Amyotrophic lateral sclerosis: symptoms and causes

Amyotrophic lateral sclerosis: causes, symptoms, photos, diagnosis and treatment

Amyotrophic lateral sclerosis is a disease of the nervous system, in which motor (motor) neurons are affected.

Such nerve cells are in the spinal cord and the brain and control the fulfillment of deliberate movements, for example, when walking.

The lateral (lateral) amyotrophic sclerosis in 1869 was described by the French psychiatrist Jean-Martin Charcot, which gave the disease another name - "Charcot's disease".

Inclusion in the name of the disease epithet "lateral" ("lateral") is due to the location of neutrons, which are more prone to injury (in special lateral protrusions of the spinal cord).

Another "nominal" term, which is used in Canada and the United States, is "Lou Gehrig's disease." Louis Guerig is an outstanding baseball player, whose tragic ending at the age of 36 was due to ALS (amyotrophic lateral sclerosis).

According to ICD-10, ALS is referred to as a motor neuron disease (code G 12.2).

Separately, there is a syndrome of amyotrophic lateral sclerosis.

Such a syndrome is a consequence of another disease, and in a situation where such a cause is known, therapy is aimed at eliminating the underlying pathology.

With BAS, as a result of the destruction of motoneurons, the signal to the muscles from the brain ceases to be transmitted, which weaken and atrophy. ALS is a chronic disease with constant progression.

The first manifestations of the disease patients can feel about after 40 years, in 8-10% of the episodes is family. Prevalence is 4-6 cases per 100 thousand population.

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Causes

Why the pathology develops is not reliably established.

There are a number of alleged causal factors:

• mutation of genes, inherited;
• the generation and accumulation of abnormal proteins that cause neuronal death;
• autoimmune reactions (when the immune system attacks the nerve cells of one's own organism);
• violation of biochemical processes in the body, resulting in it accumulates glutamic acid, which has a harmful effect on neurons;
• defeat of nerve cells by a virus.

Risk factors:

1. hereditary predisposition;
2. aging;
3. male gender (after 70 years this factor is leveled);
4. smoking (especially with long experience);
5. Military service;
6. work related to the use of lead.

Photo of the main lesions in amyotrophic lateral sclerosis

Symptoms and complaints of patients

ALS begins with the defeat of the limbs, which later spreads to other parts of the body. The initial weakening of muscles quickly builds up, which eventually leads to paralysis. It does not affect the sphincter of the bladder and the ocular muscles.

Symptoms of an early stage:

• weakness and atrophy of the hands, a violation of motility;
• weakness of the muscles in the feet and ankles;
• sagging of the foot;
• twitching of muscles, cramps of shoulders, arms, tongue;
• speech disorders and difficulties with swallowing.

In patients with the development of the disease there are attacks of spontaneous crying or laughing, imbalance, atrophy of the tongue.

It is believed that the BAS does not suffer from higher mental activity. However, in about 1-2% of episodes cognitive functions are violated (even before the appearance of usual symptoms) and dementia develops.

In the later stages appear:

1. depression;
2. loss of ability to move;
3. interruptions in breathing.

Typical for the disease is the asymmetry of the symptoms.

Forms of the disease

There are many approaches to determining the types and forms of ALS.

One such approach is based on where the affected muscles are located, while:

• about half the episodes of the disease are in the cervicothoracic form;
• a quarter - on the bulbar form (due to the defeat of the sublingual nerve, glossopharyngeal, etc.);
• 20 - 25% of cases - on the lumbosacral;
• up to 2% - on cerebral.

Diagnosis: Ways

Amyotrophic lateral sclerosis is the diagnosis of an exception.

To exclude other diseases use:

Differential diagnostics

The disease of amyotrophic lateral sclerosis must be differentiated with:

1. Guillain-Barre syndrome;
2. cervical myelopathy;
3. tumors of the spinal cord;
4. post-poliomyelitis syndrome;
5. intoxication by mercury, lead, manganese;
6. endocrinopathies;
7. malabsorption syndrome;
8. diabetic amyotrophy, and others.

Treatment

ALS - not cured. To slow its progression, Riluzole (Rilutec) is used.

The active substance of the drug blocks the release of glutamine, preventing damage to neurons and, thereby, slowing the development of the disease itself. Riluzole is taken daily twice a day until, 5th year.

