Myasthenia gravis contraindications medication. Myasthenia gravis: Modern approaches to treatment. How to treat a disease

Changes in myasthenia gravis depend on antibodies to acetylcholine receptors. They decrease the amount of acetylcholinesterase at the synapse.

What is myasthenia gravis

How does the onset of neuromuscular pathology occur:

• The joints between muscles and nerves are called synapses. The nerve endings at the end of the synapse are equipped with vesicles that contain the neurotransmitter acetylcholine.
• On the other hand, the muscles contain receptors for the mediator.
• When a command is given to a muscle to contract, an impulse travels along the nerve. Acetylcholine is released, the impulse is connected to the desired receptor.
• Myasthenia gravis occurs when the transmission of a nerve impulse is blocked. It is not inherited, it increases after physical exertion.
• In case of illness, the number of nicotinic acetylcholine receptors in the muscle end plates is only 30%.

Myasthenia gravis

In neurology, the disease was given its code according to ICD 10. The forms of myasthenia gravis are divided into two groups: local and generalized. The latter type is expressed by the selectivity of the lesion site during pathogenesis: some muscles suffer more often than others. For example:

1. Among the eye muscles, a pathology is common that raises the upper eyelid.
2. In the hand, the triceps muscle of the shoulder suffers much more than others.
3. Children's myasthenia gravis is distinguished as a separate form, the symptoms of which appear at birth.

Myasthenia gravis - ocular form

Asthenic ophthalmoplegia is associated with such ailments as lagophthalmos, ptosis, impaired eye movement, diplopia. These symptoms are caused by ocular myasthenia gravis, in which the muscle fibers that lift the upper eyelid, the circular muscle of the eyelids and the oculomotor muscles are affected. Signs of weakness of the eye muscles are pathological fatigue, which can increase with:

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• physical activity;
• a surge of emotions, frustration;
• stress;
• after a course of anticholinesterase drugs.

Myasthenia gravis - generalized form

A common form of myasthenia gravis is generalized, in which all muscles are affected. First, the work of the oculomotor muscles is disrupted, then the muscle fibers of the neck and facial expressions. Generalized myasthenia gravis prevents the patient from holding his head, he does this with great difficulty. A transverse smile is displayed on the face, deep wrinkles appear on the forehead. Drooling and fatigue of the muscles of the arms and legs increase. The patient cannot look after himself, he walks poorly.

Bulbar myasthenia gravis

Violation of the processes of chewing, swallowing, speech change, voice exhaustion are signs that characterize the bulbar form of myasthenia gravis (asthenic bulbar palsy, pseudobulbar syndrome). The disease is characterized by dysarthria (speech impairment), dysphonia (nasalness, hoarseness) and dysphagia (impaired swallowing), that is, symptoms associated with the muscles of the soft palate and larynx, facial muscles, and oculomotor muscles. External manifestations: youthfulness of the face, smile "growl", lethargy of facial expressions.

Lambert-Eaton myasthenic syndrome

This phenomenon belongs to the category of rare forms of myasthenia gravis. Lambert-Eaton syndrome is a disease in which the process of neuromuscular transmission is disrupted. The condition is characterized by rapid muscle fatigue, bouts of weakness, ophthalmoplegia (paralysis of the eye muscles), myalgia, and autonomic disorders. Myasthenic syndrome can occur in small cell lung cancer, malignant tumors, and autoimmune diseases. Patients with this syndrome find it difficult to get up from a lying or sitting position.

Causes of myasthenia gravis

Myasthenia gravis can be congenital or acquired. Congenital occurs as a result of a change in the COLQ gene (a mutation in the genes of proteins responsible for muscle contraction). In age, women suffer from this disease more often than men. In an autoimmune process directed against acetylcholine receptors, human immunity produces antibodies in muscle tissue. The causes of acquired myasthenia gravis are described below:

• tumor of the thymus gland (thymoma, thymus);
• various autoimmune processes;
• severe stressful situations;
• viral diseases.

Myasthenia gravis symptoms

To learn more about the disease, to understand myasthenia gravis - what it is, to recognize the cause will help a consultation with a neurologist who will reliably determine the signs of myasthenia gravis. Myasthenic symptoms are of two types: people complain of weakness in different muscle groups and dysfunction of the nervous system. The following problems are signs of illness:

• with repetitive movements - fatigue;
• diplopia associated with weakness of the eye muscles;
• ptosis of the eyelids;
• reduced vision clarity;
• difficulty swallowing;
• dysarthria, blurred pronunciation;
• breathing is disturbed;
• increased heart rate;
• stool problems, diarrhea, constipation.

Myasthenia gravis diagnostics

Before the diagnosis is made, other diseases that may accompany the pathology are excluded. The most reliable diagnostic method is the proserin test. How does this happen? The patient is injected subcutaneously with the drug proserin. It will briefly improve the neuromuscular transmission function. Diagnosis of myasthenia gravis includes other ways to detect the disease:

• a blood test for antibodies;
• patient survey, clinical examination;
• decrement test (needle electromyography);
• computed tomography of the thymus gland.

Decrement test

One of the options for studying the process of neuromuscular transmission is the decrement test for myasthenia gravis, in which five muscle groups are examined. Each of them is subjected to the process of rhythmic stimulation, that is, the muscle fibers make contractions caused by artificiality. In this process, indicators of muscle responses to stimulation are recorded. The term "decrement" refers to the decrease in the frequency of muscle responses in response to stimulation, which is characteristic of myasthenia gravis. The presence of a decrement indicates a neuromuscular pathology.

Proserine test

A drug that dramatically improves the work of neuromuscular synapses is proserin. When the question of the presence of pathology is questioned, a proserin test is used for the test for myasthenia gravis. The test consists of the following steps:

1. A decrement test is in progress.
2. Proserin is injected subcutaneously or intramuscularly with a syringe.
3. Against the background of proserin action, the decrement test is repeated.
4. If there is a significant improvement in the decrement test results, then the braking of the transmission is justified.

