Amyotrophic Lateral Sclerosis (ALS, ALS): Causes, Symptoms, and Treatment

Amyotrophic lateral sclerosis (ALS, Charcot's disease, Lou Gehrig's disease, motor neuron disease) is a progressive neurological disease that he suffered from, among others. the outstanding physicist, Stephen Hawking. In his case, amyotrophic lateral sclerosis took an extremely chronic form. More than 50 years passed from the moment of her diagnosis at the age of 21 to her death. The essence of ALS is the paralysis of motor neurons, which leads to the gradual impairment of the functions of all muscles in the body and, consequently, to death. What are the causes and symptoms of amyotrophic lateral sclerosis? How is the treatment and rehabilitation of ALS patients? What is the prognosis for ALS?

Contents:

1. Amyotrophic lateral sclerosis (SLA, ALS) - causes
2. Amyotrophic lateral sclerosis (ALS, ALS) - symptoms
3. Amyotrophic lateral sclerosis (SLA, ALS) - diagnosis
4. Amyotrophic lateral sclerosis (ALS, ALS) - treatment, rehabilitation and prognosis
5. Amyotrophic lateral sclerosis (ALS, ALS) - diet

Amyotrophic lateral sclerosis(LatinSLA sclerosis lateralis amyotrophica , English ALS amyotrophic lateral sclerosis , Charcot's disease, Lou Gehrig's disease, motor neuron disease) is a progressive neurological disease. Its essence is the gradual paralysis of peripheral nerve fibers responsible for the motor functions of the muscles and neurons in the brain responsible for movement. This paralysis inevitably leads to death.

Amyotrophic lateral sclerosis usually affects the elderly (60-70 years of age), usually men. Young people are diagnosed much less frequently, but it happens that - as was the case with Lou Gehring - he died 2 years after diagnosis, at the age of 38. However, perhaps the most famous patient in the world with amyotrophic lateral sclerosis was Stephen Hawking, who has lived for over 50 years since his diagnosis at the age of 21 (in 1963).

>>>Stephen Hawking is dead. The outstanding scientist died at the age of 76

>>>Stephen Hawking - quotes, wife, movie, illness, children. An outstanding scientist is dead

Amyotrophic Lateral Sclerosis - Causes

For the most part, the cause of the disease is unknown, albeitthere are genetic risk factors for this disease.¹Most authors support the hypothesis of a complex etiology for ALS that arises from the interaction of genetic factors with environmental factors.¹

Amyotrophic lateral sclerosis (ALS, ALS) - symptoms

Damage to peripheral nerve fibers leads to:

• muscle weakness, paresis or atrophy - then there are movement problems, i.e. more frequent dropping of objects, stumbling or falling over due to weakness and partial atrophy of muscles and possibly their stiffness. Partial atrophy of the muscles of the hands, arms and legs are noticeable, especially in the so-called the shoulder girdle, i.e. arms, shoulder blades, collarbones

SLA is progressive, which means that the muscle weakness and wasting mentioned above are starting to build up. A patient who was unable to perform precise movements with his hand, e.g. fasten buttons, will not be able to perform this activity as the disease develops.

• muscle contracture
• muscle tremors - As the disease is progressive, the muscle weakness may initially affect, for example, only the hand muscles. Then the patient may experience hand muscle spasms, weakness and other strange sensations. Tremors are sometimes described by patients as "worms crawling under the skin", appearing at first mainly on the hands. Only with time does paresis of the hand appear, followed by the paresis of the entire limb. It should be realized that the patient, although unable to move, does not lose feeling because the disease does not attack the neurons responsible for receiving stimuli
• the paralysis affects all muscles, including the head and neck, therefore the symptom of ALS also means difficulties with holding the head, talking, chewing and swallowing (due to atrophy of the muscles of the tongue, mimic muscles and the muscles of the esophagus)

Slower walking due to more frequent falls will lead to the use of the walking frame until the use of the stroller is a necessity.

When the disease affects the intercostal muscles and the diaphragm, it leads to respiratory failure and death.

