Amyotrophic lateral sclerosis symptoms diagnosis treatment. Amyotrophic lateral sclerosis (ALS, Charcot's disease)

ALS disease is a rare disease. It is also called amyotrophic lateral sclerosis or Charcot's disease. This ailment affects the central nervous system and was first described in 1865. Its manifestations are almost always very severe and dangerous. It is good that the percentage of cases is relatively small.

ALS disease - what is it?

Amyotrophic lateral sclerosis is a rare disease, but this does not prevent it from being considered the most common type of motor neuron dysfunction. Other alternative names for the disease include Lou Gehrig's disease (in honor of the first patient with such a diagnosis), motor neuron disease, and motor neuron disease. The most famous person with ALS is Stephen Hawking.

What is amyotrophic lateral sclerosis? It develops when degenerative changes begin in the motor nerve cells, due to which the muscles contract normally and correctly. Destructive processes reach the neurons located in the cerebral cortex and anterior spinal horns, the nuclei of the brain. When some motor nerve cells die, others do not come to replace them. As a result, the function for which they were responsible simply ceases to be performed.

Amyotrophic lateral sclerosis - causes

Although ALS disease has been studied for a very long time, it is still difficult to talk about where it comes from. The most accurate fact is that several reasons affect the development of the disease. One of the most important is the change in superoxide dismutase-1. This enzyme protects cells in the body from damage and destruction. When it starts to figure out, ALS disease, what it is, where it comes from, mutations in the chromosomes responsible for coding SOD often become visible.

Why is this happening? In most cases, the tendency to deformities is inherited. In some, the anomaly develops as a result of exposure to some adverse factors, such as:

• autoimmune processes;
• lack of neurotrophic factors;
• the appearance of some excess amount of potassium ions inside the cell;
• smoking;
• destructive effects of exotoxins;
• negative effects of glutamate.

ALS syndrome and ALS disease - the difference

Although the names of the two diagnoses are similar, they are different things. Lou Gehrig's disease appears for reasons still unexplained and can spread to both the spinal cord and the brain. The syndrome develops against the background of pathologies of the spine. In addition, this diagnosis affects only the spinal cord and has a very favorable prognosis, while ALS disease is considered incurable.

Amyotrophic Lateral Sclerosis - Symptoms

The disease in each organism develops in its own way. At different stages of development, ALS disease has different symptoms. As a rule, all of them concern only some physical abilities of a person. Lou Gehrig's disease does not affect the mental development in any way, but it can lead to a very serious one: it is extremely difficult to watch how the body gradually refuses to work, to understand and accept it.

ALS disease can be distinguished by the following features:

• weakness in the body;
• atrophy;
• problems with swallowing;
• speech disorder;
• imbalance;
• sudden mood changes;
• hanging foot;
• spasticity;
• fibrillation.

How does amyotrophic lateral sclerosis start?

The very first signs of amyotrophic lateral sclerosis are usually associated with muscles. In some patients, they begin to go numb, in others they twitch. What is scary, even after the appearance of these symptoms, it is almost impossible to diagnose the disease. In most cases, ALS neuronal disease begins in the lower extremities. The hands are less affected. The muscles of the speech apparatus are affected only with a more complex - bulbar form. As the disease progresses, the symptoms become more pronounced. At some point, the muscles atrophy completely.

What is characteristic of ALS is that the following systems and organs continue to function normally:

• touch;
• smell;
• hearing;
• intestines;
• bladder;
• genitals (in many patients, sexual function is preserved until the last - before the onset of the terminal stage);
• eye muscles;
• heart.

ALS disease - stages

Since the diagnosis of ALS is not an easy process, it is customary to distinguish only two stages of the disease:

• period of harbingers;
• local period.

During the latter, in most cases, when ALS is diagnosed, the last stage occurs, therefore it is very difficult not to determine it. At this point, specialists begin to identify where the lesion is located. During the precursor period, the main symptoms appear, but they are not pronounced enough for doctors to suspect ALS. This is a rare ailment, and many professionals do not have to deal with it even once in their lives.

Clinical variants of the lesion are different. If it develops along the spinal cord, the disease is classified into:

• cervicothoracic;
• lumbosacral;
• bulbar;
• cerebral.

When propagating across, the forms are as follows:

• polymyelitic;
• spastic;
• classical.

Amyotrophic Lateral Sclerosis - Diagnosis

The disease in each organism develops in its own way. That is, there is no list of specific signs by which ALS could be accurately identified. In addition, early symptoms are not always similar to symptoms - clumsiness, for example, or awkwardness in the hands can be attributed to the personal characteristics of a person. The disease, meanwhile, will develop safely.

When suspicions nevertheless arise, the therapist should send the patient to a neurologist who will prescribe a complete examination, including:

1. Blood analysis. Most of all, experts should be interested in the level of creatine kinase, an enzyme that begins to be produced quickly and in large quantities when muscles are destroyed.
2. ENMG. Electroneuromyography helps to find muscles that are losing innervation. Their electrical activity differs markedly from healthy ones.
3. Magnetic resonance imaging. When diagnosed with amyotrophic lateral sclerosis, MRI is needed to rule out other diseases with similar symptoms. This method is not suitable for determining ALS - it will not be able to detect any specific changes in this disease.
4. TMS. Transcranial magnetic stimulation, as a rule, is carried out together with ENMG. The method evaluates the state of motor nerve cells. Its results can be useful in confirming the diagnosis.

ALS - how to treat this disease?

The first question asked by patients with this diagnosis and their relatives is ALS disease, is it curable, and if so, by what means and ways can it be dealt with. The bad news is that there is no one perfect solution to a problem. The disease is rare and not fully explored. The good news is that if you hit three pathological processes in the body at the same time, the disease will develop more slowly.

You need to act on:

• accelerated aptosis;
• disturbed energy metabolism;
• autoimmune inflammatory processes.

There are no drugs that would effectively, once and for all treat ALS, but there are drugs that can:

• relieve symptoms;
• slow down the development of the disease;
• stabilize the normal level of quality of life.

Now the treatment of Charcot's disease is carried out with such medicines as:

• Levocarnitine (solution intended for oral administration);
• Kartinin (capsules);
• Creatine;
• Trimethylhydrazinium propionate (intravenously);
• multivitamin complexes.

Treatment of ALS with stem cells

This method has been gaining popularity in recent years. They are trying to use it in various medical fields and in the fight against ALS too. Stem cells are called those that are not yet fully formed and cannot perform specific functions. But they are able to independently renew themselves and help produce various other cells - nerve, muscle, blood.

There is no confirmation of this, but it is not excluded that in the near future the treatment of amyotrophic lateral sclerosis will be one of the most effective methods. It is expected that it will help to achieve the following results:

• activate the protective and trophic function;
• replace damaged neurons;
• replace glial cells;
• enrich neurons with nutrients.

Amyotrophic lateral sclerosis - naturopathic treatment with folk remedies

Naturopathy is an ancient medical method. Some patients with ALS amyotrophic lateral sclerosis try to treat with it and talk about good results. Which is not strange, given the essence of such therapy. The latter consists in the following - the body's own internal forces may well cope with any ailment, the main thing is to activate them and direct them in the right direction.

