What is ALS Disease? What are the Symptoms?

What is ALS Disease? What are the Symptoms?

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Amyotrophic lateral sclerosis is a condition that adversely affects the brain and spinal cord. This rare disease is also called ALS. ALS affects the nerves that control speech, swallowing and body movements, causing voluntary muscles to lose their function. The course and speed of the disease varies from person to person. The average life expectancy for people with ALS is 3 to 5 years, but many people can live 10 or more years.

What is ALS Disease? Why Does It Happen?

ALS can strike at any age in adulthood, but it is usually seen between the ages of 40 and 70. Since the nerve cells called motor neurons are located in the anterior horn region of the spinal cord called the medulla spinalis, the disease is also called motor neuron disease or anterior horn disease. Due to the loss of motor neurons that move the muscles, there is a progressive weakness in the muscles.

What are the Symptoms of ALS Disease?

ALS Disease usually manifests itself as progressive muscle weakness. Symptoms of ALS disease are usually as follows. ALS disease can manifest itself in different places. Some of these symptoms are as follows;

  • Progressive muscle weakness manifests itself
  • Difficulty lifting heavy objects
  • Quick fatigue
  • Difficulty swallowing
  • Breathing difficulties
  • Muscle wasting and thinning

Twitching of the muscles can also occur very often, but it should be noted that muscle twitching is also common in normal individuals and does not necessarily indicate a serious disease.

What are the Types of ALS Disease?

ALS symptoms are divided into familial and non-hereditary. 90-95% of ALS patients have no family history. It is not known why ALS disease is caused in findings without a history. The types of ALS disease are as follows;
Classical ALS: It is a progressive neurological disorder characterized by deterioration of neurons. Classic ALS accounts for more than two-thirds of all people with ALS.
Primary Lateral Sclerosis: A progressive neurological disorder in which upper motor nerve cells deteriorate. If nothing happens to the lower motor neurons within two years, the disease usually remains a pure upper motor neuron disease.
Progressive Bulbar Paralysis (PBP): Due to the deterioration of the lower motor nerve cell (neuron), speech, chewing and swallowing become difficult. This problem accounts for about 25% of all patients with ALS.
Progressive Muscular Atrophy (PMA): A progressive neurological disorder in which lower motor neurons lose their function. If the upper motor neurons do not deteriorate within two years, the disorder remains a pure lower motor neuron disorder.
Familial ALS: A progressive neurological disorder that affects many members of the same family. It accounts for 5% to 10% of patients with ALS.

Is it a genetically inherited disease?

There are some genetic types ofALS. Families carrying certain genetic mutations may have ALS frequently. However, genetic cases constitute a very small portion of the total cases. People who have ALS in one of their relatives do not need to fear that they will also develop the disease. Because only 10 percent of ALS cases are caused by genetic causes. The remaining 90 percent of patients develop this disease even though their relatives do not have any disease.

How is ALS treated?

There is not yet a treatment that completely eliminates the disease and provides a chance of complete cure. However, since intensive research is being carried out on this disease, there is the possibility of new treatments being found all the time. In ALS, a drug called riluzole is used, which is known to be effective on the progression of the disease. Studies have shown that this drug slows down the loss of muscle strength to a significant extent. The drug edaravone was also approved for use in the USA last year. Apart from these drugs, there are treatments that can be recommended for respiratory problems that are common in ALS. Since decreased respiration, especially at night, can negatively affect the quality of life of patients, it is recommended to take precautions in the early period. In the later stages of the disease, special treatments may be required for conditions such as difficulty swallowing, muscle wasting and difficulty breathing.

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CreatorNP Istanbul Hospital Editorial Board
Updated At05 March 2024
Created At11 January 2023
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