home Thyroid Symptomatic treatment of bass syndrome. Symptoms and causes of amyotrophic lateral sclerosis. Tips for improving the condition of patients with ALS

Symptomatic treatment of bass syndrome. Symptoms and causes of amyotrophic lateral sclerosis. Tips for improving the condition of patients with ALS

Update: December 2018

Amyotrophic lateral sclerosis, or Lou Gehrig's disease, is a rapidly progressive disease of the nervous system characterized by damage to motor neurons. spinal cord, cortex and brain stem. also in pathological process motor branches of cranial neurons (trigeminal, facial, glossopharyngeal) are involved.

Epidemiology of the disease

The disease is extremely rare, approximately 2-5 people per 100,000. It is believed that men over 50 years of age are more often affected. Lou Gehrig's disease makes no exceptions for anyone; it affects people of all sizes. social status and various professions (actors, senators, laureates Nobel Prize, engineers, teachers). The most famous patient was world baseball champion Loi Gering, after whom the disease took its name.

In Russia, amyotrophic lateral sclerosis received wide use. Currently, the number of sick people is approximately 15,000-20,000 in the population. Among famous people Russia, having this pathology, we can mention the composer Dmitry Shostakovich, the politician Yuri Gladkov, and the pop singer Vladimir Migulya.

Causes of amyotrophic lateral sclerosis

The disease is based on the accumulation of pathological insoluble protein in the motor cells of the nervous system, leading to their death. The cause of the disease is currently unknown, but many theories exist. The main theories include:

Viral - this theory was popular in the 60-70s of the 20th century, but was never confirmed. Scientists in the USA and USSR conducted experiments on monkeys, injecting them with extracts of the spinal cord of sick people. Other researchers tried to prove participation in the formation of the disease.
Hereditary - in 10% of cases the pathology is hereditary;
Autoimmune - this theory is based on the discovery of specific antibodies that kill motor nerve cells. There are studies proving the formation of such antibodies against the background of other severe illness(for example, when lung cancer or Hodgkin's lymphoma);
Genetic - 20% of patients have disorders of genes encoding very important enzyme Superoxide dismutase-1, which converts toxic nerve cells Superoxide to oxygen;
Neuronal - British scientists believe that glial elements, that is, cells that ensure the vital activity of neurons, are involved in the development of the disease. Research has shown that when astrocytes, which remove glutamate from nerve endings, the likelihood of developing Lou Gehrig's disease increases tenfold.

Classification of amyotrophic lateral sclerosis:

Symptoms of amyotrophic lateral sclerosis

Any form of the disease has the same onset: patients complain of increasing muscle weakness, decreased muscle mass and the appearance of fasciculations (muscle twitching).

Bulbar form of ALS characterized by symptoms of damage to the cranial nerves (9, 10 and 12 pairs):

Those who are ill have deteriorated speech and pronunciation, and it becomes difficult to move their tongue.
Over time, the act of swallowing is disrupted, the patient constantly chokes, and food can pour out through the nose.
Patients feel an involuntary twitching of the tongue.
The progression of ALS is accompanied by complete atrophy of the muscles of the face and neck; patients completely lack facial expressions, they cannot open their mouths or chew food.

Cervicothoracic variant The disease primarily affects the patient’s upper limbs, symmetrically on both sides:

Initially, patients feel a deterioration in the functionality of the hands, it becomes harder to write, play musical instruments, perform complex movements.
At the same time, the arm muscles are very tense, increased tendon reflexes.
Over time, weakness spreads to the muscles of the forearm and shoulder, they atrophy. The upper limb resembles a hanging whip.

Lumbosacral form usually begins with a feeling of weakness in the lower extremities.

Patients complain that it has become more difficult for them to do work, stand on their feet, walk long distances, climb the steps.
Over time, the foot begins to sag, the leg muscles atrophy, and patients cannot even stand on their feet.
Pathological tendon reflexes (Babinsky) appear. Patients develop urinary and fecal incontinence.

Regardless of which variant predominates in patients at the beginning of the disease, the outcome is still the same. The disease progresses steadily, spreading to all muscles of the body, including the respiratory ones. When the respiratory muscles fail, the patient begins to need artificial ventilation lungs and constant care.

In my practice, I observed two patients with ALS, a man and a woman. They were distinguished by their red hair color and relatively young age (up to 40 years). Outwardly, they were very similar: there was no hint of muscles, an amicable face, and their mouth was always slightly open.

Such patients die in most cases from concomitant diseases(pneumonia, sepsis). Even with proper care, they develop bedsores (see), hypostatic pneumonia. Realizing the severity of their illness, patients fall into depression, apathy, and cease to be interested in the outside world and their loved ones.

Over time, the patient's psyche is subjected to strong changes. The patient I observed for a year was distinguished by his capriciousness, emotional lability, aggressiveness, and lack of restraint. Carrying out intelligence tests showed a decrease in his thinking, mental abilities, memory, and attention.

Diagnosis of amyotrophic lateral sclerosis

The main diagnostic methods include:

MRI of the spinal cord and brain– the method is quite informative; it reveals atrophy of the motor parts of the brain and degeneration of pyramidal structures;
cerebrospinal puncture– usually reveals normal or increased content squirrel;
neurophysiological examinations– electroneurography (ENG), electromyography (EMG) and transcranial magnetic stimulation (TCMS).
molecular genetic analysis– studies of the gene encoding Superoxide dismutase-1;
biochemical blood test– reveals a 5-10-fold increase in creatine phosphokinase (an enzyme formed during muscle breakdown), a slight increase in liver enzymes (ALT, AST), accumulation of waste in the blood (urea, creatinine).

What happens in ALS

Due to the fact that ALS has similar symptoms to other diseases, differential diagnosis is made:

brain diseases: posterior tumors cranial fossa, multiple system atrophy,
muscle diseases: oculopharyngeal myodystrophy, Rossolimo-Steinert-Kurshman myotonia
systemic diseases
diseases of the spinal cord: lymphocytic leukemia or lymphoma, spinal cord tumors, spinal amyotrophy, syringomyelia, etc.
diseases peripheral nerves: Personage-Turner syndrome, Isaacs neuromyotonia, multifocal motor neuropathy
myasthenia gravis, Lambert-Eaton syndrome - diseases of the neuromuscular junction

Treatment of amyotrophic lateral sclerosis

Treatment of the disease is currently ineffective. Medications and proper care for the patient only prolong life expectancy without ensuring complete recovery. Symptomatic therapy includes:

Riluzole (Rilutek)– a well-proven drug in the USA and Great Britain. Its mechanism of action is to block glutamate in the brain, thereby improving the functioning of Superoxide Dismutase-1.
RNA interference is a very promising method of treating ALS, the creators of which were awarded the Nobel Prize in Medicine. The technique is based on blocking the synthesis of pathological protein in nerve cells and preventing their subsequent death.
Stem cell transplantation– studies have shown that stem cell transplantation into the central nervous system prevents the death of nerve cells, restores neural connections, improves the growth of nerve fibers.
Muscle relaxants – eliminate muscle spasms and twitching (Baclofen, Sirdalud).
Anabolics (Retabolil)– to increase muscle mass.
Anticholinesterase drugs(Prozerin, Kalimin, Pyridostigmine) – prevent the rapid destruction of acetylcholine in neuromuscular synapses.
B vitamins(Neurorubin, Neurovitan), vitamins A, E, C - these drugs improve the conduction of impulses along nerve fibers.
Antibiotics wide range actions(3-4 generation cephalosporins, fluoroquinolones, carbopenems) – indicated for the development of infectious complications, sepsis.

IN complex therapy necessarily include feeding through a nasogastric tube, massage, exercise therapy with a doctor, and consultations with a psychologist.

Forecast

Sadly, the prognosis for amyotrophic lateral sclerosis is unfavorable. Patients die literally within a few months or years, average duration life in patients:

only 7% live more than 5 years
with bulbar debut - 3-5 years
for lumbar - 2.5 years

A more favorable prognosis for hereditary cases of the disease associated with mutations in the superoxide dismutase-1 gene.

The situation in Russia is overshadowed by the fact that patients are not provided with proper care, as evidenced by the fact that Riluzote, a drug that slows the course of the disease, was not even registered in Russia until 2011, and only in the same year the disease itself was included in the list " rare." But in Moscow there are:

Fund for helping patients with amyotrophic lateral sclerosis at the Marfo-Mariinsky Mercy Center
G.N.Levitsky Charitable Foundation for ALS Patients

Finally, I would like to add about the Ice Bucket Challenge charity event that took place in July 2014. It was aimed at raising funds to support patients with amyotrophic lateral sclerosis and became quite widespread. The organizers managed to raise more than $40 million.

The essence of the action was that a person either doused himself with a bucket ice water and captures it on video, or donates a certain amount of money to a charitable organization. The event became quite popular due to the participation of popular performers, actors and even politicians.

Represents a severe organic pathology unknown etiology. It is characterized by damage to lower and upper motor neurons and a progressive course. ALS (amyotrophic lateral sclerosis) ends invariably with death. Next, we will find out how the pathology manifests itself and whether there is a possibility of getting rid of the disease.

General information

Amyotrophic lateral sclerosis is considered incurable pathology. It affects the central nervous system. Pathology also has some other names: Louis-Gehrig, for example. not always simple. The fact is that recently the range of ailments has increased significantly, in certain clinical manifestations of which it is not pathology as such, but the syndrome of lateral amyotrophic sclerosis. Due to this, modern specialists consider the most important task differentiation and clarification of the etiology of the disease.

Amyotrophic lateral sclerosis: symptoms

Manifestations of pathology are associated primarily with damage to the lower motor neuron. Amyotrophic lateral sclerosis, the symptoms of which include atrophy, weakness, fasciculations, has signs of damage to the corticospinal canal. The latter represent spasticity and strengthening of the tendon and without. The corticobulbar tracts may be involved. This, in turn, will accelerate the development of pathology at the level brain stem. Amyotrophic lateral sclerosis affects older people. The pathology does not develop until the age of 16.

Features of manifestation

In people suffering from amyotrophic lateral sclerosis, initial stages pathology is noted (progressive course) with hyperreflexia. This sign is the most important clinical manifestation. Pathology can begin to develop from any striated muscle fibers. Amyotrophic lateral sclerosis can have several forms: bulbar, high, lumbosacral and cervicothoracic. Reason fatal outcome As a rule, the respiratory muscles become affected after approximately 3-5 years. Amyotrophic lateral sclerosis has several characteristic features. One of the most common manifestations is the progressive weakness of muscle fibers of one of the upper limbs. As a rule, it begins in the hand. When developing from proximally located tissues, the course of the pathology is more favorable. At the onset of the disease, associated with the development of muscle weakness in the hand, thenar fibers are involved in the process. The condition is manifested by weakness of adduction (adduction) and opposition thumb. As a result, grasping with the index and thumb, fine motor control is impaired. Patients with amyotrophic lateral sclerosis experience difficulty getting dressed (buttoning up) and picking up small objects. If the dominant hand is affected, difficulties arise in writing and everyday activities at home. The lumbosacral form is considered relatively favorable.

Typical course of pathology

In this case, there is a stable progressive involvement of the muscles of the same limb in the process. Subsequently, it begins to spread to the second arm before affecting the bulbar fibers or legs. Amyotrophic lateral sclerosis may begin in the muscles of the tongue, mouth, face, or lower limbs. At the same time, subsequent lesions do not “catch up” with the initial ones. In this regard, life expectancy is considered to be the shortest with the bulbar form. Patients die while remaining essentially on their feet, without experiencing paralysis of the lower extremities.

Further development

Amyotrophic lateral sclerosis is accompanied by various combinations of signs of paralysis (bulbar and pseudobulbar). This is mainly manifested by dysphagia and dysarthria, and subsequently respiratory disorders. A characteristic feature Almost all forms of pathology are considered to be early strengthening of the mandibular reflex. When swallowing liquid food Dysphagia is observed more often than when taking solids. At the same time, it should be said that the use of the latter becomes significantly more difficult as the pathology progresses. Weakness appears in the masticatory muscles, the soft palate begins to droop, the tongue becomes atrophic and immobile. Amyotrophic lateral sclerosis begins to be accompanied by a continuous flow of saliva and anarthria. Swallowing becomes impossible, and the likelihood of aspiration pneumonia increases. It should also be noted that cramps (periodically occurring cramps in the calves) are observed in all patients and are often considered the first signs of pathology.

Spread of atrophy

It should be said that it is quite selective. The patient's hands show damage to the hypothenar, thenar, interosseous and deltoid muscles. On the legs, atrophy develops in areas that dorsiflex the foot. Tissue from the bulbar muscles is damaged soft palate and language. Oculomotor fibers are considered the most resistant to atrophy.

Practical observations

In pathology, sphincter disorders are also rare. One of amazing features disease is the absence of bedsores in bedridden patients paralyzed for a long time. It has also been established that dementia appears extremely rarely in pathology. Only a few subgroups are considered an exception: the familial form and the “parkinsonism-AD sclerosis-dementia” complex. Cases with uniform involvement of lower or upper motor neurons have also been described. In this case, damage to any one zone may predominate. For example, the upper motor neuron may be more affected. In this case, they talk about primary lateral sclerosis. The predominant damage may be in the lower motor neuron. In this case, they talk about anterohorn syndrome.

Electromyography

This method has a special diagnostic value among paraclinical methods of studying pathology. Using electromyography, widespread damage is detected in the cells of the anterior horns (including in preserved (clinically) muscles) with fasciculations, fibrillations, changes in the potentials of motor units, positive waves against the background of the normal speed of propagation of excitation along the fibers in the sensory nerves.

Diagnostics

To conduct research to identify pathology, you must have:

Signs of damage to the lower motor neuron. It is also necessary to have EMG confirmation in preserved (clinically) muscles
Symptoms of progressive upper motor neuron damage.

