Overview: is myasthenia gravis myasthenia gravis (myasthenia gravis, MG) is a point at the neuromuscular junction of striated muscle conduction disturbances in autoimmune diseases, muscle fatigue in the morning twilight light weight, rest or after treatment with cholinesterase inhibition is characterized by ease. Often involving the extraocular muscles, masticatory muscles, swallowing muscles and respiratory muscles. Severe palsy. The distribution of muscle involvement by people with time, rather than a paralysis that occurs when nerve damage performance. The disease should be referred to as acquired autoimmune myasthenia gravis, often referred to as myasthenia gravis.[Cause]
myasthenia gravis is caused by what the?
(A) etiology 1. myasthenia gravis is a neuromuscular transmission effects of autoimmune disease, its exact pathogenesis is still unclear, but on the disease is still a lot of research, of which the most studied is the relationship between myasthenia gravis and thymus, and acetylcholine receptor antibodies in myasthenia gravis in the role, and the large number of studies have found that patients with myasthenia gravis neuromuscular junction sudden Aftertouch membrane acetylcholine receptors (AchR) reduce the number of anti-AchR antibody receptor site exists, and the postsynaptic membrane complexes of igg and C3 deposition. And proved that the serum anti-AchR antibodies increased and postsynaptic membrane caused by the deposition of an effective reduction in the number of AchR is the main reason Benbingfasheng. The thymus is the main site AchR antibodies, therefore, the incidence of this disease generally have a close relationship with the thymus. Therefore, the regulation of human AchR, the number increased to resolve the postsynaptic membrane deposition, inhibit the production of anti-AchR antibodies is the key to cure the disease. 2 In recent years, genetic susceptibility of human leukocyte antigen (HLA) study, MG incidence may be related to genetic factors, according to MG onset age, sex, associated with thymoma, AChR-Ab-positive, HLA and other relevant and comprehensive assessment of treatment response , MG can be divided into two subtypes: with HLA-A1, A8, B8, B12, and DW3 of MG patients, mostly women, 20 to 30 years of age onset, combined thymic hyperplasia, AChR-Ab positive rate is low, taking poor efficacy of anti-cholinesterase drugs, early removal of the thymus is better; with HLA-A2, A3 of MG patients are male, 40 to 50 year-old disease, and more with thymoma, AChR-Ab detection rate was higher cortical steroids is effective; in diagnosis and treatment of 850 cases of domestic xu Xianhao MG patients, two pairs of twins (both sisters). 3 Recent studies have found, MG and non-MHC antigen genes, such as T-cell receptor (TCR), immunoglobulin, cytokines, and apoptosis (apoptosis) and other related genes, TCR gene rearrangement is not only associated with MG and may be related to thymoma associated MG patients to determine the TCR gene rearrangement can not only provide help for the early diagnosis of thymoma, MG-specific treatment is based. 4.MG peripheral blood mononuclear cells (MNC) glucocorticoid receptor decreased plasma cortisol levels were normal, animal experiments suggest that glucocorticoid receptor to reducing vulnerability to trigger EAMG. (B) normal pathogenesis of muscle fatigue is the number of consecutive contractions decrease the release of Ach, MG of pathological muscle weakness or muscle fatigue is due to reduced transmission AchR at the NMJ disorders. Ach and AchR combined produce enough to make the muscle fiber contraction of the endplate potential, MG of the NMJ as AchR reduction in the number and antibody competition effect, so that the end-plate potential can not be effectively expanded to muscle fiber action potential, motor end plate to pass blocked the muscle contraction decreased This change is reflected in the first movement the highest frequency, AchR minimal muscle and brain control muscles. 125I labeled with α-bungarotoxin and extracted human skeletal muscle acetylcholine receptor binding compound can be measured in serum anti-acetylcholine receptor antibodies, antibody blocking degradation of postsynaptic receptors, autoantigen (nAchR) activity decreased, reducing the surface area of postsynaptic membrane. As the nerve - neuromuscular transmission disorder, resulting in symptoms of myasthenia gravis. degeneration of muscle fiber atrophy later, replaced by fibrous tissue. Fambrough et al (1973) confirmed that the basic defect MG NMJ postsynaptic membrane is the apparent lack of AchR and EAMG animals were detected in serum AchR-Ab, using immunofluorescence can be found in the postsynaptic membrane AchR and AchR-Ab and complement immune complex deposition. MG patients with muscle biopsy also found significantly reduced AchR, AchR-Ab to determine the pathogenicity of the autoimmune theory for the MG to provide strong evidence. The human AchR-Ab into normal animals can make the disease, the evidence to meet the autoantibody-mediated disease diagnostic criteria (Drachman). About 85% of the systemic MG and 50% of ocular MG patients can be detected AchR-Ab, MG mothers of newborns can be found in AchR-Ab, making the diagnosis of MG antibodies as sensitive and reliable indicators. MG's own immune response abnormalities are not yet clear, about 70% of MG patients with thymic abnormalities, of which 10% to 15% with thymoma, 50% to 60% of the combined thymus hypertrophy and hyperplasia of lymphoid follicles, the condition improved after removal of the thymus. central to the immune thymus in the pathogenesis of myasthenia gravis play an important role. Whether epithelial cells (including muscle-like cells) or thymus (lymphoid) cells were immune attack, break immune tolerance, the occurrence of humoral immunity (such as nAchR-Ab) and cell-mediated immunity (eg, sensitized T cells), are caused by for nAchR autoimmune response, and thus disease. Thymic T cell maturation is place in the thymus was detected in AchR subunit mRNA, MG patients with thymus-derived t cells and b cells in peripheral blood of AchR response than similar cells strong. In addition, the normal and hyperplastic thymus contain muscle-like cells (myoid cells), similar to the striated muscle cells and containing AchR. Genetic predisposition in certain individuals, some kind of virus on the thymus contains muscle-like cells AchR tendency (tropism), which can damage cells and lead to cell surface AchR conformational changes, induced AchR-Ab formation, but also the potential danger caused by the tumor may be about 10% of MG patients with thymoma reasons. MG patients with thymic t cells sensitized AchR-rich, IgG-type AchR-Ab by antigen-specific T helper cells (CD4) activated by the peripheral lymphoid organs, bone marrow and thymus in the plasma cells. But