Therapy methods are aimed at alleviating the symptoms and include:

• tranquilizers and antidepressants in depression;
• muscle relaxants with muscle spasms;
• anesthesia with nonsteroidal anti-inflammatory drugs, and at the late stage - with opiates;
• benzodiazepine preparations for sleep disturbance;
• antibiotic therapy for the development of bacterial complications (bronchopulmonary diseases in ALS often have no symptoms);
• reduction of salivation with the help of saliva ejector and some medications (amitriptyline, etc.);
• use of devices that facilitate the movement of the patient (beds with different functions, armchairs, walking sticks), fixing collars;
• speech therapy;
• diet, ensuring sufficient fluid intake, probe feeding;
• artificial lung ventilation;
• tracheostomy (surgically in the trachea creates a hole that allows the patient to breathe).

The patient and his family members are required to provide psychotherapeutic assistance.

Alternative medicine (herbal medicine, acupuncture, homeopathy, etc.) is ineffective in the true form ALS, but if the symptoms are caused by the syndrome of ALS, it is possible to improve the condition depending on the primary pathology.

Forecast and consequences

The prognosis of amyotrophic lateral sclerosis is disappointing. Depending on the type of disease in 2 to 12 years, a lethal outcome occurs, due to the development of respiratory failure, severe pneumonia, etc.

In the case of the bulbar form of ALS, as well as in elderly patients, this period is reduced to one to three years.

Measures to prevent amyotrophic lateral sclerosis are not known.

ALS, a rapidly progressing disease caused by the defeat of nerve cells responsible for the motor function of the body, has no examples of successful recovery. The gradual increase in muscle weakness significantly complicates the life of the patient and his loved ones.

Despite the comforting prognosis of the disease, family members and the patient himself must be fully responsible for timely and adequate measures to alleviate his course.

Given the scientific interest in the problem under consideration, one can hope to create more effective therapeutic agents in a relatively close time.

It is necessary to carefully differentiate the true ALS from the corresponding syndrome, for which the prognosis for recovery is much better.

A source: http://gidmed.com/bolezni-nevrologii/skleroz/bokovoj-amiotroficheskij.html

Amyotrophic lateral sclerosis: symptoms, treatment, diagnosis, prognosis

Amyotrophic lateral sclerosis or Lou Gehrig's disease is a rapidly progressing disease of the nervous system, characterized by defeat motor neurons of the spinal cord, cortex and trunk of the brain the brain. Also in the pathological process, the motor branches of craniocerebral neurons (trigeminal, facial, glossopharyngeal) are involved.

Epidemiology of the disease

Diseases are extremely rare, about 2-5 people per 10, 00. It is believed that men are more often sick after 50 years.

Lou Gehrig's disease does not make any exceptions to anyone, it affects people of different social status and various professions (actors, senators, Nobel Prize winners, engineers, teachers).

The most famous patient was the world champion in baseball Loi Gering, in honor of which the disease got its name.

In Russia, amyotrophic lateral sclerosis has become widespread. At present, the number of sick people is about 1, 00-2, 00 among the population.

Among the famous people of Russia who have this pathology, you can note the composer Dmitry Shostakovich, the politician Yuri Gladkov, pop artist Vladimir Migulyu.

Causes of amyotrophic lateral sclerosis

At the heart of the disease is 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 there are many theories. The main theories are:

1. Viral - this theory was popular in the 60-70s of the 20th century, but it was not confirmed. Scientists of the USA and the USSR conducted experiments on monkeys, introducing them extracts of the spinal cord of sick people. Other researchers have attempted to prove the involvement of the poliovirus in the formation of the disease.
2. Hereditary - in 10% of cases, pathology is hereditary;
3. Autoimmune - this theory is based on the detection of specific antibodies that kill motor nerve cells. There are studies proving the formation of such antibodies against the background of other serious diseases (for example, in lung cancer or Hodgkin's lymphoma);
4. Gene - in 20% of the patients there are violations of genes encoding a very important enzyme Superoxide dismutase-1, which converts the superoxide poisonous to nerve cells into oxygen;
5. Neural - British scientists believe that the development of the disease involves elements of glia, that is, cells that provide the vital activity of neurons. Studies have shown that with insufficient function of astrocytes, which remove glutamate from nerve endings, the likelihood of Lou Gehrig's disease increases tenfold.