To make a diagnosis, the absence of:

The diagnosis is confirmed by fasciculations in 1 or more zones, EMG signs, normal speed propagation of excitation along sensory and motor fibers. Among the categories it should be noted:

Reliable BAS. In this case, we are talking about the identified signs of damage in the lower motor neuron and the upper motor neuron in three areas of the body.
Likely. In this case, there are symptoms of damage in the lower motor neuron and the upper one in two parts of the body.
Possible. Signs of damage to the lower and upper motor neurons in one area of the body or signs of damage to the second in 2-3 zones were revealed. In the latter case we're talking about about manifestations of ALS in one limb, progressive bulbar palsy and the primary form.

Treatment of amyotrophic lateral sclerosis

The first and only drug used today for pathology is Riluzole. It is approved in Europe and the USA, but is not registered in the Russian Federation and cannot be officially recommended by a doctor. The medication does not eliminate the pathology. But this is the only drug that has a beneficial effect on the life expectancy of patients. The drug "Riluzole" helps reduce the concentration of glutamate (a neurotransmitter in the central nervous system) released during the passage of a nerve impulse. An excess of this compound, as was established during observations, has a destructive effect on the neurons of the spinal cord and brain. According to the results clinical trials It was found that those who took the medication lived on average 2-3 months longer than those who used a placebo.

Antioxidants

This class of nutritional compounds helps the body prevent damage that can cause free radicals. It is believed that individuals with amyotrophic lateral sclerosis may be more susceptible to their negative influence. Today, research is being conducted aimed at identifying, detecting beneficial effects supplements containing antioxidants. Some of these drugs that have been tested have not proven to be effective.

Concomitant therapy

Various activities make life easier for patients and make it more comfortable. In particular, we are talking about relaxation. It is believed that reflexology, aromatherapy and massage help eliminate stress and reduce anxiety, relax muscles, and normalize lymph and blood flow. Such procedures help get rid of pain due to the synthesis of endogenous painkillers produced by the body itself, as well as endorphins. This or that medicine or any procedure must be recommended by a doctor. For amyotrophic lateral sclerosis, self-medication is not recommended at all.

Amyotrophic lateral sclerosis (ALS) is an incurable, progressive disease of the central nervous system in which the patient experiences damage to the upper and lower motor neurons, which causes muscle atrophy and paralysis. The frequency of this pathology is about 2-7 cases per 100 thousand people. Most often, the disease is diagnosed in patients over 50 years of age.

Classification of amyotrophic lateral sclerosis

Scientists have not yet created a unified comprehensive classification of ALS. There are several approaches to classifying the disease. For example, the North American approach involves highlighting the following types ALS: sporadic, familial, sporadic endemic. The classification of amyotrophic lateral sclerosis provides for the following forms of the disease: bulbar, lumbosacral, cervicothoracic and primary generalized. There are also several variants of the disease: mixed, pyramidal and segmental-nuclear.

Clinical picture of amyotrophic lateral sclerosis

To the most common initial symptoms diseases include cramps (painful muscle spasms), lethargy and weakness in the distal arms, bulbar disorders, atrophy of the leg muscles, weakness in the shoulder girdle. In addition, for different options The disease is characterized by various clinical manifestations.

Classic variant of ALS (with cervical onset). The first sign of the disease is the formation of asymmetric paraparesis with pyramidal signs. In addition, spastic paraparesis appears, which is accompanied by hyperreflexia. Over time, the patient begins to show signs of bulbar syndrome.
Segmented variant of ALS (with cervical onset). This type of disease is manifested by the formation of asymmetrical flaccid paraparesis, which is accompanied by hyporeflexia. At the same time, patients retain the ability to move independently for some time.
Classic variant of ALS (with diffuse onset). This variant of the pathology usually begins to manifest as flaccid asymmetric tetraparesis. In addition to this, patients are also diagnosed with bulbar syndrome, which manifests itself in the form of dysphagia and dysphonia. The patient often experiences a sharp decrease in body weight, shortness of breath and fatigue.
Classic variant of ALS (with lumbar onset). This variant of pathology begins with lower flaccid paraparesis. Later, symptoms such as muscle hypertonicity and hyperreflexia are added. At the onset of the disease, patients can still move independently.
Pyramidal variant of ALS (with lumbar onset). This type of disease begins with the occurrence of lower asymmetric paraparesis, which is then joined by upper spastic paraparesis.
The classic variant of ALS (at the onset of the disease there is bulbar palsy). This disease is characterized by dysphagia, dysphonia, dysarthria, upper and lower asymmetric paraparesis. The patient is rapidly losing weight and has respiratory problems.
Segmented variant of ALS (with bulbar palsy). Nasophonia, dysphagia, and dysarthria are considered characteristic of this variant of the disease. As in the previous case, the patient loses body weight and develops respiratory pathologies.

Etiology and pathogenesis of amyotrophic lateral sclerosis

The exact causes of amyotrophic lateral sclerosis are still being investigated by scientists. However, several factors can be named that provoke the disease. For example, about 5% of diseases have a hereditary etiology. At least 20% of cases are associated with mutations in the superoxide dismutase-1 gene. Scientists have proven that important role High activity of the glutamatergic system plays a role in the onset of the disease. The fact is that excess glutamic acid provokes overexcitation and sudden death of neurons. The molecular genetic mechanism of the pathology has also been proven. It is caused by an increase in the level of DNA and RNA in cells, which ultimately leads to disruption of protein synthesis.

Scientists also identify several predisposing factors that play an important role in the occurrence of ALS. First of all, such factors include age. The fact is that the disease usually develops in patients aged 30-50 years. It is worth remembering that only about 5% of patients have hereditary predisposition to BAS. In the vast majority of cases of ALS, the cause of the pathology cannot be determined.

For early course The disease is characterized by symptoms such as convulsions, twitching, muscle numbness, difficulty speaking, and weakness in the limbs. Since such symptoms are typical for many neurological diseases, diagnose ALS on early stage difficult. In most cases, the disease can be diagnosed at the stage of muscle atrophy.

Depending on the disease affected different parts body, distinguish limb ALS and bulbar ALS. In the first case, patients experience deterioration in ankle flexibility, awkwardness when walking, and they begin to stumble. Bulbar ALS is manifested by difficulty speaking (nasal sound, difficulty swallowing). Soon the patient finds it difficult to move or can no longer move independently. Usually the disease does not have a detrimental effect on the patient’s mental abilities, but it leads to severe depression. In most cases, about three to five years pass from the appearance of the first symptoms to death.

Diagnosis of amyotrophic lateral sclerosis

Since ALS is an incurable disease that rapidly shortens a person’s life, the patient’s examination must be comprehensive and accurate. It is extremely important to put correct diagnosis the patient in order to begin timely relief of his main symptoms, as this can prolong the patient’s life. The examination plan usually includes a history of life and illness, a neurological and physical examination, MRI of the spinal cord and brain, EMG, and laboratory tests.

History taking and examination

Diagnosis of the disease begins with a detailed interview with the patient. Namely, the doctor needs to clarify whether the patient complains of muscle spasms and twitching, weakness and stiffness, impaired movement of the hands, speech, walking, swallowing, salivation, frequent lack of air, weight loss, fatigue, shortness of breath during exercise physical exercise. In addition, the doctor should ask whether the patient has noticed double vision, memory impairment, crawling sensations on the body, or urinary problems. It is imperative to ask the patient about his family history - whether he has any relatives who are sick chronic disorders movements.

