What is amyotrophic lateral sclerosis?
Amyotrophic lateral sclerosis (ALS) is a degenerative disease. It is a disease of the neurons in the brain, brain stem and spinal cord that control the movement of voluntary muscles, causing disability.
Approximately 5 out of every 100,000 people in the world are affected by ALS. It is also known as Lou Gehrig’s disease.
Prognosis of the disease
The prognosis is severe. The disease usually results in death 3 to 5 years after diagnosis. There are exceptions and it is estimated that 1 in 4 people survive more than 5 years after ALS is diagnosed. There are even cases that survive for several more years, usually with the help of a ventilator or similar.
ALS affects the neurons of the brain.
Symptoms of amyotrophic lateral sclerosis
Symptoms do not usually occur before the age of 50, but may occur in younger people. The most common symptom is loss of muscle strength and coordination, which progressively worsens.
This weakness initially affects the legs or arms, or when swallowing or breathing. As the disease progresses, more parts of the body are affected. When muscle weakness is present, the following symptoms are seen:
- Difficulty breathing
- Difficulty climbing stairs, walking or lifting weights
- Difficulty swallowing, drooling or gagging
- Hoarseness and voice changes
- Speech problems, slower speech
- Neck muscles can no longer support the weight of the head, which droops
In addition, other symptoms such as depression, muscle stiffness (spasticity), muscle twitching (fasciculations), muscle cramps or weight loss may also occur.
It rarely causes dementia and most people with the disease can think normally and have their senses intact.
Medical tests for amyotrophic lateral sclerosis
If any of the above symptoms are present, a physical examination will be done to check for:
- There is weakness, usually in a specific area.
- Fasciculations of the tongue
- Muscle spasms or tremors
- Stiff or awkward gait
- Increased or decreased joint reflexes
- Difficulty controlling crying or laughter
- Loss of gag reflex
Other evaluations may also include the following:
- Blood tests to rule out other conditions
- Checking for lung involvement with a breathing test
- Check for disease or injury to the neck
- Electromyography to check for improperly functioning nerves or muscles
- Check for family history of the disease
- CT or MRI scan of the head
- Swallowing analysis
- Spinal tap
What are the causes of amyotrophic lateral sclerosis?
In most cases, the origin of the disease is unknown. It is estimated that one in ten cases is due to a genetic defect.
It should also be noted that if a family member has it, it can be transmitted hereditarily and is therefore a risk factor.
Can it be prevented?
It is not possible to prevent the disease, but a geneticist can be consulted to detect a history of ALS.
Treatments for amyotrophic lateral sclerosis
There is no specific cure for ALS and it must be treated by a multidisciplinary team. The goal is to try to delay symptoms and to improve the patient’s lifestyle.
Rehabilitation and physical therapy may be important to improve muscle function and the patient’s overall health. In addition, the use of a wheelchair or various orthopedic devices may also be necessary to help with this muscle function.
What specialist treats it?
ALS should primarily be treated by a neurologist, although many specialists such as physical therapists, speech therapists or digestive system specialists will be involved.