Naturopaths are convinced that diseases develop when a person leads an unhealthy lifestyle. That is, if you reconsider your habits, behavior, attitude to life, the diagnosis may disappear by itself. In addition to exploring your own capabilities, naturopathy involves:

• hydrotherapy;
• air baths;
• physical procedures;
• sunbathing;
• prophylactic intake of dietary supplements;
• color meditation;
• bioresonance therapy and the use of many other means.

New in the treatment of amyotrophic lateral sclerosis

The study of the characteristics of this disease is ongoing. More recently, scientists in North Carolina have made progress towards making ALS an effective treatment. They managed to find the part of DNA that is responsible for sending signals along the nerve endings. Just its destruction and causes disease. That is, if everything works out, a medicine will be created that prevents the deformation of nerve cells, and at the same time the disease.

ALS syndrome (amyotrophic lateral sclerosis) is a rare neurological disease. According to statistics, the frequency of pathology is 3 people per 100 thousand. The formation of degenerative-dystrophic abnormalities is due to the death of nerve axons, through which impulses are transmitted to muscle cells. An abnormal process of destruction of neurons occurs in the cerebral cortex, the horns (anterior) of the spinal cord. Due to the lack of innervation, muscle contraction stops, atrophy, paresis develops.

Jean-Martin Charcot was the first to describe the disease, giving the name "lateral (lateral) amyotrophic sclerosis (ALS)." Based on the results of the study, he concluded that in most cases the etiology is sporadic. In 10% of patients, the cause was a hereditary predisposition. It develops mainly after 45 years, in women it is less common than in men. The second name - Lou Gehrig's syndrome - is common in English-speaking countries, an anomaly was assigned in honor of the famous baseball player, who ended his career in a wheelchair due to illness at the age of 35.

Classification and characteristic features

The classification of pathology depends on the location of the lesion. Two types of neurons are involved in motor activity: the main one, located in the cerebral hemispheres, and the peripheral one, located at different levels of the spinal column. The central one sends an impulse to the secondary one, and it, in turn, to the cells of the skeletal muscles. The appearance of ALS will differ depending on the center where transmission from motor neurons is blocked.

At an early stage of the clinical course, the signs are the same regardless of the type: spasms, numbness, muscle hypotension, weakness of the arms and legs. General symptoms include:

1. Episodic appearance of cramps (painful contractions) on the affected area.
2. The gradual spread of atrophy to all parts of the body.
3. Disorder of motor function.

Types of the disease proceed without loss of sensitive reflexes.

lumbosacral shape

It is a manifestation of myelopathy (destruction of the spinal cord), caused by the death of peripheral neurons located in the sacral spine (anterior horns). ALS syndrome is accompanied by symptoms:

1. Weakness of one, then both lower limbs.
2. Lack of tendon reflexes.
3. The formation of initial muscle atrophy, visually determined by a decrease in mass (“shrinkage”).
4. Wavy fasciculations.

The process involves the upper limbs with the same manifestations.

cervicothoracic form

The syndrome is characterized by the death of axons of secondary neurons located in the upper spine, leading to the manifestation of signs:

• decrease in tone in one hand, after a period of time the pathological process spreads to the second;
• muscle atrophy is noted, accompanied by paresis, fasciculation;
• the phalanges are deformed, acquiring the appearance of a "monkey brush";
• foot signs are manifested, characterized by a change in motor function, muscle atrophy is absent.

A symptom of a lesion of the cervical region is a constantly tilted forward head.

bulbar form

This type of syndrome is characterized by a severe clinical course, motoneurons die in the cerebral cortex. The life expectancy of patients with this form does not exceed five years. The debut is accompanied by:

• violation of articulatory function, speech apparatus;
• fixation of the tongue in a certain position, it is difficult for them to move, rhythmic twitching is noted;
• involuntary spasms of mimic muscles;
• swallowing dysfunction due to spasms in the esophagus.

The progression of amyotrophic lateral sclerosis of the bulbar type forms a complete atrophy of the facial and neck muscles. The patient cannot independently open his mouth for eating, communication capabilities are lost, the ability to clearly pronounce words. Increased gag and jaw reflex. Often the disease occurs against the background of involuntary laughter or lacrimation.

high shape

This type of ALS begins with damage to the central neurons, in the process of development it covers the peripheral ones. Patients with a high form of the syndrome do not live to the stage of paralysis, because the muscles of the heart and respiratory organs quickly die off, and abscesses form in the affected areas. A person cannot move independently, atrophy covers the entire skeletal muscles. Paresis leads to uncontrolled defecation and urination.

The condition is aggravated by the constant progression of the syndrome, in the terminal phase the respiratory act is impossible, ventilation of the lungs is required using a special apparatus.

Causes

The syndrome of amyotrophic lateral sclerosis in most cases proceeds with an uncertain genesis. In 10% of patients with this diagnosis, the cause of development was the transmission of a mutated gene from the previous generation in an autosomal dominant way. The etiology of the formation of the disease can be a number of factors:

1. Infectious lesion of the brain, spinal cord with a stable little-studied neurotropic virus.
2. Insufficient intake of vitamins (hypovitaminosis).
3. Pregnancy can provoke ALS syndrome in women.
4. Growth of cancer cells in the lungs.
5. Gastric bypass.
6. Chronic form of osteochondrosis of the cervical region.

The risk group includes people who are constantly in contact with concentrated chemicals, heavy metals (lead, mercury).

Diagnostic studies

The examination provides for the differentiation of ALS syndrome from ALS disease. Independent pathology proceeds without violation of internal organs, mental abilities, sensitive reflexes. For adequate treatment, it is necessary to exclude diseases with similar symptoms by diagnostics:

• spinal craniovertebral amyotrophies;
• residual effects of poliomyelitis;
• malignant lymphoma;
• paraproteinemia;
• endocrinopathy;
• cervical myelopathy with ALS syndrome.

Diagnostic measures to determine the disease include:

• chest x-ray;
• magnetic resonance imaging of the spinal cord, brain;
• electrocardiograms;
• electroneurography;
• studies of the level of functioning of the thyroid gland;
• cerebrospinal, lumbar puncture;
• genetic analysis to detect mutations;
• spirograms;
• laboratory study in the blood of protein, ESR, creatine phosphokinase, urea.

Effective Treatments

It is impossible to completely get rid of the disease, in Russia there is no patented drug that can stop the clinical development. In European countries, Riluzole is used to slow the spread of muscle atrophy. The task of the drug is to inhibit the production of glutamate, a high concentration of which damages brain neurons. Tests have shown that patients taking the drug live a little longer, but still die of respiratory failure.

The treatment is symptomatic, the main task of therapy is to maintain the quality of life, prolong the ability of self-care. In the process of development of the syndrome, the muscles of the organs responsible for the respiratory act are gradually affected. Oxygen deficiency is compensated by the BIPAP, IPPV apparatus used at night. The equipment facilitates the patient's condition, it is easy to use and used at home. After complete atrophy of the respiratory system, the patient is transferred to a stationary lung ventilation apparatus (NIVL).