The main purpose of the physical examination is to assess the patient's constitution, weigh him, measure his height, and calculate his body mass index. The neurological examination usually includes neuropsychological testing. When assessing bulbar functions, the doctor pays attention to the timbre of the voice, speed of speech, pharyngeal reflex, the presence of tongue atrophies, and paresis of the soft palate. In addition, during the examination, the strength of the trapezius muscles is checked.

Instrumental research methods

Main instrumental method Needle EMG is considered to diagnose the disease. This technique allows you to identify signs of the disease such as acute or chronic denervation. In the early stages of the disease, stimulation EMG is ineffective because it does not detect noticeable signs of ALS.

In the process of diagnosing the disease, doctors also use neuroimaging methods. Great importance MRI of the spinal cord and brain plays a role in the differential diagnosis of ALS. During MRI, in 17-67% of patients it is possible to identify symptoms of degeneration of the pyramidal tracts and atrophy of the motor cortex of the brain. However, it is worth noting that this technique is ineffective when diagnosing the disease in patients with bulbar syndrome.

In the process of diagnosing ALS, many laboratory tests. In particular, doctors can prescribe clinical and biochemical tests blood, cerebrospinal fluid examination, serological studies. However, the only effective and reliable method of analysis is still considered to be molecular genetic analysis. The presence of mutations in the superoxide dismutase-l gene is considered a suspicion for ALS.

Differential diagnosis

Since the symptoms of amyotrophic lateral sclerosis are similar in many respects to the manifestations of other neurological pathologies, doctors must carry out differential diagnosis. The most accurate diagnosis can be made using MRI of the brain and spine. First of all, ALS must be differentiated from muscle diseases, which include Rossolimo-Steinert-Kurshman dystrophic myotonia, myositis with cellular abnormalities, and oculopharyngeal myodystrophy.

It is also necessary to distinguish ALS from spinal cord pathologies:

chronic vertebrogenic ischemic myelopathy;
Kennedy bulbospinal amyotrophy;
syringomyelia;
tumors;
familial spastic paraplegia;
chronic lymphocytic leukemia;
hexosaminidase deficiency;
lymphoma.

Differential diagnosis is also necessary in order to distinguish the disease from systemic pathologies, lesions of the neuromuscular synapse, and brain pathologies such as multiple system atrophy, dyscirculatory encephalopathy, and syringobulbia.

Treatment of amyotrophic lateral sclerosis

The main goals of treatment for amyotrophic lateral sclerosis are considered to be to slow the progression of the disease, as well as eliminate its symptoms, which significantly worsen the patient’s quality of life. It should be remembered that ALS is a serious incurable disease that shortens a person’s life expectancy. That is why the doctor has the right to inform the patient of the diagnosis only after a comprehensive and thorough examination.

Treatment of the disease includes drug and non-drug therapy. The latter implies security measures. The patient must limit physical exercise, which may accelerate the progression of ALS. In addition, it is very important to eat properly and nutritiously. Drug therapy is divided into two types: pathogenetic and palliative.

Pathogenetic therapy

To date, the only drug that can slow the progression of ALS is riluzole. It has been proven that taking it can prolong the patient’s life by an average of three months. This drug is indicated for patients whose disease duration is less than 5 years. The patient should receive 100 mg of the drug daily. To avoid the risk of drug-induced hepatitis, every three months it is necessary to check the levels of AST, ALT and LDH. Since men and smokers have lower concentrations of riluzole in their blood, they should either limit their smoking or quit smoking altogether. bad habit. You will need to take the drug for life.

Scientists have repeatedly tried to use pathogenetic therapy and other drugs. However, such experiments did not prove effective. Among them were:

The effectiveness of taking it has also not been proven high doses Cerebrolysin, despite the fact that this drug is able to slightly improve the condition of patients.

Palliative care

Palliative therapy is intended to eliminate a complex of symptoms of the disease and thereby improve the patient’s quality of life. To eliminate certain symptoms of ALS, the following techniques are used:

spasticity - baclofen and tizanidine are prescribed;
fasciculations (muscle twitching) - in addition to baclofen and tizanidine, carbamazepine is also prescribed;
depression and emotional lability- fluoxetine and amitriptyline;
walking impairment - walkers, canes and strollers are indicated to eliminate this symptom;
deformation of the feet - the patient should wear orthopedic shoes;
neck paresis - a rigid or semi-rigid head holder is indicated;
thrombosis of the veins of the lower extremities - elastic leg bandaging is prescribed;
fatigue quickly - execution gymnastic exercises, as well as taking amantadine and ethosuximide;
humeroscapular periarthrosis - compresses with procaine, dimethyl sulfoxide solution, hyaluronidase are prescribed;
oral hypersecretion syndrome - to eliminate this symptom, correction of dehydration, portable suction, mucolytics and bronchodilators are indicated;
sleep apnea syndrome - fluoxetine;
respiratory disorders - periodic non-invasive ventilation is prescribed;
dysphagia - compliance special diet(exclusion of dishes with hard and dense ingredients, preference for pureed dishes, soufflés, porridges, purees);
dysarthria - taking muscle relaxants, applying ice to the tongue, using electronic typewriters, a special computer typing system, following speech recommendations compiled by the British ALS Association;
drooling - regular sanitation oral cavity(you need to brush your teeth three times a day, rinse the cavity often antiseptic solutions), restriction of use fermented milk products, taking atropine and amitriptyline.

To improve muscle metabolism, a patient with ALS can be prescribed the following medications: creatine, carnitine, levocarnitine solution, trimethylhydrazinium propionate. Multivitamin therapy is also indicated for patients, which involves taking multivitamins (neuromultivit, milgamma) and thioctic acid.

In most patients with ALS, the disease is accompanied by serious motor impairments, including limited mobility. Of course this delivers severe discomfort a patient who constantly needs help from other people. Eliminate some movement disorders Orthopedic correction techniques help. The doctor must explain to the patient that the use aids does not indicate his disability, but only reduces the difficulties caused by the disease.

The most life-threatening symptom of the disease is considered to be respiratory failure. Its earliest symptoms will be morning fatigue, vivid dreams, daytime sleepiness, and dissatisfaction with sleep. For detection respiratory failure At an early stage, polysomnography and spirography are performed. To eliminate apnea, medication and non-invasive ventilation are indicated. It has been proven that these techniques can prolong a patient’s life by one year. If the patient needs assisted breathing for more than 20 hours, the doctor raises the question of a complete transition to invasive ventilation.

Patients who have passed initial examination or a repeated diagnosis of the disease, must remain under outpatient observation. As any new symptoms appear, they should also receive qualified advice. Patients must take most medications regularly. Only vitamins and myotropic drugs are taken in courses in stages.

Every three months the patient must undergo spirography. If he takes riluzole regularly, he needs to have LDH, AST, and ALT checked every six months. If the patient has dysphagia, blood glucose levels and trophic status should be measured periodically. Patients have a choice of treatment regimen: they can either stay at home or stay in a hospice.