the thymus is not the only source of AchR-Ab, after removal of the thymus all patients still long AchR-Ab, possibly through stimulation of antigen-specific helper t cells in peripheral lymphocytes AchR-Ab. MG patients may have natural killer t cells (nature killer T-cell, NKT) and other lymphocyte phenotypic changes, such NKT cell number and dysfunction in the pathogenesis of MG is unclear. MG patients often accompanied by other autoimmune diseases such as systemic lupus erythematosus, rheumatic and rheumatoid arthritis, Sjogren's syndrome, hyperthyroidism, thyroiditis, and polymyositis, and some autoimmune MG patients, while not merging disease, but can be detected autoantibodies such as anti-thyroid microsomal and thyroglobulin antibody, anti-nuclear antibody, anti-gastric parietal cell antibodies and anti-islet b-cell antibodies. MG patients with HLA-B8, DR3 and DQB1 genotype frequencies higher, suggesting that the incidence may be related to genetic factors are also relevant. The main pathological changes are as follows: MG muscle to change into coagulation necrosis, fibrosis and inflammatory lymph overflow three stages. 8% to 20% of MG patients with muscular atrophy, a common neurogenic and myogenic damage, visible muscle fiber diameter sizes of the fracture, proliferation, nuclear translocation to the central, glass-like degeneration and connective tissue hyperplasia. Young patients with a higher incidence of muscle damage, about 42%, only 12% of children. The most important lesions in the motor endplate ultrastructure level, Engel et al (1976) electron microscope and size of the nerve endings of the disease reduced, NMJ presynaptic membrane widened, the number of vesicles and the amount of ACh contained in the normal range. Postsynaptic membrane extension, the primary synaptic gap widened from the normal 200A ° to 400 ~ 600A °, reduced synaptic folds, shallow, broken and wrinkled surface, the lack of secondary folds, basement membrane of the synaptic gap can be seen like substances accumulate to form the basis of neuromuscular block, known as synaptic loss of nerve function. About 60% of MG patients with thymic lymphoid hyperplasia (lymph oid hyperplasia), limited to the thymic medullary germinal centers, the young patient is high. 10% to 15% of MG patients with thymoma, MG with thymoma histopathological changes can be divided into three types: epithelial cell type, cell type and mixed cell type (epithelial cells and lymphocytes), rare thymoma shuttle shaped cell tumors and Hodgkin's granuloma, each about 1%. thymoma is generally benign.[Sign]
What are the early symptoms of myasthenia gravis?
the disease can be seen at any age, onset age of our patients were more common in childhood, age of onset 20 to 40 more women, mostly middle-aged men after the onset , associated with thymoma were more common. Infants born to female patients, of which about 10% of transfusion through the placenta to obtain the nicotinic acetylcholine receptor antibody (nicotiniacetylcholine receptorantibody, nAchR-Ab) may be temporarily unable to symptoms. Small number of family history. Insidious onset, those who have Jiqi outbreak. 1 The traditional type: ocular, and bulbar muscular body type. (1) of ocular myasthenia gravis: clinical features of pathological involvement of skeletal muscle fatigue were the symptoms more tired in the afternoon or evening after the increase in the morning and rest after mitigation, the law was twilight morning light weight fluctuated. muscle weakness is usually late morning light weight, can be varied, the latter can in incomplete paralysis; muscles are not evenly affected, most commonly involving the extraocular muscles, the early symptoms, but also long-term restricted to muscle. Light eyes, weakness, ptosis, showing asymmetric distribution, the amount of compensatory muscle contraction on the mention. eye movement is limited, there strabismus and diplopia, severe eye stationary. eye muscle is generally not affected, the pupil reflex were normal. (2) medullary type (or ball) myasthenia gravis: facial muscles, tongue, masticatory muscles and sore muscles are also likely involved; eyes closed, incomplete, and the amount of grain flattened nasolabial fold, after the laughter when the mouth than the upper lip raised constrictor muscle More muscle weakness, raised lips grinning like roar like; chewing weakness, difficulty swallowing, tongue movement does not ease; soft palate muscle weakness, pronunciation was nasal; low tone, after a short conversation or hoarseness. (3) generalized myasthenia gravis: cervical, trunk and limbs muscles may also be sick, showing the rise of difficult, often hand to support the head; chest tightness, shortness of breath, walking, fatigue, not a long line; face, hair, clothing difficult to support; tendon reflex, no sensory disturbances; respiratory muscles, diaphragm involvement, cough, weakness, difficulty breathing, severe & may be due to respiratory muscle paralysis secondary to aspiration pneumonia can be fatal. myocardial involvement even sudden death can be, smooth muscle and bladder sphincter is generally not involved; severe limb weakness, upper limb weight in the lower limbs, proximal more important than the distal. Occasionally muscular atrophy. Above type is not radically separated, there is often mixed, and to certain types of symptoms are more prominent. Protracted course of the disease extension, during which can be alleviated, recurrence or deterioration. Colds, diarrhea, agitation, fatigue, and menstruation, childbirth or surgery often sicker, or even life-threatening crisis. Quinine, quinidine, procainamide, penicillamine, propranolol, phenytoin, lithium salt, tetracycline and aminoglycoside antibiotics can exacerbate symptoms, avoid using. Clinical examination involved muscle fatigue, muscle weakness continued activity lead to a temporary increase in the short term improved after the break of the performance characteristics of the MG; involvement gravis does not meet any single nerve, nerve root or the distribution of the central nervous system lesions; progress of cases involved muscle may Mild muscle atrophy, feeling normal, usually without reflex. Clinical classification: 1.Osserman classification: Ⅰ type: ocular (15 ~ 20%), extraocular muscle involvement alone. The main symptoms are purely extraocular muscle involvement, manifested as unilateral or bilateral ptosis, diplopia or strabismus phenomenon. Corticosteroid treatment is effective, the prognosis is good. Ⅱ: body type, involving more than one set of muscles control the medulla oblongata, severe illness than type I, involving the neck, items, back and limbs, trunk muscles. according to its severity can be divided into Ⅱ a and Ⅱ b type. Ⅱ A type: mild systemic (30%), slow, no crisis, often accompanied by extraocular muscle