Conservative treatment of symptoms contributes to:

1. Relief of seizures "Carbamazepine", "Tizanil", "Phenytoin", "Isoptin", "Baclofen", injection of quinine sulfate.
2. Normalization of metabolic processes in the muscle mass with anticholinesterase agents ("Berlition", "Espa-Lipon", "Glutoxim", Lipoic acid, "Cortexin", "Elkar", "Levocarnitine", "Prozerin", "Kalimin", "Pyridostigmine" " Milgamma", "Thiogamma", Vitamins of group B A, E, C).
3. Removal of fasciculations (Elenium, Sirdalud, Sibazon, Diazepam, Mydocalm, Baklosan).
4. Improvement of swallowing function ("Prozerin", "Galantamine").
5. Elimination of pain syndrome with the analgesic "Fluoxetine" with subsequent transfer of the patient to morphine.
6. Normalization of the amount of saliva secreted by Buscopan.
7. Increase muscle mass "Retabolil".
8. Removal of mental disorders with antidepressants (Paxil, Sertraline, Amitriptyline, Fluoxetine).

If necessary, antibiotic therapy is prescribed with the antibiotics Fluoroquinol, Cephalosporin, Carbapenem. Also, the course of treatment includes nootropic drugs: Nootropil, Piracetam, Cerebrolysin.

Patients with ALS syndrome need special devices to make life easier, including:

Neurodegenerative disease, which is accompanied by the death of central and peripheral motor neurons. The main manifestations of the disease are skeletal muscle atrophy, fasciculations, spasticity, hyperreflexia, pathological pyramidal signs in the absence of pelvic and oculomotor disorders. It is characterized by a steady progressive course, leading to death. Amyotrophic lateral sclerosis is diagnosed on the basis of neurological status data, ENG, EMG, MRI of the spine and brain, analysis of cerebrospinal fluid and genetic studies. Unfortunately, today medicine does not have an effective pathogenetic therapy for ALS.

If amyotrophic lateral sclerosis is suspected, the following is necessary: ​​anamnesis (both personal and family); physical and neurological examination; instrumental examinations (EMG, MRI of the brain); laboratory tests (general and biochemical blood tests); serological tests (antibodies to HIV, Wasserman reaction, etc.); research of liquor; molecular genetic analysis (mutations in the superoxide dismutase-1 gene).

When taking an anamnesis, it is necessary to pay attention to the patient's complaints about stiffness and / or weakness in certain muscle groups, muscle twitches and spasms, weight loss of certain muscles, episodes of acute lack of air, speech disorders, salivation, swallowing, shortness of breath (during physical exertion and in the absence of it), a feeling of dissatisfaction with sleep, general fatigue. In addition, it is necessary to clarify the presence (or absence) of double vision, chills, memory impairment.

Neurological examination for suspected amyotrophic lateral sclerosis should include selective neuropsychological testing; assessment of cranial innervation, checking the mandibular reflex; assessment of bulbar functions; the strength of the sternomastoidal and trapezius muscles; assessment of muscle tone (according to the scale of the British Medical Research Council), as well as the severity of motor disorders (according to the Ashfort scale). In addition, it is necessary to study pathological reflexes and coordinating tests (static and dynamic).

Attempts to pathogenetic therapy of amyotrophic lateral sclerosis with other drugs (including anticonvulsants, metabolic agents, antiparkinsonian agents, antioxidants, calcium channel blockers, immunomodulators) were unsuccessful.

The task of palliative therapy is to stop the progression of the main symptoms of amyotrophic lateral sclerosis - dysphagia, dysarthria, fasciculations, spasticity, depression. To improve muscle metabolism, it is recommended to prescribe carnitine, levocarnitine, creatine in courses of 2 months three times a year. To facilitate walking, patients are advised to use orthopedic shoes, walkers, a cane, and in case of deep vein thrombosis of the lower extremities, bandaging the legs with elastic bandages is indicated.

Dysphagia is a fatal symptom of amyotrophic lateral sclerosis leading to cachexia. First, frequent sanitation of the oral cavity is carried out, subsequently the consistency of the food is changed. At the same time, at the earliest stages of the development of dysphagia, it is necessary to have a conversation with the patient, explaining to him the need for endoscopic gastrotomy, focusing on the fact that it will improve his condition and prolong life.

The need for tracheostomy and mechanical ventilation is a signal of an imminent death. Arguments against mechanical ventilation can be the improbability of the subsequent removal of the patient from the device, the high cost of caring for such a patient, technical difficulties, as well as postresuscitation complications (pneumonia, posthypoxic encephalopathy, etc.). Arguments for mechanical ventilation - the desire of the patient to prolong his life.

Forecast

With amyotrophic lateral sclerosis, the prognosis is always unfavorable. An exception may be hereditary cases of ALS associated with certain mutations in the superoxide dismutase-1 gene. The duration of the disease with a lumbar debut is about 2.5 years, with a bulbar one - about 3.5 years. No more than 7% of patients diagnosed with ALS survive more than 5 years.

Amyotrophic lateral sclerosis (ALS, "Hehrig's disease", "motor neuron disease") was first described by the French psychiatrist Martin Charcot in 1869.

In the US and Canada, there is another term - "Lou Gehrig's disease", a famous baseball player who had to end his career at the age of 36 due to amyotrophic lateral sclerosis.

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis is a nervous system disease that rapidly affects the motor neurons of the spinal cord, brainstem, and cortex.

The motor nerves of the cranial neurons (facial, ternary, glossopharyngeal) are involved in the pathological process.

Amyotrophic lateral sclerosis is rare (2-3 people per 100,000) and progresses rapidly.

In medicine, there is another concept - the syndrome of amyotrophic lateral sclerosis. It is provoked by another disease, so the treatment in this case is aimed at eliminating the underlying pathology. If a patient has symptoms of ALS, but their causes are not known, doctors are not talking about the syndrome, but about the disease.

In ALS, motor neurons are destroyed, they stop sending signals from the brain to the muscles, as a result, the latter begin to weaken and atrophy.

The reasons

The reasons that provoke the appearance of this disease have not yet been established. However, scientists offer several theories:

hereditary

It has been established that in 10-15% of cases the disease is hereditary.

Viral

This theory became widespread in the 60s of the 20th century in the USA and the USSR. At this time, experiments were carried out on monkeys. Animals were injected with extracts of the spinal cord of sick people. It was also assumed that the disease can be provoked by the polio virus.

Gennaya

Gene disruption is found in 20% of patients with ALS. They encode the enzyme Superoxide dismutase-1, which converts Superoxide, which is dangerous for nerve cells, into oxygen.

autoimmune

Scientists conducted research and found antibodies that kill motor nerve cells. It has been proven that these antibodies can be formed in severe diseases (Hodgkin's lymphoma, lung cancer, etc.).

neural

This theory was developed by British scientists who believe that the formation of ALS can provoke elements of glia - cells responsible for the vital activity of neurons. If the function of astrocytes that remove glutamate from nerve endings is impaired, the risk of Charcot's disease increases several times.

Among the risk factors, doctors distinguish: hereditary predisposition, age over 50 years, smoking, work associated with the use of lead, and military service.

ALS symptoms

Regardless of the form of the disease, all patients feel muscle weakness, muscle twitches appear, and muscle mass decreases.

