04:58,23,Jul,2005 | (2898/0/0) | Original

myasthenic crisis vs cholinergic crisis


Thymoma patients with myasthenia gravis crisis in nursing
Of Pharmaceutical Sciences 1 2000, Volume 23 Medical Care
Of: Zhong Min Wang Yue Zou will
Unit: Zhong Min (First Affiliated Hospital of Jiamusi University, Thoracic Surgery); Zou Council (the First Affiliated Hospital of Jiamusi University, Thoracic Surgery); Wang, Yue (Jiamusi Chinese medicine hospital operating room)
Key words: thymoma; myasthenia gravis; postoperative care
Thymoma patients with about 1 / 3 with myasthenia gravis, thymoma resection is prone to lead to the risk of myasthenic crisis, thymoma after doing the treatment for respiratory care is the prevention and treatment of thymoma with myasthenia gravis crisis the key. Is combined with surgical treatment of thymoma in our hospital experience, focusing on the thymoma patients with myasthenia gravis crisis in nursing.
1 Clinical data
Our department from 1989 to 1999 were treated thymoma in 25 cases, including nine cases with myasthenia gravis, 6 females, 3 males, aged 9 to 40 years old, complete surgical removal of thymus and anterior mediastinal fat pad, after all with varying degrees of respiratory muscle paralysis, myasthenic crisis occurred in 3 cases, 2 cases of cholinergic crisis, 4 patients in the 18 ~ 48h after tracheotomy and artificial mechanical ventilation. 1 case of respiratory failure caused by a lung infection, died after saving invalid.
2 nursing
2.1 strengthen the respiratory tract
Narcotic drugs after general anesthesia may aggravate symptoms of myasthenia gravis. Pharyngeal secretions increase pharyngeal muscle weakness may occur, respiratory muscle weakness can cause breathing difficulties, they should first ensure airway patency, clear the respiratory secretions, suction time, if necessary, indwelling gastric tube, continuous vacuum, to avoid regurgitation of stomach contents, airway obstruction caused by suffocation, can be injected neostigmine restored swallowing reflex. Respiratory muscle weakness, note the application of neostigmine, to help patients turn over call-back, in order to facilitate sputum expectoration. To observe the patient breathing rate and ventilatory function, such as patients with myasthenic crisis, neostigmine ineffective, with doctors, tracheotomy and artificial respiration to mechanical ventilation, 3 patients in this group appeared after 18h myasthenic crisis, tracheotomy and artificial mechanical ventilation, rescue a success.
2.2 The use of artificial breathing machine
Our department mainly of high frequency jet ventilator and ventilator Star card. The choice should be based on the patient's respiratory status to decide, the former used in spontaneously breathing patients, the latter type of constant pressure constant volume ventilator, can be used in patients without spontaneous breathing, the use of breathing machine, to observe the patient breathing rate, rhythm, note atomization, preventing scab sputum, suction time. Strong spontaneo
us breathing patients can be intermittent mechanical ventilation, exercise, respiratory muscle function, easy to produce some of ventilator dependent patients, for lung breath sounds clear, no infection can be shutdown and try to extended downtime, and gradually remove the breathing machine, such as condition occurs repeatedly, drug difficult to control, can be used again. 1 patient in this group tracheotomy ventilator 9d, the shutdown 7d discharged 10d repeated illness after discharge, cut, once again applied 8d, offline, discharged.
2.3 Note myasthenic crisis and cholinergic crisis in the observation and identification of
Myasthenia gravis crisis in the rescue at the same time, due to the application of more neostigmine, should pay attention to prevent the occurrence of cholinergic crisis, such as neurological symptoms, sweating, salivation, heart rate, muscle tremors may be intramuscular atropine Commodities relief. Care in myasthenia gravis crisis and to observe the different manifestations of cholinergic crisis, and to identify, provide the basis to accurately determine the condition for the rescue.
(Received :1999-09-28)
myasthenic crisis vs cholinergic crisis

Myasthenic crisis
Refers to the symptoms of myasthenia gravis gravis suddenly worsened respiratory muscles, swallowing, progressive muscle weakness or paralysis, and those life-threatening. Myasthenic crisis caused by what?