involvement, without chewing, swallowing, and dysarthria, lower limb weakness was, CASTLE leg weakness, chest tightness or shortness of breath without symptoms. Good response to medication, the prognosis is good. type Ⅱ B: moderate body type (25%), severe skeletal and bulbar muscle involvement, significant weakness, life can still take care of themselves, accompanied by mild dysphagia, when the flow of juice into the improper cough, feel chest tightness, breathing poor. No crisis, poor drug sensitivity. Ⅲ: severe aggressive type (15%), critical symptoms progress rapidly, within a few weeks or months to reach its peak, the high incidence of thymoma. crisis can occur, efficacy poor, often require tracheotomy or ventilation, and high mortality. Ⅳ: severe late-type (10%), symptoms of the same type Ⅲ, from the development of type Ⅰ Ⅱ A, Ⅱ B-type, advanced by more than 2 years gradually evolved. drug treatment is poor, and poor prognosis. Ⅴ type: muscle atrophy, muscle atrophy onset six months of life can not take care of themselves, difficulty swallowing, food being diverted to the trachea and choked out by the nostrils. slurred speech accompanied by chest tightness or shortness of breath. Occur due to chronic weakness and muscle atrophy secondary does not belong to this type. course repeated more than 2 years, often by the type Ⅰ or type Ⅱ evolved. crisis is a sudden increase muscle weakness, particularly in the respiratory muscles (including the diaphragm, intercostal muscles), and severe weakness of throat muscles, causing breathing difficulties, throat and airway secretions increase and can not discharge, sputum, or be artificial respiration. On the basis of more in the heavy-induced muscle weakness, associated with thymoma were more susceptible to crisis. crisis can be divided into three kinds: (1) myasthenia gravis crisis is aggravated due to the disease itself, then cholinesterase inhibitor drugs are often insufficient, increase the dose or after intravenous injection of muscle Tang Hei Long strength improved. Often induced by the cold, may also occur in the application of nerve - muscle blockers (eg, streptomycin), high-dose corticosteroids and thymus after radiation therapy or surgery. (2) cholinergic crisis due to excessive cholinesterase inhibitor, from the hydrolysis of Ach in the synaptic accumulation of too much, the performance of cholinergic toxicity; weakness increased, muscle bundle fibrillation (nicotine-like reaction , over the end-plate membrane depolarization); miosis (diameter less than the natural light 2mm), sweating, increased saliva (muscarinic response), headache, nervous (central nervous system response). injection Tang Hei Long inability symptoms did not improve and actually worsened. (3) anti-bend crisis a temporary failure of cholinesterase inhibitors, increase the dosage to no avail. (2) Other types: (1) Neonatal MG: about 12% MG mothers of newborns have difficulty sucking, crying, weakness, paralysis of limbs, especially respiratory insufficiency to be the typical symptoms. Symptoms within 48h after birth, for several days to several weeks, symptoms of improvement, until they disappear. Mother, children can be found AchR-Ab, antibody titers decreased with symptoms disappear. plasma exchange can be used to treat children with severe respiratory insufficiency, mechanical ventilation and nutrition is key to the treatment. (2) congenital MG: rare, but severe symptoms. Usually asymptomatic in the neonatal period, infancy, the main symptoms are ophthalmoplegia, limb weakness is also evident, with a family history. AchR-Ab-negative, but positive response to repeated electrical stimulation. gene mutations known to AchR ion channel diseases resulting from slow-channel syndrome and fast channel syndrome. The former ion channels open and to extend the period of abnormal enhancement of the Ach response, particularly in the forearm extensor muscle selectively weakened, quinidine is effective, can extend the open period shortened; the latter diminished Ach response. Known AChR w subunit of the 24 mutations are autosomal recessive inheritance, caused by a serious lack of endplate AchR. Anti-cholinesterase drugs may be effective. (3) drug-induced MG: can occur in the use of penicillamine treatment of rheumatoid arthritis, scleroderma, liver degeneration patients. Clinical symptoms and AchR-Ab titers and adult-MG is similar to withdrawal symptoms. Diagnosis is based in part or in whole body skeletal muscle fatigue, fluctuating muscle weakness, increased after the event, rest after mitigation, and twilight morning light weight characteristics, physical examination no other signs of nervous system, decreasing amplitude low-frequency repetitive stimulation and cholinesterase inhibition drug treatment is effective and ultra-sensitive drugs such as curare pharmacological characteristics, and increased serum AchR-Ab and so on. A diagnosis of early disease signs include ptosis, diplopia, speech effort, difficulty swallowing, and mild limb muscle weakness, etc., the brain control muscles fatigue after sustained activity, such as staring at the ceiling can be increased ptosis, gaze or reading 2 diplopia after 3min, take a break after the resumption. Can be difficult to diagnose cases of fatigue (Jolly) test, Tang Xi-Long or neostigmine test, AchR-Ab determination, repetitive nerve stimulation screening.[Aftertreat]
myasthenia gravis ate?
suitable for the food we eat: the spleen and stomach deficiency in patients with myasthenia gravis, should eat Gan Wen Buyi of the goods, can play a benefit, and in the priorities of the role of common benefit Food:
① meat: beef, pork, dog, rabbit, chicken, etc.;
unfit to eat food:
pathogenesis of myasthenia gravis and closely related deficiency, it is more important to adjust the diet , not too hungry or too full, but should be appropriate to deploy a variety of nutrients, not a partial eclipse.
after eating the following foods aggravate the disease, should avoid eating: radish, mustard, green beans, kelp, seaweed, Jianhua, watercress, lily, watermelon, bitter gourd, melon, cabbage, soy milk, soy milk, cold drinks, etc., especially the radish and mustard most critical;
(2) myasthenia gravis therapeutic bone black female, the cock of the workers only, the chamber 9 g ginseng stuffed, raw astragalus 60 grams of ginger 6 grams, to suture, add wine, water, half and half, emergency fire boiling into the casserole, skim foam, slow fire Jiong stew, crisp flesh rotten to the bone, boiled soup, a little seasoning, divided on sub-Dayton, decoction and chicken, dressed in uniforms.
(3) myasthenia gravis therapeutic lamb, 500 grams, to the fascia wash, add Jiujin night, cut meat, ginseng powder, add 30 grams of yam 500 grams, a little seasoning into the sauce, pack into ravioli, served daily 5 to 10, dressed in uniforms.
