and other sensory disorders. These can occur in all regions of the body and thus restrict everyday life. Those affected appear clumsy and continue to suffer from speech disorders or swallowing disorders. These disorders can also make it much more difficult to take food and fluids, leading to malnutrition or deficiency symptoms.
In many cases, the disease cannot be diagnosed early because the symptoms are slow and insidious. Communication with other people can also be significantly restricted by the illness. Patients often continue to suffer from severe fatigue or headaches. The tiredness can usually not be compensated by sleep. Disturbances in concentration and coordination also occur.
As a rule, it cannot be predicted whether direct treatment is possible. Some complaints can be limited. A complete cure is usually not possible.
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➔ Medicines for muscle weaknessprevention
It is not possible to prevent amyotrophic lateral sclerosis. However, treatment at an early stage of the disease has a very positive effect on the course and slows down the progression of symptoms.
Aftercare
Since amyotrophic lateral sclerosis (ALS) is progressive and always fatal in the end, the term aftercare is actually incorrect. Rather, it is a matter of making the symptoms, which are spreading ever wider, more bearable and more bearable for the patient through long-term treatment. The symptoms of this neurodegenerative disease are complex. The medical care is correspondingly intensive during the entire time that the ALS patient has ahead of him.
Instead of follow-up care, which conceptually assumes a later cure or improvement, in amyotrophic lateral sclerosis only co-treatment by physiotherapists, psychologists or other bodies may be indicated. Those affected are dependent on extensive help and support measures - including technical ones - during the entire duration of the illness and in the advanced stage of the disease. As a result, they can be given the best possible quality of life. For example, people with ALS need a special diet at an advanced stage.
With ventilation measures and the help of voice computers or electric wheelchairs, ALS patients can at least ensure their mobility and participation in life to the extent possible. However, it is doubtful whether all therapeutic options are available to all ALS patients. A lot can be done for patients with amyotrophic lateral sclerosis with measures of physiotherapy or occupational therapy. Symptomatic treatment and care is possible, but nothing more.
You can do that yourself
The diagnosis of "amyotrophic lateral sclerosis" is a great burden for those affected. Psychotherapeutic counseling or an exchange in a self-help group can help to adjust to the new situation.
Maintaining mobility is extremely important in order to master everyday life for as long as possible without assistance. Regular physical and occupational therapy helps to learn movement sequences that are adapted to the disease and to compensate for any restrictions that may already arise with the use of aids. By adapting the home environment at an early stage, usual daily routines can often be retained for a longer period of time.
Gripping aids, special cutlery and electrical household appliances are available to help with household activities, which compensate for a lack of muscle strength to a certain extent. Personal hygiene is also made easier through the use of aids. Mobility can be maintained with walking aids or - in an advanced stage - an electric wheelchair.
Speech therapy can counteract speech disorders; if the muscles become increasingly weak, the speech therapist will help you choose a suitable means of communication. If swallowing causes problems, swallowing therapy and the preparation of meals tailored to individual needs can bring relief. Physiotherapeutic measures, which in many cases are combined with the use of a suction device and the assistance of a caregiver, support breathing and coughing up secretions.
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