Dejerine's symptom is a disease that is quite rare. It has a genetic predisposition. Is incurable. This is due to the fact that any pathology associated with gene mutation is considered irreversible.
Neurologist Dejerine was the first to describe this disease. He noticed that the problem is transmitted within the same family. Therefore, there was an assumption about the relationship of pathology with genetics. Due to modern devices, it is possible to know in advance whether a child will be born he althy or not.
It is impossible to prevent the development of the problem. If it is passed on to a child, it will undoubtedly develop. According to ICD-10, Dejerine's symptom code is G60.
Epidemiology
To date, many varieties of the described syndrome are known. They are similar to each other in special signs and appear up to seven years. In 20% of cases, the first symptoms appear already in the first year of a child's life. On the second - up to 16%.
The most common Dejerine-Sott syndrome. It is diagnosed in 43% of cases. During treatment, up to 95% of patients remain disabled.
On the secondplace is the so-called paralysis of Dejerine-Klumpke. Symptoms appear quite quickly, the disease progresses at lightning speed. Occurs in 30% of cases. In third place is Rousseau's syndrome. It occurs in 21% of cases. This pathology is formed during the year in those patients who had previously suffered a stroke or other disease associated with cerebral circulation problems.
Pain syndrome in each person grows in its own way. In half of the cases, discomfort occurs a month after a stroke. In 40%, pain develops from 1 month to 2 years. In 11% - two years later.
Causes of the problem
Dejerine's symptom is considered a gene mutation. The disease affects the person as a whole, especially the brain. The causes of the problem include the following factors:
- constant jumps in intracranial pressure;
- fractures of those bones that are near the skull;
- injuries that affect the nerves located in the skull and in the brain (for example, a concussion);
- inflammation of the meninges of acute and chronic forms.
First signs of illness
Often the problem is actively manifested already in preschool age. However, its first signs can be noticed from the moment of birth. For example, a child will develop slowly when compared with peers. His first step will be late, and independent movement will be difficult.
The child has lowered muscles located on the face. The limbs begin to deform. They are weakrespond to external stimuli. Over time, the muscles completely atrophy.
It should be noted that the symptom of Dejerine in neurology is considered as a rather serious disease that needs to be tried to slow down from the first manifestation.
Problem stages
There are several stages of the disease. There is an elementary, intermediate and heavy. The mild stage first appears in infancy.
At the middle stage, the patient has a delay in the development of motor and speech muscles. Sensitivity begins to drop, reflexes slow down, and there are also problems with visual reactions.
At a severe stage, the patient becomes disabled. The disease progresses rapidly. Skeletal deformities occur, muscle tone is disturbed, and hearing loss sets in.
Klumpke Syndrome
Dejerine-Klumpke symptom is accompanied by problems with the shoulder connection. In this case, not the entire limb is paralyzed, but only part of it. Over time, the problem spreads to the hands. All adjacent areas begin to lose sensitivity. Vessels suffer, there are problems with pupillary reflexes.
Paralysis extends to the muscular frame. First, only the hands go numb, then the forearms and elbows. Then the sensitivity of the thoracic nerve is lost.
Neri-Dejerine Syndrome
With the development of this problem, the patient has a violation of the functionality of the roots of the spinal cord. With osteochondrosis, Dejerine's symptom of this form often occurs. Also it cancause tumors that press on the brain. Hernia, pinching and various kinds of injuries affecting the roots are also provoking factors. The main manifestation is considered severe pain at the site of pressure.
Most often, the symptom of Neri-Dejerine is considered a manifestation of a more serious problem. For example, the same osteochondrosis. If we are talking about just such a medical history, then the patient will have a sharp pain near the lower back, as well as discomfort near the neck and head. Over time, the sensitivity of these areas will fall and the pain syndrome will decrease. Muscle spasm may occur.
Dejerine-Roussy Syndrome
This problem affects perforating arteries. Areas of the brain are also affected. A person constantly has intense and piercing pain. The patient may experience nausea. Hyperpathy develops. Because of it, all the muscles come in a strong tone. But due to reduced sensitivity, the patient does not particularly notice this condition. With the development of the Dejerine-Roussy symptom, sharp crying, laughter, screaming may occur.
Paralysis usually affects only one limb. There is not only pain, but also a burning sensation. Sensations are intensified under the influence of external factors, as well as internal sensations.
Diagnosis is difficult in most cases. Often the correct diagnosis is made only after the clinical picture is fully built.
Diagnosis
The problem is diagnosed based on complaints. It is possible to suspect the development of this particular problem already at the firstexternal examination. However, the complexity of diagnosis lies more in the inability to immediately diagnose. Symptoms may be similar to other neurological problems. It is important to conduct a subjective, objective examination and complete anamnesis. The final diagnosis can only be made after laboratory tests.
Treatment
Given that the problem is genetic, it is completely impossible to cure it. The disease will progress and it is even difficult to slow it down. However, this does not mean that you should give up. Treatment must be thorough and rational. It is only through this approach that suffering can be alleviated.
Therapy is not aimed at getting rid of the problem, but at eliminating the symptoms. It is necessary to improve the patient's condition, as well as to alleviate the manifestations of the problem. Treatment regimens differ for each patient. This is due to the fact that the symptoms are always different.
When carrying out complex therapy, drugs against pain are prescribed. Also needed are those means that will help maintain the condition of blood vessels, tissues, metabolic processes, and so on. Particular attention should be paid to maintaining the condition of the muscles. Because they are the first to suffer. Also needed are those drugs that will maintain the state of the nervous system.