1. In recent years, according to ultrastructural findings, the disease is mainly postsynaptic acetylcholine receptor (AChR) induced lesions occurred.
2. Many of the clinical phenomena suggest mechanisms of disease and immune disorders. What are the performance of myasthenic crisis and how to diagnose?
1. Myasthenic crisis: the lack of neostigmine crisis, often due to infection, trauma, reduction caused. Respiratory muscle paralysis, inability to swallow sputum life-threatening.
2. Cholinergic crisis: the new crisis of the next over. In addition to the myasthenic crisis are still outside the accumulation of excessive acetylcholine symptoms:
â‘  muscarinic poisoning: nausea, vomiting, diarrhea, abdominal pain, small pupils, sweating, salivation, tracheal secretions and more slow heart rate;
â‘¡ nicotine-like symptoms: muscle tremors, convulsions, feeling tight places;
â‘¢ central nervous system symptoms: anxiety, insomnia, mental confusion and convulsions.
3. Anti-bend crisis: difficult to distinguish the nature of crisis and who can not stop or increase the drug dose used to improve symptoms, the more the higher dose in the long term after treatment. Myasthenic crisis should do what check?
1. â‘  neostigmine test. â‘¡ edrophonium test.
2. Electrophysiological examination: continuous electrical stimulation of common sense, impaired muscular response and fast disappearing.
3. Other: Determination of serum anti-AChRab increased about 85% of patients. Chest X-ray or CT examination of thymus, thymic hyperplasia, or associated with thymic tumor, but also diagnostic value. Myasthenic crisis and the diseases easily confused?
Myasthenic crisis, cholinergic crisis, anti-bend of the differential between the crisis. Three kinds of crisis can identify the following methods: â‘  Tang Xi-Long Test. â‘¡ atropine test. â‘¢ electromyography. Myasthenic crisis can be complicated by the diseases?
Myasthenic crises, and by the development of the disease caused by lack of anti-cholinesterase drugs. Clinical swallowing, cough can not, respiratory distress, difficult even to stop a serious situation. Examination shows dilation of pupils, sweating, abdominal distension, bowel sounds normal and after injection of neostigmine symptoms improved and so on.
Cholinergic crisis of crisis about the number of cases of 1.0% and 6.0%, caused by the excessive anticholinesterase. In addition to common features of myasthenia gravis, the patient miosis, sweating, muscle twitching, hyperactivity bowel sounds, after intramuscular injection of neostigmine increased symptoms and other features.
Anti-bend of the crisis is by infection, poisoning, and electrolyte imbalance caused by the application of anti cholinesterase drugs may be temporarily reduced, followed by another increase in the critical state. Myasthenic crisis should be treated?
1. Myasthenic crisis: neostigmine methyl sulfate 1-2mg IM or 0.5-1mg VD, on the total 6mg.
2. Cholinergic crisis: stop using anti-cholinesterase drugs, atropine 0.5-2mg VD or intramuscularly 15-30 minutes can be repeated until muscarinic symptoms were relieved or disappeared. Against nicotine-like symptoms, pralidoxime 400-500mg in 5% glucose or saline infusion, until the muscle relaxation.
3. Anti-bend crisis: Disable all the anti-cholinesterase drugs for at least 3 days from the original starting dose of half the amount of administration, and use or in combination with hormones. Myasthenic crisis should be how to prevent?
1. Light should avoid over-exertion, cold, infection, trauma and anger, etc., should not be too long in the hot sun to prevent the occurrence of myasthenic crisis.
2. Myasthenic crisis episodes, they should stay in bed, stay calm and quiet, smooth and keep indoor air fresh, the timely removal of nasal and oral secretions, keep the airway open.
3. Myasthenic crisis, the immediate intramuscular injection of neostigmine 1 mg, if necessary, repeat after use of oral symptoms.
4. Cholinergic crisis, the disable all anti-cholinesterase drugs, while intramuscular or intravenous injection of atropine 0.5 to 2 mg daily for 15 to 30 minutes, repeat.