The weakening of the muscles increases rapidly, but the eye muscles and bladder sphincter are not affected.

At an early stage of the disease occurs:

• muscle weakness in the ankles and feet;
• hand atrophy;
• impaired motor skills and speech;
• difficulty in swallowing;
• muscle twitching;
• spasms of the tongue, arms and shoulders.

With the development of ALS, bouts of laughter and crying appear, balance is disturbed, atrophy of the tongue appears.

Cognitive functions worsen only in 1-2% of cases of the disease; in other patients, mental activity does not change.

In the later stages, the patient develops depression, interruptions in breathing begin, and the ability to move independently is lost.

Patients with ALS cease to be interested in their loved ones and the outside world, they become capricious, unrestrained, emotionally labile and aggressive. When the respiratory muscles stop working, the person needs mechanical ventilation.

Course of the disease

Initially, symptoms appear that prevent a person from leading a full life: muscle numbness, convulsions, twitching, difficulty speaking. But, as a rule, at the very beginning it is difficult to determine the exact cause of these disorders.

In most cases, ALS is put at the stage of muscle atrophy.

Gradually, muscle weakness spreads and covers new parts of the body, the patient cannot move independently, breathing problems begin.

Patients with ALS rarely suffer from dementia, but their condition leads to severe depression in anticipation of death. At the last stage, a person can no longer eat, walk and breathe on his own, he needs special medical devices.

Forms of the disease

Forms of the disease are distinguished by the location of the damaged muscles.

Bulbarnaya

The cranial nerves are affected (9,10,12 pairs).

Patients with the bulbar form of ALS begin to have problems with speech, they complain of difficulty in pronunciation, it is difficult for them to move their tongue.

With the progression of the disease, the act of swallowing is disturbed, food can pour out through the nose. At the late stage of the disease, the muscles of the face and neck completely atrophy, facial expressions disappear, and patients with ALS cannot open their mouths and chew food.

cervicothoracic

The disease progresses in the upper limbs on both sides.

Initially, discomfort in the hands appears, it becomes difficult for a person to perform complex movements with his hands, write, play musical instruments. On examination, the doctor notices that the muscles of the patient's arms are tense, and tendon reflexes are increased.

In the advanced stages of the disease, muscle weakness progresses and spreads to the forearms and shoulders.

lumbosacral

The first symptom is weakness in the lower extremities.

It becomes more difficult for the patient to do work while standing, climb stairs, ride a bicycle and walk long distances.

Over time, the foot begins to sag, the gait changes, then the leg muscles completely atrophy, the person cannot walk, urinary and fecal incontinence develops.

Almost 50% of patients suffer from the cervicothoracic form of ALS, 25% each are lumbosacral and bulbar.

Diagnostics

The neurologist uses as the main diagnostic methods:

MRI of the brain and spinal cord

Using this method, it is possible to detect degeneration of pyramidal structures and atrophy of the motor parts of the brain.

Neurophysiological examinations

To detect ALS, TKMS (transcranial magnetic stimulation), ENG (electroneurography), EMG (electromyography) are used.

Cerebrospinal puncture

The level of protein content (normal or elevated) is determined.

Biochemical blood tests

In patients with ALS, an increase in creatine phosphokinase by 5 or more times, accumulation of creatinine and urea, and an increase in AST and ALT are found.

Molecular genetic analysis

The gene encoding Superoxide dismutase-1 is being investigated.

But these methods are not enough to identify the diagnosis, in parallel differential diagnosis is used to confirm or exclude diseases:

• brain: dyscirculatory encephalopathy, tumors of the posterior cranial fossa, multisystem atrophy.
• spinal cord: tumors, syringomyelia, lymphocytic leukemia, spinal amyotrophy, etc.
• muscles: myositis, oculopharyngeal myodystrophy, myotonia Rossolimo-Steiner-Kurshman.
• peripheral nerves: multifocal motor neuropathy, Parsonage-Turner syndrome, etc.
• neuromuscular synapse: Lambert-Eaton syndrome, myasthenia gravis.

Treatment

There is no cure for ALS, but it is possible to slow the progression of this disease, increase life expectancy and alleviate the condition of a person.

For this, complex therapy is used:

The drug, which was first used in the treatment of ALS in the UK and the USA. Active substances block the release of glutamine and slow down the process of neuronal damage. The drug must be taken 2 times a day for 0.05 g.

Muscle relaxants and antibiotics help with muscle weakness. To eliminate muscle spasms and twitches, Mydocalm, Baclofen, Sirdalud are prescribed.

To increase muscle mass, the anabolic "Retabolin" is used.

Antibiotics if sepsis develops or infectious complications occur. Doctors prescribe fluoroquinolones, cephalosporins, carbopenes.

vitamins groups B, E, A, C to improve the impulse along the nerve fibers.

Anticholinesterase drugs, which slow down the process of destruction of acetylcholine ("Kalimin", "Prozerin", "Pyridostigmine").

In some cases, it is used stem cell transplant. It prevents the death of nerve cells, promotes the growth of nerve fibers and restores neural connections.

In the later stages, use antidepressants and tranquilizers, non-steroidal painkillers and opiates.

If sleep is disturbed, benzodiazepine preparations are prescribed.

To facilitate movements, chairs and beds with various functions, canes, fixing collars are needed. Doctors advise speech therapy. In the later stages of the disease, a saliva ejector will be needed, and then a tracheostomy so that the patient can breathe.

Non-traditional methods of treatment for ALS do not give a positive result.

Forecast and consequences

Prognosis for ALS patients adverse. The lethal outcome occurs in 2-12 years, as severe pneumonia, respiratory failure or other serious diseases provoked by Gehrig's disease develop.

With the bulbar form and in elderly patients, the period is reduced to 3 years.

Prevention

Measures to prevent ALS medicine are still unknown.

The disease progresses very quickly, there are no cases of successful treatment and restoration of the motor functions of the body. Muscle weakness, which increases gradually, completely changes the life of a person and his family.

But despite the disappointing prognosis and insufficient study of the disease, loved ones should hope that effective therapeutic methods of treatment will be developed in the near future. In the meantime, it is necessary to take measures to alleviate the condition of a person with ALS.

Amyotrophic lateral sclerosis is a dangerous disease that can immobilize a person and lead to death.

Doctors still cannot determine the exact causes of the disease and find effective treatments. At the moment, all that medicine can do is to alleviate the condition of patients with ALS. Not a single patient could completely recover from this disease. It is important to distinguish "ALS disease" from "ALS syndrome". In the second case, the prognosis for recovery is much better.

Let us discuss in detail such a disease as amyotrophic lateral sclerosis. Find out what it is, what are the symptoms and causes. Let's touch on the diagnosis and treatment of ALS disease. And also there will be many other useful recommendations on this topic.

is a neurodegenerative disease accompanied by the death of central and peripheral motor neurons. This leads to gradual atrophy of skeletal muscles, dysphagia, dysarthria, alimentary and respiratory failure. The disease progresses steadily and is accompanied by death.

This determines the relevance of studying the problem. It was first described by the French psychiatrist Jean-Martin Charcot in 1869. For which it received such a second name as Charcot's disease.

