Myasthenia gravis (MG) is a chronic autoimmune disease caused by dysfunctional transmission between the nerve and muscle junction. It is an autoimmune disease mainly mediated by antibody to acetylcholine receptor (antiboby,Ab), cellular immune-dependent, and complement-involved, and pathologically involves acetylcholine receptor (AchR) on the postsynaptic membrane at the neuromuscular junction (MG). In other words, the nerves that innervate muscle contraction cannot transmit “signal instructions” to the muscles normally, causing the muscles to reduce or lose their contractile function, and may also involve the heart muscle and smooth muscle, showing corresponding visceral symptoms. A few cases of myasthenia gravis may have a family history (familial inherited myasthenia gravis).
The immunological etiology of myasthenia gravis is inconclusive; autoimmune diseases mostly occur on a genetic basis, and genetics may be its endogenous cause; among the exogenous causes, most believe it is related to chronic viral infection of the thymus. Patients with HLA-A1, A8, B8, B12, Dw3 myasthenia gravis are mostly female, young, with thymic hyperplasia, no tumor, low detection rate of acetylcholine receptor antibodies, ineffective with anticholinesterase drugs, early thyme removal is effective; patients with HLA-A2, A3 myasthenia gravis are mostly male, starting after 40 years old, mostly combined with thymoma, high detection rate of acetylcholine receptor antibodies. The detection rate is high.
MG occurs in all age groups, but mostly in 15-35 years old, with a male to female sex ratio of about 1:2. The onset of the disease is variable and insidious, mainly manifesting as skeletal muscle abnormalities and easy fatigue, often with good muscle strength in the morning and worsening symptoms in the afternoon or evening, most patients involve the extraocular muscles, with the levator muscle being the most susceptible, and as the disease progresses, more extraocular muscles can be involved, with diplopia appearing, and finally the eye can be fixed, with the intraocular muscles generally not being involved. The intraocular muscles are usually not involved. In addition, the medullary innervated muscles, cervical muscles, scapular muscles, trunk muscles and upper and lower limb muscles can be involved. Depending on the extent and degree of muscle involvement, the disease is generally divided into oculomotor type, medullary muscle involvement type and generalized type. In children, myasthenia gravis refers to neonatal to adolescent onset, and is mostly limited to the extraocular muscles, except for the generalized type.
The Osserman typology is often used clinically.
I oculomotor type; IIa mild generalized type; IIb moderate generalized type; III acute severe type.
IV late-onset severe type; V myasthenic type.
Adult myasthenia gravis: Type I is the simple ocular muscle type, which is a purely extraocular muscle involvement, without clinical and electrophysiological changes of other muscle groups involved, with good response to adrenocorticotropic hormone treatment and good prognosis. Type II is a generalized muscle weakness type, and type IIA is a mild generalized type, in which the extremity muscle groups are mildly involved, often accompanied by extraocular muscle involvement, and those who are not accompanied by medullary muscle paralysis live on their own, and respond to drug therapy and have a general prognosis. Type IIB is a moderate generalized type with moderate involvement of the extremity muscles, often with extraocular muscle involvement, and generally with chewing, swallowing, dysarthria and other medullary muscle paralysis. There are difficulties in self-care, and the response to medication is average. Type III is an acute progressive type with rapid progression, most of which develop ball palsy within weeks or months of onset, often with extraocular muscle involvement, and respiratory muscle paralysis within six months, with poor response to drug therapy and poor prognosis. Type IV is a late-stage generalized muscle weakness type, which is developed from the above Ⅰ, ⅡA and ⅡB. It responds poorly to drug therapy and has a poor prognosis. Type V is myasthenia gravis, which occurs within six months after the onset of the disease.
The clinical classification is also based on the range of muscle groups involved: simple ocular muscle type, medullary muscle type, spinal muscle type, generalized muscle type, and myasthenia gravis type. Clinically, regardless of any type of MG, the clinical course is generally divided into three phases: ① fluctuating phase: within 5 years after onset, especially within the first 1-2 years, there are large fluctuations in the disease, and it is prone to myasthenia gravis crisis and high mortality rate. ②Stable phase: the disease duration is within 10 years. ③Chronic stage: the disease duration is more than 10 years, the latter two stages of the patient’s condition is stable, rarely occurs in crisis, the prognosis is better.