By frequency of occurrence:	By inheritance:
sporadic - single, unrelated cases family - patients had family ties	autosomal recessive autosomal dominant
By the level of the CNS lesion:	Nosological forms of the disease:
bulbar cervical thoracic lumbar diffuse respiratory	Classic ALS Progressive Bulbar Palsy Progressive muscular atrophy Primary lateral sclerosis West-Pacific Complex (BAS-Parkinsonism-Dementia)

Any form of the disease has the same beginning: patients complain of increasing muscle weakness, a decrease in muscle mass and the appearance of fasciculations (muscle twitching).

Bulbar form of ALS is characterized by symptoms of defeat of cranial nerves (, 0 and 12 pairs):

1. The sick worsen speech, pronunciation, it becomes hard to move your tongue.
2. Over time, the swallowing act is broken, the patient is constantly choking, food can pour through the nose.
3. Patients feel involuntary jerking of the tongue.
4. Progression of ALS is accompanied by complete atrophy of the muscles of the face and neck, the patients completely lack facial expressions, they can not open their mouths, chew food.

The cervico-thoracic variant of the disease affects, in the first place, the upper limbs of the patient, symmetrically on both sides:

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

The lumbosacral form usually begins with a sensation of weakness in the lower limbs.

1. Patients complain that it became harder for them to do the job, standing on their feet, walking long distances, climbing the stairs.
2. With the passage of time, the foot begins to fall, the muscles of the legs are atrophied, patients can not even stand on their feet.
3. Appear pathological tendon reflexes (Babinsky). In patients develop incontinence of urine and feces.

Regardless of which variant prevails in patients at the beginning of the disease, the outcome is still one.

The disease progresses steadily, spreading to all the muscles of the body, including the respiratory ones.

When the respiratory muscles are refused, the patient begins to need artificial ventilation and constant care.

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

Over time, the patient's psyche undergoes strong changes.

The patient, whom I observed for a year, differed capriciousness, emotional lability, aggressiveness, incontinence.

Conducting intellectual tests showed a decrease in his thinking, mental abilities, memory, attention.

Diagnosis of amyotrophic lateral sclerosis

The main methods of diagnosis include:

• MRI of the spinal cord and brain - the method is quite informative, reveals the atrophy of motor parts of the brain and the degeneration of pyramidal structures;
• Cerebrospinal puncture - usually reveals a normal or increased protein content;
• neurophysiological examinations - electroneurography (ENG), electromyography (EMG) and transcranial magnetic stimulation (TKMS).
• 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 at decay of muscles), a slight increase in liver enzymes (ALT, AST), the accumulation of slag in the blood (urea, creatinine).

What happens with ALS

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

1. diseases of the brain: tumors of the posterior cranial fossa, multisystem atrophy, dyscirculatory encephalopathy
2. muscular diseases: oculofarengial myodystrophy, myositis, myotonia Rossolimo-Steinert-Kurshmana
3. systemic diseases
4. spinal cord diseases: lymphocytic leukemia or lymphoma, spinal cord tumors, spinal amyotrophy, syringomyelia, etc.
5. peripheral nerve diseases: Persononeja-Turner syndrome, neuroaxiotonia Isaac, ultlofocal motor neuropathy
6. myasthenia gravis, Lambert-Eaton syndrome - neuromuscular synapse diseases

Treatment of amyotrophic lateral sclerosis

Treatment of the disease is currently ineffective. Medications and proper care of the patient only prolong life expectancy, without providing a full recovery. Symptomatic therapy includes:

• Riluzole (Rilutec) is a well-established drug in the US and the UK. The mechanism of its action is to block glutamate in the brain, thereby improving the work of Superoxide dismutase-1.
• RNA interference is a very promising method for the treatment of ALS, the creators of which were awarded the Nobel Prize in the field of 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 The nervous system prevents the death of nerve cells, restores neural connections, improves the growth of nervous fibers.
• Muscle relaxants - eliminate muscle spasm and twitching (Baclofen, Midokalm, Sirdalud).
• Anabolic (Retabolil) - to increase muscle mass.
• Anticholinesterase drugs (Prozerin, Kalimin, Pyridostigmine) - prevent the rapid destruction of acetylcholine in the neuromuscular synapses.
• B group vitamins (Neurorubin, Neurovitan), vitamins A, E, C - these agents improve the impulse conduction on nerve fibers.
• Antibiotics of a wide spectrum of action (cephalosporins of 3-4 generations, fluoroquinolones, carbopenems) - are shown at development of infectious complications, sepsis.