In the US and Canada, it is also known as Lou Gehrig's disease. For 17 years he was a top-notch American baseball player. But unfortunately, at the age of 36, he fell ill with amyotrophic lateral sclerosis. And the next year he passed away.

It is known that the majority of ALS patients are people with high intellectual and professional potential. They quickly become severely disabled and die.

The motor analyzer is affected. This is part of the nervous system. It transmits, collects and processes information from the receptors of the musculoskeletal system. It also organizes coordinated human movements.

If you look at the figure below, you will see that the propulsion system is organized in a very complex way.

In the upper right corner we see the primary motor cortex, the pyramidal tract that goes to the spinal cord. It is these structures that are affected in ALS.

Anatomy of the pyramidal tract

Here is the anatomy of the pyramidal tract. Here you see an additional motor area, the premotor cortex.

These transformations transmit signals from the brain to the motor neurons of the spinal cord. They innervate skeletal muscles and regulate voluntary movements.

Amyotrophic Lateral Sclerosis is an unusual neurodegenerative disease! ICD code 10— G12.2.

All the worst with him happens when a person still does not feel anything. At this preclinical stage 50 - 80% of motor neurons die after a genetic failure is realized with the participation of environmental factors. Then, when 20% of resistant motor neurons remain, the disease itself begins.

The pathogenesis of amyotrophic lateral sclerosis

If we talk about the pathogenesis of amyotrophic lateral sclerosis, then first of all we must understand the following. There are various (largely unknown) genetic factors.

They are realized in conditions of selective vulnerability of motor neurons. That is, in those conditions that ensure the normal vital and physiological work of these cells.

However, under pathological conditions, they play a role in the development of degeneration. This subsequently leads to the main mechanisms of pathogenesis.

Motoneurons- These are the largest cells of the nervous system with long conducting processes ( up to 1 meter). They need high energy costs.

Each such motor neuron is a special power plant. It takes on a huge number of impulses and then transmits them to implement coordinated human movements.

These cells need a lot intracellular calcium. It is he who ensures the work of many motor neuron systems. Therefore, the production of proteins that bind calcium is reduced in cells.

The expression of certain glutamate receptors (ampa) and the expression of proteins (bcl-2) that prevent the programmed death of these cells is reduced.

Under pathological conditions, these features of the motor neuron work on the process of degeneration. As a result, there is:

• Toxicity (glutamate excitotoxicity) stimulating amino acids
• Oxidative (oxidative) stress
• Disruption of the cytoskeleton of motor neurons
• Degradation of proteins is disturbed with the formation of certain inclusions
• There is a cytotoxic effect of mutant proteins (sod-1)
• Apoptosis or programmed cell death of motor neurons

Types of disease

Family ALS(Fals) - occurs when the patient in the history of the family has similar cases of this disease. It makes up 15%.

In other cases, when they have more complex inheritance paths (85%), we are talking about sporadic ALS.

Epidemiology of motor neuron disease

If we talk about the epidemiology of motor neuron disease, then the number of new patients per year is somewhere around 2 cases per 100,000 people. The prevalence (the number of patients with ALS at the same time) ranges from 1 to 7 cases per 100,000 people.

As a rule, people from 20 to 80 years old get sick. Although exceptions are possible.

average life expectancy:

• if ALS disease begins with a speech disorder (with a bulbar debut), then they usually live 2.5 years
• if it starts with some kind of motor disorders (spinal debut), then it is 3.5 years

However, it should be noted that 7% of patients live longer than 5 years.

Genetic loci of familial ALS

Here we see many types of familial ALS. More than 20 mutations have been discovered. Some of them are rare. Some are common.

Genetic loci of familial ALS

There are also new ALS genes. We have not shown some of them. But the bottom line is that all these mutations lead to one final path. To the development of damage to the central and peripheral motor neurons.

Classification of motor neuron diseases

Below is a classification of motor neuron diseases.

Norris classification (1993):

• Amyotrophic lateral sclerosis - 88% of patients:
  • bulbar debut of ALS - 30%
  • chest debut - 5%
  • diffuse - 5%
  • cervical - 40%
  • lumbar - 10%
  • respiratory - less than 1%
• Progressive bulbar paralysis - 2%
• Progressive Muscular Atrophy - 8%
• Primary lateral sclerosis - 2%

Bass Variations by Hondkarian (1978):

1. Classic - 52% (when lesions of the central and peripheral motor neurons are evenly represented)
2. Segmental-nuclear - 32% (small signs of a central lesion)
3. Pyramidal - 16% (we see signs of a peripheral lesion not as bright as signs of a central one)

Natural pathomorphosis

If we talk about different debuts, then it should be noted that the sequence of development of symptoms is always certain.

At bulbar debut first there are speech disorders. Then swallowing problems. Then there is paresis in the limbs and respiratory disorders.

At cervical debut the process of violation begins with one hand and then passes to the other. After this, bulbar disturbances and movement disorders in the legs may occur. It all starts on the side on which the primary hand suffered.

If speak about thoracic debut of ALS, then the first symptom that patients usually do not notice is weakness of the back muscles. The state is broken. Then paresis occurs in the arm along with atrophy.

At lumbar debut one leg is affected first. Then the second is captured, after which the disease passes to the hands. Then there are respiratory and bulbar disorders.

Clinical manifestations of Charcot's disease

The clinical manifestations of Charcot's disease include:

1. Signs of damage to peripheral motor neurons
2. Signs of damage to the central motor neurons
3. Combination of bulbar and pseudobulbar syndromes

Fatal complications leading to death:

1. Dysphagia (impaired swallowing) and alimentary (nutritional) malnutrition
2. Spinal and stem respiratory disorders due to atrophy of the main and accessory respiratory muscles

Signs of damage to the central motor neuron

Signs of damage to the central motor neuron include:

1. Loss of dexterity - the disease begins with the fact that a person begins to have difficulty fastening buttons, tying shoelaces, playing the piano or threading a needle
2. Then muscle strength decreases
3. Increased spastic muscle tone
4. Hyperreflexia appears
5. Pathological reflexes
6. Pseudobulbar symptoms

Signs of damage to the peripheral motor neuron

Signs of damage to the peripheral motor neuron are combined with signs of damage to the central:

1. Fasciculations (visible twitches in the muscles)
2. Cramps (painful muscle cramps)
3. Paresis and atrophy of the skeletal muscles of the head, trunk and limbs
4. Muscle hypotension
5. Hyporeflexia

Constitutional symptoms of ALS

The constitutional symptoms of ALS include:

• ALS-associated cachexia(loss of more than 20% of body weight in 6 months) is a catabolic event in the body. It is associated with the death of a large number of cells of the nervous system. In this case, patients are prescribed anabolic hormones. Even cachexia can develop from malnutrition.
• Fatigue(restructuring of the end plates) - in a number of patients, a decrement of 15 - 30% is possible with EMG

Rare symptoms in amyotrophic lateral sclerosis

Here are some of the rare symptoms of amyotrophic lateral sclerosis:

• Sensory disorders. However, it has been shown that in 20% of cases, ALS patients (especially in the elderly) have sensory impairments. This is polyneuropathy. In addition, if the disease begins with the hands, then the patient simply hangs them. They have impaired circulation. Potentials may decrease in these sensory nerves as well.
• Violation of oculomotor functions, urination and stool are extremely rare. Less than 1%. However, more frequent secondary disorders are possible. This is a weakness of the pelvic floor muscles.
• Dementia (dementia) occurs in 5% of cases.
• Cognitive impairment - 40%. In 25% of cases they are progressive.
• Bedsores - less than 1%. As a rule, they occur with severe alimentary malnutrition.