In addition, myasthenia gravis can also occur in children and can be classified as follows: (1) Neonatal myasthenia gravis: myasthenia gravis appears on the first day after birth, manifested by difficulty in sucking and low cry. (2) Congenital myasthenia gravis: infantile myasthenia gravis appears at birth or shortly after birth and persists with extraocular muscle paralysis. (3) Juvenile myasthenia gravis refers to MG that starts at the age of 14 to 18 years, with simple ptosis or strabismus, diplopia, etc. Swallowing difficulties or generalized muscle weakness are more common than myasthenia in children, and there are also cases where only spinal muscle weakness is present.
The course of MG is chronic and prolonged, and the temporary reduction, remission, recurrence and deterioration of symptoms often alternate and constitute important signs of the disease. Most patients can be clinically cured after treatment; some patients can have a long remission period, but the disease often relapses or worsens due to various factors such as trauma, systemic infections, overexertion, pregnancy or childbirth, endocrine disorders, and menstrual periods of women. Some antibiotics, such as polymyxin, aminoglycosides such as streptomycin, kanamycin, quinolones (various kinds of fascias), sedatives and other drugs can have the effect of aggravating myasthenia gravis and should be noted.
Myasthenia gravis crisis
When the condition suddenly worsens or is not treated properly, causing respiratory muscle weakness or paralysis and resulting in severe respiratory distress, it is called myasthenia gravis crisis. There are three types.
1, myasthenia gravis crisis: that is, neostigmine deficiency crisis, which is induced by various triggers and drug reduction. Respiratory weakness, cyanosis, irritability, difficulty in swallowing and coughing up sputum, low speech until the inability to make a sound, and finally complete cessation of breathing. It may recur or become chronic.
2. Cholinergic crisis: i.e. neostigmine overdose crisis, which occurs after a momentary overdose. In addition to the above-mentioned symptoms of dyspnea, there are also symptoms of acetylcholine overaccumulation, including toxic alkaloid symptoms (vomiting, abdominal pain, diarrhea, narrow pupils, excessive sweating, salivation, increased tracheal secretions, slowed heart rate, etc.), nicotine-like symptoms (muscle tremors, spasms and tightness, etc.) and central nervous symptoms ( anxiety, insomnia, confusion, unconsciousness, convulsions, coma, etc.).
3.Reflexive crisis: It is difficult to distinguish the nature of crisis and cannot improve the symptoms by stopping or increasing the dosage. Most of them occur after long-term use of larger doses of drugs.
Three kinds of crisis can be identified by the following methods: ①Tensilon test. (2) Atropine test. ③Electromyography.
Auxiliary tests
1, muscle fatigue test (Jolly test) affected random muscle rapid repetitive contraction, such as blinking 50 times in a row, the eye fissure can be seen gradually become smaller; make the patient supine continuous head lifting 30 to 40 times, can be seen sternocleidomastoid muscle contraction force gradually weakened head lifting weakness; raise arm action or eye upward gaze for several minutes, if there is temporary paralysis or muscle weakness significantly aggravated, rest after recovery is positive; such as If the masticatory muscle strength is weak, the masticatory action can be repeated more than 30 times, if the muscle weakness is aggravated to the extent that it cannot be chewed, the fatigue test is positive.
2, anticholinesterase drugs Tensilon test and neostigmine test have the same diagnostic value and are used for the diagnosis of MG and identification of various types of crises.
(1) tensilon test: tensilon (ethyl-2-methyl-3-hydroxyphenylamine chloride) is also known as edrophonium chloride. The test should be preceded by a muscle strength assessment of specific cerebral innervated muscles such as levator aponeurosis and extraocular muscles, measurement of limb muscle strength (using a grip strength meter), and spirometry in severe cases.
Tensilon 10mg diluted to 1ml, first 2mg (0,2ml) intravenously, if there is no adverse reaction and no improvement in muscle strength after 45s, the remaining 8mg (0,8ml) will be injected slowly for about 1min. Side effects included mild muscarinic effect, such as nausea, vomiting, increased intestinal motility, excessive sweating and salivation, etc., which could be counteracted by prior subcutaneous injection of atropine 0,8mg. The results were determined: most patients had relief of symptoms after injecting 5mg, if it was myasthenia gravis, respiratory muscle weakness improved within 30-60s, and symptom relief lasted only 4-5min; if it was cholinergic crisis, it would be temporarily aggravated and accompanied by muscle bundle tremor; there was no response in antalgic crisis. A positive Tensilon test should include a significant reduction or disappearance of objective muscle contraction, lid ptosis and diplopia.