5. Anti-bend of the crisis, the disabled anti-cholinesterase drugs, the motor end plate acetylcholine receptor function in receptor function was restored, at least 72 hours, then started using small doses of anti-cholinesterase drugs.
6. Such as the temporary crisis of unknown nature, may suspend the anti-cholinesterase drugs, trial of oral prednisone.
7. Prohibiting the use can affect the nervous
The names of diseases (English) myasthenia gravis Chinese Pinyin ZHONGZHENGJIWULI alias: eyelid waste, cell down, depending on the manifold, head tilting, atrophy card, atmospheric subsidence, Western neuromuscular disease ICD code ICD code for Western medicine defines disease name myasthenia gravis (myasthenia gracis , referred to as MG) is caused by autoantibodies in autoimmune diseases, in order to pass obstacles at the neuromuscular junction caused by chronic diseases. Unusual clinical manifestations of affected skeletal muscle fatigue, weakness, easy fatigue, not at liberty to exercise, rest or by taking anti-cholinesterase drugs to reduce or disappear after the symptoms temporarily. Chinese Buddhism were based on clinical manifestations of the disease, quite different from traditional Chinese medicine syndromes, such as drooping eyelid weakness in medicine mainly belongs to the "eyelid waste" or "cells down"; see weight film was "as discrimination"; the rise of weakness in the "head tilt"; limb weakness rash soft in the "atrophy card"; breathing difficulties, myasthenic crisis was "atmospheric subsidence" and other syndromes. Cause of a Western, postsynaptic acetylcholine receptor (AchR) of the changes: normal neuromuscular transmission at the exciting, â‘  the end of the motor end plate acetylcholine synthesis (Ach), and stored in synaptic vesicles in; â‘¡ when communicated to the peripheral nerve excitability, causing hundreds of synaptic vesicles into the synaptic cleft while Ach, diffuse to the postsynaptic membrane; â‘¢ Ach two molecules of a mouth with the postsynaptic membrane of Ach receptor, AchR channel opening, rapid influx of sodium ions, causing end-plate region of muscle fiber depolarization, membrane potential generation, hundreds of small endplate potential superimposed to form the end plate potential and action potential of muscle contraction is complete. Any part of the transfer process can lead to muscle weakness disorders. Electrophysiological changes of MG's is a small end-plate potential amplitude decreased when the low frequency limit of repeated electrical stimulation of the phenomenon of a gradual decay, end-plate potential amplitude and the amount of each molecule the number of Ach release, postsynaptic density and the sensitivity of the membrane AchR related. The study found that patients with MG Ach content contained within synaptic vesicles, and each nerve impulse induced Ach release were normal. Therefore, the end-plate potential amplitude decreased due to lower density of postsynaptic membrane caused by AchR; pathological observation has confirmed that MG patients reduce the number of postsynaptic AchR.
2, autoimmune said as early as 1960, Simpson and other clinical data summarized on the basis of a large number and found this disease with other autoimmune disease with a higher incidence of survivors, probably the first time MG is an autoimmune disease. And later measured in the serum of MG patients to the AchR antibodies (AchR a Ab), and the antibodies produced in experimental animals into an experimental animal model of MG.
3. MG said that the incidence of hereditary and genetic factors are now attention has been paid, MG has certain family recurrent. Back in 1898 the first report of familial MG Oppenheim since there are many cases of familial MG report on genetic factors in the pathogenesis of MG have a certain role, MG have a certain genetic predisposition, but the disease has not yet been found positive mode of inheritance.
4. Clinical Endocrinology found that MG patients with endocrine disorders have a certain relationship, female patients often aggravated during menstruation, amenorrhea, and reduce symptoms of pregnancy, delivery or postpartum if the symptoms but also increase; The study suggests that female hormones follicle stimulating hormone can promote the synthesis of Ach , the female hormone progesterone diketone effective for some patients with MG; in MG patients, about 2.1% to 18% of patients with hyperthyroidism, thyroid hyperthyroidism significantly increased serum cholinesterase, MG symptoms.