Myasthenia gravis - treatment and prognosis

Progressive striated muscle weakness must be treated with drugs. In some cases, nothing but surgery will help. Treatment of myasthenia gravis with adequate therapy can translate the exacerbation of the disease into remission. Neuromuscular pathology has a generally favorable prognosis, which may worsen with the wrong medication. However, this does not apply to myasthenic crises, which are characterized by acute respiratory failure. The forecast in this case is not encouraging.

Medicines for myasthenia gravis

Medicines, doses, duration of therapy should be selected by the attending physician, since with different forms of the disease and the severity of the condition, its purpose is prescribed. During an exacerbation of the disease, the procedure for plasmapheresis is prescribed (500 ml every other day with replacement with albumin and plasma) and intravenous immunoglobulin is administered. Common drugs for myasthenia gravis include arresting the defect in the transmission of impulses from nerves to muscles:

• anticholinesterase drugs (kalymin);
• potassium salts;
• glucocorticoid therapy (prednisolone);
• cytostatic therapy (azathioprine, cyclosporine);
• mycophenolate mofetil (cellcept).

Thymectomy for myasthenia gravis

It has long been discovered that muscle pathology is associated with a violation of the thymus gland. Thymectomy (surgery to remove the thymus gland) is now considered standard surgery. In 75% of cases, patients experience improvement after surgery. For complete removal of the thymus gland, a transsternal surgical approach is used (with a dissection of the sternum). Less commonly, a transcervical access (catheter) is used.

Myasthenia gravis - treatment with folk remedies

Official medicine categorically prohibits treating the disease with folk drugs. They cannot cure the pathology, but they can alleviate the course of the disease. It is wiser to use traditional medicines in combination with drugs prescribed by a doctor. You need to find out if the remedy can be used with your medical history. Treatment of myasthenia gravis with folk remedies is made with the help of products such as oats, onions with garlic, dried fruits.

Diet for myasthenia gravis

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 nutrition with myasthenia gravis plays an important role in recovery. It is important to consume baked potatoes, raisins, bananas and dried apricots. It will not hurt to find sources for obtaining such trace elements as phosphorus, calcium. Calcium intake must be combined with phosphorus, so there is a better assimilation of substances. It is important to take potassium supplements and vitamins.

Myasthenia gravis in children

It is difficult to treat myasthenia gravis in children because they cannot reliably describe their symptoms. It all starts with disorders of the oculomotor, chewing, and facial muscles. Pathological fatigue sets in in the muscle fibers of the pelvis, arms, and neck. What does the face of a child with a disease look like? There are no emotions, lifelessness is visible, the gaze remains motionless, the upper eyelid of the eye is lowered. Muscle fatigue and weakness are more pronounced in the evening.

The actions prohibited in pathology include excessive hobby for sports, heavy physical activity, prolonged exposure to direct sunlight (insolation). In addition to limiting motor functions, there are drugs that are contraindicated for myasthenia gravis:

• magnesium (magnesia, asparkam);
• D-penicillamine;
• antipsychotics;
• curariform muscle relaxants;
• diuretics, except for veroshpiron;
• fluoride corticosteroids;
• quinine derivatives;
• antibiotics.

Video: Myasthenia gravis disease

The information presented in the article is for informational purposes only. The materials of the article do not call for self-treatment. Only a qualified doctor can diagnose and give recommendations for treatment based on the individual characteristics of a particular patient.

Myasthenia gravis

1. Presynaptic terminal

emerg / 325 emerg / 325 (emergency), med / 3260 med / 3260 (pregnancy), oph / 263 oph / 263 (eye)

Myasthenia gravis (lat. myasthenia gravis; Old Greek μῦς - "muscle" and ἀσθένεια - "powerlessness, weakness") is an autoimmune neuromuscular disease characterized by pathologically rapid fatigue of striated muscles.

Myasthenia gravis (asthenic bulbar palsy, asthenic ophthalmoplegia, bulbar palsy, Erb-Goldflam disease) is a classic human autoimmune disease. The main clinical manifestation of myasthenia gravis is the syndrome of pathological muscle fatigue (increased manifestations of myasthenia gravis after exercise and decrease after rest).

History

The disease was first described by Thomas Willis in 1672. Antibodies for myasthenia gravis were first isolated by Strauss in 1960. The definitive autoimmune origin of the disease was proved by Patrick and Lindstrom in an experiment on rabbits immunized with Torpedo californica AChR, which showed signs of myasthenia gravis.

Epidemiology

The disease usually begins between the ages of 20 and 40; more often women are ill. Recently, the incidence of myasthenia gravis has been growing, at the moment the prevalence is approximately 5-10 people per population.

Etiology

Myasthenia gravis is both congenital and acquired. Congenital myasthenia gravis is caused by mutations in the genes of various proteins responsible for the construction and operation of neuromuscular synapses. In synapses (in particular, in the terminal plates of neuromuscular synapses), acetylcholinesterase is present as a tetramer of the T isoform attached to a collagen-like protein encoded by a separate COLQ gene. Mutation of this gene is one of the most common causes of hereditary myasthenia gravis. Another common cause of myasthenia gravis is various mutations in the nicotinic acetylcholine receptor subunit.

Sometimes, more often in young people, there is a tumor of the thymus gland, which is removed surgically.

Pathogenesis

In the mechanism of development of myasthenia gravis, autoimmune processes play a role; antibodies are found in muscle tissue and thymus gland. The muscles of the eyelids are often affected, ptosis appears, which varies in severity throughout the day; chewing muscles are affected, swallowing is impaired, gait changes. It is harmful for people to feel nervous as it causes chest pain and shortness of breath.