(4) aries kidney 2, or son of a kidney pair, the first kidney gravy boiled, filtered crude residue, add a little wine, and then the next rice 500 grams, thick sticky porridge three times a day, every day service points. (1) bone marrow powder: pigs or cattle bone powder 500 grams of black sesame seeds 500 grams a little saute of research to fine, add sugar, mix well, 10 grams per serving, 2 times a day, under warm water. lungs and kidney marrow can fill.
myasthenia gravis should be how to prevent?
1. spirit, keeping emotions comfortable
mental and emotional activity is closely related to the body's physiological changes, fine-chi comfortable and cheerful, then the air flow, blood reconcile, the coordination function of organs, upright strong and difficult to occur, even if happen, it would soon be restored; the contrary, if the poor emotional, mental depression, will enable the air against the chaos, yin and yang, blood disorders, organs dysfunction, righteousness decreased to occur. Clinically, the disease often occurs in patients with long-term mental stress or over-thought, sadness and other emotional changes, and if the recovery period, patients often can cause fluctuations in emotional development or worsening of the disease, so patients in the treatment and recovery process, must pay attention to the spirit nursed back to health, to maintain ideological purity and stability, not greed, delusion, so infuriating rolls, keep within the spirit, the only way to make a speedy recovery of the disease.
2. reasonable, not partial addicted
reasonable and adequate to ensure the necessary conditions for human growth and development. "Gomi entrance, hidden in the stomach, to keep Wu Zang gas." Less than that is missing, affecting blood biochemistry, is leading to physical weakness, on the contrary, too much can also damage the stomach, the course of time lead to deterioration in health, therefore, muscle weakness in patients on to meat and vegetables, refined grains with whole grains, children must be under the negative correction habits, so as to enhance the patient physique, vigorous upright, so that the disease speedy recovery.
3. work and rest, living there often
the incidence of muscle weakness and excessive fatigue has a lot to the patients are often associated with overwork, overuse, worked hard day and night, or because of back and forth while living disorders, Hao Shang blood, take advantage of declining physical evils resulting Benbingfasheng and development, and therefore the patients in the recovery process, some to be living there often, work and rest, the only way to meet the drug treatment, and gradually increase Taiwan constitution, early recovery.
4. clear diagnosis of the patients, who can affect neuromuscular transmission function of the drug should be avoided, such as: aminoglycoside antibiotics - streptomycin, kanamycin neomycin and gentamicin, etc., peptide polymyxin, tetracyclines - chlortetracycline, oxytetracycline, and reduce sarcolemma excitability drugs - quinine, quinidine, pu Luoka due and so on. In addition, disabled propranolol, phenytoin, and penicillamine, etc.[Treat]
myasthenia gravis prevention
1, boost morale, keep emotions at ease
mental and emotional activity is closely related to the body's physiological changes, fine-chi comfortable and cheerful, then the air flow, blood reconcile, the coordination function of organs, upright vigorous, less prone to disease, even disease, can be quickly restored; the contrary, if the poor emotional, mental depression, will enable the air against the chaos, yin and yang, blood disorders, organs dysfunction, righteousness decreased to outbreak of diseases. Clinically, the disease often occurs in patients with long-term mental stress or over-thought, sadness and other emotional changes, and if the recovery period, patients often can cause fluctuations in emotional development or worsening of the disease, so patients in the treatment and recovery process, must pay attention to the spirit nursed back to health, to maintain ideological purity and stability, not greed, delusion, so infuriating rolls, keep within the spirit, the only way to make a speedy recovery of the disease.
2, a reasonable diet, not partial addicted
reasonable diet and adequate nutrition is to ensure that human growth and development necessary conditions. "Gomi entrance, hidden in the stomach, to keep Wu Zang gas." Inadequate diet is the lack of nutrition, affecting blood biochemistry, is leading to physical weakness, on the contrary, eating too much can also damage the stomach, leading to physical decline in the course of time, so gravis in the diet to meat and vegetables, refined grains with whole grains, some children To correct the bad eating habits, so as to enhance the patient body, vigorous upright, so that the disease speedy recovery.
3, work and rest, living there often
the incidence of muscle weakness and excessive fatigue has a lot to the patients are often associated with overwork, overuse, worked hard day and night, or because of back and forth while living disorders, Hao Shang blood, take advantage of declining physical evils resulting Benbingfasheng and development, and therefore the patients in the recovery process, some to be living there often, work and rest, the only way to meet the drug treatment, and gradually increase Taiwan constitution, early recovery.
traditional Chinese medicine:
muscle rehabilitation soup; codonopsis 20g grass 25g's Snow River 15g; yi Wood governs 10g 15g 10g Shan yu fructus 10g 10g silkworm dodder 10g; tangerine peel yam 12g medlar 12g 8g 8g cohosh Zhi Cao 6g; placental 10-inch sub-10g cordyceps ephedra 1g 3 A beads scattered strychnos 10 0.2 compound granules
with over 1958 cases of patients with myasthenia gravis treated by medication 2-4 treatments, which were 1031 cases cured, markedly effective 875 cases, up 97.35% efficiency, the more the latter follow-up after 3 years, none of recurrence.
usage 】 Shuijianbi each pay fried three times, against one, divided 3 times, 2-3 times daily for 15 to pay a course
myopathy based on clinical performance will be divided into the spleen and stomach, blood deficiency, liver and kidney deficiency and spleen deficiency four common syndromes, according to the "virtual you supplemented, the loss of those benefits "of treatment principles, to spleen Qi, replenishing qi, nourishing the liver and kidney, kidney yang as the basic treatment, supplemented by blood stasis and promoting blood circulation, Shujin other therapies, the choice of Astragalus, Ginseng, Atractylodes, Angelica, Bupleurum, Cimicifuga, medlar, monkshood, Epimedium, Curculigo, etc., and follow through by oral administration of topical drugs bath, massage, acupuncture and other comprehensive treatment of the disease.
(1) preferred solution: removal of the thymus, if after a marked deterioration in the condition, can be supplemented by plasma exchange, a large dose of immune globulin, adrenal corticosteroids and cholinesterase inhibitor treatment.
(2) times the alternatives: a serious condition were not available thymus removed plasma exchange or high-dose immunoglobulin therapy with adrenal corticosteroids, a gradual transition to corticosteroids alone , her condition improved and stable removal of the thymus underwent 2 months, after 2 months to maintain the original dose, and then the slow reduction 2 to 4 years, to the disabled.
