Myasthenia is an autoimmune disease characterized by impaired neuromuscular transmission and manifested by weakness and pathological fatigue of striated muscles.
As a result of this disturbance, healthy muscles become weak, leading to muscle paralysis. Practice shows that with this pathology any muscles can be affected, but the eye muscles are affected most often, facial, labial and lingual muscles, and also the muscles of the pharynx, larynx and neck are more rarely affected. With the defeat of the respiratory muscles, as well as the muscles of the trunk and extremities, a generalized myasthenia occurs – a very dangerous condition that can even be fatal.
For the first time the disease was described back in 1672 by an English doctor, anatomical specialist Thomas Willis. The number of cases of detection of myasthenia gravis is steadily increasing and currently stands at 5-7 people per 100 thousand of population.
According to statistics, women suffer from myasthenia much more often than men (70% to 30%), the disease first appears usually in adolescence, but is most pronounced at the age of 20-30 years. The disease has an autoimmune nature, therefore, hormone therapy is used for treatment.
By the way, this disease is not only diagnosed in humans – myasthenia is often fixed in dogs and cats.
Causes of myasthenia
To date, the causes of myasthenia is not fully understood. In medical practice, cases of family myastheniaus are diagnosed, but the heredity of the disease has not been proven. Quite often, patients with asthenic bulbar paralysis are diagnosed with hyperplasia or neoplasm of the thymus gland. Another factor contributing to the development of myasthenia is the penetration of viruses and mycoplasmas into the human body.
The main pathogenetic feature of this disease is dysfunction of the neuromuscular transmission, which causes the characteristic clinical manifestations. The synaptic block is interconnected with a failure in the synthesis of acetylcholine. In the serum, antibodies to skeletal muscles and thymus epithelial cells are determined, which gives grounds for attributing myasthenia to autoimmune diseases.
The results of the study of the causes of myasthenia gravis prove that an insufficient level of potassium in the body, hyperthyroidism and hormonal imbalance significantly increase the risks of this disease.
Symptoms of myasthenia
Weakness and severe fatigue can affect any muscle in the human body. More often – the muscles of the face and neck.
The characteristic symptoms of myasthenia gravis (see photo) are:
- ptosis (drooping of the eyelids);
- double vision;
- nasal voices and speech disorders;
- difficulty swallowing, masticatory muscle fatigue;
- neck muscle fatigue (difficult to keep head straight);
- weakness in arms and legs.
Symptoms appear after the load, and even after the most minimal. The greater the load, the worse the condition of the patient. For example, at first speech disturbances are minimal – a person is slightly nasal, but he says the words quite clearly, then he begins to “swallow” certain sounds, and then he cannot speak at all. Rest can reduce these manifestations. Especially severe symptoms occur 3-4 hours after waking up.
Depending on the manifestations, the following forms of the disease are distinguished:
- ophthalmic (the disease is manifested only by omission of the eyelids, double vision; for such patients, therapy with anticholinesterase preparations is usually sufficient);
- bulbar (impaired speech, chewing, swallowing, breathing – the danger of this form is that breathing difficulties can increase rapidly);
- generalized (the defeat of all muscles, this form of myasthenia is meant when they talk about myasthenia gravis or Erb-Goldfla disease; the disease affects all muscles, starting with facial and neck muscles and ending with the muscles of the arms and legs);
- fulminant (the most dangerous form, as it leads to disability and even death, it is usually caused by a malignant thymus gland, all muscles, including skeletal muscles, are affected, and the therapy simply does not have time to act, to prevent the dangerous effects of myasthenia) is simply impossible).
Often there is a generalized form, which usually begins with weakness of the facial muscles.
Symptoms in children
A special place is occupied by myasthenia in children, the early symptoms of which have some of its features:
1) Congenital myasthenia. Fatigue of the muscular system can still be suspected in utero during an ultrasound. In myasthenia, fetal movements are less active, if not absent. After birth, the symptoms of this myasthenia are similar to those of the newborn. Death occurs due to impaired breathing.