A provoking factor can be stress suffered by ARVI, as well as a dysfunction of the body's immune system, which leads to the formation of antibodies against the body's own cells - acetylcholine receptors of the postsynaptic membrane of neuromuscular junctions (synapses). Autoimmune myasthenia gravis is not inherited.

Most often, the disease manifests itself during the transitional age in girls (11-13 years old), less often in boys at the same age. More and more often, the disease is detected in preschool children (5-7 years old).

Forecast

Previously, myasthenia gravis was a serious disease with a high mortality rate - 30-40%. However, with modern methods of diagnosis and treatment, mortality has become minimal - less than 1%, about 80%, against the background of correct treatment, achieve complete recovery or remission. The disease is chronic and requires careful monitoring and treatment.

Clinical picture

There are several forms of myasthenia gravis (ocular, generalized, bulbar and myasthenic Lambert-Eaton syndrome (with lung cancer, etc.). The disease often begins with eye symptoms (drooping eyelids, double vision). A feature is the dynamism of symptoms: in the morning, ptosis may be less than in the evening, double vision changes in severity. Then the weakness of the proximal muscles of the extremities joins more often (it is difficult to climb the stairs, get up from the chair, raise your arms up). At the same time, against the background of physical activity, weakness clearly increases in all muscle groups (after a test with 10 squats, weakness increases not only in the muscles of the legs, but also in the arms, ptosis intensifies.) Bulbar disorders may join (against the background of a long conversation or during a meal, the voice acquires a nasal tint, dysarthria appears, it is difficult to pronounce "P", "W", "S" After rest, these phenomena disappear.) Further, bulbar disorders can become more pronounced (there is a violation of swallowing, pop jerking, getting liquid food in the nose).

Taking anticholinesterase drugs (Kalimin, Proserin) significantly improves the condition of patients - such patients try to eat at the peak of the effect of anticholinesterase drugs.

Diagnostics

In standard cases, the diagnosis of myasthenia gravis includes:

1. Clinical examination and clarification of the medical history.
2. Functional test to identify the syndrome of pathological muscle fatigue. Electromyographic study: decrement test
3. Proserine test
4. Repeated decrement test to detect a reaction to proserin
5. Clinical examination to identify the reversibility of myasthenic changes in the presence of proserin
6. A blood test for antibodies to acetylcholine receptors and antibodies to titin
7. Computed tomography of the organs of the anterior mediastinum (thymus, synonym: thymus).

In cases of complex differential diagnosis, needle electromyography, a study of the conductive function of nerves, electromyography of individual muscle fibers (jitter), biochemical studies (creatine phosphokinase, lactate, pyruvate, 3-hydroxybutyrate) are performed.

Treatment

In cases of mild newly diagnosed myasthenia gravis and ocular form, only Kalimin and potassium preparations are used in the treatment.

Kalimin 60N 1 tab 3 times a day with an interval of at least 6 hours. Potassium chloride 1 g 3 times a day or potassium-normin 1 tab 3 times a day.

In cases of severe muscle weakness or the presence of bulbar disorders, glucocorticoid therapy is used: prednisolone at a dose of 1 mg / kg of body weight strictly every other day in the morning (usual doses are 60-80 mg per day, the minimum effective dose is 50 mg per day every other day).

One tablet of prednisolone contains 5 mg, respectively, the daily dose of prednisolone is 12-16 tablets. A metipred tablet contains 4 mg, but in terms of effectiveness it is equal to 1 tab of prednisolone 5 mg, therefore, in terms of metipred, the number of tablets is the same 12-16 tab, and the total dose will be less.

Prednisolone 60 mg in the morning every other day.

Taking prednisolone is long-term, remission can occur in 1-2 months, then the dose of prednisolone 0.5 tab is reduced to a maintenance dose of 10-40 mg every other day. And then slowly with caution, 0.25 tablets until the drug is completely excluded.

Taking prednisolone requires control of blood sugar and control by a local therapist (blood pressure, prevention of steroid ulcers, osteoporosis).

In the first 1-2 years from the onset of the disease, with a generalized form of myasthenia gravis, surgery is performed to remove the thymus gland (thymectomy). The effect of thymectomy develops within 1-12 months from the moment of thymectomy; the effectiveness of thymectomy is assessed after 1 year.

In old age, with insufficient effectiveness of prednisolone therapy, if it is impossible to prescribe prednisolone and if prednisolone is canceled, cytostatic therapy is prescribed. In mild cases - azathioprine 50 mg (1 tab) 3 times a day. In more serious cases - cyclosporine (sandimmun) at 200-300 mg per day or cellcept at 1000-2000 mg per day.

With exacerbation of myasthenia gravis, plasmapheresis and the introduction of intravenous immunoglobulin are permissible and justified. Plasmapheresis is advisable to carry out 500 ml every other day N5-7 with replacement with plasma or albumin.

Immunoglobulin is injected intravenously at a dose of 5-10 g per day to a total dose of 10-30 g, on average 20 g. Immunoglobulin is injected slowly, 15 drops per minute.

Contraindications for myasthenia gravis

Karen van Rensburg, BPharm

Myasthenia gravis is a neuromuscular disease characterized by pathological rapid fatigue of striated muscles. A few years ago, this disease had a very poor prognosis. Currently, there are various methods of treating myasthenia gravis, which significantly improves the quality of life of patients suffering from this disease.

Clinic

Myasthenia gravis is a disorder of neuromuscular transmission. It is classified as an autoimmune disease because the body begins to produce antibodies that interfere with the nerve endings in the muscle, which are acted upon by the neurotransmitter acetylcholine. These antibodies can block, modify, and destroy acetylcholine receptors, preventing muscles from contracting.