(3) three program options: the removal of the thymus can not or refuse to MG patients, the preferred critical plasma exchange or high-dose immunoglobulin, non-critical corticosteroid treatment of choice, reduction in the course of corticosteroids may be appropriate to increase with azathioprine and other immunosuppressive drugs, reduce or mitigate the rebound phenomenon.
(4) four selected programs: the thymus can not be refused or removed, they refuse or can not tolerate corticosteroid treatment of MG patients, cyclophosphamide and other immunosuppressive drugs available the treatment.
2. the main treatment
(1) cholinesterase inhibitors: common neostigmine, pyridostigmine bromide, Imber chloramine (enzyme inhibition Ning). Pyridostigmine bromide the most commonly used, fewer side effects, the initial dose for adults 60mg orally every 4h1 times. May increase the dose based on clinical manifestations, patients with eating difficulties in medication 30min before meals, early morning walking difficult if the patient can get up before the service in the long-term pyridostigmine bromide 180mg. Its muscarinic side effects manifested as abdominal pain, diarrhea, nausea, vomiting, salivation, increased bronchial secretions, tears, sweat and other benefits miosis, pre-atropine 0.4mg can relieve their muscarinic symptoms, but the Alto products can cause excessive mental symptoms. There is no evidence that two drugs better than monotherapy. Although the efficacy of anticholinergic drugs is better, but there are limited pieces, such as the ocular MG drooping eyelids can be improved after treatment, but in some cases double vision often persists; generalized MG can significantly improve symptoms, but it is difficult to eliminate. These drugs can inhibit cholinesterase activity, from the hydrolysis of Ach, can improve neuromuscular transmission of neurotransmitters, so that a temporary improvement in muscle strength, as an effective symptomatic therapy. Usually with pyridostigmine (pyridostigmine) 60 ~ 120mg, 3 ~ 4 times / d, taking half an hour before a meal. Small dose and gradually increase the amount of transferred muscle strength improved, able to maintain daily activities, eating and appropriate, to avoid overdose. To ease the pain, salivation and other side effects, which can serve a small amount of atropine. Furthermore, it should prevent long-term abuse of cholinesterase inhibitors, in order to avoid impeding nAchR repair.
(2) immunosuppressive drugs:
① corticosteroids: a strong inhibitory effect on T cells. inhibition of thymic germinal center formation, reducing the nAchR-Ab synthesis. Applicable to all types of MG, ocular children markedly faster and obvious, but each time in cold recurrence. before and after surgery can also be used to thymoma. adrenal corticosteroids are usually of all ages to severe MG patients, especially middle-aged over the age of 40, whether done thymectomy, are more effective and safe, and often also combined anti-cholinesterase drugs. Commonly used in the preoperative thymectomy treatment or surgery due to appear later, the transition period after thymectomy can also be used. With this therapy, patients should intake of high protein, low sugar, and add potassium-rich diet, if necessary, need to take antacids. treatment methods currently used are three kinds; ① decreasing every other day high-dose therapy: prednisone every other day serving 60 ~ 80mg / d beginning, the symptoms improved more in a month there, often in a few months after the peak effect, at this time can be gradually reduce the dose until the next day serving 20 ~ 40mg / d maintenance dose. Maintain the amount of selection criteria is the minimum dose causing symptoms worsening. ② small dose increments every other day therapy: Overnight service prednisone 20mg / d beginning, weekly increments 10mg, until the next day serving 70 ~ 80mg / d or made noticeable effect so far; slowed down the law to improve the condition, the maximum effect after treatment is common in 5 months; less risk of exacerbations, but the date of disease progression may be delayed, so that doctors and patients vigilance weakened, is more respected high-dose therapy every other day. ③ high-dose therapy: dose every other day therapy does not alleviate the occurrence or repeated cases of crisis, to try methylprednisolone 1000mg / d, used in conjunction 3d shock therapy. experience has shown that a course of treatment often can not obtain satisfactory results, every 2 weeks you can repeat a course, can be 2 to 3 courses. Dose, interval and number of treatment should be done according to the specific circumstances of the individual patient's treatment. Corticosteroid side effects such as Cushing's syndrome, hypertension, diabetes, ulcers, cataracts, osteoporosis, and withdrawal syndrome should not be ignored.
② cytotoxic drugs: treatment of corticosteroid drugs ineffective or associated with thymoma patients, can be used cytotoxic drug treatment. cyclophosphamide (CTX) more significant inhibition of B cells. Usual dose for adults is 100 mg / d. Repeated occurrence of the crisis by intravenous infusion of 200 ~ 400mg / d, the effective amount is about 10g. thymoma resection poor efficacy, can be combined with chemotherapy: cyclophosphamide 800mg/m2, doxorubicin 50mg/m2, vincristine 1.4mg/m2, can be added with prednisone 40mg/m2, every 3 ~ 4 weeks as a cycle, and some have been significant benefits. Should be noted that side effects and blood. azathioprine (AZA) inhibition of cd4 T cells, usually 8 weeks after markedly.
(3) plasma exchange: plasma exchange or thymectomy commonly used in patients with preoperative treatment to avoid or improve postoperative respiratory crisis. Generally 5% of preoperative blood replacement body to ensure that patients can withstand surgery; also used for other types of crisis, most patients have symptoms of varying degrees of improvement for days or months. Although the safety of the law, but they are costly.
(4) immune globulin: 0.4g / (kg · d) intravenous infusion, once every 5d therapy for various types of crisis. side effects are headache, aseptic meningitis, flu-like symptoms, l ~ 2d of symptoms can be alleviated. The method is simple compared with plasma exchange therapy, the condition worsened when the two treatments are all in use.
(5) immunosuppressive drugs: no improvement within six months of hormone therapy, use of azathioprine or cyclophosphamide should be considered. The initial adult dose of azathioprine 1 ~ 2mg / (kg · d), maintenance dose of 3mg / (kg · d), taken with food to prevent nausea. Should be noted that the bone marrow suppression and susceptibility to infection, blood should be regularly checked, - once WBC less than 3 × 109 / L that is disabled, but also pay attention to the liver and kidney function.