Prognosis for amyotrophic lateral sclerosis

The prognosis for patients with ALS largely depends on the course of the disease. It has been proven that about 80-90% of patients who have severe respiratory complications, die within 3-5 years after the first signs of the disease appear. The remaining 10% of patients have a benign course of the disease. The duration of the disease is significantly reduced in the presence of the following factors: the patient’s age is less than 45 years, bulbar onset of ALS, rapid progression of the disease.

Slow down the progression of the disease and prolong the period of illness during which the patient does not need constant outside care.
Reduce the severity of individual symptoms of the disease and maintain a stable level of quality of life.

Indications for hospitalization

Primary examination.
Carrying out percutaneous endoscopic gastrostomy.

Ethical and deontological aspects of managing patients with amyotrophic lateral sclerosis

The diagnosis of ALS can be communicated to the patient only after a thorough examination, which is not always a one-time examination. Sometimes a repeat EMG is necessary. According to the Helsinki Convention on Bioethics (1997), doctors must notify patients with incurable diseases about a diagnosis that requires decisions related to impending death. The diagnosis of ALS should be communicated in a sensitive manner, emphasizing the variability in disease progression. There are known cases of extremely slow progression (with homozygous carriage of the D90A mutation) and in isolated sporadic cases. It should be remembered that 7% of patients live longer than 60 months. The neurologist needs to establish close contact with the patient and his family and communicate the diagnosis in the presence of family and friends, in a calm, comfortable environment for the patient, without haste. The patient's questions should be answered with an expectation of his emotional reaction. You cannot tell a patient that nothing can be done to help him. On the contrary, he should be convinced to be observed by a neurologist or in a specialized center every 3-6 months. It is necessary to emphasize that individual symptoms respond well to treatment.

Drug therapy

Pathogenetic therapy

The only drug that significantly slows the progression of ALS is riluzole, a presynaptic inhibitor of glutamate release. The use of the drug allows to prolong the life of patients by an average of 3 months. Riluzole is indicated for patients with definite or probable ALS, after excluding other probable causes lesions of peripheral and central motor neurons, with a disease duration of less than 5 years, forced vital capacity (FVC) of more than 60%, without tracheostomy. For patients with possible ALS lasting less than 5 years, FVC<60% и трахеостомией для предотвращения аспирации без зависимости от аппарата ИВЛ рилузол, согласно мнению экспертов, также может быть показан. Препарат назначают в дозе 100 мг в день вне связи с приёмом пищи. Каждые 3 мес необходимо мониторировать уровень АЛТ, АСТ и ЛДГ из-за риска развития лекарственного гепатита. Концентрация рилузола в сыворотке крови несколько ниже у мужчин и курильщиков, поэтому рекомендуется уменьшить количество выкуриваемых сигарет или прекратить курение. Рилузол следует принимать пожизненно.

Attempts have been made to pathogenetic therapy of ALS with other drugs, but all of them, including neutrotrophic factors, xaliproden (a low-molecular ligand of neurotrophic factor receptors), anticonvulsants (lamotrigine, gabapentin, topiramate, etc.), metabolic agents (gangliosides, branched amino acids, creatine), antiparkinsonian drugs (selegiline), antibiotics (cyclosporine), antioxidants (acetylcysteine, vitamin E), calcium channel blockers (nimodipine, verapamil), immunomodulators (interferon beta, immunoglobulin) and others were ineffective.

There is no convincing data on the effectiveness of high doses of Cerebrolysin, although its use led to a general activation of patients.

Palliative care

Methods for correcting the main symptoms of ALS are presented in table. 34-5.

Table 34-5. Palliative care for ALS

Symptom/indication	Correction methods
Fasciculations, cramps	Carbamazepine 100 mg twice a day, baclofen 10-20 mg per day or tizanidine with a gradual increase in dose to 8 mg / day
Spasticity	Baclofen 10-20 mg per day or tizanidine with a gradual increase in dose to 8 mg/day, diazepam at a dose of 2.5-5 mg 3 times a day
Depression, emotional lability	Amitriptyline up to 100 mg/day at night, fluoxetine 20 mg/day at night
Improved muscle metabolism	Carnitine 250 mg, three capsules four times a day. Creatine 3 g/day for pyramidal, 6 g/day for classic and 9 g/day for segmental-nuclear variant of ALS. Levocarnitine 20% solution, 15 ml 4 times a day. Course therapy for two months three times a year. Trimethylhydrazinium propionate 10% solution, 10 ml per 200 ml of 0.9% sodium chloride solution intravenously (course - 10 infusions, 1-2 times a year)
Multivitamin therapy	Thioctic acid 600 mg daily for 2 weeks 1-2 times a year. Multivitamins (milgamma 2 ml intramuscularly daily for 2 weeks 1-2 times a year, neuromultivit 2 capsules 3 times a day for 2 months 2 times a year)
Peroneal paresis, equinovarus foot deformity	Orthopedic shoes
Neck extensor paresis	Semi-rigid or rigid head holder
Walking disorders	Canes, walkers, strollers
Fatigue	Amantadine 100 mg/day for a month, if ineffective - ethosuximide 37.5 mg/day, if ineffective - gymnastics 2 times a day for 1 5 minutes (exercises with passive contraction)
Deep vein thrombosis of the lower extremities	Elastic leg wrapping
Spastic contracture of the hand	Relaxing splints
Humeroscapular periarthrosis	Compresses with dimethyl sulfoxide 30% (one teaspoon), procaine 0.25% (two teaspoons), 3 ml of hyaluronidase (dissolve 64 units of powder) for 30-40 minutes for 3-5 days
Salivation	Mechanical or medicinal sanitation of the oral cavity (frequent rinsing with antiseptic solutions, brushing teeth three times a day). Limiting fermented milk products. Amitriptyline up to 100 mg/day at night. Atropine 0.1% 1 ml, two drops in each corner of the mouth 10-20 minutes before meals and at night. Systemic use of atropine is fraught with side effects (tachycardia, constipation)
Oral hypersecretion syndrome	Portable suction units. Bronchodilators and mucolytics (acetylcysteine 600 mg orally per day). Correction of dehydration
Dysarthria	Muscle relaxants (see « Spasticity"). Ice applications on the tongue. British ALS Association speech guidelines. Electronic typewriters. Etran tables with letters or words. Computer system for typing characters using touch sensors installed on the eyeballs
Dysphagia	Mashed and ground dishes, purees, soufflés, jellies, porridges, liquid thickeners. Exclusion of dishes with liquid and solid components contrasting in density. Percutaneous endoscopic gastrostomy
Obstructive sleep apnea syndrome	Fluoxetine 20 mg/day at night
Respiratory disorders (FVC)<60-70%)	Intermittent non-invasive ventilation

An improvement in the emotional state according to the quality of life scale for ALS ALSAQ-40 and, as a consequence, general activation of patients was revealed when patients were treated with a 1% solution of Semax (methionyl-glutamyl-histidyl-phenylalanyl-prolyl-glycyl-proline) intranasally at a dose of 12 mg/day (two 10-day course with a break of 2 weeks). This drug from the group of nootropics has no effect on the progression of the disease.