The main clinical manifestations of myasthenia gravis are the result of weakness (often unstable) of various groups of striated muscles: oculomotor (drooping of the eyelids, strabismus, "double vision", limited mobility of the eyeballs), chewing and facial muscles (incomplete closing of the eyes, inability to puff out the cheeks, grin teeth, violation of chewing, etc.), bulbar muscles (difficulty swallowing, nasal, food getting into the nose, hoarseness, articulation disorders), skeletal muscles (muscles of the legs, arms, neck, trunk). With weakness of the intercostal muscles, diaphragm, muscles of the larynx, accompanied by the accumulation of sputum and saliva in the airways, breathing disorders occur. The most common lesions are oculomotor muscles (in 40-50% of patients at the first clinical examination and up to 95% as the disease progresses), bulbar (about 40%), muscles of the limbs and trunk (20-30%) of patients. The severity of symptoms in patients with myasthenia gravis is very variable.

The extreme degree of myasthenic disorders is a crisis (in 10-15% of patients) - a rapid aggravation of movement disorders, combined with impaired breathing and swallowing. If emergency care is not provided to the patient at this time, there is a real threat of death.

By the nature of the course, a stationary form of myasthenia gravis is distinguished, as a rule, proceeding for many years without aggravating the symptoms of the disease, and progressive (in most patients) with the release of the following types: rapidly progressing (developing within 6 months), rapidly progressing (within 6 weeks) and malignant, characterized by an acute onset and rapid development with the addition of bulbar and respiratory disorders within 2-3 weeks. The form of myasthenia gravis can be local (usually ocular) and generalized, with varying degrees of severity of damage to certain muscle groups. In addition, taking into account the degree of muscle dysfunction, it is possible to distinguish (very conditionally) mild, moderate and severe forms of myasthenia gravis.

Diagnosis

Muscle weakness and a number of other symptoms observed in myasthenia gravis can occur with various other neurological and endocrine diseases. In this regard, a qualified neurological examination with the definition of weakness and pathological fatigue of various muscle groups plays a major role in the diagnosis of myasthenia gravis.

In order to exclude a tumor of the thymus gland (thymoma), as well as to determine changes in the thymus gland (hyperplasia, cyst, fatty involution), a number of special research methods are used. Thymoma is observed in 10-12% of the examined patients, hyperplasia in 60-70%, and myasthenia gravis in the rest. There is speculation that the thymus may be the source of the original acetylcholine receptors, to which antibodies are produced.

Treatment

There are different ways to treat myasthenia gravis, depending on factors such as age, type of myasthenia gravis, rate of progression.

The main methods of treating myasthenia gravis

1. Anticholinesterase drugs (proserin, oxazil, kalimin, etc.) improve neuromuscular transmission, are the basis for symptomatic therapy of myasthenia gravis. The effect of anticholinesterase drugs is enhanced by the simultaneous administration of potassium salts.
2. Corticoid therapy (adrenocorticotropic hormone, prednisolone and its derivatives) is carried out while taking anticholinesterase drugs, but their dosages require a significant reduction in order to avoid a cholinergic crisis. Due to the potassiumuric effect of glucocorticoids, patients should receive potassium supplements.
3. Thymectomy. Removal of the thymus gland is a widespread and quite effective method of treating myasthenia gravis. The operation is effective only with a relatively short duration of the disease (8-10 years) in patients not older than 50-60 years.
4. Radiation therapy is used to suppress the function of the thymus gland. This is the method of choice for the treatment of patients with inoperable thymomas with contraindications to surgical treatment.
5. Immunosuppressive therapy. Azathioprine, methotrexate are used when anticholinesterase drugs and corticosteroids are ineffective, in elderly people with acute myasthenia gravis who are not indicated for thymectomy.
6. Plasmapheresis. The improvement noted in this case is explained by the removal, together with the plasma, of antibodies to acetylcholine receptors, as well as other plasma components that have a pathological effect. With the right treatment, patients may experience recovery in muscle strength, the ability to lead a normal life, and even prolonged remission.

The role of the pharmacist

Since the pharmacist can be approached by patients with myasthenia gravis or their relatives, he should have the latest information on the safe use of drugs prescribed by the doctor, medications to avoid, and be able to give advice to help the patient cope with myasthenia gravis.

1. Emotional support. It is important to warn family members of patients with myasthenia gravis about the need for their support. They should be encouraged to gather as much information as possible about the disease and its treatment. The family should be prepared to meet the needs of the patient that may arise and offer their help. For example, many patients are reluctant to seek help with shopping and childcare. Basic tips, such as using an electric toothbrush, can make life easier for patients with myasthenia gravis. A visit to the doctor with the patient can also be helpful, as family members can always get information about the problems that may arise.
2. Nutrition. Factors such as diet and food choices must be considered to ensure proper nutrition for the patient when the muscles needed to chew and swallow food are affected by myasthenia gravis. If the patient experiences fatigue while eating, this can lead to malnutrition or food choices that do not provide a healthy and healthy diet. Meals should be planned for a time when patients are feeling well and muscle weakness is at a minimum. When the swallowing muscles are affected, food can become stuck in the throat, and the presence of a family member who knows how to perform the Heimlich maneuver can be life-saving.
3. Visit to the dentist. Some dental procedures need to be slightly modified to accommodate the changed muscle strength in the mouth. Certain medications should be avoided in patients with myasthenia gravis, and care should be taken to avoid any articulatory side effects caused by the drugs that patients with myasthenia gravis take. If a patient with severe myasthenia gravis is facing a major dental procedure, preoperative plasmapheresis is sometimes recommended. For local anesthesia, amide derivatives are preferred over esters. Intravascular anesthesia should be avoided.
4. Side effects of drugs for the treatment of myasthenia gravis. Some medications given for myasthenia gravis can cause gastrointestinal discomfort and diarrhea. At this time, you can give small portions of rice, bananas, applesauce and toasted croutons. If symptoms persist, you should definitely see a doctor.
5. Drugs that should be used with caution in patients with myasthenia gravis. With myasthenia gravis, more than with any other disease, you should pay attention to the fact that many drugs cause a deterioration in the condition of patients, in particular benzodiazepines, quinine, quinidine, morphine, muscle relaxants, inhalation anesthetics, neomycin, streptomycin, tetracycline, sulfonamides, D-penicillamine, hydantoin, magnesium salts, radiopaque substances and citrates (table). Therefore, it is recommended to closely monitor whether patients experience an increase in muscle weakness after the appointment of any new drug.
6. Other factors provoking myasthenia gravis. Exacerbation of myasthenia gravis can occur under the influence of infection, overheating, alcohol intake, menstruation, pregnancy, somatic diseases.
7. Patient safety. Patients with generalized myasthenia gravis often have difficulty walking, and patients with ocular myasthenia gravis often have diplopia (double vision). This can lead to the fact that the patient is not safe at home, the home can become a place where accidents and injuries often occur to him.