(6) thymus or thymectomy radiotherapy: radiotherapy or surgery for the removal of the thymus, can directly inhibit or remove the thymus source of autologous immune response and tumor lesions. So far the MG thymus or radiation treatment for a really effective method, known as "non-surgical surgical treatment." For poor drug efficacy, easy to relapse patients, large or multiple thymoma can not surgery, or cancer may be added after this treatment. The usual dose is 40 ~ 50Gy, using 60 Co (60Co), linear accelerator and electron beam irradiation, a more stable effect.
thymectomy: MG, thymectomy is still the basic treatment for generalized MG, poor efficacy or more occurrences of crisis patients. 3 to 5 years the incidence of middle-aged female patients very good effect. Associated with various types of thymoma MG patients, although the efficacy of surgery is poor, surgical resection of the lesion should strive as much as possible. Blalock and other 20 cases of myasthenia gravis patients concluded that the implementation of thymectomy, myasthenia gravis patients in thymectomy for myasthenia gravis is an important treatment. Surgery in symptom control compared with non-surgical treatment has obvious advantages. Most authors believe that medical therapy, or adverse side effects of drug treatment, and restricted, then the implementation of thymectomy. Some authors (Jaretzki etc. 1988, Cop-per, etc. 1988) advocated an operation early on, the shorter duration of surgery, the better. Because severe cases, poor resistance to high surgical risk patients, need to regulate the scope of surgical treatment to reduce the period of short-term mortality surgery.
① preoperative preparation and precautions: In addition to routine preoperative smoking, hypoproteinemia, anemia, the application of anti-cholinesterase drugs to control symptoms. 1 month before surgery started with corticosteroids, injection of human gamma globulin (gamma globulin), with the application of plasma exchange or filter weight to lose. All patients before surgery to be done x-ray and CT scan assessment, to exclude the presence of thymoma. Avoid possible interference with neuromuscular conduction between the aggravated symptoms of myasthenia gravis drugs such as quinidine, quinine, propranolol (Inderal), polymyxin, tetracycline antibiotics, amino glycoside antibiotic sugar; anesthesia drug ether, muscle relaxants, including tubocurarine (curare); avoid the use of central nervous system depressants such as morphine. Preoperative routine examination AchRab, PsMab and CAEab.
② anesthesia: the preferred general anesthesia, due to surgery may damage the pleura and into the chest. anesthesia for short-acting barbiturates induced, then maintained under general anesthesia, intraoperative continuous monitoring of ECG, blood pressure and blood gas analysis. Surgery to avoid the use of anti-cholinesterase drugs.
③ incision: via median sternotomy incision or through the neck incision, extended thymectomy can be the incision. The neck incision combined with video-assisted thoracoscopic resection of thymus and convenient operation, trauma, disease rate, easy for patients to accept. Most surgeons use sternal incision, or cut part of the sternum, or breastbone fourth intercostal transverse incision. Many reported that thymectomy for myasthenia gravis and the effect of complete removal of thymic tissue have some relevance. Because ectopic thymus and the vagus in the mediastinum can be widely distributed among the fat, so that the cleaning to remove the neck and mediastinal ectopic thymic tissue fat can improve long-term efficacy.
④ surgical methods:
A. the sternal thymectomy: supine semi-recumbent or low, by the middle of the sternum from manubrium to the fourth rib level, sawing the sternum, if necessary, to do full sternotomy. On the huge thymoma, the second surgery, short-necked or obese patients, can be complemented by transverse cervical incision. Coated on both sides of the incision in the sternum with bone wax, sternal retractor to expose the roots of the anterior mediastinum and neck.
incision as tumors located in the upper left and right pleura open, to expose the thymus was pale pink and yellow surrounding mediastinal fat was significantly different. Lower pole of the thymus from the side of the sharp upward anatomical separation of muscle below the sternum thyroid make sure mediastinal extension of the deep cervical fascia, the separation process to encounter left, right and middle thymic artery ligation. Free to avoid the phrenic nerve laterally, otherwise it will cause postoperative diaphragmatic paralysis. Separation of 1 to 2 lead off the left innominate vein into the bottom of the thymus vein. One or both sides of the upper pole of the thymus can even enter the innominate vein behind, on the very top on both sides, often from the inferior thyroid artery branches of the artery, pay attention not to damage, to avoid accidental injury under the parathyroid glands. Carefully examined whether the ectopic thymus or remnants of mediastinal thymus, studies have shown that the fat from the neck to the diaphragm may exist in small tissue thymoma or thymic hyperplasia tissue, surgery should be clear.
complete hemostasis with particular attention to the sternum and sternum cross-section of the film before and after bleeding. The lowest position in the anterior mediastinal drainage tube placement, pleural resection can not be repaired due to the expansion gap pleural chest drainage. Stainless steel wire fixed split of the sternum, not to damage the internal mammary artery on both sides of the sternum, sternal wire through the parts of the bleeding can be coagulation. Note the wire around the stump placed in the right position, so as not to cause the patient discomfort after surgery, layered suture the periosteum, the pectoralis major fascia and skin.
B. thymectomy by the neck: now more than supplemented by video-assisted thoracoscopic resection of the thymus. supine position, raise the shoulders, head and later Yang. 2cm of taking suprasternal fossa skin fold along the middle line of curved incision on both sides of the sternocleidomastoid muscle. Platysma flap in the plane under free, from the thyroid level, issued sternum level. Middle of the muscle strip separately, under the sternum can be seen thymus thyroid muscle. After ligation of the inferior thyroid veins you can see the very top left thymus. Stay ligature for traction. Anatomy of right upper pole to the same method, and cut off before the ligation, the poles fully free to the sternal notch level, in front of the innominate vein blood. Posterior wall of the thymus free from the sternum, the two pulled up on the pole when you can see the thymic vein, given ligation. First the right side free thymus, extending down to the lower right with the pole. The thymus and the pericardium in front of the aorta and the free, finally free and very left end, the Department often extending down to the aortic pulmonary window. All free thymus, the thymus together with the total removal of the anterior mediastinal fat, mediastinal tube drainage after the sutured incision. Drainage tubes were removed after the suture the platysma, the full expansion of the lung, so one or both sides, although a small rupture of the pleura, chest tube drainage are not home.
