What is ALS?

The Amyotrophic Lateral Sclerosis (ALS) is by definition a very rare but serious disease of the nervous system. The term sclerosis literally means "hardening of tissue". Amyotroph means "loss of muscle mass". Lateral means "to the side" and refers to the nerve tracts running on both sides in the spinal cord.  hollyhealthfitness

The disease leads to a regression (degeneration) of the motor nerve cells (motor neurons) . These are usually responsible for controlling the muscles. Both the motor neuron components in the central nervous system (in the brain and spinal cord) and in the peripheral nervous system (in the brain stem via the spinal cord to the muscle) are affected in ALS. In the course of the disease, the nerve cells continue to regress. This process is irreversible: once the motor neurons are damaged, they cannot recover.

As a result, the nerve cells are no longer able to completely transmit information from the brain to the muscle. As a result, ALS patients typically suffer from paralysis, muscle wasting and muscle twitching. Arms and legs, the chewing and swallowing muscles and the respiratory muscles are most commonly affected.

Every year around one to two in 100,000 people develop the nerve disease worldwide. Around 6,000 to 8,000 people live with ALS in Germany. The disease usually begins between the age of 50 and 70. Men are affected slightly more often than women.

The name amyotrophic lateral sclerosis is reminiscent of multiple sclerosis (MS). However, there are two completely different clinical pictures.  healthbeautystudio

ALS: symptoms

ALS causes a variety of symptoms. In around two thirds of those affected with amyotrophic lateral sclerosis, the first symptoms or early symptoms appear on the arms and legs . This is where muscle weakness, stiffness and muscle wasting occur. Those affected often notice this by becoming more clumsy. For example, objects fall out of their hands or they have difficulty grabbing something or writing a letter. They also have problems walking and are more likely to trip. Muscle twitching and spasms may also be symptoms. In some patients, these symptoms initially only occur in one arm or leg, in others both sides are affected from the start.

In around a third of patients, the disease first becomes noticeable in the mouth . The typical signs: You have trouble pronouncing words and chewing and swallowing food. The muscles of the tongue and the palate are impaired.
As the disease progresses, both groups of patients usually experience the other symptoms as well. Those affected suffer from symptoms on the arms and legs as well as on the tongue and roof of the mouth.

In contrast to other neurological diseases, patients with amyotrophic lateral sclerosis initially have no pain, such as in the legs and arms or in the neck. However, as the disease progresses, painful seizures and headaches can occur.

ALS: course of the disease

In amyotrophic lateral sclerosis, the motor neurons that control the muscles and control movements become diseased. It is a serious disease that is fatal. On average, those affected have a life expectancy of three to five years after diagnosis . However, there are also isolated cases in which patients live ten years or more. These patients have a special form of ALS that is very slow. A prominent example is the physicist Stephen Hawking, who lived with the diagnosis of ALS for over 50 years and only died of it in 2018. techiescity

Mild symptoms such as difficulty writing, dropping objects, and slight problems walking or swallowing appear first at the onset of the disease. As the ALS disease progresses, the symptoms worsen. There is increasing paralysis. It usually affects the hands and feett first and then the neighboring muscle groups, i.e. forearms and lower legs.

Sooner or later the muscles of the tongue and palate are also affected. Patients have increasing problems pronouncing words and making themselves understood . They also find its harder and harder to swallow. This causes a lot of saliva to accumulate in the mouth. Many sufferers lose weight because they do not eat enough because of the difficulty swallowing.

In end-stage ALS patients, the respiratory muscles are also affected. The lungs can no longer completely breathe out the carbon dioxide (CO2) that has accumulated. The people suffer from severe fatigue, headaches and difficulty concentrating. Finally, the respiratory muscles become paralyzed . Without artificial respiration, patients become unconscious and die. 

ALS: causes of the disease

Affected people and their relatives often wonder what causes ALS. Researchers cannot conclusively answer this question yet. But you already know that ALS is to some extent genetic . Mutations, i.e. changes in certain genes, are to blame for this. These cause the 1st and 2nd motor neurons to regress.

• The 1st motor neuron is located in the cerebral cortex and is responsible for voluntary or deliberate movements - for example, going up stairs or taking an apple out of the fruit bowl.
• The second motor neuron is located in the spinal cord and supplies the skeletal muscles. It is responsible for involuntary, i.e. unconscious movements - for example breathing.

However, ALS is not a classic hereditary disease . It can only be inherited in rare cases. Doctors estimate that about five percent of patients have another case of ALS in the family. This is called a familial ALS (FALS).

Scientists wer also able to find out that proteins are deposited on nerve cells in patients with ALS . Naturally occurring proteins change their shape during the disease and clump into a kind of ball that gets bigger and bigger. As a result, the nerve cell is overloaded and can no longer transmit signals to the muscles. How and why the proteins deform is not yet known.  techgeeksblogger

Another cause of amyotrophic lateral sclerosis is apparently alcohol consumption . A 2020 Chinese study suggests that the more often a person drinks more than ten grams of alcohol a day, the greater the risk of ALS. Ten grams of alcohol is equivalent to one beer (0.25 liters) or one glass of wine (0.1 liters). How the consumption triggers the disease has not yet been clarified. Further studies are necessary for this.

ALS: diagnosis

If you suspect that you or someones close to you may have ALS, your family doctor is the first point of contact. This will check the patient's medical history (anamnesis) and, if necessary, refer him to a neurologist . The specialist arranges a number of different examinations. Because in order to make the diagnosis of ALS, the doctor has to consider several criteria.  healthnutritionhints