In conclusion, MG is a multifactorial disease, autoimmune responses play a major role in the pathogenesis, in addition to anti-AchR antibodies, but there may be other non-direct role in the AchR antibodies in disease, such as genetic and endocrine pathogenic factors may be important factors involved, these are worthy of further study. TCM etiology 1. Six Exogenous external organs function caused by an imbalance of the evil, blood loss of pathogenicity.
2. Impassioned by the internal injury, the situation go rather Shu, involving internal organs, if any "anger harms liver," "hi sad", "thinking injured spleen," "sad lung", "fear of harm to the kidneys," said ("Q to be as big of yin and yang ").
3, no inside and outside the regimen due to careless, improper diet, Feiganhouwei, loss, and stomach, caused by weak spleen and stomach; Laojuan excessive dysfunctional organs, gas consumption disability allowance.
4. Congenital lack of factor endowments, ferrite weak stomach, spleen and kidney yang, liver and kidney and so on. Seasonal population with MG can occur at any age, but the most common 10 to 40 years of age, more women than men. Intensity and spread of disease incidence was 0.5/10 million, the prevalence was 10/10 million. TCM pathogenesis 1, spleen and stomach of the spleen was the day after tomorrow, and fluid, the source of qi and blood. Such as ferrite weak stomach, or a chronic illness caused by empty stomach, or Laojuan excessive loss and stomach, spleen and stomach by the operation of the dysfunctional sodium, and fluid, the source of qi and blood deficiency, the deficiency of qi and blood, tendons and muscle dystrophy, it is muscle weakness, eyelid sag or limb weakness, or breathing difficulties.
2, spleen deficiency of the kidney is the innate, hidden essence marrow, the spleen is acquired and the source of the water transport Valley subtle; Congenital deficiencies, deficiency losses, not very inviting spleen yang, deficiency of water can not be shipped subtle Valley , How to Run the muscle tendons, so the limbs, muscle atrophy soft weakness.
3. Liver and kidney liver stores the blood, reinforcement; kidney essence, the main bone; essence and blood, Chong Sheng, the bones and muscles strong, normal activity. Jiubingtixu, liver and kidney injuries, the sex fluid losses: because of yin deficiency heat, even burning liquid disability allowance, loss of energy and blood can not support irrigation wing muscle atrophy is caused by the soft and weak.
4. Blood for blood deficiency Yin Ye, with moisture, Rong Yang effectiveness; gas as the commander of blood, the blood gas lines, the movement of human body fluid and gas driving all closely related to Qi can not push blood, blood is not enough Rong Yang muscle tendons, then the induced atrophy. Pathology MG is an autoimmune disease, the pathological change is systemic, focusing on skeletal muscle, neural and thymus at a neuromuscular junction. Naked eye: early muscle did not change significantly, advanced muscular atrophy muscle volume reduced. Microscope, muscle fiber coagulation, necrosis, swelling, and muscle fiber striated muscle disappear, swallowed cell infiltration, muscle fibers and Lymphocyte infiltration around small blood vessels, called lymphatic leakage; widened synaptic cleft, postsynaptic membrane folds split scarce , shallow, narrow nerve endings magnum; about 80% MG patients have thymic hyperplasia, lymphoid nodules microscope center hyperplasia occurred; about 10% to 20% of patients with thymoma. Often pathological thymoma cell type in the epithelial cell type and mixed type, the latter two types is often accompanied by myasthenia gravis. Traditional Chinese Medicine diagnostic criteria for pathological diagnosis of Physiology 1, spleen and stomach Qi:
Syndrome: ptosis, early evening light weight, soft limb weakness, the rise of the difficulties, inability to chew, eat less, loose stools, less gas lazy words, pale tongue body fat, while a scalloped, thin white fur, thready and weak pulse .
Syndrome analysis: a long illness caused by weak stomach, spleen deficiency, or ferrite, the operation of the right, the source of qi and blood deficiency, the water can not be on the Valley Wing craniofacial subtle, so ptosis, inability to chew, the first suppressed; spleen main limbs, spleen lost health movement, tendons dystrophy, limb soft so weak; spleen can not be shipped wet, so the surface float; so weak stomach, loose stools deprivation; less gas lazy words, pale, thin white fur, pulse thin, both the spleen and stomach qi deficiency of the levy.