Metabolic myotropic drugs that can be prescribed for ALS include carnitine capsules, levocarnitine (oral solution) or trimethylhydrazinium propionate (intravenous drip), as well as creatine, depending on the variant of the disease. However, a recent clinical trial of creatine did not confirm its positive effect on the decrement of muscle strength identified in the original study_ In the segmental-nuclear variant of ALS with lumbar onset, pronounced myolysis occurs and the serum level of CPK increases, therefore it is believed that the use of carnitine preparations in such cases is safer from -due to the risk of developing acute renal failure during treatment with creatine. If there is a significant decrease in motor activity, myotropic drugs are discontinued, since otherwise they will increase muscle catabolism. For the same reason, it is not recommended to prescribe nandrolone, which, in addition to the indicated negative effect, leads to the development of impotence. Also, for ALS, it is customary to prescribe multivitamin preparations or combinations of B vitamins with thioctic acid preparations. Any positive effect of myotropic and vitamin preparations on the course of the disease has not been established.

A complex of motor disorders in patients with motor neuron disease requires the use of orthopedic correction methods. In addition, in specialized centers abroad there are sets of tableware and other household appliances convenient for patients. Patients should be explained that the use of these aids does not “glue” » labeling them “disabled”, but, on the contrary, allows them to reduce the difficulties associated with the disease, keep patients in the circle of social life, and also improve the quality of life of their relatives and friends.

It has been shown that the use of gymnastics for 15 minutes twice a day slows down the decrement of muscle strength and helps correct peripheral fatigue in ALS. Some authors also consider medicinal methods used for multiple sclerosis, which make it possible to correct fatigue of central origin in ALS (see Table 34-5).

One of the most important areas of palliative care is the treatment of bulbar and pseudobulbar disorders. They occur at the onset of the disease with progressive bulbar palsy (bulbar form of ALS) and are associated in 67% of cases with spinal onset of ALS.

Saliva production in ALS is less compared to healthy individuals. At the same time, as dysphagia progresses, drooling develops due to the inability to swallow and spit out excess saliva. Palliative treatment of drooling is important because this symptom contributes to the development of opportunistic infections in the oral cavity, which in turn increases the manifestations of dysphagia and dysarthria, increases the risk of developing aspiration pneumonia and, finally, creates emotional discomfort and increases depression, since the image of a person with saliva flowing from the mouth is associated among ordinary people with dementia, which patients with motor neuron disease do not suffer from.

In addition to amitriptyline (see Table 34-5), methods of combating salivation include the use of portable suction, subcutaneous injections of botulinum toxin in a dose of up to 120 units per parotid gland and up to 20 units per submandibular gland, irradiation of the parotid salivary glands, application of fluorouracil to the salivary glands, tympanotomy. All of these treatments are considered to be inferior in effectiveness to amitriptyline therapy, although comparative clinical trials have not been conducted. Salivation is only a component of such a symptom as oral hypersecretion, which is caused by impaired sanitation of the tracheobronchial tree. Correction of dehydration in patients with dysphagia and nutritional deficiency is carried out using infusions of 5% glucose, but not sodium chloride, to prevent central pontine myelinolysis, manifested by acute vestibular syndrome in the presence of pre-existing bulbar disorders.

The earliest symptom of this group is dysarthria. It can be spastic and accompanied by nasophonia in the classic and pyramidal variants of ALS, or flaccid and accompanied by hoarseness in the segmental nuclear variant. Dysarthria, unlike dysphagia, is not a life-threatening symptom, but it significantly worsens the patient’s quality of life and limits his ability to participate in social life. Dysarthria in ALS with the presence of deep tetraparesis significantly worsens the quality of life of people who care for the patient, due to the difficulties that arise in this case in mutual understanding between the patient and the relative. However, dysarthria is the most difficult to treat.

Dysphagia is a fatal symptom of motor neuron disease, as it leads to the development of nutritional deficiency (cachexia), secondary immunodeficiency, which simultaneously increases the risk of developing aspiration pneumonia and opportunistic infections. At the initial stages, frequent sanitation of the oral cavity is carried out, and later the consistency of food is changed.

The patient must be explained that food should always be taken while sitting with the head upright in order to ensure the most physiological act of swallowing and to prevent the development of aspiration pneumonia. From the earliest stages of dysphagia, the patient is discussed with the patient about the need to perform percutaneous endoscopic gastrostomy. It has been shown to improve the condition of ALS patients and prolong their life.

This operation is indicated for a decrease in body weight of more than 2% per month in the presence of dysphagia; a pronounced slowdown in the act of swallowing (eating a bowl of porridge for more than 20 minutes); pronounced restriction of fluid intake with the threat of dehydration (less than 1 liter of fluid per day); the presence of hypoglycemic fainting; FVC >50%.

A contraindication to endoscopic gastrostomy is a decrease in FVC<50%, поскольку во время операции при раздувании желудка возможна острая дыхательная недостаточность из-за воздействия на диафрагму и плевру с включением пульмонокардиального рефлекса. Перед операцией необходимо провести исследование трофического статуса пациента и назначить пероральную искусственную питательную смесь, чтобы предотвратить нарушения заживления послеоперационной раны на фоне иммунодефицита, а также антибиотики. к сожалению, пациенты с БАС редко соглашаются на проведение гастростомии в силу эмоциональных проблем, обусловленных невозможностью принимать пищу через рот. После операции проводят энтеральное питание искусственными питательными смесями в зависимости от трофического статуса и питательных потребностей больного, а также жидкими пищевыми продуктами (бульон, кисель в объёме до 400 мл) . При отказе от гастростомии проводят периодическое зондовое кормление искусственными питательными смесями с повышенным содержанием углеводов, парентеральное и ректальное питание. Назначаются эубиотики и пробиотики, растительные слабительные и большое количество жидкости.

The main fatal symptom of ALS is respiratory failure, which occurs as a result of paresis and atrophy of the diaphragm and accessory respiratory muscles or degeneration of the respiratory center of the medulla oblongata. First of all, they join with progressive bulbar palsy, diffuse and thoracic onset of ALS. In the latter case, they occur faster than with the cervical onset, due to the initial damage to the auxiliary and then the main respiratory muscles. With the onset of cervical ALS, the weakness of the main respiratory muscles, as a rule, is compensated for a long time by the function of the auxiliary muscles. Patients with ALS develop restrictive respiratory failure associated with a decrease in the ventilated surface of the lungs, which subsequently turns into restrictive-obstructive due to a violation of the passage of tracheobronchial secretions. With the bulbar onset of ALS, the opposite situation occurs, when obstructive respiratory failure becomes mixed due to the addition of a restrictive component.

Early signs of breathing problems include symptoms such as vivid dreams, morning grogginess, dissatisfaction with sleep and daytime sleepiness. To detect early breathing disorders, spirography and polysomnography are performed. If sleep apnea is present, fluoxetine 20 mg is prescribed at night for 3 months. In the future, it is recommended to use periodic non-invasive ventilation devices (BiPAP). Unfortunately, these devices are expensive and therefore inaccessible. The duration of sessions ranges from 2 hours for mild disorders to 20 hours, including night time, for severe ones. Peak fluometry, determination of blood gases, and oxygen therapy are performed. It has been shown that non-invasive ventilation of the lungs, started before the fall of FVC<60%, может продлить жизнь при БАС на 1 год. Гипербарическая оксигенация не эффективна. При потребности во вспомогательном дыхании свыше 20 ч ставят вопрос о переходе на инвазивную ИВЛ.