Patients with unsteady gait can feel more confident with the walking frame. In the apartment, you need to remove all free carpets on the floor, and carefully place the cables outside of the places where they walk. The sole of the patient's shoe should be non-slip, without high heels. Handrails on stairs, in the bathroom and in the shower can also provide additional support. A medical alert bracelet should also be worn to warn that the patient is suffering from myasthenia gravis.

Causes drug-induced myasthenia gravis in 1-7% of patients

Causes drug-induced myasthenia gravis.

After treatment with interferon-alpha, myasthenic crisis may occur

By interfering with the release of acetylcholine, magnesium can interfere with neuromuscular transmission.

Usually, an increase in myasthenia gravis symptoms occurs with parenteral administration of magnesium, but sometimes it is observed with oral administration.

Patients with myasthenia gravis should avoid parenteral magnesium administration

and take oral magnesium medications with caution

Possible electrolyte imbalance due to loss of potassium

General anesthesia drugs

In patients with myasthenia gravis, general anesthesia may increase the effects of drugs that block neuromuscular transmission. Inhalation anesthesia can act directly on the transmission of excitation from the nerve to the muscle (enflurane, halothane, isoflurane)

Preparations for local anesthesia

Intravenous administration of lidocaine and procaine may enhance the effects of drugs that block neuromuscular transmission

Non-depolarizing muscle relaxants (mivacurium, atracurium, tubocurarine)

Cause long-term neuromuscular blockade, which can lead to neurogenic muscle atrophy, peripheral neuropathy, and myopathy

Depolarizing muscle relaxants (succinylcholine)

Can be used, but with extreme caution

Aminoglycoside antibiotics (amikacin, gentamicin, kanamycin, neomycin, netilmicin, streptomycin, tobramycin)

Hinder neuromuscular transmission

May cause severe muscle weakness

Can directly block muscle contractility

Exacerbates the symptoms of myasthenia gravis

Quinidine and quinine

Hinder the formation or release of acetylcholine

Beta blockers (including timolol, eye drops)

May cause muscle fatigue, transient diplopia

Reduces the release of acetylcholine from the motor nerve endings

Patients with myasthenia gravis for whom treatment with corticosteroids is indicated should be administered the drug gradually, gradually increasing the dose, in order to avoid the initial exacerbation of the disease. And just as gradually after the end of treatment, it is necessary to reduce the dose.

Based on materials from sapj.co.za

Translation by Svetlana Bondareva

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Contraindicated drugs for myasthenia gravis

A neuromuscular disease with an autoimmune nature of development is myasthenia gravis - from the Latin Myasthenia Gravis. Ten people will suffer from this ailment. More than 50% of patients achieve remission.

Causes

Some are prone to myasthenia gravis - a risk group. It includes:

• Young people aged 20 - 40;
• Female sex - according to statistics, women get sick 3 times more often than men, but in adulthood, these indicators are equalized.

The main reason for the development of myasthenia gravis is the body's autoimmune reaction to its own tissues. Such a process can be started by:

Depending on the provoking factor, myasthenia gravis is divided into 2 large classes:

The first type is the outcome of a genetic mutation. Failure leads to a violation of the contractile function of the muscles. The second form is often a consequence of a tumor of the thymus. Myasthenia gravis can affect muscle fibers in any part of the body. The ocular form of the disease is more common. Children rarely suffer from this ailment. They account for less than 3% of the total number of patients.

Clinical picture

Whichever muscle group is captured by the disease, patients will notice general symptoms in the affected area:

• Excessive fatigue;
• Increased weakness;
• Decreased performance and functionality.

The pathological focus causes discomfort to a person. This defective part does not cope with the tasks assigned to it. The muscles of any part of the body can suffer. But especially often the eyes are involved in the process. This brings a lot of inconvenience to the patient. However, resting the muscles, relieving tension from them, gives relief. But it is short-lived.

Over time, even prolonged relaxation will not reduce the severity of the patient's symptoms. Advances in pharmacology enable patients to fight disease. In clinical practice, the following forms of myasthenia gravis are distinguished:

Diagnostics

Symptoms alone are not enough for a doctor to record the fact of a disease. Any study of the pathological process is not limited to instrumental methods. The doctor examines the patient, finds out the clinical signs of the disease. Examines the patient's medical history. And then he assigns him laboratory and instrumental diagnostics. A standard set of tests for detecting myasthenia gravis consists of the following:

• Functional tests aimed at detecting pathological muscle fatigue;
• Electromyographic examination, which indicates activity in the affected areas;
• Decrement test, which allows you to identify the blockade of the neuromuscular signal, and at the same time the severity of the process;
• Proserine test;
• Biochemical blood test;
• Immunograms;

If it is difficult to make a diagnosis, a differential study may be prescribed by:

• Needle electromyography;
• Study of the conductive functions of nerve fibers;
• Electromyography of specific muscles - jitter.