2, spleen deficiency:
Syndrome: ptosis, limited eye movement, limb weakness. Backache spontaneous sweating, cold limbs, pale Koushi white, swallowing difficulties, accept less loose stools, urine long, pale tongue body fat, thin white fur, pulse.
Syndrome analysis: a long spleen and kidney lesions and two dirty, or daily deficiency, spleen yang yang rely on warmth, kidney is less than the spleen yang deficiency, spleen governs the transportation and transformation, transportation of stolen Secretary spleen, the blood biochemical the source, then the water is not rong Yang Valley subtle craniofacial, so ptosis, difficulty in swallowing; spleen limbs, spleen governs the muscles, limbs atrophy spleen is soft; spleen to transport and can not. So eat less poor appetite, loose stools dilute Ambassador; deficiency is cold limbs, backache, spontaneous perspiration, pale tongue body fat, while a scalloped, thin white fur, the pulse was small, the deficiency of the spleen sign.
3, liver and kidney:
Syndrome: two face down, difficulty in swallowing, chewing, weakness, towards the evening light weight, weakness, weak waist; dizziness, ringing in the ears; less sleep soundly dreams, head dry and astringent, five upset hot, red tongue, little coating, thready pulse number.
Syndrome analysis: chronic illness of liver and kidney loss, or ferrite deficiency, liver and kidney, essence and blood loss, muscular dystrophy, the weak, the two face drooping, swallowing difficulties, inability to chew; liver and kidney, brain displaced keep the dizziness, tinnitus, less sleep soundly dreams, head of liver failure has raised the yin head dry and astringent; back to the House of kidney, kidney so weak waist, five upset hot, red tongue, little coating, pulse breakdown are heat of the symptoms of liver and kidney.
4, qi and blood deficiency:
Syndrome: Shenpi fatigue, palpitations shortness of breath, less gas lazy words, looking Koushi white, eyelid weakness, chewing difficulties, muscle weakness wilt, spontaneous sweating, pale tongue, the tongue has scalloped body fat and tender, thin white fur, the pulse Fine.
Symptoms of: lack of blood, brain failure has raised, so Shenpi fatigue, eyelid weakness, difficulty chewing, looking Koushi white; lack of effort, the heart palpitations shortness of breath, spleen governs the muscles, spleen governs the transportation and transformation, transportation of stolen Division spleen deficiency, the Water Valley is not subtle Ru Yang limbs, muscle, so muscle atrophy; tongue pale, tongue has scalloped edges and tender body fat, thin white fur, the pulse was small, all the signs of blood deficiency. Western diagnostic criteria for myasthenia gravis diagnostic criteria:
Japanese Ministry of Health survey of a particular class of myasthenia gravis diseases, 1979 revision
1. The necessary symptoms: ophthalmoplegia caused by repeated movements, swallowing and other parts of the muscle strength or body weakness, resting after a transient recovery.
2. Reference
(1) more of the following symptoms ① ptosis; ② eye movement disorder or double vision; ③ difficulty swallowing; ④ speech impairment; ⑤ walking or movement disorders; ⑥ difficulty breathing.
(2) symptoms within 1 day fluctuations.
(3) non-pyramidal tract signs and sensory impairments.
(4) anticholinergic drugs (edrophonium 2 ~ 1Omg intravenous or intramuscular injection of neostigmine 0.5mg) a transient improvement of the symptoms.
(5) shows attenuation of EMG (50Hz voluntary contraction or repeated to give the following maximum stimulation, the evoked EMG pm).
(6) the following combination of symptoms or symptoms of concurrent thymoma or thymic hyperplasia â‘ ; â‘¡ hyperthyroidism; â‘¢ muscular atrophy; â‘£ own autoimmune diseases.
[With] three crisis identification of myasthenia gravis
Myasthenia gravis gravis cholinergic crisis and the identification of anti-bend
Gravis phenomenon: large pupils, sweating less, no salivation, no abdominal pain, diarrhea, no meat or muscle impulse to jump, good anti-cholinesterase drug reactions.
Cholinergic crisis: the pupil is small, sweating, salivation and more obvious abdominal pain, diarrhea, meat or muscle impulses common hop, anti-cholinesterase drug reactions increase.