(2) neostigmine test: neostig mine methylsulfate is a synthetic compound with a chemical structure similar to that of toxic lentil base. This test is sometimes preferable to the Tensilon test because of the long duration of action, and the results can be evaluated accurately and repeatedly. 1 to 1,5 mg of intramuscular injection, either several minutes in advance or simultaneously with intramuscular injection of atropine sulfate 0,8 mg (average 0,5 to 1,0 mg), is used to counter muscarinic-like side effects and arrhythmias. Judgment of results: The symptoms usually improve 10-15 min after injection, with a peak at 20 min and last for 2-3 h. The degree of improvement can be carefully assessed.
Laboratory tests
1, blood, urine and cerebrospinal fluid routine examination are normal.
2.Suspicious MG can be measured for thyroid function.
3.Serum autoantibody profile examination
(1) Measurement of serum AchR-Ab: AchR-Ab titer increases significantly in MG patients, with a positive rate of 70% to 95% reported abroad, and is a highly sensitive and specific diagnostic test. The positive rate of AChR-Ab in systemic MG is 85% to 90%. Except for patients with Lambert-Eaton syndrome or patients with thymoma without clinical symptoms or those in remission, there are generally no false positive results. In contrast, some patients with oculomotor type, patients in remission after thymoma resection, or even those with severe symptoms may not have antibodies measured. Antibody titers are also inconsistent with clinical symptoms, and patients in complete clinical remission may have high antibody titers.
Antibodies to myocilin (myocardin, myosin, and actin antibodies) are also seen in 85% of patients with thymoma and are the earliest manifestation in some patients with thymoma. Other serologic abnormalities are also of interest, with antinuclear antibodies, rheumatoid factor, and thyroid antibodies being more common in patients than in those with normal.
(2) AchR-binding antibody (AchR-binging Ab), which is also seen in 13% of patients with Lambert-Eaton myasthenia gravis syndrome, is not recommended as a screening test, as is this antibody or rhabdomyosin autoantibody.
Electromyography
The muscle contraction force is reduced and the amplitude becomes smaller on EMG in MG patients. Low frequency (1-5Hz) repetitive nerve stimulation (RNS) is a commonly used neuromuscular conduction physiology test and is the most common method to detect NMJ disease. 2-3Hz low frequency repetitive electrical stimulation of peripheral nerves causes a rapid decrease in action potentials in innervated muscles, resulting in a decrease in EPPs due to local Ach depletion in NMJs.
Single-fiber electromyography reveals inconsistent excitation transmission between muscle fibers or conduction block.
Imaging
X-rays show thymoma in 15% of MG patients, especially those older than 40 years old, and CT or MRI of the chest: thymic hypertrophy or thymic tumors may be present.
Pathological examination
The pathological pattern of myasthenia gravis includes three major parts: muscle fibers, neuromuscular junction and thymus gland.
(1) Myofiber changes: early in the course of the disease, there are mainly lymphocytes infiltrating between muscle fibers and around small blood vessels, mainly small lymphocytes, this phenomenon is called lymphatic leakage; in acute severe disease, there is coagulative necrosis of muscle fibers, accompanied by the exudation of polymorphonuclear leukocytes of macrophages; in the late stage, muscle fibers can have different degrees of denervation changes, and muscle fibers are small.
(2) Alterations at the neuromuscular junction: morphological changes at the neuromuscular junction are the most characteristic changes in the pathology of myasthenia gravis, mainly manifested by the disappearance, flattening, or even breaking of the postsynaptic membrane folds and the decrease in the number of AchR.
(3) Thymic changes: About 30% of patients with myasthenia gravis have combined thymoma, 40%-60% have thymic hypertrophy, and more than 75% have central hyperplasia of thymic tissue. Adenomas are classified according to their cell types: lymphocytic type, epithelial type, and mixed cell type, the latter two often associated with myasthenia gravis.