In complex therapy must include feeding through a nasogastric tube, massage, exercise with a physician LFK, counseling psychologist.

Forecast

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

1. Only 7% live more than 5 years
2. at bulbar debut - 3-5 years
3. with lumbar year

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

The situation in Russia is clouded by the fact that patients do not receive proper care, as evidenced by the fact that Rylusot is a drug slowing the course of the disease, until 2011 in Russia was not even registered, and only in the same year the disease itself was introduced into list of "rare". But in Moscow there are:

• Fund for the care of patients with amyotrophic lateral sclerosis at the Marfo-Mariinsky Mercy Center
• Charity fund for the help of G.N. Levitsky to patients with ALS

In the end I would like to add about the charity event Ice Bucket Challenge, which took place in July 2014.

It was aimed at collecting funds in support of patients with amyotrophic lateral sclerosis and was widely used.

Organizers managed to collect more than 40 million dollars.

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

A source: http://zdravotvet.ru/bokovoj-amiotroficheskij-skleroz-simptomy-lechenie-diagnostika-prognoz/

Amyotrophic lateral sclerosis, Charcot's disease, symptoms and causes of the syndrome

At the present time, there are still diseases from the section of neurology that are not amenable to treatment and lead to a fatal outcome in a short time. One such pathology is amyotrophic lateral sclerosis (ALS).

This pathology in medicine is called Charcot's disease or motor motoneuron's disease. It occurs extremely rarely, namely 3-10 cases per 400 thousand. person every year.

It is inherent in the gradual weakening and atrophy of muscle tissue.

Motoneurons are volumetric nerve cells in the spinal cord, which are responsible for the movement and tone of the muscles.

They are localized in the anterior horns and have a name, depending on the innervated group of muscle tissue.

According to the ICD (International Classification of Diseases) 10 revision, the ALS disease has the code G12.2.

Causes of ALS

Despite all the modern methods of examination and diagnosis, doctors still could not understand why Charcot's disease occurs. Judging by the confirmed information, the most common version of the development of pathology is the collection of several reasons.

The main fault lies on the antioxidant enzyme SOD1 (superoxide dismutase-1). It serves to protect the cells of the body from oxidation, but when changing the composition of such a function is no longer satisfied.

This phenomenon arises because of the mutation of the 21 chromosomes, which is responsible for the encoding of SOD1. This anomaly occurs in 30% of patients with ALS.

There are other causes of ALS, namely, anomalies in the development of the nerve cell (an improperly formed skeleton) or a violation of the functions of the proteins of vesicular transport. Such versions are less studied, but also are basic.

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

• Damage to nerve cells due to glutamate. This substance is responsible for the transport of information in the nervous system;
• Excessive amount of calcium inside cells. Because of this failure, the extracellular and intracellular balance of this element is disturbed;
• Lack of neurotrophin (substances stimulating nerve cells);
• Autoimmune pathological processes;
• Negative effects of exotoxins, which are extremely toxic to humans, for example lead or pesticides;
• Smoking.

To date, for amyotrophic lateral sclerosis, the causes of the onset of doctors have so far not been fully understood, but there are confirmed versions that can be oriented. If you can not remove the genetic factor, you should prevent its development, focusing on the list of catalysts for the occurrence.

Symptoms

Usually, with amyotrophic lateral sclerosis, symptoms arise depending on the location of the damaged motoneuron.

If the disease touched the peripheral neuron, then the person develops weakness and atrophic changes in the muscle tissue.

Sometimes fasciculations (involuntary contractions) of separate groups of muscles are observed, which eventually develop into a generalized form.

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

1. Increased muscle tone;
2. Manifestation of pathological reflexes in the lower and upper limbs;
3. Deepening of reflexes;
4. The appearance of abnormally fast movements (clones), arising from jerky contractions of muscle tissue.

Often several motoneurons are damaged. With anomalies in the central and peripheral neuron, there is a crossing of their characters.