Treatment

The choice of a line of therapy for myasthenia gravis depends on many factors:

• Forms of the disease;
• The patient's condition;
• Concomitant pathologies;
• The prevalence of the process.

Medication is usually prescribed. However, in some cases, surgery is recommended. Autoimmune processes in the thymus can cause thymectomy surgery. Among the effective drugs are known "Proserin", "Kalinin" and drugs with a high potassium content. And also prescribe medications that stimulate the immune system.

Symptomatic treatment that relieves the patient's condition includes the following medications:

• Anticholinesterase - "Ipigrix";
• Cytostatics;
• Glucocorticoids - "Prednisolone", "Metipred";
• Immunoglobulins.

In the case of rapidly progressing changes, extracorporeal hemocorrection is prescribed - a method that helps cleanse the patient's blood from antibodies against his own tissues. Even the first procedure gives people a chance to feel better. Further therapy helps to achieve a lasting effect.

Cryophoresis is an effective method. This procedure allows you to cleanse the blood from harmful substances by influencing with the help of a lowered temperature. Such treatment is carried out in a course for 5-7 days in a row. The method of cascade plasma filtration has become widespread. This procedure is carried out using nano cleaners. They purify the blood and then return it to the patient.

Another modern method of treating myasthenia gravis can be considered extracorporeal immunopharmacotherapy. It involves the extraction of lymphocytes from the patient, their drug treatment and subsequent sending to the blood system. With the help of a similar technique, it was possible to induce a stable remission in patients within a year. It is especially important to adhere to the doctor's recommendations, since with myasthenia gravis, there are contraindicated drugs, the use of which is fraught with dangerous consequences.

Eye shape

Orbital disease is one of the most common types of disease. Often, it is with her that the process of myasthenia gravis begins, and then spreads to other organs. The main symptoms noted by patients:

• Diplopia, that is, double vision. Patients see more than one complete image;
• Decreased visual acuity and clarity;
• Violation of the rotational and motor functions of the orbits;
• Ptosis, that is, drooping of the eyelids. As a result, the palpebral fissure is unable to open and close normally.

All the features described can apply to one or both orbits. Usually, by closing their eyes for a short time, patients experience relief. However, mild stress associated with reading or watching TV is uncomfortable.

Bulbar form

This type of myasthenia gravis can be life-threatening. It entails:

• Dysphonia - a disorder of the vocal function;
• Dysphagia - swallowing disorder;
• Dysarthria - disorganization in the work of the muscular apparatus of the pharynx, larynx, and also the soft palate.

The described symptomatic manifestations entail dangerous consequences. Dysphagia can turn into a complete inability to swallow. The list of food items for such patients is extremely scarce. The food is prescribed by the doctor. Patients have to be tube fed, they lose weight and become weak. This means that their general condition is deteriorating, which does not contribute to recovery.

Disorder of voice formation reduces the social sphere of life of patients. And dysatria can be fatal due to respiratory problems caused by paresis of the vocal cords that close the larynx. This is fraught with asphyxiation - suffocation.

Generalized form

The most unfavorable type of disease is systemic, that is, common. This dangerous type of myasthenia gravis invariably causes up to 1% of deaths among patients with this pathological process. The generalized form captures a large number of muscles, including the respiratory ones - this can cause failure and death if care is not provided.

This disease is often accompanied by the prevalence of the process. Over time, the limited form progresses to systemic. And although remissions are not uncommon in patients, they, as a rule, occur and end suddenly. Therefore, myasthenic episodes and conditions are distinguished.

The first ones start and end quickly. The latter represent a continuous ongoing process, up to several years. However, this myasthenic condition is not prone to progression.

Contraindications

Patients suffering from this ailment are forced to have some restrictions. These include:

• Excessive physical activity;
• Insolation, that is, stay in direct sunlight;
• Medicines with magnesium - "Magnesia" and "Panangin", "Asparkam";
• Curariform muscle relaxants;
• Antipsychotics, tranquilizers and drugs that enhance their action - "Gidazepam", "Corvalcaps";
• Diuretics, with the exception of Veroshpiron and Spironolactones;
• The use of some antibiotics like aminoglycosides - "Gentamicin" and "Streptomycin", fluoroquinolones - "Enoxacin" and "Ciprofloxacin";
• Vaccinations.

Contraindicated drugs for myasthenia gravis should not be ignored. There are tables and lists of medications that answer the question "What antibiotics can be used for myasthenia gravis?" Funds that have this disease on the list of complications should be avoided. These drugs include "Glutalite". So the use of these pills is a contraindication. Compliance with the doctor's prescriptions is the key to a favorable course of the disease.

Myasthenia gravis is a severe autoimmune disease that manifests itself as pathological weakness of the muscles and progresses slowly. More often children suffer from it, but this pathology also occurs in adults.

A little about the reasons

Myasthenia gravis is a congenital hereditary disease. Its signs appear in early childhood. The syndrome can develop at different rates and severity. Due to genetic abnormalities, the connection between neurons and muscle fibers is disrupted. Due to the fact that the muscles actually turn off, do not function, their atrophy gradually develops.

Scientists have not yet been able to fully identify the mechanism of the onset of the disease, but it is known for certain that the reason lies in the deficiency of a gene that is responsible for the work of myoneural connections. First of all, visual functions suffer, as the muscles of the eyes atrophy. Then the process moves on to the facial muscles, neck, muscles of the arms, legs, swallowing muscles.

Often, this congenital syndrome leads to serious consequences and even death of the patient, but with proper treatment, recovery or temporary remission is possible. This pathology can be inherited from one of the parents or through a generation.