Differential diagnosis
1, diseases with oropharyngeal limb muscle weakness: such as myotonic dystrophy, amyotrophic lateral sclerosis neurosis or hyperthyroidism caused by muscle weakness other causes of eye muscle paralysis, eye muscle spasm occasionally seen with mild eye muscle weakness but their eyelid closure force is weak involving the upper and lower eyelids These diseases are not difficult to distinguish from MG based on the history neurological examination electrophysiological examination and neostigmine test
Lambert-Eaton syndrome: most of the disease starts after 50 years of age and is more common in men; it is associated with tumors, with small cell lung cancer being the most common; mainly weakness of the trunk muscles in the proximal extremities; symptoms in the lower extremities are heavier than those in the upper extremities; emaciation and easy fatigue, slow movement. Muscle fatigue is felt after activity; however, muscle strength can be temporarily improved if contraction continues; occasional extraocular and medullary innervated muscles are involved; about 1/2 cases have sensory abnormalities in the extremities oral dryness impotence; cholinesterase inhibitors are ineffective in treatment; tendon reflexes are weakened but there is no muscle atrophy. In contrast, myasthenia gravis is common in women under 40 years of age; it is often associated with thymic tumors; all muscles of the body can be involved with the most active muscles being involved at the earliest; myasthenia gravis is light in the morning and heavy in the afternoon; it is aggravated after activity and decreases or disappears after rest; tendon reflexes are usually not affected; cholinesterase inhibitor therapy is effective
3, drug poisoning: botulinum poisoning organophosphorus pesticide poisoning snake bites caused by neuromuscular transmission disorders with neostigmine or epsilon chloride will also improve clinical symptoms, but these diseases have a clear medical history, including botulinum poisoning has an epidemiological history, its toxin action in the presynaptic membrane affect the transmission of nerve-muscle junction Function appears as skeletal muscle paralysis; it should be promptly treated with guanidine hydrochloride, intravenous glucose and saline.
Treatment
MG is a chronic disease, the patient can not adhere to work and study for a long time, there are certain difficulties in life, the ideological burden is great, the patient should establish the spirit of long-term struggle with the disease; for the systemic type MG patients should be bed rest, breathing difficulties to oxygen, sputum can not cough can pat the back to assist in sputum removal or aspiration, serious cases should be hospitalized. Commonly used treatment methods are as follows.
(a) Drug therapy.
1, anticholinesterase drugs is the treatment of MG temporary effective drugs, but this drug can only treat the symptoms but not the root cause, only temporary improvement of symptoms can not suppress immunity. Commonly used are.
(1) Neostigmine tablets: 15~30mg each time, 2~4 times a day; 0.5mg~1.0mg each time of injection, the effect will be seen after 30 minutes of injection, and the best effect will be seen after 1 hour, generally used for emergency or experimental use.
(2) Bromipyridamole tablets: 60～120mg each time, 3～6 times a day, the blood concentration increases one hour after taking the drug, and reaches the highest peak in 1,5～2 hours, the half-life is 4,25 hours, the drug has the characteristics of long duration of action and less side effects.
(3) Mestinon (enzyme inhibitor): 5-10 mg each time, 2-4 times a day, is the strongest anticholinesterase drug, about 2-4 times as strong as neostigmine, with a long duration of action, can be maintained for 6-8 hours. The drug has large side effects and has recently been found to have insulin-promoting effects, which can lead to hypokalemia and clinical application should be noted.
In the application of anticholinesterase drug therapy, the dose of the drug should be selected specifically according to individual differences, generally starting with a small dose and gradually increasing to maintain the best effect and maintain the ability to eat as the standard. Side effects include pupil narrowing, salivation, abdominal pain, diarrhea, sweating, muscle jumping, etc.
2. Immunosuppressants
(1) Adrenocorticotropic hormone: mechanism of action ① hormone can inhibit the synthesis of AchR antibodies, so that the AchR on the postsynaptic membrane is protected from or less damaged by autoimmune attack; ② can make the presynaptic membrane easy to release Ach; ③ can make the end plate regenerate and increase the number of AchR on the postsynaptic membrane. It is indicated for adults with generalized muscle weakness, especially after 40 years of age, who are not satisfied with anticholinesterase drug therapy; or before or after surgery for thymoma or thymic hyperplasia; or who have an active immune function before thymic radiation therapy. The method of administration should be based on the doctor’s recommendation.
Adrenal corticosteroid therapy is highly effective in the near future, with an effective rate of 96% and a remission and efficacy rate of 89%, but because the drug cannot remove the initiating antigen in the thymus, it is easy to relapse after stopping. In order to reduce relapse, we should pay attention to: ① hormone reduction should not be too fast, at least 3-6 months; ② maintenance amount should not be too small, at least 10-20mg/day; ③ do not stop the drug too early, the treatment period is at least 1 year, in order to improve the efficacy can be used with other drugs.
