Symptom Dejerine is a disease that is quite rare. It has a genetic predisposition. It is incurable. This is due to the fact that any pathology associated with a gene mutation is considered irreversible.
Neurologist Degerin 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 connection of pathology with genetics. Due to modern devices, you can know in advance whether a child will be born healthy or not.
It is impossible to prevent the development of the problem. If it is transmitted to a child, then, undoubtedly, it will develop. According to ICD-10, the Degerin symptom code is G60.
Epidemiology
To date, many varieties of the described syndrome are known. They are similar to each other with special signs and manifest 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%.
Most often, Degerin-Sott syndrome occurs. It is diagnosed in 43% of cases. During treatment, up to 95% of patients remain disabled.
In second place is the so-called paralysis of Dejerine-Klumpke. Symptoms appear rather quickly, the disease progresses with lightning speed. It 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 have previously suffered a stroke or other disease associated with problems of cerebral circulation.
The pain syndrome in each person grows in his own way. In half the cases, discomfort occurs a month after a stroke. In 40% of the pain is formed from 1 month to 2 years. In 11% - two years later.
Causes of the problem
Symptom Dejerine 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 affecting the nerves located in the skull and brain (for example, concussion);
- inflammation of the meninges of acute and chronic form.
First signs of the disease
Often the problem is actively manifested already in preschool age. However, its first signs can be seen from the moment of birth. For example, a child will develop slowly when compared with peers. His first step will be late, and independent movements are difficult.
In the child, the muscles located on the face are omitted. The limbs begin to deform. They react poorly 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 should be tried to slow down from the first manifestation.
Problem stages
There are several stages of the disease. There is an initial, medium and heavy. The mild stage manifests itself for the first time in infancy.
At the middle stage, the patient exhibits a delay in the development of motor and speech muscles. Sensitivity begins to decline, reflexes slow down, and there are also problems with visual reactions.
In a severe stage, the patient becomes disabled. The disease progresses rapidly. There are deformations of the skeleton, muscle tone is disturbed, hearing loss occurs.
Klumpke syndrome
The symptom of Dejerine-Klumpke is accompanied by problems with the brachial joint. In this case, not the entire limb is paralyzed, but only a part of it. Over time, the problem spreads to the brushes. All adjacent areas begin to lose sensitivity. The vessels suffer, there are problems with pupillary reflexes.
Paralysis extends to the muscular skeleton. At first, only the hands are 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, a Dejerine symptom of this form often occurs. It can also be caused by tumors that press on the brain. The hernias, pinching, and various injuries affecting the roots are also provocative factors. The main manifestation is severe pain at the site of pressure.
Most often, a symptom of Neri-Dejerine is considered a manifestation of a more serious problem. For example, the same osteochondrosis. If we are talking about such a medical history, then the patient will have a sharp pain near the lower back, as well as discomfort near the neck, head. Over time, the sensitivity of these areas will fall and the pain syndrome will decrease. Muscle spasm may occur.
Degerin-Russi syndrome
This problem affects perforating arteries. Brain zones are also affected. A person constantly appears intense and piercing pain. The patient may experience aches. Hyperpathy develops. Because of it, all the muscles come in a strong tone. But because of the reduced sensitivity, the patient does not particularly notice such a condition. With the development of the Dejerine-Russi symptom, sharp crying, laughter, screaming can occur.
Paralysis affects, as a rule, only one limb. There is not only pain, but also a burning sensation. Sensations are amplified by external factors, as well as internal sensations.
Diagnosis in most cases is difficult. Often the correct diagnosis is made only after the clinical picture is fully built.
Diagnostics
The problem is diagnosed based on complaints. One can suspect the development of precisely this problem already at the first external examination. However, the complexity of the diagnosis lies more in the inability to immediately make a diagnosis. Symptoms may be similar to other neurological problems. It is important to conduct a subjective, objective examination and fully collect the anamnesis. A definitive diagnosis can only be made after laboratory examinations.
Treatment
Given that the problem is genetic, it is completely impossible to cure it. The disease will progress and even slow it down is difficult. However, this does not mean that you need to give up. Treatment should be thorough and rational. Only through this approach can suffering be alleviated.
Therapy is aimed not 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 are different for each patient. This is due to the fact that the symptoms are always different.
When conducting complex therapy, pain medications are prescribed. Also necessary are those funds that will help maintain the condition of blood vessels, tissues, metabolic processes, and so on. Particular attention should be paid to maintaining muscle condition. So how exactly do they suffer in the first place. Also needed are those drugs that will maintain the state of the nervous system.