Amyotrophic lateral sclerosis (SLA, ALS) - diagnosis

In order to assess muscle performance and disease progression, the following is performed:¹

• electrophysiological tests - are performed in patients with suspected ALS in order to confirm the involvement of the lower motor neuron and to exclude other disease processes
• EMG (Muscle Electromyography) - Provides evidence of motor cell involvementin the brain
• ENG (muscle electroneurography) - performed in order to exclude diseases of the peripheral nerves and diseases of the neuromuscular junction
• Transcranial Magnetic Stimulation (TMS) - allows for non-invasive assessment of cortico-motor pathways
• magnetic resonance imaging (MRI)

The symptoms of amyotrophic lateral sclerosis can resemble those of other diseases. About 10% of patients initially suspected of ALS have another condition.¹Therefore, in the differential diagnosis, the following should be considered:

• conduction block multifocal motor neuropathy
• myasthenia gravis
• multiple sclerosis
• pusky band
• postpolio team
• Denny-Brown-Foley syndrome (mild fasciculations and cramps, occurring in he althcare professionals who are familiar with the disease; they do not develop muscle weakness or atrophy)
• Hirayama syndrome (one limb muscle wasting)

Amyotrophic lateral sclerosis (ALS, ALS) - treatment, rehabilitation and prognosis

Treatment of amyotrophic lateral sclerosis aims to alleviate the symptoms of the disease and slow down its course. The therapy uses Riluzole - a drug that reduces muscle spasticity. It is also necessary to rehabilitate (preferably in water), which e.g. will activate the preserved muscle functions and prevent joint stiffness.

In the advanced stage of the disease, when the muscles in the mouth and esophagus are paralyzed, speech therapy and a special diet are necessary. In the event of a significant weakening of the swallowing reflex, feeding through a gastric tube or gastrostomy is used.

The average survival time from the onset of disease symptoms is 3 years, but it happens that the patient lives longer than 10 years.

Stephen Hawking survived half a century of ALS

Amyotrophic lateral sclerosis (ALS, ALS) - diet

A common symptom in ALS is dysphagia, a swallowing disorder. It causes an increased risk of aspiration, malnutrition, weight loss and dehydration. In ALS there is an increased metabolic effort, therefore patients require an increased supply of calories.¹

What can we do to avoid trouble swallowing?²

If you have trouble taking pills in the form of pills, crush them and try to take them with yoghurt or thick juices (not clear liquids).

• avoid eating lying down as it may lead to choking
• eat sitting upright. You can use a cushion that is inserted between the back and the backrest of the lumbar seatspine, which will make it easier to maintain a straight position. If you cannot hold your head on your own, you can wear a brace during a meal
• When planning meals, assume that the eating time will be longer, if you eat calmly and without stress
• do not eat in a hurry. Make sure the atmosphere during the meal is cheerful
• do the best you can, chew every bite
• avoid eating dry and powdery foods, e.g. shortbread cookies or wholemeal bread, as they can choke
• do not tilt your head back when swallowing as it is easier to choke in this position. If, however, you have a problem with swallowing a bite, try tilting your head slightly forward, because in this position it is easier to swallow
• when you have difficulty swallowing solid foods, try to modify your diet to semi-liquid, i.e. mix food, use soups but dense and nutritious, meals, porridges, etc. Remember that this diet contains adequate amounts of nutritional value needed by the patient

In accordance with current guidelines, consideration of introducing an alternative route of feeding should be considered when body weight is more than 10%.¹

In the case of severe dysphagia, percutaneous endoscopic gastrostomy (PEG,percutaneus endoscopic gastrostomy ), percutaneous radiological gastrostomy (PRG,percutaneus radiologic gastrostomy) ) and nasogastric tube (NGT).¹

How do patients deal with the symptoms of amyotrophic lateral sclerosis?

Source: x-news.pl/Dzień Dobry TVN

Bibliography:

1. Kubiszewska J. Kwieciński H., Amyotrophic lateral sclerosis, "Postępy Nauk Medycznych" 2010, no. 6

2. Guide for SLA / MND Patients, Dignitas Dolentium Association.