⑤ after treatment: postoperative indwelling endotracheal intubation is not clear to return to the ward and for respiratory care, clear respiratory secretions, such as 3 to 5 days can not remove the auxiliary breathing, tracheotomy should be done, gastric tube feeding and medication; awake throat muscles back to normal, namely: to spontaneous breathing, coughing, blood gas analysis of the indicators shows the normal respiratory function may extubation, to encourage patients to cough, cough sputum, enhanced care.
patients with myasthenia gravis due to surgical trauma, postoperative immune function and sensitivity to change, can cause or aggravate symptoms of myasthenia gravis there muscarine-like reaction, after to anti-cholinesterase drug treatment. dose adjusted according to the disease. Serving surgery plus prednisone in patients before and after only steroids, oral or intravenous administration. Until the last symptoms disappear after the withdrawal. If the surgery were found in thymoma, intraoperative findings surrounding tissue infiltration, postoperative symptoms of unstable patients, after conventional radiotherapy to the anterior mediastinal area.
⑥ surgical results: good results after early mortality was 0% to 2%, on the patient's age, gender, disease classification and other factors on the effect of surgery, clinical Data Report: myasthenia gravis associated with thymoma is generally believed that, its results of operations as there were no tumor; young women associated with hypertrophy or hyperplasia of the thymus of myasthenia gravis patients with type Ⅱ surgical better; preoperative acetylcholine receptor antibody titers with higher degrees decreased to normal levels after surgery was better below. As the extent of disease myasthenia gravis difficult stage, not on drug therapy, plasmapheresis, and thymectomy for thymic irradiation compared and evaluated.
(7) Other treatments: In addition to these commonly used therapies, there are thymus microwave percutaneous intervention, plasma exchange or purified gamma globulin high-dose intravenous immunoglobulin adsorption and anti-CD4-McAb treatment. MG is considered by Chinese dialectical spleen deficiency, often buzhongyiqitang as the basic governing law, re-Astragalus.
(8) the treatment crisis: should emergency rescue. First, keep the airway open and maintain effective breathing. Mild respiratory muscle paralysis, encourage expectoration, oxygen, or prone position, head lowered, chest pressure artificial respiration. If breathing and severe weakness expectoration, sputum, should be early or tracheal intubation, artificial respiration and suction. Check the original cholinesterase inhibitor doses and time, pay attention to inadequate or excessive medication. For different types of crisis, given the appropriate drug treatment: such as myasthenia gravis crisis, the first intramuscular injection of neostigmine 1mg, then according to the disease every 0.5 to 1 hour injection 0.5 ~ 1mg. medication a few times cholinergic crisis can be avoided. If the muscle weakness but heavier, it indicates that cholinergic crisis has occurred, immediately disable the cholinesterase inhibitors, intravenous injection of atropine 1 ~ 2 mg. crisis on the anti-bend, should be stopped for more than medication, intravenous dexamethasone or methyl prednisolone sodium succinate 500 mg, 1 times / d, for 6 days, muscle motor end plate can function recovery, after recovery cholinesterase inhibition re-established medicinal. crisis caused by infection, more than can be given to penicillin and other anti-infectives. myasthenia gravis may be complicated by cardiac dysfunction, should also pay attention to blood pressure.
① ocular. Patients, especially children, should use prednisone "dose impact, low-dose maintenance" therapy.
② bulbar muscular. Should be first choice AChEI and corticosteroid therapy.
③ ocular and bulbar muscular patients. Use of corticosteroids or pyridostigmine repeated symptoms after treatment, the effect is gradually reduced efficiency, the appropriate use of other immunosuppressive agents such as cyclophosphamide, azathioprine, or cyclosporine and so on.
⑤ for the crisis (especially in myasthenic crisis associated with cholinergic crisis), thymus pre-operative preparation, increased symptoms after thymectomy or systemic drugs bad treatment, the choice of plasma exchange therapy.
⑦ of the thymoma, thymus disease or other types of systemic therapy in patients with MG were gradually reduced efficiency. Thymectomy can be used (to be carried out according to standard perioperative management).
⑨ thymus of all MG patients, including patients, in order to improve efficacy and reduce the amount of hormones to prevent relapse and consolidate the therapeutic effect, can be added with traditional Chinese medicine (such as: righting gluten film) adjuvant therapy.
(b) the prognosis
3. effective in clinical symptoms and signs of significant improvement, the patient can take care of themselves, adhere to the study or engage in light work, MG treatment reduction 1 / 2 or more, no more than 1 month recurrence.
5. invalid clinical symptoms and signs do not improve, or even worsening.[Examine]
myasthenia gravis should be how?
1. blood, urine and cerebrospinal fluid routine examination were normal. x-ray shows 15% of MG patients, especially those older than 40 years have thymoma, chest CT can be found in addition to all other micro-adenoma thymoma. 2 MG suspicious thyroid function can be determined. 3 spectral examination of serum autoantibodies (1) determination of serum AchR-Ab: MG patients with AchR-Ab titer increased significantly, foreign reports positive rate of 70% to 95%, which is a highly sensitive and specific diagnostic tests. Generalized MG in AChR-Ab-positive rate of 85% to 90%. In addition to Lambert-Eaton syndrome, or thymoma patients without clinical symptoms, or symptoms, generally no false positive results. Some ocular thinking person, remission after resection of thymoma patients, even those with severe symptoms, may be undetectable antibodies. antibody titer is also inconsistent with the clinical symptoms, clinical complete remission in patients whose antibody titer may be high. actin antibody (myosin, myosin, actin antibody) is also seen in 85% of thymoma patients, but also some of the earliest manifestations of patients with thymoma. serological abnormalities also have some significance. Antinuclear antibodies, rheumatoid factor, thyroid antibodies were more common in patients than normal. (2) is not recommended AchR binding antibodies (AchR-binging Ab) as a screening test, the antibody or antibodies is also found in skeletal muscle 13% of patients with Lambert-Eaton myasthenic syndrome. (3) actin (such as myosin, myosin, actin) antibodies seen in 85% of thymoma patients is an early manifestation of some of thymoma. 