In patients, there is a weakness in the muscle tissue with the presence of abnormal reflexes, there is an excessively high muscle tone prone to atrophic changes, etc. The syndrome of ALS in a neglected stage is manifested a little differently.

Symptoms of central motoneuron damage are usually hidden behind signs of failure in the peripheral neuron.

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

Pyramidal syndrome with such a disease as amyotrophic lateral sclerosis occurs at an early stage. It is typical for him to decrease tendon reflexes and weaken the lower limbs. As the pathology develops, the abdominal reflexes gradually disappear.

Forms of pathology

The disease is divided not only by the localization of injuries to the motor neuron, but also to certain forms.

All of them have their own symptoms, stages of development, treatment scheme and prognosis.

Bulbar form is the first and its main feature is dysarthria or speech device disruption. In addition to it, we can distinguish the following symptoms:

• Violations associated with pronunciation of sounds and swallowing;
• Involuntary contraction of the tongue followed by atrophy of its tissues;
• Failures in work and atrophic changes in the muscles of the mouth;
• Elevated 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 loosening of the muscles.

Over time, both limbs are affected and deep reflexes are extremely aggravated.

As the pathology develops, other groups of muscles will also become weaker, whose innervation comes from neighboring parts of the spinal cord.

During the diagnosis, doctors have to differentiate the diagnosis of ALS with other diseases, for example, with damage to the peroneal nerve.

As the disease develops, the weakness also appears in the other muscles, which are innervated by neighboring parts of the spinal cord and the achilles reflexes gradually deteriorate.

The fourth form is called high. It is characterized by symptoms of central motoneuron damage, namely convulsions with symmetrical weakening of muscle tissue. In fact, there are no manifestations of peripheral neuronal damage and a weakly marked deterioration of memory and mental abilities.

Among the common features can be identified:

1. Loss of body weight;
2. Violation of coordination of movements;
3. Hyperactivity of the sebaceous glands;
4. Changing the shade of the skin;
5. Decrease in skin temperature of upper and lower extremities;
6. Disturbance of sensitivity in the region of segmental innervation.

Pathology has an exceptionally progressive course and can be divided into 2 stages:

• Local weakening of muscle tissue;
• The generalized stage, in which bulbar and pseudobulbar failures are combined with the weakening of 4 limbs (tetroparesis).

The appearance of the second stage indicates that amyotrophic lateral sclerosis is rapidly progressing, as well as the early onset of the terminal stage of the disease. It is characterized by the paralysis of individual muscle groups, problems with swallowing, breathing and speech.

It is the problems with the respiratory system that are the main factor that often leads to the patient's death. For other reasons, you can include severe pneumonia, bleeding in the gastrointestinal tract, bedsores with infection, as well as the formation of thrombi.

Diagnostics

Differentiate among other diseases ALS is not so simple and for this doctors use such research methods:

• Tomography (computer, magnetic resonance);
• Diagnosis of muscle tissue activity;
• Blood test;
• X-ray;
• Cell fetching (biopsy) of muscle tissue.

During the examination of the patient, the specialist should exclude the following diseases that are similar in their manifestations:

1. Failures in the work of endocrine glands (endocrinopathy);
2. Guillain-Barre disease;
3. The Bruns-Garland Syndrome;
4. Myelopathy of the cervical department;
5. Neoplasms in the spinal cord;
6. Primary lateral sclerosis (PBS);
7. Poisoning by lead, manganese or mercury;
8. Post-poliomyelitis syndrome (PPS);
9. Loss of nutrients (malabsorption syndrome).

For all listed diseases, it is inherent in the gradual weakening of muscle tissue, especially this, of a pathology such as primary lateral sclerosis.

It is rare enough and it is typical for it to damage corticospinal, as well as corticobulbar lines.

In its course, the disease can manifest itself virtually identical to ALS or have a more favorable prognosis, in which one can live for 10 years or more. Differentiate the pathological process will have instrumental methods, and based on the flow of the disease.

Course of therapy

Symptomatic treatment of ALS should begin in a timely manner, as the disease progresses rapidly.

To date, there has not been invented a full-value medicine for pathology, and there are only drugs to slow the course of the type of Riluzole.

Due to its composition, the drug does not allow the production of glutamine in full, thus protecting neurons. Take it regularly, and the dosage is chosen by the attending physician.