It is worth saying that in the presence of these symptoms, one can doubt the diagnosis.

However, it should always be remembered that if a patient has a typical clinical picture of this disease and has these symptoms, then a diagnosis of ALS with features can be made.

Moderate cognitive impairment and dementia

Let's talk more about mild cognitive impairment and dementia in Lou Gehrig's disease. Here we often see gene mutations C9orf72. It leads to the development of ALS, frontotemporal dementia and their combination.

There are three options for the development of this disorder:

1. behavioral variant- this is when the patient's motivation decreases (apato-abulic syndrome). Or vice versa, there is disinhibition. A person's ability to actively and adequately communicate in society is reduced. Criticism is down. The flow of speech is impaired.
2. executory- Violation of action planning, generalization, fluidity of speech. The logical processes are broken.
3. Semantic (speech)- Fluid and meaningless speech is rarely manifested. However, dysnomia often occurs (they forget words), phonemic paraphasias (damage to the frontal speech zones). They often make grammatical errors and stutter. She has paragraphia (a writing disorder) and oral apraxia (cannot put her lips around the spirograph tube). There is also dyslexia and dysgraphia.

Diagnostic criteria for frontotemporal dysfunction according to D.Neary (1998)

Diagnostic criteria for frontotemporal dysfunction according to D.Neary include such mandatory features as:

• Insidious onset and gradual progression
• Early loss of self-control of behavior
• The rapid emergence of interaction difficulties in society
• Emotional flattening in the early stages
• Early decline in criticism

The diagnosis does not contradict the fact that such a disorder can occur before the age of 65. The diagnosis is called into question when the patient abuses alcohol. If such disorders are divorced acutely, they were preceded by a head injury, then the diagnosis of frontotemporal dysfunction is removed.

The patient is shown below. She has a symptom of empty eyes. This is not a specific symptom. But with ALS, when a person cannot speak or move, this is something to look out for.

To the right is an example where the patient writes in telegraph style. She writes single words and makes mistakes.

See how thick the skin is in ALS. It is difficult for patients to pierce the skin with a needle electrode. In addition, there are difficulties in performing a lumbar puncture.

Revised El Escorial Criteria for ALS (1998)

Reliable ALS is set when signs of damage to the peripheral and central motor neurons are combined at three levels of the central nervous system out of four possible (trunk, cervical, thoracic and lumbar).

Likely- this is a combination of signs at two levels of the central nervous system. Some signs of damage to the central motor neurons are very high.

Probable laboratory susceptible- a combination of signs at the same level of the central nervous system in the presence of signs of damage to peripheral motor neurons in at least two limbs and the absence of signs of other diseases.

Possible ALS- a combination of features at the same level. Either there are signs of damage to the central motor neuron rostral to signs of damage to the peripheral motor neuron, but there are no ENMG data at other levels. Requires exclusion of other diseases.

Suspect- these are isolated signs of damage to the peripheral motor neuron in two or more parts of the central nervous system.

Progression of motor neuron disease

The progression of motor neuron disease can be divided into three types:

1. Rapid - loss of more than 10 points in 6 months
2. Average - loss of 5 - 10 points for six months
3. Slow - loss of less than 5 points in a month

Instrumental methods for diagnosing ALS

Instrumental diagnostic methods are designed to exclude diseases that are potentially curable or have a benign prognosis.

There are two methods of ALS diagnostics:

1. (EMG) - verification of the generalized nature of the process
2. Magnetic resonance imaging(MRI) of the brain and spinal cord - exclusion of focal lesions of the central nervous system, the clinical manifestations of which are similar to those of the onset of MND

Amyotrophic Lateral Sclerosis - Treatment

Unfortunately, there is currently no complete treatment for amyotrophic lateral sclerosis. Therefore, this disease is still considered incurable. At least, cases of recovery from ALS have not yet been recorded.

But medicine does not stand still!

Various studies are constantly being carried out. Clinics use methods that help the patient to ease the tolerance of the disease. We will discuss these clinical guidelines below.

There are also drugs that prolong the life of an ALS patient.

But it is worth saying that it is not necessary to carry out treatment with methods that have not yet been thoroughly investigated. There are some drugs that at first glance can improve the patient's condition. However, over time, everything comes full circle and the disease still begins to progress.

The same applies to stem cells. Studies have been conducted that have shown improvement at first. However, then, the person got worse and ALS disease began to progress again.

Therefore, at the moment, stem cells are not the way of treatment to be chosen. In addition, the procedure itself is very expensive.

Pathogenetic therapy for Lou Gehrig's disease

There are drugs that slow the progression of Lou Gehrig's disease.

- presynaptic inhibitor of glutamate release. Extends the life of patients by an average of 3 months. You need to take while a person maintains self-service. Doses of 50 mg 2 times a day before meals every 12 hours.

In 3 - 12% of cases, the drug causes drug-induced hepatitis, increased pressure. Metabolized in men and smokers. They need a higher dose.

It is worth saying that the slowdown in progression cannot be felt. The drug does not make a person better. But the patient will be sick longer and later ceases to serve himself.

The drug is contraindicated in patients with certain and probable ALS with a disease duration of less than five years with a forced vital capacity of more than 60% and without tracheostomy.

The price of Riluzole in different pharmacies ranges from 9,000 to 13,000 rubles.

NP001 The active substance is sodium chlorite. This medicine is an immune regulator for neurodegenerative diseases. Suppresses macrophage inflammation in vitro and in patients with amyotrophic lateral sclerosis.

3 months after droppers at a dose of 2 mg/kg, sodium chlorite stabilizes the course of the disease. It seems to stop progression.

What can not be applied

For ALS, do not use:

• Cytostatics (aggravate immunodeficiency in malnutrition)
• Hyperbaric oxygenation (aggravates already disturbed carbon dioxide washout)
• Physiological saline infusions for hyponatremia in patients with ALS and dysphagia
• Steroid hormones (cause respiratory muscle myopathy)
• Branched-chain amino acids (shorten life)

Palliative therapy for Charcot's disease

The goal of palliative therapy for Charcot's disease is to reduce individual symptoms. As well as prolonging the life of the patient and maintaining the stability of its quality at a certain stage of the disease.

1. Treatment of fatal symptoms (dysphagia, nutritional and respiratory failure)

Treatment of Non-Fatal Symptoms of ALS

Now consider the treatment of ALS with non-fatal symptoms.

