Amyotrophic lateral sclerosis (ALS) is a chronic progressive disease of the central nervous system. The disease occurs as a result of the death of motor nerve cells in the brain and spinal cord. In this case, that part of the nervous system is affected, which is responsible for the voluntary movements of the skeletal muscles. In a small number of patients, amyotrophic lateral sclerosis is hereditary. In most cases, the causes of ALS are unknown. Currently, various hypotheses of ALS occurrence are being discussed, for example, the accumulation of toxic metabolic products in the nervous system, which causes the death of neuromotor cells (motoneurons).

In this disease, the first and second motor neurons are affected. The first motor neuron is of central origin, located in the brain, and continues its process in the spinal cord. A second neuron connects the spinal cord to the skeletal muscles. The main symptoms of the disease differ depending on which cells are affected – in the brain or spinal cord. There are 4 stages of the disease (early stage, middle stage, late-stage, and final stage). 

Stages of ALS development

ALS is a relentlessly progressive disorder. The rate of progression between individuals is variable and history usually reflects a gradual deterioration over time until death occurs.

Early stages:

  1. Musculature
  • Muscles can be weak and soft, as well as stiff, tense, and spastic. Muscle cramps and twitching (fasciculation) occur, and muscle atrophy is also possible.
  • Symptoms may be limited to one area of the body, or mild symptoms may affect more than one area. 
  1. Physical effects
  • The person may experience fatigue, poor balance, speak indistinctly, stumbling when walking, or other minor symptoms.
  • Sometimes this stage occurs before the diagnosis shows ALS.

Middle stages:

  1. Musculature
  • Symptoms are becoming more common.
  • Some muscles are paralyzed. Fasciculations may continue.
  1. Physical effects
  • Unused muscles can cause contractures that become stiff, painful, and sometimes deformed.
  • In the event of a fall, the person will not be able to stand up on his own.
  • Driving is prohibited.
  • Weakness in the swallowing muscles can cause choking, difficulty eating, and salivation.
  • Weakness of the respiratory muscles can cause respiratory distress, especially when lying down.
  • Some people experience bouts of uncontrollable and inappropriate laughing or crying (pseudobulbar affect) but the person usually does not feel particularly sad or happy. 

Late stages:

  1. Musculature
  • Most of the muscles are paralyzed.
  • The muscles that help move air into and out of the lungs are severely damaged. 
  1. Physical effects
  • Mobility is extremely limited and to meet the most personal needs the patient needs help.
  • Poor breathing can cause fatigue, blurred thinking, headaches, and sensitivity to the monograph. (Respiratory failure is the leading cause of death in ALS.)
  • It is impossible to speak, eat and drink.

Final stage:

  • Most deaths from ALS are the result of respiratory failure, a process that slowly progresses over several months. 
  • Much less common causes of death in ALS include malnutrition due to difficulty swallowing, pulmonary embolism (blockage of one of the arteries in the lungs), abnormalities in the electrical system of the heart called cardiac arrhythmias, and pneumonia due to aspiration (when eating), or fluid enters the lungs).
  • Hospice care (in-house) aims to provide comfort and quality of life by supporting the physical, emotional, and spiritual needs of the person with ALS and their families.  

Symptoms of ALS 

Symptoms of ALS

Symptoms usually do not appear before the age of 50, but there are precedents of the disease in people at an earlier age. People who suffer from ALS have less muscle strength and increase, which decrease with the progression of the disease, and in the last stages of the disease, a person can no longer perform elementary actions: move around, get up from a chair and even swallow. The muscles that are responsible for breathing and swallowing may also be the first muscles affected by the disease. With the course of the disease, more and more group lesions. ALS does not affect the functioning of the senses. Disturbances in the functioning of the bladder and intestines, as well as disturbances in a person’s ability think, are rare. Symptoms include:

  • difficulty breathing,
  • problems with swallowing: a feeling of slight suffocation, increased salivation, vomiting,
  • weakening of all muscle groups,
  • muscle contractions (fasciculation),
  • paralysis,
  • speech problems
  • voice change, hoarseness,
  • weight loss.

Conclusions

Due to the fact that with ALS, irreversible processes occur in the body, the main principle of treatment is to slow down the development of symptoms. It is important to note that therapies and devices have been developed to control symptoms and disease occurrence. Medical developments help preserve patients’ independence in daily life and can prolong life. There is no specific model for the development of amyotrophic lateral sclerosis; the disease proceeds individually for each patient.