The need for tracheostomy and mechanical ventilation is a signal of approaching death. Arguments against performing mechanical ventilation for motor neuron disease include the unlikelihood that the patient will be removed from the device, technical difficulties and high costs of caring for a patient dependent on a mechanical ventilation device, the development of extramotor disorders in patients on mechanical neuron disease (dementia, cerebellar, extrapyramidal, sensory, pelvic disorders), as well as post-resuscitation complications (posthypoxic encephalopathy, pneumonia, deep vein thrombosis of the lower extremities, bedsores). In the United States, the cost of caring for a ventilated patient at home is $200,000 per year. Arguments for performing mechanical ventilation are the desire of individual patients to prolong their lives, as well as rare cases of preservation of cognitive functions and even partial performance in a number of patients with ALS after transferring them to mechanical ventilation. In Japan, 80% of patients with ALS are transferred to mechanical ventilation, in the USA - 10%, in the UK - 1%. In no country in the world is ventilation included in health insurance; it is provided only at the expense of the patient’s family at home or in a hospice setting. In addition, transfer to mechanical ventilation for ALS is carried out only if the patient, in the presence of a lawyer and legal representative, has agreed on the conditions for disconnection from the device.

Clinical indications for transfer to mechanical ventilation are isolated bulbar syndrome with respiratory disorders or isolated spinal respiratory failure with tetraparesis, but without bulbar disorders. In the presence of tetraparesis and bulbar disorders, i.e. “locked-in syndrome”, transfer to mechanical ventilation is not indicated. An emergency transfer to mechanical ventilation, if it is impossible to obtain instructions from the patient regarding further tactics, is not carried out.

Non-drug therapy

There are no specific recommendations for the regimen for ALS. It is believed that excessive physical activity, including sports, is not indicated, since such a lifestyle before the development of the disease is associated with the risk of its development. Food should be sufficient, high in calories, mechanically and thermally gentle and varied.

FURTHER MANAGEMENT OF THE PATIENT

After the initial or repeated final examination, which establishes the diagnosis of ALS, patients should remain under outpatient observation (once every 3-6 months), and they should be provided with counseling in stages as new symptoms appear. Treatment with myotropic metabolic drugs and vitamin therapy is carried out in courses, other drugs are taken continuously. It is recommended to perform spirography every 3 months and, if the patient is taking riluzole, after 3 months, and then every 6 months to determine the activity of ALT, AST and LDH. In the presence of dysphagia and nutritional deficiency, the trophic status and blood glucose level should be assessed. To perform percutaneous endoscopic gastrostomy, the patient is hospitalized for a short time in the hospital, where after the operation the optimal volume and frequency of enteral nutrition is selected. If the patient refuses this operation, he can be hospitalized for a short period to undergo infusion therapy to correct dehydration or periodic tube feeding. If periodic non-invasive mechanical ventilation is not available to the patient and tracheostomy with transfer to mechanical ventilation cannot be performed for legal or medical reasons, oxygen therapy is indicated. If oxygen therapy in a volume of 2-4 l/min does not eliminate shortness of breath at rest while lying down or sitting, the prescription of narcotic analgesics is indicated (morphine at a dose of 5 mg/day in tablets, or in the form of a rectal suppository, or subcutaneously 1 ml of a 0.1% solution; chlorpromazine at a dose of 25 mg/day in tablets or lorazepam at a dose of 2 mg/day in tablets; the latter two drugs can also be prescribed as an oral solution or rectal suppository). The patient may be at home or placed in hospice care.

FORECAST

The prognosis for ALS is always unfavorable, with the exception of rare hereditary cases associated with certain mutations in the superoxide dismutase -1 gene (D90A and some others). The duration of the disease with a bulbar onset is on average 2.5 years, and with a spinal onset - 3.5 years.

Only 7% of patients live longer than 5 years. Taking riluzole can prolong a patient's life by an average of 3 months. The duration of the disease is shorter with the bulbar onset of ALS (progressive bulbar palsy), with an age of onset younger than 45 years, as well as with a rapid type of progression on the ALS-FRS-R scale (loss of more than 12 points per year).

At the present time, there are still diseases from the branch of neurology that cannot be treated and lead to death in a short time. One of these pathologies is amyotrophic lateral sclerosis (ALS). This pathology in medicine is called Charcot's disease or motor neuron disease. It occurs extremely rarely, namely 3-10 cases per 400 thousand people annually. It is characterized by gradual weakening and atrophy of muscle tissue.

Motor neurons are bulky nerve cells in the spinal cord that are responsible for muscle movement and tone. They are localized in the anterior horns and have a name depending on the group of muscle tissue innervated. According to the ICD (International Classification of Diseases) 10th revision, ALS disease is coded G12.2.

Despite all the modern methods of examination and diagnosis, doctors still have not been able to understand why Charcot's disease occurs. Judging by confirmed information, the most common version of the development of pathology is considered to be a combination of several causes.

The main culprit lies with the antioxidant enzyme SOD1 (superoxide dismutase-1). It serves to protect the body's cells from oxidation, but if the composition changes, this function is no longer performed. This phenomenon occurs due to a mutation on chromosome 21, which is responsible for encoding SOD1. This anomaly occurs in 30% of patients with ALS. In 20-25% of cases, the mutation is inherited, and in other people it occurs due to other factors.

There are other causes of ALS, namely abnormalities in the development of a nerve cell (an incorrectly formed framework) or dysfunction of vesicular transport proteins. Such versions are less studied, but are also basic.

Among the factors that cause ALS and contribute to its development, the most basic can be identified:

Damage to nerve cells due to glutamate. This substance is responsible for transporting information in the nervous system;
Excessive amounts of calcium inside cells. Due to such a failure, the extracellular and intracellular balance of this element is disrupted;
Lack of neurotrophin (a substance that stimulates nerve cells);
Autoimmune pathological processes;
The negative impact of exotoxins, which are substances that are extremely toxic to humans, such as lead or pesticides;
Smoking.

To date, doctors have still not been able to fully unravel the causes of amyotrophic lateral sclerosis, but there are confirmed versions that can be guided by. If it is impossible to remove a genetic factor, then its development should be prevented, focusing on the list of catalysts for its occurrence.

Symptoms

Typically, with amyotrophic lateral sclerosis, symptoms occur depending on the location of the damaged motor neuron. If the disease affects a peripheral neuron, then the person experiences weakness and atrophic changes in muscle tissue. Sometimes fasciculations (involuntary contractions) of individual muscle groups are observed, which over time develop into a generalized form.

If the lesion is localized in the central motor neuron, then the symptoms of ALS will look like this:

Increased muscle tone;
Manifestation of pathological reflexes in the lower and upper extremities;
Deepening reflexes;
The appearance of abnormally fast movements (clonuses) resulting from jerky contractions of muscle tissue.