Anti-bend of the crisis: the pupil is normal or too large, the number of uncertain sweating, salivation small, no abdominal pain, diarrhea, no meat or muscle impulses jump, anti-cholinesterase drug reactions uncertain.
Western medicine diagnosis is based on based on the typical history, involved in skeletal muscle fatigue easily, are heavy towards the eve of the laws of light, the rest or with anti-cholinesterase drugs can be improved after the diagnosis. On clinical symptoms only, while the lack of objective signs or symptoms of atypical cases, diagnostic tests should be done to further confirm the diagnosis.
1, the muscle fatigue test involved the patient's skeletal muscle easy to repeat as a continuous action (such as the open eyes closed eyes, chewing and swallowing, lifter arm, hand make a fist and other actions), and repeat it after the corresponding muscle fatigue, the symptoms after the break reduced.
2, drug test neostigmine methyl sulfate 0.5 ~ 1.0mg subcutaneous injection (available atropine 0. 5mg injected at the same time to reduce muscarinic effects), 30 to 60 minutes after injection, symptoms of myasthenia gravis improved significantly.
3, EMG repeated electrical stimulation of low frequency (3Hz) or high frequency (30 ~ 50Hz) stimulation of the ulnar nerve or facial nerve, distal evoked potential recording, low-frequency attenuation of more than 10% after stimulation, no change or slight high-frequency stimulation increased, but not more than 50% can be diagnosed, the positive rate was 70% examination. The single fiber EMG is a special single-fiber needle electrodes by measuring the "trembling", recorded in a muscle joint nerve function, if more than 55 microseconds, the shaking widened. When a serious block. Muscle shivering in record 20 2> 55 ms was an exception.
4. Identification of the three MG crisis
Myasthenic crisis: Intravenous Tang Xi-Long (1 10mg), the first intravenous injection of 2mg, without adverse reaction, then 30 seconds all of the remaining 8mg injection, 0.5 MG symptoms after 1 minute better, 4 to 5 minutes after symptoms appear gravis; such as after injection, a temporary increase, there muscle tremors, compared with cholinergic crisis; such as injecting Long Teng hi no significant changes in symptoms, compared with anti-bend of the crisis. Because the drug can enhance gastrointestinal motility, it should be 2 hours later in the meal; because of bronchial smooth muscle spasm and can lead to heart rhythm changes, and therefore, bronchial asthma and cardiac arrhythmias with caution. MG disease often has chronic or subacute onset of disease, spread from one muscle to other muscle groups showed highly involved in skeletal muscle fatigue. History of symptoms and signs (a) symptoms:
MG often has chronic or subacute onset of disease, spread from one muscle to other muscle groups showed highly involved in skeletal muscle fatigue, such as ocular involvement of the eyelids droop; throat muscles affected the speech, weakness, difficulty swallowing, choking water cough, inability to chew; neck muscle weakness are suppressed; limbs, muscle weakness involved extremities, such as the lifting arm weakness, lower limb problems or easy to fall down stairs; intercostal muscles involved are breathing difficulties, speech sound small and weak. Above symptoms and activities in the afternoon or evening after the increase, or early morning break or after the symptoms with anti-cholinesterase drugs to reduce or disappear. Incidence of MG can be any age, who a few days minimum, maximum, 70 years old. China 1458 cases of MG by the statistics there are two peak age of onset, first 10 years of age, accounting for 41.2%, and the other is 20 to 30 years, accounting for 20.5%. 40 women before the age of onset than men, more men than women after the age of 40, and those associated with thymoma and more.
(B) of the signs:
MG patients about 70% to 90% of patients show ptosis, can be unilateral or bilateral, or alternating left and right. Eyelid movement is limited, or diplopia, strabismus; facial muscle involvement is manifested smile face, lax eyelid closure, whistle difficulties, can not drum gills; throat muscles involved partial visible uvula, soft palate drooping bow;
Clinical classification: the classification of myasthenia gravis according to Osserman's classification number.
1. Divided into five types of adult MG: â…  type is a simple ocular, extraocular muscle involvement is simply no other muscle group involvement, clinical and electrophysiological changes in response to treatment of adrenal cortical hormone is good, good prognosis. MG-type body type â…¡, â…¡ A mild systemic type, this type of mild limb muscle involvement, often accompanied by involvement of extraocular muscles, bulbar muscle paralysis are not associated with self-care, drug treatment response and prognosis is usually . â…¡ B moderate systemic type, this type of limb muscles moderately affected, often accompanied by involvement of extraocular muscles, usually with chewing, swallowing, dysarthria and other bulbar muscle paralysis by. Self-care difficulties, the general response to drug therapy. Acute progressive type â…¢, rapid progress of the disease, most in the onset of several weeks or months appear palsy, often accompanied by involvement of extraocular muscles of life can not take care of themselves, more than six months, respiratory muscle paralysis, response to drug therapy poor prognosis is not good. Type â…£ type of late-onset myasthenia gravis the body is determined by the â… , â…¡ A, â…¡ B developed from the poor response to drug treatment, the prognosis is not good. Muscular atrophy type â…¤, this type of patient appears within six months after the onset of muscle atrophy.
2. Children gravis
(1) Neonatal myasthenia gravis: In day 1 after birth appears gravis, showing difficulties in sucking, crying deep.
(2) congenital weakness: that life or the baby after birth, the short term weakness, and persistent external ophthalmoplegia.
(3) juvenile myasthenia gravis mean onset of 14 to 18 years old MG, a simple ptosis or strabismus, diplopia and other more common, dysphagia, muscle weakness or systemic weakness more common than the children, also showed only the spinal cord gravis.
MG crisis, MG patients with breathing difficulties, swallowing difficulties and can not maintain basic living, life, known as myasthenic crisis. The total incidence of about MG 9.8% to 26.7%. According to myasthenic crisis occurred in the clinical reasons, there are three cases: â‘  weakness of the crisis: the development or because of disease caused by lack of anti-cholinesterase drugs, clinical manifestations, swallowing, coughing can not, respiratory distress, and even to stop the serious difficulties conditions. Examination shows dilation of pupils, whole body sweating, abdominal distension, injection of neostigmine symptoms improved. â‘¡ cholinergic crisis: caused by the excessive anti-cholinesterase drugs, in addition to common features of myasthenia gravis, the patient miosis, muscle twitching, hyperactivity bowel sounds, Tang Xi-Long after the intravenous injection of symptoms. â‘¢ anti-bend of the crisis: from infection, poisoning, electrolyte imbalance and other causes, using anti-cholinesterase drugs did not change significantly.
Range of clinical muscle involvement is also to be classified: simple ocular, bulbar muscular, spinal muscular, muscular body, muscle atrophy. Regardless of any types of clinical MG, generally divided into three clinical course of: â‘  fluctuations: onset within 5 years after, especially the first 1 to 2 years, the condition have a greater volatility, and risk prone gravis as high mortality. â‘¡ Stabilization: disease within 10 years. â‘¢ chronic phase: duration more than 10 years, after two patients in stable condition, rare crisis, the prognosis is good. EMG power diagnostic examination: MG patients EMG muscle contraction decreased, the amplitude of smaller, low-frequency limit of the ulnar nerve repetitive stimulation potential gradually decay, single fiber electromyography examination revealed muscle fibers excitation transmission block phenomenon of inconsistent or CT or MRI diagnostic imaging examination: suggestive of thymus hypertrophy or thymic tumors. 1 laboratory diagnosis of blood, peripheral blood T lymphocyte subsets exception, MG auxiliary T lymphocytes in peripheral blood of patients (T4) increased inhibition of T lymphocytes (T8) decreased, T4/T8 ratio increased.
2, a serum AchR Ab-positive, about 63% ~ 95% MG patients with a serum AchR Ab-positive.