In addition to slowing down the course, with amyotrophic lateral sclerosis treatment should be aimed at relief of symptoms, namely:

• To remove the patient from depression, antidepressants and, if necessary, tranquilizers are used;
• Relieve muscle spasms will allow muscle relaxants;
• Anti-inflammatory drugs can remove pain, and in the late stages of opiate development (narcotic alkaloids);
• Normalize the sleep will help drugs based on benzodiazepines;
• If a bacterial infection occurs, antibacterial drugs must be drunk;
• Reduce the allocation of saliva will help drugs like Amitriptyline or saliva ejector;
• Simplify the movement of the patient will be able to special canes or a wheel chair at a more neglected stage;
• The speech therapist will help to resolve problems with speech;
• Artificial ventilation should be avoided. Sometimes it is necessary to surgically make a hole in the trachea so that the patient can breathe independently;
• Replenish the supply of vitamins and vitamins should a special diet. In later stages, food is administered through a probe.

To treat ALS it is necessary in a complex and it is desirable to pass a course of psychotherapy to the patient and people who take care of him.

After all, the disease is extremely difficult and it greatly depresses a person's mental state.

Close people should understand how to adjust the patient to a positive mood and to know what better not to talk in conversations with him.

Methods of traditional medicine usually do not give any result and they can be used only to improve the overall condition. They should be used only after consultation with the attending physician.

Prognosis and prevention measures

Amyotrophic lateral sclerosis is an incurable disease with a severe and progressive course.

Depending on the form of the pathological process, the life expectancy of people can vary from 1-3 years to 10-15.

The course and rate of development of ALS is often influenced by other factors, for example, the age of the patient, hormonal changes and external pathologies.

Prevention of such a disease to date has not been invented, since the reasons for its occurrence have not been fully studied. Instead, doctors gave several tips on how to slow the progression of the disease:

1. As far as possible, engage in physical therapy;
2. Refuse any bad habits (smoking, drinking alcohol);
3. Be under strict control of a neurologist and visit it at least every 3 months;
4. Strictly follow the treatment schedule prescribed by the doctor;
5. It is right to make a diet in order to receive all the substances necessary for the body, but at the same time give up fried, smoked, spicy, hot and canned food.

Lateral amyotrophic sclerosis virtually always leads to complete disability and death. The patient should undergo a course of psychotherapy and realize the full severity of the disease in order to ease his condition a little.

Treatment for this ailment consists in slowing progression and in relieving symptoms, but scientists are trying to find more effective medications.

This nuance of the moment means the rapid appearance of medications from ALS, which can help people suffering from this severe pathology.

A source: http://NashiNervy.ru/perifericheskaya-nervnaya-sistema/simptomy-i-prichiny-bokovogo-amiotroficheskogo-skleroza.html

Amyotrophic lateral sclerosis: symptoms and treatment

Category: Neurology and psychiatry 9533

• Convulsions
• Weakness in the legs
• Weakness in the hands
• Memory malfunction
• Sense of speech
• Inability to swallow food
• Decreased muscle tone
• Violation of the musculoskeletal system
• Inability to chew food properly
• Unclear speech
• Twitching of the arm muscles
• Twitching of leg muscles
• Partial hand paralysis
• Atrophy of the muscles of the extremities
• Uncontrollable laughter
• Hanging Head
• Uncontrollable crying
• Feeling of the waves on the leg
• Twitching of the tongue
• Suspension of lower jaw

Amyotrophic lateral sclerosis is a neurodegenerative disorder in which peripheral as well as central motoneurons (motor nerve fibers) are affected.

Due to the progression of this syndrome, the patient has atrophy of skeletal muscles, fasciculations, hyperreflexia and other disorders.

Stop the course of pathology at this time is not possible, so the gradual increase in symptoms inevitably leads to a fatal outcome.

For the first time the description of amyotrophic lateral sclerosis was presented in the medical field in 1896. This was done by the French psychiatrist Jean-Martin Charcot. Later, this syndrome was renamed in his honor, and he became known as "Charcot's disease."

Specification in the name of the affliction "lateral" (or "lateral") directly indicates the localization of nerve fibers, which most often undergo changes.

In the international classification of diseases, amyotrophic lateral sclerosis has its own code - G 12.2.

Etiological factors

For today, the true causes of the onset and progression of amyotrophic lateral sclerosis in man remain unknown, but research in this area continues.

In particular, such interest in the disease is caused by its prevalence, as well as the inability to conduct its treatment so far (since the cause of development is unknown).

But still, scientists continue to research in this area and to date have several assumptions regarding the causes of the progression of amyotrophic lateral syndrome. So, in their opinion, the syndrome can be manifested due to the following factors:

• attack of nerve fibers by various viral agents;
• mutation of genes of hereditary type (scientists tend to believe that this reason can act as the main one in the progression of amyotrophic lateral sclerosis);
• secretion and accumulation by the body of specific protein compounds that cause the death of nerve cells;
• violation of biochemical processes occurring in the human body. A lot of reasons can contribute to this, but the result is always the same - a large amount of glutamic acid gradually accumulates in the body. Increasing its concentration leads to the death of neurons;
• the course of autoimmune reactions, as a result of which the human immune system attacks its own nerve endings.

Risk factors for the development of such a syndrome:

1. work related to close contact with lead;
2. hereditary predisposition;
3. the aging process of the body (more often amyotrophic lateral sclerosis begins to develop in people who have crossed the forty-year boundary);
4. tobacco smoking;
5. male.

Varieties

At the moment, clinicians distinguish 4 types of amyotrophic lateral sclerosis:

• cerebral, also referred to as high;
• bulbar;
• lumbosacral;
• cervicothoracic.

Symptomatology

Normal and damaged neuron with amyotrophic lateral sclerosis

Each of the above forms of amyotrophic lateral sclerosis has its own symptoms and the nature of their expression. But there is a group of symptoms that are characteristic of any of the varieties of the ailment:

1. the occurrence of seizures of different intensity in the affected parts of the body;
2. motor disorders;
3. no sensitivity disorders are observed;
4. disorders of the urinary system are not observed;
5. amyotrophic lateral sclerosis progresses constantly, and it does not matter which particular form it affects a person. The disease attacks all the new muscle structures, and in the end, a person can completely become immobilized or die.

Lumbosacral type

This kind of syndrome in a sick person can occur in two variations:

• The course of the disease begins with an attack only peripheral motoneuron.

Symptoms of amyotrophic lateral sclerosis of this type are manifested in the patient gradually. First, he notes that in one of the legs there was a muscle weakness, which gradually spreads to the second. At the same time, there is a decrease in tendon reflexes characteristic of the lower limbs. It is about the knee and Achilles.

Reduced muscle tone in the limbs, and manifested atrophy. All these pathological processes are necessarily accompanied by fasciculations. Fasciculation is a muscle twitch that a person can not consciously control. The patient notes that there was a feeling of "waves" on the leg, or it seems to him that "the muscles are moving."

Further, the musculature of the hands is involved in the process, with the manifestation of the same symptomatology as described above. Further, the bulbar group of motoneurons is attacked, which leads to the appearance of the following symptoms: problems with chewing incoming food, the voice becomes nasal, speech is fuzzy, lower jaw hangs down;

• second variationoccurs less frequently.Amyotrophic lateral sclerosisis manifested by the simultaneous damage of both peripheral and central motoneurons, which are responsible for the motor function of the legs.

As a result, in the limbs there is atrophy of the musculature, increased reflexes, and also muscle tone. All these same symptoms later appear on the hands. In the pathological process may be involved motor neurons of the brain. In this case, there will be symptoms such as twitching of the tongue, speech disturbance, a malfunction of chewing food, uncontrollable crying or strong laughter.

Cervico-thoracic form

This type of syndrome also occurs in two variations:

• peripheral motor neuron attack. It is manifested by the appearance of paresis, fasciculation, and also a decrease in the tone of the musculature in one hand. As the disease progresses, this symptomatology also appears on the second hand. The affected brushes acquire a characteristic appearance - "monkey's paw". Simultaneously with such signs, the muscular structures of the legs are affected - they are weakened. Further, the bulbar part of the brain is affected. The clinical picture is supplemented by the following symptoms: tongue paresis, speech impairment and swallowing problems. The defeat of the muscular structures of the neck is manifested by the dangling of the head;
• one-stage lesion of peripheral and central motoneurons.Immediately amazed and changed two brushes. In the legs, reflexes first increase, and later muscular strength decreases, pathological stop signs are manifested. Then the bulbar part of the brain is affected.

