One of the very rare and dangerous diseases is amyotrophic lateral sclerosis. This disease causes paralysis of most muscles of the human body, while the consciousness remains perfectly clear. The most famous example is the famous theoretical physicist Stephen Hawking, which is an exceptional case, since amyotrophic sclerosis usually leads to death within 3-5 years, and Hawking managed to stabilize the condition for a much longer period.
The main symptoms of amyotrophic lateral sclerosis
To date, scientists have not been able to establish the exact causes of amyotrophic lateral sclerosis. Some consider this disease hereditary, some - viral. Due to the fact that ALS occurs in about 3 people per 10 000 and progresses rather quickly, the study of the symptoms is somewhat difficult. There is evidence that amyotrophic lateral sclerosis is of autoimmune origin, but in each case the causes of the disease may be different and not always clear.
The disease can not be fixed with a macroscopic examination, therefore a computed tomography in this case does not give a result. Diagnosis of amyotrophic lateral sclerosis is based on a microscopic analysis of the cells of the cerebral cortex and the entire stem of the cerebrospinal cord. Only in this way the disease can be identified and distinguished from other lesions of the central nervous system with similar symptoms.
In the early stages, ALS proceeds almost unnoticeably, can only be manifested by numbness of the limbs and confusion of speech. Over time, signs become more pronounced:
- partial paralysis of some muscles of the upper, or lower limbs;
- the sense of balance deteriorates;
- a spasticity occurs;
- intensified deep reflexes;
- there are sudden and uncontrollable bouts of laughter, or crying;
- the freezing of the foot;
- Atrophy of the muscles of the shoulder and hip belts, the press;
- speech becomes illegible and incoherent;
- difficulty breathing;
- there is weakness and depression .
The final diagnosis is made after the unambiguous signs of the defeat of the central and peripheral motoneurons in the patient are fixed. This means that the process of destruction of motor neurons has begun and soon complete paralysis will occur. Often until this point, patients do not live out, as death occurs as a result of difficulty in respiratory function due to atrophy of the corresponding muscles.
Treatment of amyotrophic lateral sclerosis
Since there are no reasons for the development of the disease, its treatment is not effective. You can only slow down the process a little, using supportive therapy to facilitate its manifestations. First of all it concerns artificial ventilation of lungs. This method is actively used in the West and allows to prolong the life of the patient for 5-10 years. In the countries of the former CIS, this technique is practically not used because of the high cost of the equipment.
There is only one medicine that can slow the progression of the disease. This is Riluzol, which includes rilutec. It stops the production of the patient's glutamate by the body, as a result of which the damage to motoneurons becomes less significant. Riluzole has been introduced into use since 1995 in the US and many countries in Europe, but this drug has not yet been registered and is not being used.
Even if you managed to get a medicine, do not expect that it will significantly affect the course of the disease. On average, Riluzole therapy removes the need to connect the ventilator for about a month.