Amyotrophic amyotrophic 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 performance of deliberate movements, for example, walking.
Amyotrophic lateral sclerosis - history and description of
The lateral( amyotrophic lateral) 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).
In BAS, as a result of 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 after about 40 years, in 8-10% of the episodes is family. Prevalence is 4-6 cases per 100 thousand population.
Amyotrophic lateral sclerosis is very important to distinguish from a sharp narrowing of the cerebral vessels, because this determines the choice of the correct method of treatment and its outcome.
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Why the pathology develops is not reliably established.
There are a number of alleged causative factors:
- gene mutation inherited;
- development and accumulation of abnormal proteins causing neuronal death;
- autoimmune reactions( when the immune system attacks the nerve cells of one's body);
- biochemical processes in the body, resulting in it accumulates glutamic acid, which has a harmful effect on neurons;
- lesion of nerve cells by virus.
- hereditary predisposition;
- is aging;
- male gender( after 70 years this factor is leveled);
- smoking( especially with long experience);
- service in the army;
- work related to the use of lead.
Symptoms and complaints of patients
ALS begins with lesions 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 early stage:
- weakness and atrophy of the hands, motor disturbance;
- muscle weakness in the area of the feet and ankles;
- the hanging of the foot;
- muscle twitching, shoulder cramps, arms, tongue;
- speech disorders and difficulty swallowing.
In patients with the development of the disease there are attacks of spontaneous crying or laughing, imbalance, atrophy of the tongue.
In the later stages there are:
- loss of ability to move;
- shortness of breath.
The asymmetry of symptoms is typical for the disease.
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 occur in the cervicothoracic form;
- a quarter - on the bulbar form( due to the defeat of the hypoglossal nerve, glossopharyngeal, etc.);
- 20 - 25% of cases - on the lumbosacral;
- up to 2% - on cerebral.
Amyotrophic lateral sclerosis is the diagnosis of an exception.
To exclude other diseases use:
- blood tests;
- muscle biopsy;
- tests to determine muscle activity;
- X-ray examination;
- MRI( magnetic resonance imaging).
Differential diagnosis of
The disease of amyotrophic lateral sclerosis must be differentiated with:
- Guillain-Barre syndrome;
- with cervical myelopathy;
- with spinal cord tumors;
- post-poliomyelitic syndrome;
- intoxicating exposure to mercury, lead, manganese;
- by malabsorption syndrome;
- with diabetic amyotrophy, etc.
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ALS is not curable. 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 daily for 0.05 g.
Therapy methods are aimed at alleviating the symptoms and include:
- tranquilizers and antidepressants in depression;
- muscle relaxants in muscle spasms;
- anesthesia with non-steroidal anti-inflammatory drugs, and at the late stage - with opiates;
- preparations of benzodiazepine for sleep disturbances;
- antibiotic therapy for the development of bacterial complications( bronchopulmonary diseases in ALS often have no symptoms);
- decrease in salivation with the help of saliva ejector and some medications( amitriptyline, etc.);
- use of devices facilitating the movement of the patient( beds with different functions, armchairs, walking sticks), locking collars;
- speech therapy;
- diet, ensuring sufficient fluid intake, probe feeding;
- for artificial ventilation;
- tracheostomy( surgically a hole is created in the trachea, allowing the patient to breathe).
Alternative medicine( herbal medicine, acupuncture, homeopathy, etc.) is ineffective in the true form of ALS, but if the symptoms are caused by the syndrome of ALS, it is possible to improve the condition depending on the underlying pathology.
Prognosis and consequences of
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 this problem, one can hope to create more effective therapeutic agents in a relatively close time.
The true ALS should be carefully differentiated from the corresponding syndrome, for which the prognosis for recovery is much better.
In the video dms. Alexei Sergeevich Kotov talks about amyotrophic lateral sclerosis: