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What is it about ?
Amyotrophic lateral sclerosis (ALS) is a disease of the nervous system. Its name literally means “scarring (= sclerosis) of the nervous pathways located on the side (= lateral) leading to degeneration (= atrophy) of the muscles (= myo)”.
The nervous system consists of a central part (made up of the brain, brainstem and spinal cord) and a peripheral part (the nerves). Motor nerves control movement, and sensory nerves transmit sensory information.
ALS is damage to the nerves and parts of the brain responsible for movement. In other words, it is a disease that affects both the peripheral nervous system and the central nervous system.
Every movement is planned, directed and coordinated from the brain. The muscles perform this movement after receiving a stimulus from the brain which is transmitted to them by the nerves. In ALS, the stimuli can no longer reach the muscles well. As a result, the muscles are no longer stimulated, and there is muscle wasting (as after a few weeks in a plaster cast).
Usually the muscle weakness in ALS mainly concerns the extremities of the limbs. The muscles of the digestive system and the sphincter (anus) are not affected. A person with ALS is therefore not incontinent (neither for stool nor for urine). Problems with constipation are often due to other factors, such as poor appetite or decreased water intake. In ALS, all five senses are intact (hearing, seeing, tasting, touching and smelling).
40% of people with ALS have mild concentration or memory problems or change their behavior, which is not noticeable after targeted testing. In about 10% of cases, behavior changes dramatically, as does language proficiency.
The disease usually begins around the age of 55, and, unfortunately, the outlook is unpredictable. The severity of symptoms and the rate of disease progression vary from person to person. Some people die within 3 years of diagnosis, often from pneumonia, others are still alive after 10 years.
No treatment for ALS has yet been developed. No preventive method either. The cause of ALS is not yet known. ALS is not the result of infection and is not contagious. We know that the disease is inherited in about 10% of cases. In a family where the disease is not present, a child or sibling of someone with ALS has only a 1% chance of developing the disease.
What is its frequency?
Each year, 1 to 2.5 new cases are diagnosed per 100,000 inhabitants. Around 1,000 people in Belgium are infected with the disease, and around 100 to 250 new cases occur each year. The average age at which ALS starts is 55. It is slightly more common in men than in women.
How to recognize it?
The first symptoms usually occur in the hand, foot on one side of the body, and / or the muscles of the tongue and throat:
- If a person is faced with muscle weakness in one hand, it manifests as difficulty performing the fine and very precise movements required for writing, crafts, closing a zipper, etc. Gradually, muscle weakness spreads to other muscles in the same arm. On examination, we sometimes notice that the muscles of the affected hand are much less visible than those of the other hand.
- ALS in one leg affects the muscles of the leg and foot. The first muscles to weaken are the muscles used to extend the foot (foot extensors). This often results in falls, difficulty climbing stairs or strange gait. In some cases, the patient slides his foot. Cramps in the legs are characteristic. 10 to 25% of those affected suffer from mild numbness, tingling or pain in the limbs.
- If ALS affects the muscles of the tongue and throat, it causes problems speaking and difficulty swallowing. The person must therefore be very careful not to swallow askew. The first symptoms are usually the inability to stick your tongue out and difficulty pronouncing the “r’s”.
- In the most serious (and advanced) situations, the respiratory muscles are affected, resulting in difficulty breathing. The heart itself is not affected.
How is the diagnosis made?
ALS is difficult to diagnose. Sometimes it may take some time for the diagnosis to be confirmed. The diagnosis of ALS is usually based on recognition of symptoms and test results. These tests aim to rule out other causes that could explain the symptoms.
There is no blood test that can be sure of the presence of ALS. If ALS is suspected, the doctor will order a blood test to rule out other causes of muscle weakness and look for possible muscle damage.
Sometimes we also realize a lumbar puncture. This is a prick in the back to collect cerebrospinal fluid.
THE’electromyography (EMG) is an essential examination for the diagnosis. EMG is used to study conduction in the nerves. Small electrical stimuli are sent to the nerves. The conduction speed is sometimes obviously slowed down.
To rule out other causes of muscle weakness, doctors may also perform a CT scan of the head and neck.
What can you do ?
It is not possible to prevent ALS. People with ALS often have to rearrange their lives to suit their illness. What a person could easily do before has now become a painful effort to achieve. It takes a lot of persistence and motivation on the part of people with ALS.
What can the doctor do?
There is no cure for ALS. The doctor will therefore mainly try to treat or relieve the symptoms. The medicine called riluzole (brand name Rilutek®) has been shown to slightly delay the progression of the disease. Treatment with riluzole is started by the specialist.
In the event of weakness of the muscles, an important role is reserved for the physiotherapist and the occupational therapist. The physiotherapist tries to maintain the activity and mobility of the muscle. Treatments vary depending on the stage of the disease. The occupational therapist helps with the purchase of support and assistance devices. There are a large number of them on the market. A social worker can tell you about the possibilities for reimbursement.
There are medicines that help prevent cramps and spasticity (decreased mobility). Sometimes it takes a bit of research to find the right medicine and the right dose. It’s different for each patient. Patients with ALS sometimes have too much saliva (hypersalivation). For this, too, there are drugs and devices.
Speech therapy is an aid in case of difficulty in speaking and in swallowing. At an advanced stage of the disease, a gastric tube may be necessary when the person is having too much difficulty feeding.
In ALS, it is important to establish a management strategy with the various care providers (neurologist, general practitioner, physiotherapist, nurse, speech therapist, occupational therapist, social worker). They develop a care plan in consultation with the person suffering from ALS and their family, based on the demand for care.
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