Often several motor neurons are damaged at the same time. When there is an anomaly in the central and peripheral neurons, a crossover of their characteristics is observed. Patients develop weakness in muscle tissue with the presence of abnormal reflexes, excessively high tone of muscles susceptible to atrophic changes, etc. ALS syndrome in its advanced stage manifests itself a little differently. Symptoms of central motor neuron damage are usually hidden behind signs of peripheral neuron damage.

Amyotrophic lateral sclerosis is usually accompanied by movement disorders.

In most cases, it is difficult to understand their degree, because sometimes muscle atrophy is not as pronounced as it is difficult for a person to move. Convulsive attacks occur periodically, which are accompanied by severe pain.

Pyramid syndrome in a disease such as amyotrophic lateral sclerosis occurs at an early stage. It is characterized by a decrease in tendon reflexes and weakening of the lower extremities. As the pathology develops, abdominal reflexes gradually disappear.

Forms of pathology

The disease is divided not only according to the location of motor neuron damage, but also into certain forms. They all have their own symptoms, stages of development, treatment regimen and prognosis. The bulbar form is the first and its main symptom is dysarthria or a disorder of the speech apparatus. In addition to this, the following symptoms can be identified:

Disorders associated with pronunciation and swallowing;
Involuntary contraction of the tongue with subsequent atrophy of its tissues;
Malfunctions and atrophic changes in the muscles of the mouth;
Increased mandibular reflex.

The second form is called cervicothoracic. Initially, it manifests itself in one of the upper limbs in the form of atrophic changes, twitching and weakening of the muscles. Over time, both limbs are affected and deep reflexes are extremely exacerbated. As the pathology develops, weakening of other muscle groups whose innervation comes from neighboring areas of the spinal cord will also appear.

The third form is called lumbosacral and is characterized by weakness and dystrophy of the foot muscles. Mostly the areas responsible for extension (fingers, feet) undergo changes. During diagnosis, doctors have to differentiate the diagnosis of ALS from other diseases, for example, damage to the peroneal nerve. As the disease develops, weakness also appears in other muscles that are innervated by neighboring parts of the spinal cord and the Achilles reflexes gradually worsen.

The fourth form is called high. It is characterized by symptoms of damage to the central motor neuron, namely seizures with symmetrical weakening of muscle tissue. There are virtually no manifestations of peripheral neuron damage and there is little noticeable deterioration in memory and mental abilities.

Common signs include:

Loss of body weight;
Impaired coordination of movements;
Overactivity of the sebaceous glands;
Change in skin tone;
Decrease in skin temperature of the upper and lower extremities;
Impaired sensitivity in the area of segmental innervation.

The pathology has an exclusively progressive course and 2 stages can be distinguished:

Local weakening of muscle tissue;
Generalized stage, in which bulbar and pseudobulbar disruptions are combined with weakening of 4 limbs (tetroparesis).

The appearance of the second stage indicates that amyotrophic lateral sclerosis is rapidly progressing, as well as the imminent beginning of the terminal stage of the disease. It is characterized by paralysis of individual muscle groups, problems with swallowing, breathing and speech. It is problems with the respiratory system that are the main factor that often leads to the death of the patient. Other causes include severe pneumonia, bleeding in the gastrointestinal tract, bedsores with infection, and blood clots.

Diagnostics

It is not so easy to differentiate ALS from other diseases, and for this, doctors use the following research methods:

Tomography (computer, magnetic resonance);
Diagnostics of muscle tissue activity;
Blood analysis;
X-ray;
Cell collection (biopsy) of muscle tissue.

During the examination of the patient, the specialist must exclude the following diseases with similar manifestations:

Malfunctions of the endocrine glands (endocrinopathy);
Guillain-Barre disease;
Bruns-Garland syndrome;
Cervical myelopathy;
Neoplasms in the spinal cord;
Primary lateral sclerosis (PLS);
Lead, manganese or mercury poisoning;
Post-polio syndrome (PPS);
Loss of nutrients (malabsorption syndrome).

All of these diseases are characterized by a gradual weakening of muscle tissue, especially for such pathologies as primary lateral sclerosis. It is quite rare and is characterized by damage to the corticospinal as well as corticobulbar lines. In its course, the disease can manifest itself virtually identical to ALS or have a more favorable prognosis, with a life expectancy of 10 years or more. The pathological process will have to be differentiated using instrumental methods and based on the course of the disease.

Course of therapy

Symptomatic treatment of ALS should begin in a timely manner, as the disease progresses rapidly. To date, no complete cure for the pathology has been invented, and there are only drugs to slow down the course of the disease, such as Riluzole. Due to its composition, the medication prevents glutamine from being fully produced, thereby protecting neurons. It must be taken regularly, and the dosage is selected by the attending physician.

In addition to slowing down the course, for amyotrophic lateral sclerosis, treatment should be aimed at relieving symptoms, namely:

To lift the patient out of depression, antidepressants and, if necessary, tranquilizers are used;
Muscle relaxants will help relieve muscle spasms;
Anti-inflammatory drugs, and in the later stages of development, opiates (narcotic alkaloids) can relieve pain;
Benzodiazepine-based medications will help normalize sleep;
If a bacterial infection occurs, you will need to take antibacterial drugs;
Medicines like Amitriptyline or a saliva ejector can help reduce saliva production;
Special canes or a wheelchair at a more advanced stage can make the patient’s movement easier;
A speech therapist will help eliminate speech problems;
Artificial ventilation should relieve breathing problems. Sometimes it is necessary to surgically make a hole in the trachea so that the patient can breathe on his own;
A special diet should replenish the supply of nutrients and vitamins. In the later stages, food is administered through a tube.

ALS needs to be treated comprehensively and it is advisable to undergo a course of psychotherapy for the patient and the people who care for him. After all, the disease is extremely serious and it greatly depresses a person’s mental state. Close people must understand how to set the patient up in a positive mood and know what it is better not to talk about in conversations with him.

Traditional medicine methods usually do not give any results and can only be used to improve the general condition. They should be used only after consultation with your doctor.

Forecast and preventive measures

Amyotrophic lateral sclerosis is an incurable disease with a severe and progressive course. Depending on the form of the pathological process, the lifespan of people can vary from 1-3 years to 10-15. The course and rate of development of ALS are often influenced by other factors, such as the patient’s age, hormonal changes and third-party pathologies.

To date, prevention of such a disease has not been invented, since the causes of its occurrence have not been fully studied. Instead, doctors gave some advice on how to slow down the development of the disease:

Whenever possible, engage in physical therapy;
Give up any bad habits (smoking, drinking alcohol);
Be under strict supervision of a neurologist and visit him at least every 3 months;
Strictly follow the treatment regimen prescribed by the doctor;
Correctly create a diet to receive all the substances necessary for the body, but at the same time refuse fried, smoked, spicy, hot and canned foods.

Amyotrophic lateral sclerosis virtually always leads to complete disability and death. The patient must undergo a course of psychotherapy and realize the severity of the disease in order to alleviate his condition a little. Treatment for this disease involves slowing the progression and relieving symptoms, but scientists are trying to find more effective drugs. This nuance means the imminent appearance of medications for ALS that can help people suffering from this severe pathology.