2) Myasthenia in newborns. It occurs in those children whose mothers themselves suffer from myasthenia. It begins from the first days of life and can last about 2 months. Its main symptoms may be the following:
- the baby is more sluggish than other newborns,
- breathes shallowly
- periodically choked,
- his cry is faint, more like a squeak,
- he also sucks badly and gets tired quickly,
- his mouth parted,
- look almost still
- In some cases, swallowing can also be difficult.
The child is more likely to die from choking.
- Early childhood myasthenia. According to experts, it is found at the age of 2 years, when, against the background of complete health, vision problems begin to appear: the eyelids involuntarily fall, the eye paralysis and strabismus develop. In some cases, the baby may refuse to walk, run, it is difficult for him to climb or descend the stairs, often asking for his hands. Refusal to eat can occur if the chewing muscles are involved in the process.
- Youth and childhood myasthenia. It develops from 2 to 10 years or in adolescence mainly in girls. Among the early signs, it is necessary to pay attention to the child’s complaints about sudden tiredness, blurred vision, difficulties with running, walking long distances, squats, and doing physical chores.
Myasthenia is a chronic disease, it is constantly progressing. If the patient does not receive the correct treatment, his condition worsens. A severe form of the disease may be accompanied by the onset of myasthenic crisis. It is characterized by the fact that the patient experiences a sharp weakness of the muscles responsible for swallowing and moving the diaphragm. Because of this, his breathing is difficult, his heartbeat quickens, and salivation is often observed. Due to paralysis of the respiratory muscles, the patient may die.
In case of an overdose of anticholinesterase drugs, a cholinergic crisis may occur. It is expressed in slowing heartbeat, drooling, convulsions, increased intestinal motility. This condition also threatens the patient’s life, so he needs medical attention. The anticholinesterase drug must be canceled, and its antitode, an Atropine solution, must be administered intramuscularly.
It is necessary to consult a neurologist. First, the doctor will examine the patient, ask about complaints and other symptoms identified. At the initial examination of the patient, it is rather difficult to identify the myasthenia, because its symptoms are similar to the symptoms of many other diseases. Therefore, a comprehensive survey.
The easiest and most valuable method for diagnosing myasthenia is a prozerin test. When performing this test, a solution of prozerin (0.05%, 1-2 ml) is injected subcutaneously into the patient, then after 20-30 minutes the neurologist re-examines the patient, determining the body’s response to the sample. Prozerin blocks cholinesterase, the increased activity of which leads to disruption of neuromuscular joints, so the symptoms of myasthenia quickly recede, and the person feels completely healthy. It is worth noting that the effect of prozerin, although powerful, is short-lived, therefore it is not used for treatment, but it is indispensable for diagnosis.
Also for the diagnosis of conduct the following studies:
- Electromyography (decrement test) – registration of bioelectric potentials in skeletal muscles. It is advisable to carry out electromyography twice: first, before carrying out a prozerin test, then 30-40 minutes after it, since this can reveal a neuromuscular transmission disorder.
- Electroneurography is the study of the speed of impulses passing through nerves.
- A blood test for specific antibodies, sometimes a biochemical blood test is also required.
- Genetic studies – are conducted to identify congenital myasthenia.
- Computed tomography of the mediastinum organs – to study the thymus gland for its increase in volume or the presence of a tumor (thymoma).
Special tests (tests) have been developed for the diagnosis of myasthenia, which allow the neurologist to identify the disease at the initial stages. Although such tests you can perform independently at home:
- Quickly open and close your mouth within 40 seconds – normally you should have time to do 100 cycles of such movements, and with muscle weakness much less.
- You need to lie on your back, slightly lift your head and keep it on the weight for 1 minute.
- Do 20 squats with the same amplitude.
- Quickly squeeze and unclench brushes – often due to muscle weakness in myasthenia such an exercise leads to drooping of the eyelids.
Differential diagnosis is carried out with diseases with similar symptoms: bulbar syndrome, encephalitis, meningitis, brain glioma, hemangioblastoma, Guillain-Barre syndrome, etc.
Treatment of myasthenia
The main task of treating myasthenia is to increase the amount of acetylcholine. It is rather difficult to synthesize this component; therefore, treatment is used to prevent its destruction. For this purpose, neurostigmin containing medications are used in neurology.
If the disease progresses rapidly, medications that block the immune response are prescribed, this is the treatment in this case.