The side effects of hormone include Cushing’s-like performance (full moon face, obesity, hairiness), hypertension, diabetes, cataract, ulcer, gastrointestinal bleeding, psychiatric symptoms, osteoporosis, osteonecrosis, etc. To improve the effect and reduce the side effects of hormone can be used with other drugs, such as giving potassium chloride orally to improve the membrane potential; to prevent gastric ulcer and gastric bleeding, mecamylguanidine can be given orally to inhibit the production of gastric acid. Calcium gluconate can be given to promote Ach release; nandrolone phenylpropionate can be given to promote protein synthesis and inhibit protein breakdown; vitamin D and calcium can be given to prevent osteoporosis and aseptic necrosis of the femoral head. MG with active tuberculosis, peptic ulcer, severe hypertension, heart disease and other infectious diseases should be listed as contraindications to hormone therapy. Older and frailer people should be used with more caution.
(2) Cyclophosphamide, azathioprine, cyclosporine, tacrolimus, these immunosuppressive agents have varying degrees of efficacy.
Several studies in recent years have confirmed that the side effects of tacrolimus are less than those of prednisone and azathioprine, which are commonly used before.
With regard to the duration of medication, since patients will have relatively good remission of symptoms for 3-5 years after thymectomy and may have milder exacerbations afterwards, but overall it is also closely related to individual conditions. buckingham et al. reported that removal of the thymus early in the course of the disease (within 1 or 2 years) may eventually lead to complete remission in one-third of patients; about half may lead to improvement. Therefore, if symptoms gradually decrease over several years after surgery, it may be possible to try to taper tacrolimus to see if it can be discontinued, and in some patients, to stop using it. Many patients abroad have reported using it for 5 or 6 years with fewer significant side effects, but attention should still be paid to monitoring blood work, liver and kidney function, the presence of secondary tumors, etc. Since the drug has been widely used in myasthenia gravis for only the last 5-10 years worldwide, longer-term follow-up information is still scarce.
3, gammaglobulin therapy High-dose intravenous gammaglobulin can achieve significant results in the treatment of severe generalized MG rest. Intravenous gammaglobulin has been reported to increase Ts in patients with MG. It is hypothesized that intravenous gammaglobulin may protect the AchR from its antibodies by counteracting AchR I Ab or replacing AchR antibodies from their loci. Dosage: 400 mg/kg daily, side effects occasionally include headache and swelling of both feet.
4. VEP therapy, namely vincristine, cyclophosphamide and hydrogenated prednisolone (VEP) therapy, is suitable for MG patients with thymic tumors that are not suitable for surgery.
5. Prohibited and cautionary drugs Arrow toxin, succinylcholine, quinine, quinidine, chloroform, streptomycin, neomycin, kanamycin, mucin, polymyxin, vancomycin and other aminoglycoside antibiotics have neuromuscular blocking effects and should be prohibited. Morphine, dulcolax, etc. have respiratory inhibition, should be prohibited or used with caution; tetracycline and tetracycline antibiotics have increased magnesium ions and inhibit the release of Ach, should be used with caution.
(B) Non-pharmacological treatment
1, thymectomy surgery can remove the initiating antigen of autoimmune reaction in MG patients, reduce the production of immunologically active T lymphocytes, and reduce the synthesis of acetylcholine receptor antibodies, which is now considered the most fundamental treatment for MG.
Indications: (1) all types of MG with thymoma; (2) systemic MG in young women with thymic hypertrophy and high AchR antibody potency; (3) thymectomy in those who do not respond satisfactorily to anticholinesterase drug therapy, whether or not combined with thymoma or thymic hyperplasia. The remission rate after surgery increases year by year, 30% in the 1st year, 50% in the 2nd year, 65% in the 3rd year and 80% in the 6th year. Surgical efficacy: ① females are better than males; ② the milder the disease, the shorter the course of the disease, the better; ③ the more growth centers in the thymus, the more obvious the epithelial cells, the better the surgical effect; ④ the immediate postoperative effect with adrenocorticotropic hormone is good.