1 muscle fatigue test (Jolly test) involved repeated voluntary muscle contraction speed, such as continuous blink 50 times, showing the eye fissure became smaller and smaller; the patient supine continuous rise of 30 to 40 times, showing the gradual weakening of the sternocleidomastoid muscle contraction appear unable to rise; arm is raised upward gaze or eye movements continued several minutes, if a temporary paralysis or weakness significantly worse after the break to restore as positive; as weak chewing muscles can make chewing movements repeated 30 times, such as myasthenia gravis can not chew as well as increase the fatigue test was positive. (2) anti-cholinesterase drugs (anticholinesterase drugs) Tang Hei Long neostigmine test and diagnostic value of the same test for MG diagnosis and identification of various types of crisis. (1) Tang Hei Long (tensilon) test: Tang Hei Long (ethyl - 2 - methyl - 3 - hydroxy ammonium chloride), also known as phenol by ammonium chloride (edrophonium). test should be preceded by a specific brain such as levator muscle control and eye muscles to assess muscle strength, limb muscle strength measurements (with the grip strength meter), critically ill patients should check lung capacity. Tang Hei Long 10mg diluted to 1ml, the first intravenous injection of 2mg (0.2ml), absence of adverse reactions and no increase muscle strength after 45s, the remaining 8mg (0.8ml) of about 1min slow injection. side effects include mild muscarinic response (muscarinic effect), such as nausea, vomiting, bowel movements increased, sweating and excessive salivation, can advance with atropine 0.8mg subcutaneous injection of confrontation. Results found: the majority of patients after injection of 5mg symptoms have eased, the case of myasthenic crisis, respiratory muscle weakness in the 30 ~ 60s in improved symptom relief lasted only 4 ~ 5min; the case of cholinergic crisis, and will temporarily increase with bundle tremor; anti-bend crisis no response. Determine Tang Hei Long test positive should include the objective to enhance muscle contraction, such as ptosis and diplopia significantly reduced or disappeared. (2) neostigmine (neostigmine) test: methyl sulfate neostigmine (neostig mine methylsulfate) is a synthetic compound with the chemical structure similar to physostigmine. The pilot test is sometimes more preferable Tang Hei Long, long time because of the role, the results can be accurate and repeatable assessment. 1 ~ 1.5mg intramuscular injection, a few minutes in advance or while intramuscular injection of atropine (atropine sulfate) 0.8mg (average of 0.5 ~ 1.0mg), against the muscarinic side effects and arrhythmia. Results found: 10 ~ 15min after injection, usually symptom improvement, 20min peak, sustained 2 ~ 3h, can carefully assess the degree of improvement. Note reference to Tang Hei Long test. 3 emg low frequency (1 ~ 5Hz) repetitive nerve stimulation (repetitive nerve stimulation, RNS): neuromuscular transmission is commonly used physical examination, is the most common method of detecting NMJ disease. 2 ~ 3Hz low-frequency electrical stimulation of peripheral nerves caused by repeated muscle action potential decreased rapidly dominate, because NMJs Ach local consumption, resulting EPPs reduced. 4 patients with pathological diagnosis may be difficult for the muscle biopsy, electron microscopy NMJ, postsynaptic membrane folds under reduced flatten and reduce the number of such AchR can be diagnosed MG.[Diff]
myasthenia gravis and the diseases easily confused?
1. myasthenic syndrome (Lambert-Eaton syndrome) 50 male patients over the age of majority, about 2 / 3 associated with cancer, small cell lung cancer the most common; main the proximal limb muscle weakness of the trunk, lower limb symptoms and in the upper limb; thin and easy fatigue, slowness of movement. muscle fatigue after the event, but if they continue to shrink the muscle but may be temporarily improved; occasionally dominated the extraocular muscles and bulbar muscle involvement; about 1 / 2 patients had limb paresthesia, dry mouth, impotence; yang alkali inhibitors for the treatment ineffective. tendon reflexes but no muscle atrophy. The myasthenia gravis more common in women 40 years of age; often accompanied by thymic tumors; muscles may be involved, most of the muscles involved in the earliest activities; gravis morning afternoon light weight, activity increased, decreased or disappeared after the break; tendon reflexes are usually not affected; cholinesterase inhibitor treatment is effective. Differential diagnosis in table 1. 2.MG combined thyrotoxicosis (thyrotoxicosis) with the author that the MG and thyrotoxicosis, thyrotoxicosis ophthalmoplegia usually based on proptosis (early is not obvious) and neostigmine to determine non-response. 3 lupus erythematosus and polymyositis no extraocular muscle paralysis, but the MG co-exist with autoimmune disease. 4 patients complained of muscle weakness neurological disorder is actually fatigue, double vision may readme (when the short-term symptoms of fatigue) and a sense of throat tightening (hysteria ball), but no ptosis, strabismus, etc. Conversely, MG can be misdiagnosed as neurosis or hysteria. 5 progressive paralysis of the extraocular muscles and congenital myasthenic state can also be misdiagnosed as MG, the first two levator muscle of the eye such as permanent damage to the neostigmine no response. Another possibility is that no reaction against cholinesterase drug, wrong to exclude MG, pay attention to other muscles EMG. 6.MG patients with no ptosis or strabismus, but dysarthria can be misdiagnosed as MS, polymyositis, inclusion body myositis, stroke, motor neuron disease and other neurological disorders, attention should be identified. 7 muscular dystrophy eye strap muscular pharynx and Guillain-Barré syndrome Fisher variant of early, may have ptosis, but the Fisher-type tendon reflexes, or ataxia, emg examination can identify. 8 botulism (botulism) acting on the presynaptic membrane, resulting in NMJ transmission barriers and skeletal muscle paralysis, with Tang Hei Long or neostigmine symptoms improved, easy to be confused with myasthenic crisis. Early manifestation of blurred vision, diplopia, ptosis, strabismus and eye muscle paralysis, can be mistaken for MG. botulism is usually dilated pupils, light reaction disappeared, the rapid emergence of the bulbar muscles, trunk muscles and limb muscle involvement. 9 organophosphorus pesticide poisoning and snake bites can cause cholinergic crisis, but there is a clear history of poisoning, snake bite history, can be identified. 10 progressive muscular dystrophy ocular (Kiloh-Nevin Type) and more in young adults, insidious onset, progression-free fluctuations, mainly affects the eye muscles, severe eye fixed; family history, serum muscle enzymes and muscle biopsy can be identified.[Disease]
myasthenia gravis related diseases
"5P" sign "cough water" addiction "three side" syndrome asperger syndrome unresponsive to hiv infection Moro Todd paralysis (left temporarily aspirin allergy cancer rush fever septicemia accompanied by hypertension and white film mucosal bleeding baby with lively fear of pain, violence, lack of security outbreaks of impulsive behavior were forced to turn a blind eye position difficult to establish levy autism
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