There are the following causes of the disease among children:

1. Failures of biochemical processes due to pathologies of the thymus, hypothalamus.
2. The thymus is attacked by its own immune cells, due to which less acetylcholine is produced and broken down.

Please note that the condition of a sick child can worsen stressful situations, acute respiratory viral infections, and impaired immunity.

Symptoms

The symptoms of myasthenia gravis directly depend on its form. The main symptom is unusual weakness in the muscles. The patient gets tired quickly, is not able to cope with work, training. This is especially noticeable if you need to perform a series of similar movements.

After rest, the functions of the muscles are restored. Waking up in the morning, patients feel vigorous, rested, feel a surge of energy. After a while, characteristic symptoms begin to build up, the patient feels literally overwhelmed.

Myasthenia gravis

Myasthenia gravis can manifest itself in different ways, it all depends on the shape. There are three of them:

1. bulbar;
2. ocular;
3. generalized.

In the bulbar form, only one local muscle group suffers. They provide chewing, swallowing, so the patient's voice begins to change. It becomes hoarse, quiet and even almost silent.

With the ocular form of myasthenia gravis, the muscles that provide movement of the eyeballs suffer. These are the muscles that lift the eyelid, the outer circular one. A patient with myasthenia gravis is easily recognizable by drooping eyelids - he cannot lift them due to muscle damage.

If myasthenia gravis is generalized, the oculomotor, facial, and cervical muscles are gradually affected. In patients, deep wrinkles appear on the face, and the smile becomes unnatural, taut. Over time, it becomes difficult for a person to even hold his head. This is a consequence of the weakening of the neck muscles.

When the disease progresses, the muscles of the arms and legs are involved in the pathological process. Such patients practically lose the ability to walk, move, since the muscles do not experience normal stress, over time it atrophies. It is the generalized form that occurs most often.

Myasthenia gravis can be accompanied by characteristic crises. This is the most severe form of the disease. During a crisis, the pharyngeal and respiratory muscles are completely turned off. This is a direct threat to life, because the movements of the chest completely stop, as a result of which oxygen starvation of the body occurs.

Diagnostics

It is very important to conduct a thorough examination in order to understand the extent to which the disease progresses, because of what it develops. For the correct selection of the treatment regimen, it is necessary to go through all the stages of diagnosis. It includes:

1. Electromyography. It will help identify a myasthenic reaction.
2. Proserine test. The patient is injected into the muscle with cholinesterase antagonist drugs.
3. Serology research. Its purpose is to identify the antibodies of the receptors for acetylcholine in the patient.
4. CT. Helps to identify possible tumors (eg thymoma).

It is the proserin test that is the main diagnostic method that can finally confirm myasthenia gravis.

Treatment

Myasthenia gravis is a serious and life-threatening condition. When such a diagnosis is made, it is imperative to immediately begin treatment of myasthenia gravis. Often, ophthalmological treatment is also required, since the disease can provoke eye dysfunctions. You will also need to eat right.

The mechanism of therapy is based on the fact that new manifestations of myasthenia gravis are constantly taken into account and the dose of drugs is adjusted. It should not exceed the one that provides a sustained therapeutic effect. Sick children and young people respond more easily to therapy, remission occurs less often in the elderly.

It is important for parents to remember that even a common cold can cause myasthenia gravis, therefore any infectious disease should be treated. Such well-known infectious disease specialists as, for example, academician Yuri Vladimirovich Lobzin insist on this. It is important to choose a good clinic that will provide all modern methods of treatment for this difficult disease.

Proper treatment can halt the progression of the disease, and in some cases, complete recovery can be achieved. Therapy must fully comply with modern standards, since in recent years in medicine, methods for treating myasthenia gravis have been significantly improved.

The symptomatology of a particular patient must be taken into account. The disease can have quite different forms and severity. It all depends on the reason for its development. It can be not only a broken genetic code, but also an infectious lesion, head injury, snake bite, etc.

Treatment will be based on maintaining the correct level of anticholinesterase substances in the blood. These funds are constantly introduced into the body. Sometimes it is difficult to immediately determine the safe dose for a particular patient, because the administration of drugs begins with extremely low doses. Such patients need constant care and regular courses of treatment.

An overdose of these drugs is fraught with serious side effects and unpleasant phenomena from the liver and kidneys. It can even provoke a cholinergic crisis, which manifests itself in the form of seizures, miosis, bradycardia. They are accompanied by pain in the abdomen. If such a crisis occurs, the patient is immediately given the correct dose of atropine.

The essence of the treatment is that an acetylcholinesterase antagonist is selected for the patient. This selection is carried out strictly individually. It is important to consider the patient's age, weight, shape and severity of the disease. Oxazil, proserin, galantamine, or kalymin are also prescribed.

If pseudoparalytic myasthenia gravis is established, the patient is additionally administered spironolactone, potassium salts. They support the state of the body. If the patient suffers from a severe form of the disease, glucocorticosteroids and cytostatics are prescribed to him. If a thymoma is detected, the only treatment is surgical removal of the tumor.

For the relief of myasthenic crisis, proserin, mechanical ventilation, plasmapheresis, preparations based on human immunoglobulin are used. If it is gravis disease (a severe hereditary form), therapy will differ from treatment for other forms of the disease.

Most often, pyridostigmine bromide is prescribed. The drug causes a number of side effects: diarrhea, abdominal pain, muscle fasciculations. An increased dose of the drug can cause a cholinergic crisis.

Immunomodulatory treatment

One of the areas of therapy is immunity modulation. For this purpose, glucocorticoids are prescribed. They are effective, relatively safe, and inexpensive. This is the secret of their worldwide popularity. Scientists have not yet fully figured out how these drugs work, but the fact is undeniable that they can significantly alleviate the patient's condition and lead to long-term remission.