2, radiation therapy ① thymus radiotherapy: can inhibit the thymus immune function, so that the blood T lymphocytes and B lymphocytes decreased, AchR antibody reduction, so that the thymus atrophy. The method is deep X-ray or 60 cobalt irradiation, the efficacy is similar to thymectomy, the side effects include local red pain and ulceration, lower white blood cell count, general discomfort, headache, loss of appetite, etc. ②Spleen radiotherapy: Inhibiting the function of B lymphocytes and thus the production of AchR antibodies, it reduces or alleviates the symptoms of MG. The side effects include mild diarrhea and intestinal rumbling. (③) Systemic low-dose radiotherapy: it can reduce lymphocytes and AchR antibodies and normalize Ts cells. Side effects include general malaise, weakness, insomnia, loss of appetite, etc. ④Local irradiation: MG patients have excessive accumulation of AchR antibodies on the local postsynaptic membrane at the neuromuscular junction, which can be suppressed by local radiotherapy.
3.Plasma exchange Regularly replace the plasma of MG patients with normal human plasma or plasma substitutes to reduce the AchR Ab in the patient’s blood, calculate the blood volume by 5% of body weight, exchange 1000ml~2000ml of plasma each time, once a day or every other day, 5~6 times for a course of treatment. This course of treatment is fast-acting and has good recent efficacy, but it cannot be sustained. Therefore, it is used for patients with critical episodes, or those with frequent critical episodes, or patients with refractory MG. Improvement begins in the first 1-2 weeks after plasma exchange therapy, antibodies fall, and then slowly rise again, and symptoms appear gradually again, so other immunotherapy or thymectomy, or thymic radiotherapy are used after remission. Side effects: hypotension, syncope, hypoproteinemia, numbness of extremities, bleeding, thrombosis, allergy, etc. The high cost of this therapy is not easy to promote.
4.Blood replacement therapy is to release blood through a vein and then transfuse an equal amount of fresh allogeneic blood. Each blood exchange volume is 800~1000ml for adults, 100~200ml for children, 600~800ml for the elderly and teenagers, once every 3~7 days according to the condition, 3~4 times as a course of treatment, monitor blood pressure and pulse rate when bleeding, if blood pressure is lower than 12kPa (90mmHg) and pulse rate is more than 120 times/minute, stop bleeding or maintain blood pressure by drip 706 plasma. The mechanism of action is the same as plasma exchange or plasma cleansing therapy, blood exchange can partially remove disease-causing antibodies to rescue patients in critical condition, and can be carried out in both urban and rural areas.
(iii) Treatment of myasthenia gravis crisis.
Critical signs are the most critical state of MG, with a high morbidity and mortality rate of 15,4% to 50% reported. First of all, we need to clarify which kind of critical signs belong to, and then carry out active resuscitation measures.
1, keep the airway open When there is respiratory muscle paralysis, sputum discharge difficulties, to do tracheal intubation or tracheotomy, the application of artificial ventilator-assisted breathing, to ensure adequate ventilation, correct hypoxia, to remove respiratory secretions in a timely and thorough manner.
2.Actively control the infection Choose effective and sufficient antibiotics to control the infection, use lincomycin, penicillin, erythromycin, cephalosporin, fudaxin, bacteriophage, etc.
3.Hormone Choose methylprednisolone 500-2000mg/day, or dexamethasone 10-20mg/day with sugar drip, and change to oral prednisone after 3-5 days of stable condition.
4, cholinesterase inhibitors cholinergic crisis to stop the use of anticholinesterase drugs, while intramuscular or intravenous injection of atropine 0, 5 ~ 2, 0mg, 15 ~ 30 minutes after the symptoms do not improve significantly can be repeated application. In myasthenia gravis crisis, anticholinesterase drugs are increased, and when the effect is not obvious, anticholinesterase drugs are discontinued, because the sensitivity of AchR to Ach can be significantly reduced after prolonged and massive application. In the case of tracheal intubation with positive pressure oxygen to control respiration, all cholinesterase inhibitors (ChEl) should be stopped immediately, so that the AchR can get sufficient rest and gradually improve the sensitivity of AchR to Ach, and generally after 3-5 days of discontinuation, the respiratory muscle function starts to recover, and according to the degree of recovery of the patient’s muscle strength, ChEl can be applied again from small doses, and the dosage can be gradually increased to the optimal dose, which can play a better The patient’s respiratory muscle function starts to recover after discontinuation.
5. Plasma exchange Plasma exchange can remove AchAb from blood and restore AchR function. After the condition improves, continue to use other therapies to control muscle weakness.
6.Intravenous infusion can correct the imbalance of water and electrolyte balance in time.