For starters, this reduction of fasciculations and cramps:

• Quinidine sulfate (25 mg twice daily)
• Carbamazepine (100 mg twice a day)

Can give drugs reducing muscle tone:

• Baclofen (up to 100 mg per day)
• Sirdalud (up to 8 mg per day)
• Centrally acting muscle relaxants (diazepam)

Fight against joint contractures:

• Orthopedic shoes (prevention of equinovarus foot deformity)
• Compresses (novocaine + dimexide + hydrocortisone / lidase / ortofen) for the treatment of humeroscapular periatrosis

Myotropic metabolic drugs:

• Carnitine (2 - 3 g per day) course 2 months, 2 - 3 times a year
• Creatine (3 - 9 g per day) course for two months pass 2 - 3 times a year

They also give multivitamin preparations. Neuromultivit, milgamma, lipoic acid preparations by intravenous drip. The course of treatment is 2 months and 2 times a year.

Fatigue treatment:

• Midantan (100 mg per day)
• Ethosuximide (37.5 mg per day)
• Physiotherapy

To prevent subluxation of the head of the humerus with flaccid paresis of the hands, unloading bandages for the upper limbs of the Dezo type are used. You need to wear 3-5 hours a day.

There are special orthoses. These are head holders, stop holders and hand splints.

There are also assistive devices in the form of crutches, walkers or belts for lifting a limb.

There are also special utensils and devices that facilitate hygiene and everyday life.

Treatment of dysarthria:

• Speech recommendations
• Ice applications
• Give drugs that reduce muscle tone
• Use tables with alphabet and dictionaries
• Electronic typewriters
• Multifunctional voice recorders
• Voice Amplifiers
• A computer system with eyeball sensors is used to display speech as text on a monitor (Figure below)

Aspiration pneumonia in ALS

50-75% of patients with neurodegenerations and the elderly have swallowing problems, which occur in 67% of ALS patients. In 50%, aspiration pneumonia is fatal.

Diagnostic methods:

• Videofluoroscopy, APRS scales (aspiration-penetration scale) DOSS (dysphagia outcome severity scale)

Below we see the definition of the density and volume of liquid, nectar and pudding, which the patient should swallow.

Treatment of dysphagia

In the treatment of dysphagia (impaired swallowing), the following are used at the initial stage:

1. Food of a semi-solid consistency with a mixer, blender (mashed potatoes, jelly, cereals, yogurts, jelly)
2. Fluid Thickeners (Resourse)
3. Difficult to swallow dishes are excluded: with solid and liquid phases (soup with pieces of meat), solid and bulk foods (walnuts, chips), viscous foods (condensed milk)
4. Reduce products that increase salivation (fermented milk, sweet candy)
5. Exclude products that cause a cough reflex (spicy seasonings, strong alcohol)
6. Increase the calorie content of food (adding butter, mayonnaise)

Gastrostomy is used to treat progressive dysphagia. In particular, percutaneous endoscopic.

Percutaneous endoscopic gastrostomy (PEG) and enteral nutrition prolong life for ALS patients:

• ALS + PEG group — (38 ± 17 months)
• ALS group without PEG — (30 ± 13 months)

Treatment of respiratory failure

The treatment of respiratory failure is non-invasive intermittent ventilation (NIPPV, BIPAP, NIPPV), two-level positive pressure (inspiratory pressure higher than expiratory pressure).

Indications for ALS:

1. Spirography (FVC)< 80%)
2. Inspiratory manometry - less than 60 cm. Art.
3. Polysomnography (more than 10 episodes of apnea per hour)
4. Pulse oximetry (Pa CO2 ≥ 45 mmHg; decrease in nocturnal saturation ≥ 12% over 5 minutes)
5. arterial blood pH less than 7.35

Indications for breathing apparatus:

• Spinal ALS with forced vital capacity (FVC) 80-60% - isleep 22 (S)
• Bulbar BASS FVC 80-60% - isleep 25 (ST)
• Spinal ALS FVC 60-50% – isleep 25 (ST)
• FVC below 50% - tracheostomy (device VIVO 40, VIVO 50 - PCV, PSV modes)

Artificial (invasive) ventilation is safe, prevents aspiration and prolongs human life.

However, it contributes to the secretion, the risk of infection, complications from the trachea. As well as the risk of locked-in syndrome, addiction 24 hours a day and high cost.

Indications for invasive ventilation:

• Inability to adapt to NPVL or it lasts more than 16-18 hours a day
• For bulbar disorders with a high risk of aspiration
• When NVPL does not provide adequate oxygenation

Criteria limiting the transfer to mechanical ventilation in ALS:

1. Age
2. Disease progression rate
3. Possibility of communication
4. Frontotemporal dementia
5. Relationships in the family
6. Mental illness in a patient
7. Habitual intoxications
8. Fear of death

ALS patients who have been on mechanical ventilation for more than 5 years develop locked-in syndrome (18.2%), a state of minimal sociability (33.1%).

Psychotherapy for amyotrophic lateral sclerosis

Psychotherapy is also very important for amyotrophic lateral sclerosis. It is necessary for both the patient and his family members.

According to statistics, 85% of ALS patients suffer from mental disorders. And 52% suffer family members. At the same time, anxiety disorders predominate in relatives, and depressive disorders in patients.

There are alarms and addiction. That is, the abuse of tobacco, drugs or alcohol. Patients account for 49%, and family members account for 80%. As a result, all this leads to the following consequences.

Pathogenesis of mental disorders in ALS

So, what happens to the human psyche at the stage diagnosis:

• Ambivalence (split) thinking
• Development of anxiety - drug and Internet addiction
• Obsessive thoughts - obsessive syndrome (repetition of examinations with different doctors)
• Violation of the mental state of relatives - comfort disorders (psychiatric cocoon)

At the stage development of neurological deficit:

• Denial of illness (inverted hysteria-like reaction)
• Or there is an acceptance of the disease (depression)

At the stage increasing neurological deficit:

• Deepening depression
• Refusal of treatment

Treatment of mental disorders

In general, in this case, with Charcot's disease, it is imperative to deal with the treatment of mental disorders. This includes:

• Replacement of anticholinergics against salivation (atropine, amitriptyline) with butolotoxin and irradiation of the salivary glands
• Cholinomimetics (low doses of galantamine)
• Atypical antipsychotics - seroquel, possibly in drops - neuleptil
• Do not start treatment with antidepressants, as they increase anxiety. It is better to give something softer (azafen).
• Use of tranquilizers - alprazolam, strezam, mezapam
• Sleeping pills to use only when immobilized
• Pantogam

Occupational Therapy and Physical Therapy for ALS

Let's talk a little about occupational therapy and physical therapy for ALS. You will find out what it is and what it is for.

Physical Therapist helps the patient maintain optimal fitness and mobility, taking into account how this all affects life.

If physical therapy monitors how the patient's life changes, then another person is needed who will evaluate the very quality of the patient's life.

Ergotherapist is a specialist who helps the patient to live as independently and interestingly as possible. The specialist should delve into the peculiarities of the patient's life. Especially for patients with severe cases of the disease.

Stretch marks

Stretching and movements of maximum amplitude prevent contractures, reduce spasticity and pain. Including involuntary convulsive contractions.

You can do stretching with the help of another person or some external additional force. The patient can do this himself with the help of belts. But here you need to remember that with this method a person will waste energy.

Does muscle training help with ALS?