Bulbar form

The main symptoms of this form of amyotrophic lateral sclerosis are:

1. violation of articulation;
2. atrophy of the tongue and manifestation of fasciculations on it;
3. choking during eating;
4. a patient with difficulty can move the tongue;
5. strengthening of the mandibular and pharyngeal reflexes;
6. the appearance of uncontrollable crying or laughter;
7. increased emetic reflex;
8. defeat of the muscles of the hands and feet - increased tone, reflexes, as well as atrophic changes.

High shape

This form of the syndrome is characterized by a predominant lesion of the central motoneuron. In this case pareses are observed in all the muscles of the body. Simultaneously, there is an increase in their tone, as well as other pathological symptoms, as in other forms.

Also, with such a syndrome, mental disorders also appear:

• memory impairment;
• violation of rational thinking;
• decreased intelligence.

Diagnosis for suspected progression of the syndrome

If there is a suspicion of the progression of such a syndrome, the doctors carry out the following diagnostic measures:

1. collection of complaints;
2. clarification of the analysis of life and the disease itself;
3. EMG;
4. MRI;
5. investigation of cerebrospinal fluid;
6. molecular genetic analysis;
7. lab tests. It is important to establish the level of AlAT, CK, AsAT, as well as creatinine.

Healing measures

As it was said above, it is not possible to completely cure such a syndrome.

But here doctors can slow down his progression with the help of special medicines. The main drug of choice is Riluzole.

It contains in its composition active substances that block the secretion of glutamine, thereby preventing the death of neurons.

Treatment of amyotrophic lateral syndrome should be only complex and include such pharmaceuticals and medical measures:

• tranquilizers and antidepressants;
• pharmaceuticals with benzodiazepine are prescribed if there is a sleep disorder in the syndrome;
• muscle relaxants are indicated when strong muscular spasms develop with such an incurable syndrome;
• antibiotics are prescribed only if the pathology of a bacterial nature occurs concurrently with the syndrome;
• reduction of salivation by means of a special suction or pharmaceutical means;
• speech therapy;
• diet;
• use of special devices that could greatly facilitate the movement of the patient;
• In severe cases, tracheostomy or mechanical ventilation is indicated.

Diseases with similar symptoms:

Polyneuropathy (coinciding symptoms: 4 of 20)

Polyneuropathy is a complex of disorders characterized by damage to motor, sensory and autonomic nervous fibers.

The main feature of the disease is that a lot of nerves are involved in the pathogenesis process.

Regardless of the type of illness, it manifests itself in the weakness and atrophy of the muscles of the lower or upper extremities, lack of sensitivity to low and high temperatures, the emergence of painful and uncomfortable sensations. Paralysis is often expressed, complete or partial.

... Ischemic stroke (coinciding symptoms: 3 of 20)

Ischemic stroke is an acute type of impaired cerebral circulation, which arises from a lack of admission to a particular a region of the brain's brain or to the complete cessation of this process, in addition, with it there is damage to brain tissue in conjunction with its functions. Ischemic stroke, the symptoms of which, like the disease itself, are most often noted among the most prevalent types of cerebrovascular diseases, is the cause of subsequent disability and often lethal outcome.

... Microinsult (coinciding symptoms: 3 of 20)

... Hemorrhagic stroke (coinciding symptoms: 3 of 20)

Hemorrhagic stroke is a dangerous condition characterized by hemorrhage to the brain due to rupture of blood vessels under the influence of critically high blood pressure.

According to the ICD-10, the pathology is coded in I61. This type of stroke is the most severe and has a worse prognosis.

Most often it develops in individuals aged 35-50 years who have a history of hypertension or atherosclerosis.

... Ischemia (concurrent symptoms: 3 of 20)

Ischemia - a pathological condition that occurs when the blood circulation is sharply weakened in a certain area of ​​the organ, or in the whole organ. Pathology develops because of a decrease in blood flow.

The scarcity of blood circulation becomes the reason of disturbance of a metabolism, and also leads to infringement of functioning of the certain organs. It should be noted that all tissues and organs in the human body have different sensitivity to lack of blood supply.

The cartilaginous and bony structures are less receptive. More vulnerable - the brain, the heart.

A source: http://SimptoMer.ru/bolezni/nevrologiya/1622-bokovoy-amiotroficheskiy-skleroz-simptomy