When choosing drugs, be aware that myasthenic drugs are contraindicated in medicines with fluoride. People over 70 years old remove the thymus gland. In addition, drugs are selected to block individual symptoms – eyelid twitching, stopping saliva flow and
To improve the condition of the patient helps pulsotherapy. This treatment involves the use of hormonal drugs. First, a large dose of artificial hormones is discharged, but gradually it decreases and reduces to no. If a myasthenic crisis has occurred, hospitalization may be necessary. Plasmoferosis and ventilation of the lungs for the treatment of symptoms are prescribed to patients in this state.
A relatively new method for the treatment of myasthenia is cryoferrosis. Treatment involves the use of low temperatures, which help rid the blood of harmful components. Useful substances contained in the plasma, returned to the blood of the patient. The method is safe because there is no likelihood of transmission and the appearance of an allergic reaction. Cryoferrosis improves the general condition of the patient. After 5-6 procedures, a good lasting result is established.
Often, during treatment, doctors prescribe Kalimin – an anticholinesterase agent, also known as Mestinon, Pyridostigmine. As with any autoimmune disease, corticosteroids are prescribed, in particular Prednisone for treatment.
Along with drug therapy, it is possible to treat myasthenia gravis in the home with folk remedies in order to speed up recovery and alleviate the condition. However, any traditional methods of treatment should be coordinated with the observing specialist.
- Oats Grains are thoroughly washed, filled with water in a volume of 0.5 l. Next, the container is placed on the fire, the composition is brought to a boil, and steamed for at least half an hour. Next, for the preparation of the infusion should wait another 2 hours, removing the oats from the fire. The resulting broth is taken one hour before meals, at least 4 times a day. The duration of therapy is 3 months with an interval of 1 month and one more course treatment.
- Onion. The product in a volume of 200 grams (purified) is mixed with 200 grams of sugar and filled with water in a volume of 0.5 liters. The composition is placed on a slow fire and cook for 1.5 hours. Next, cool the mass, add two tablespoons of honey and take two teaspoons three times a day.
- Three heads of garlic are crushed and mixed with four lemons, a liter of honey and linseed oil (200 gr.). All ingredients must be thoroughly mixed and taken daily, one teaspoon three times a day.
Rules of conduct with myasthenia
If the diagnosis is made on time, and the patient fulfills all the prescription of the doctor, his working capacity and lifestyle almost do not change. Treatment of myasthenia gravis is the constant admission of special drugs and compliance with certain rules.
It is forbidden for such patients to sunbathe, perform heavy physical work and drink medications without a doctor’s recommendation. Patients must know what contraindications for myasthenia are for taking medication. Many drugs can cause complications in the course of the disease or the development of myasthenic crisis. These are the following medications:
- all preparations of magnesium and lithium;
- muscle relaxants, especially curariform;
- tranquilizers, antipsychotics, barbiturates and benzodiazepines;
- many antibiotics, for example, “Neomycin”, “Gentamicin”, “Norfloxacin”, “Penicillin”, “Tetracycline” and others;
- all diuretic drugs, except Veroshpiron;
- Lidocaine, Quinine, oral contraceptives, antacids, some hormones.
Patients with the disease need to adjust their diet in accordance with the stage of the disease. The protective functions of the body are weakened, so the power during myasthenia plays an important role in recovery. It is important to eat baked potatoes, raisins, bananas and dried apricots. It does not hurt to find sources of such trace elements as phosphorus, calcium.
Calcium intake should be combined with phosphorus, so there is a better absorption of substances. It is important to use potassium and vitamins.
With the help of acetylcholine, as described above, the human respiratory muscles also function. Therefore, due to problems with the work of these muscles, a sharp breathing disorder may occur, up to its stopping. As a result, death may occur.
Adults should always pay special attention to the complaints of the child. With the development of childhood myasthenia, the child often complains that he cannot pedal the bike, climb to elevation and
It is almost impossible to predict the exact outcome of this disease, since it depends on many factors: time of onset, clinical form, sex and age of the patient, as well as the presence or absence of therapeutic measures. The most favorable course is in the case of the ocular form of myasthenia, and the most severe course of the disease accompanies the generalized form.
With proper treatment and constant medical supervision, almost all forms of this disease have a favorable prognosis.