There are studies that say that the use of resistance exercises for the upper limbs in a patient with amyotrophic lateral sclerosis, led to an increase in static strength in 14 muscle groups (in 4 they did not increase).

And this is after 75 days of training. Therefore, any classes should be regular and long.

Progress in muscle strength shown in the study

As you can see, there was a significant increase in muscle strength in most groups. As a result of this activity, it became easier for a person to get items from the top shelf. Or pick up an item and put it on a shelf.

In the therapy itself, movements were made along a trajectory close to those used in PNF. This is the moment when the doctor gives resistance to the movement of the patient. Of course, such resistance should not be very strong. The main thing is to catch the right degree.

As a rule, many movements are made diagonally. For example, take your leg diagonally. The exercises themselves were done with the help of elastic bands (drums). Although they were partially made with the help of resistance with their hands.

Methodology for conducting classes

Here is the training methodology:

• The action was performed with overcoming resistance throughout the movement
• The movement in one direction took 5 seconds
• The session consisted of two sets of 10 movements.
• Rest between sets was about 5 minutes.
• Classes were held 6 times a week:
  • 2 times with a specialist (overcoming the resistance of the hands of a specialist)
  • 2 times with a family member (same arm resistance)
  • 2 times alone with elastic bandages
• The results from the table appeared after 75 days (65 lessons) of the course

What is the effect of force

Why does strength increase even when a motor neuron is damaged? A sedentary lifestyle can lead to cardiovascular detraining.

In other words, those muscles that are not affected also begin to weaken. And it is on their training that the effect of increasing strength is based.

Another detraining occurs because it falls functional support. With a sedentary lifestyle, there is little oxygen. Therefore, exercise with moderate physical activity improves the overall functioning of the body.

Intensity of training in Lou Gehrig's disease

I want to say that exercises with strong resistance do not give any effect. Not only do they not give, they can also harm a patient with Lou Gehrig's disease.

But exercises of moderate intensity lead to an improvement in the work of muscles that are not affected by significant weakness. If the muscle practically does not move, then you should not count on an increase in its strength.

However, there are muscles that make movements, but weakly.

Even people with respiratory failure using non-invasive assisted ventilation may experience improvements in muscle function as a result of moderate exercise.

Regular exercise with moderate resistance helps improve static strength in some muscles.

To determine the significant and insignificant load, the Borg scale is used.

Borg scale

From 0 to 10, we can determine the subjective sensations that a person has when he does exercises.

No matter how much effort the patients make. No matter what exercises and duration of the walk they were offered. Most importantly, their effort should feel like nothing more than "medium" or "almost heavy." That is, the load should not go above level 4.

There is such a calculation that helps determine the reserve of the heart. That is, at what heart rate you can easily and safely train.

• Load intensity= Resting heart rate + 50% to 70% heart reserve
• Heart reserve= 220 - age - resting heart rate

Aerobic training program for ALS

There is also a 16 week aerobic training program for ALS. It is done three times a week. A couple of times we do at home on a bicycle ergometer and on the steppe. Then once in the hospital under the supervision of a physical therapist.

• 1-4 weeks 15 to 30 minutes
• From 5 weeks to half an hour

Step exercises(a step that is climbed and then descended):

• First 5 weeks (3 minutes)
• 6-10 weeks (4 minutes)
• 11-16 weeks (5 minutes)

Classes in the hospital:

1. 5 minutes warm-up (pedaling without load)
2. 30 minutes of moderate exercise (15 minutes cycling, 10 minutes track, 5 minutes step)
3. 20 minutes of strength training (anterior thigh, biceps and triceps)
4. 5 minutes of the final part

And this program also led to the fact that the patients then improved their physical condition.

Principles for restoring daily activities

The exercise therapist and occupational therapist should always think of three things in order to improve a person's daily activities:

1. The specialist must evaluate and optimize the physical capabilities of the patient
2. Given these possibilities, you need to select the optimal posture for each action.
3. Adapt the patient's environment and see how physical abilities can be improved

Walking support for Charcot's disease

Very often, specialists need to solve the problem of supporting the walking of their patient with Charcot's disease. There are several solutions here. You can use elbow crutches because they are easier to lean on.

But sometimes it is enough to use ordinary canes. But they require strength because you need to hold the whole hand. And to walk on slippery surfaces, I recommend buying special "cats" at the pharmacy.

If the foot sags, then a stop holder is needed.

If a person is too tired or simply cannot walk, then a wheelchair will be needed. And what is the optimal model to choose - this issue should be jointly decided by the ergotherapist and the physical therapist.

In a wheelchair, a person should be comfortable, comfortable. In addition, the device itself should not require a lot of energy from the patient.

It is also necessary to take into account the modularity (options) of the stroller. They help carry out daily activities.

For example, a wheelchair may use an anti-tilt device to prevent it from falling. Transit wheels can also be used. So you can drive where a wide stroller does not fit.

Sometimes we have to provide brakes for the maintainer. Headrests, side belts and so on can also be included.

As you can see, the adaptation and selection of a wheelchair is also a very important part for an ALS patient.

seat height

Seat height is also important. From a higher surface, it will be much easier for the patient to get up. Also at a high level, a person will sit more evenly. Indeed, in this case, the back muscles are activated.

Therefore, it is not necessary to seat the patient in a low chair. He will be uncomfortable in it. In addition, it will be much more difficult to remove a patient from such a chair.

So in addition to the stroller, you must adjust the optimal level of the bed and chair.

meal

Eating depends on which table the patient is sitting at. Avoid round tables as they make sitting difficult.

If a person has a bad head and food falls out of his mouth, then in no case make a high headboard. This will make it very difficult for him to swallow.

If you put the patient on a high headboard, then at least make sure that his entire back lies on this headboard. That is, the back should not bend.

If a person lies completely horizontal, then he will have a very low respiratory capacity. If it is raised by 30º, it will be much better.

In the position on the side and on a high headboard it will be better than just on the side. But the worst position is on the back and horizontally.

If sitting, then breathing will be even better! Remember that we always eat sitting down. Therefore, tilting the head forward always helps the pharynx.

There are also many adapted cutlery that are easier to hold by hand. For example, devices with thickened handles. There are even special clamps on the brush and much more.

But be careful if the person has dysphagia! In this case, you need to decide whether to maintain active amateur performance or a safe sip. The latter will always take precedence.

If a person has dysphagia, then it will be difficult for him to think about a fictitious throat and, in addition, to bring this food to himself with a weak hand. Therefore, in this case, you do not need to chase adaptation to self-service.

Drink

There are cutlery that help to carry out drinking. For example, there are special glasses with a cut. As a result, you can drink from such a glass without throwing your head back. The nose drops into this notch, making drinking safer.

There is also a mug with two handles. It is very comfortable to carry with two hands.

However, a special nipple on the top cover can also be dangerous for dysphagia. If you drink from such a glass, then a person will have to throw back his head.

ALS positioning program

In order to coordinate the regime of work and rest, as well as to balance the ability of the ALS patient from excessive fatigue, it is necessary to adhere to a special program (schedule).

Here is a sample positioning program for a severely disabled person who may be upright for a while.

ALS disease - photo

Below is a photo related to ALS disease. All images are clickable to enlarge.