This group of medicines has a number of side effects, but they directly depend on the dose. Therefore, the physician must prescribe the minimum effective dosage for a particular patient. The most popular drug in this group is Prednisolone.

It is prescribed with a minimum daily dose (10-25 mg) and then the dosage is slowly increased. Ideally, the daily dose should be 60-80 mg (single dose every other day). It can be replaced with Methylprednisolone.

If the patient suffers from a severe form of the disease, treatment is immediately prescribed with a high dose of corticosteroids. The drug is administered every day. In parallel, plasmapheresis is performed or immunoglobulin is injected intravenously. The purpose of such enhanced therapy is to stabilize the patient's condition. It will take 4 to 16 weeks to reach it. After improvement, the dose of corticosteroids is gradually reduced. They are brought to the level of supportive care.

Azathioprine is a purine analogue that slows down the synthesis of nucleic acids. It acts on lymphocytes. When using the drug, it is imperative to monitor liver function, blood condition. At first, a blood test is taken every day. If the drug is well tolerated by the patient, then after 1-2 weeks the dosage is increased. The maximum dose is 2-3 mg per kg of body weight (the average daily dose is 150-200 mg).

This remedy is quite well tolerated, although sometimes it can cause nausea, lymphopenia, skin rashes, pancreatitis, pancytopenia.

Please note that the healing effect may not occur immediately. It often appears 4-12 months after starting treatment. The maximum effect is usually observed after six months or a year.

Azathioprine is used as an adjunct to Prednisolone. It is prescribed for those patients who are taking long-term immunosuppressive therapy. Thanks to this combination, the dose of corticosteroids can not be increased without losing their effectiveness. This is the so-called sparring effect, when one drug enhances the healing effect of another.

Cyclosporin is another drug that is prescribed for myasthenia gravis. It has a complex action that ultimately results in slowing down the activation of T cells. The drug can cause tremors, insomnia, kidney failure, high blood pressure, headache. These side effects depend on the dose used. If it is reduced, unpleasant manifestations can go away or be minimized.

Cyclosporine is rarely prescribed. He has much more pronounced side effects than other drugs, therefore this remedy is used if the rest have shown low efficiency. If a medication is prescribed, it is important to control the level of electrolytes in the blood, magnesium, and kidney function. They start with small doses, gradually bringing the daily dosage to a therapeutically effective one.

If cyclosporine is prescribed, diuretics (potassium-sparing) and NSAIDs should not be taken, and when corticosteroids need to be taken, their dose should be reduced as much as possible. It will not be possible to completely cancel Prednisolone.

Mycophenolate mifetil is a modern drug. Scientists have yet to fully understand how it works, but the results are encouraging. The substance slows down the replication of B-, T-cells. When using the drug, you need to take a blood test every month. Scientists agree that Mycophenolate Mifetil is as effective as Cyclosporn, but it has fewer side effects.

Cyclophosphamide is an effective immunosuppressant that is prescribed for severe disease, inhibiting T and B cells. It is rarely prescribed, only when other drugs have not shown their effectiveness. Already after a couple of months, a stable remission is observed in 50% of severe patients. If there are noticeable side effects, this remedy will have to be canceled.

Methotrexate slows down cell division, but can provoke nausea, cystitis, mucositis, alopecia, myelosuppression. Doctors see it as a back-up drug if first-line drugs are ineffective.

Rituximab is an antibody that has an increased affinity for the CD20 cell antigen. It can cause fever, skin rashes, nausea, and sometimes bronchospasm. Between taking it, you can take a fairly long break - up to six months.

Short term therapy

Along with drugs, short-term treatment is prescribed: plasmapheresis, immunoglobulin is injected intravenously.

The mechanism of action of immunoglobulin is that it neutralizes the activated compliment, autoantibodies, modulates cytokines, etc. it can cause fever, headache, and skin rashes.

The purpose of plasmapheresis is to remove autoantibodies and other components produced by the immune system from the blood. 4-5 sessions of plasmapheresis are performed. More often it is prescribed in the process of preparing cooperation, in a serious condition, when the symptoms are growing rapidly. These two methods have approximately the same effectiveness.

Surgical treatment

The surgical method is thymectomy. It is most often used to eliminate myasthenia gravis. It is carried out if an accurate diagnosis of thymoma is made, as well as if patients with a generalized type of disease have not reached the age of 60.

Please note that thymectomy is not always indicated in the generalized form.

During pregnancy, drugs are selected with great care.

Pathogenesis

In recent years, there is a lot of new information about the pathogenesis and clinical manifestation of myasthenia gravis. Scientists have made significant progress in studying the mechanism of its development, although there are still many gaps.

Now there is an opportunity not only to achieve a stable remission, but also to predict the further development of the disease in a particular patient. In therapy, it is important to take into account the nature of the course of the disease in an individual patient, the presence of therapeutic and side effects, and their ratio.

It is important that the doctor understands the essence of this problem, the main stages of the development of the disease, thoroughly knows the entire arsenal of modern means of therapy, and possesses treatment algorithms. All this will allow to provide the patient with the most effective help.

If myasthenia gravis is detected, the prognosis depends on the form of the disease, as well as the timeliness and systematicity of treatment. The worst response to treatment is the generalized form of myasthenia gravis.

The disease is of a periodic rhythmic nature. Remission is replaced by a period of exacerbation. It is important to regularly stimulate the muscles so as not to lead to their atrophy. This is facilitated by physiotherapy exercises and gymnastics.

So, for the successful treatment of myasthenia gravis, it is important to carry out a complete diagnosis and identify the cause of the pathology. Then a complex of drugs is selected that have a minimum of contraindications and side effects. This disease is extremely dangerous, therefore, only doctors should deal with its treatment. Folk remedies are categorically contraindicated.