7.Strengthen nursing care Pay attention to oral care, according to strict tracheotomy care, keep the respiratory tract humidified, prevent asphyxia, etc.
Chinese medical understanding of myasthenia gravis
The clinical manifestations of myasthenia gravis are quite different from those of Chinese medicine, for example, if the eyelid is weak and drooping, it belongs to “lid wasting” or “cell drooping” in Chinese medicine; if the object is heavily shadowed, it is “visual disorder”; if the head is raised, it is “head tilting”. If the head is weak, it is “head tilting”; if the limbs are weak, it is “impotence”; if the respiratory difficulty and muscle weakness is critical, it is “atmospheric subsidence” and “deconditioning”. “dehiscence” and other symptoms.
Common Chinese medicine classification
1. Spleen qi deficiency type: drooping eyelids, impotence and weakness of the limbs, gradually worsening, worse with exertion, relieved after rest, weakness and lassitude, little breath and lazy speech, reduced diet, choking and coughing after eating, or even difficulty in swallowing, loose stools, floating face. Fat tongue, thin white coating and weak pulse.
2.Yin deficiency of liver and kidney: drooping eyelids, strabismus or diplopia, weakness of the lower limbs, inability to stand for a long time, or even unfavorable movement, thin muscles, soreness and weakness of the lumbar spine, tinnitus and muddiness of the eyes, irregular menstruation in women, spermatorrhea and impotence in men, hot flashes and night sweats. The tongue is red and less bitter, and the pulse is fine.
3.Yang deficiency of spleen and kidney: drooping eyelids or weakness of limbs, or even general weakness, easy fatigue, cold fear of limbs, soreness and weakness of waist and knees, frequent urination or nocturnal urination, loose stools or incomplete grains, pale tongue with teeth marks on the side, thin white tongue coating, sunken and thin pulse.
4.Qi and blood deficiency type: droopy eyelids or generalized weakness, withered or pale face, thin limbs, low diet, low voice and shortness of breath, dizziness and weakness, women with irregular menstruation, low volume and light color, light and tender tongue, thin white tongue coating, and weak pulse.
We believe that although generalized myasthenia gravis belongs to the category of impotence, it has the basic characteristics of being light in the morning and heavy in the evening, remitting and recurring, and often recurs and worsens with exertion, cold and pregnancy, and belongs to the category of wind impotence. Ling Shu? The evidence of “wind impotence” contained in the chapter of “The Disease Form of the Internal Organs of Evil” seems to be similar to this disease. “Wind impotence is a condition in which the limbs are not used, but the heart is as wise as if there were no disease.” The evidence is that the limbs cannot be used at will, but “the heart is as wise as if there were no disease”, which means there is no mental disorder.
The treatment should be divided into the acute and remission phases; the acute phase is based on positive deficiency, and there are also evil realities, the positive deficiency is mainly qi deficiency, involving the two deficiencies of spleen and lung, spleen and kidney; the evil realities are mainly poisonous or phlegm and dampness, the deficiency of qi is not used, then impotence and weakness, and the invasion of turbid (phlegm) poison is hyperactive and harmful, corrupting the organism, immune attack and resulting in qi disconnection, the mental mechanism does not reach, the limbs are impotent. Treatment should be carried out in parallel to support the righteousness and dispel the evil, in order to achieve the recovery and reinstatement of impotence; in the remission period, further care should be taken to restore the righteousness, double tonification of the spleen and kidney to restore the normal immunity of the body and prevent recurrence.
According to our experience, MG acute stage and remission Qijun when the main treatment method is to nourish the spleen and kidney, it is very crucial to keep the law and keep the prescription, there is no need to adjust the prescription frequently, when the crisis occurs, despite the combined lung infection and other fever, sputum, wheezing, yellow urine and other evil heat symptoms, still should adhere to the deficiency of the main, supplementation and attack at the same time, in order to turn the crisis into peace, back to life.
In general, the effect of TCM treatment starts in 1-2 weeks, with the fastest remission in 6 weeks, and most patients are in complete or near complete remission within 6 months. 2 weeks to 8 weeks to stop taking hormones or immunosuppressants, no need to take bromipyridamole in mild cases, systemic type and severe dysphagia can be taken in small amounts, and usually reduced and stopped within 3 months. After remission, it is generally recommended to continue taking herbal medicine in reduced doses for 1-2 years, which can significantly reduce recurrence and restore and improve immunity.