3, immune globulin increased, about 2 / 3 MG patients with elevated IgG. Other diagnostic urinary CSF immunological fecal histological differential diagnosis of a Western test, ocular ① onset occult malnutrition; ② multiple young men; ③ worsening, no late morning light weight fluctuations; ④ anticholinesterase drugs invalid. 2, progressive bulbar paralysis ① there genioglossus contraction, genioglossus fibrillation; ② beating a muscle or muscle bundle fibrillation; ③ strongly associated with strong crying laughing; ④ anticholinesterase drugs is invalid. 3, a family history of periodic paralysis and recurrent ① history; ② lower limbs, muscle tone, flaccid paralysis of the lower tendon reflexes; ③ low serum potassium, intravenous potassium symptoms quickly improved transmission; ④ no change in EMG. 4, myasthenic syndrome ① with certain malignancies, in particular in common oat-cell lung cancer; ② males; ③ to proximal lower limb weakness mainly a sense of fatigue after activities, to continue activities to reduce symptoms; ④ anti-cholinesterase drugs can improve symptoms; ⑤ EMG repetitive stimulation to more than 10Hz, the muscle contraction can be identified such as the amplitude increases. 5, hysterical young women ① common weakness; ② concerned with the emotional changes; ③ easy to accept the hint; ④ irregular condition changes; ⑤ with fatigue, such as testing or anti-cholinesterase drugs can be identified. Identification of traditional Chinese medicine evaluation standard class 1 license, the general criteria:
(1) clinical cure: Clinical symptoms and signs disappeared, to normal life, study and work, disable all the drug treatment of MG, after more than 3 years without recurrence.
(2) Clinical Jinqi cured: clinical symptoms and signs disappeared, to resume normal life, study, work, disable all the drug treatment of MG, or a dose reduction of 3 / 4 or more, which lasted more than 1 month without recurrence.
(3) markedly effective: Clinical symptoms and signs of significant improvement in life can take care of themselves, adhere to the study or to complete the light work, treatment dosage of drugs reduced MG 1 / 2 or more, which lasted more than 1 month without recurrence.
(4) improvement: The clinical symptoms and signs have improved, an improvement in daily living, treatment, drug consumption decreased MG 1 / 4, which lasted more than 1 month without recurrence.
(5) is invalid: no improvement in clinical symptoms and signs, or even worse.
2, the scoring standards
Healing: clinical relative score ≥ 95%.
Basic cure: clinical relative score of 80% ~ 95% (including 80%).
Markedly: clinical relative score 50% to 80% (50%).
Improvement: clinical relative score of 25% to 50% (including 25%).
Invalid: clinical relative score <25%.
3. Comparison of two standard "general standard" simple, easy to master, but for the same patients, before treatment and after treatment, the comparability of difference. For a certain kind of therapy or a new objective evaluation of the efficacy of new drugs should be used "scoring criteria" to determine efficacy.
Prognosis 1. MG and clinical prognosis of the type, pure and simple ocular muscular spinal cord, eye muscle disease and limb muscle weakness has always been limited to a good prognosis; systemic weakness, bulbar weakness and further development of the disease risk appears like who the prognosis is not good. 2. Against cholinesterase agents, hormones and other drugs can not surgery is not sensitive to poor prognosis. 3. There infection (such as lung infection) and respiratory failure, the prognosis is not good. 4. Associated with the thymus increased, or the â… , â…¡ A type of thymoma who could cholinesterase surgery and drug sensitivity against a good prognosis, â…¡ B or type â…¢ thymoma after surgery and poor results. 5. The growth of prostitution of thymoma with invasion, metastasis or poor prognosis. 6. Short duration, medication-sensitive disease had better prognosis is good; long course, is not sensitive to those drugs with poor prognosis. 7. The clinical observation has some relationship with age, age at the time patients <15 years old curative effect and prognosis is good;> 15 years of age is poor treatment, prognosis is poor, a significant difference between the two groups. MG is a Western medicine treatment of complications of chronic diseases, the patient can not adhere to the long-term work, study and life will be difficult, thought the burden of large medical staff to be patient psychological treatment for patients, encourage patients to establish long-term fight against the disease of the spirit; the same time According to the specific circumstances of the patient choose the appropriate treatment and achieve good effect; of systemic MG patients have to stay in bed, breathing difficulties to oxygen, can not shoot back sputum assisted cough or sputum expectoration, to monitor heart function, Strict records of administration time, dose; attention to water imbalance.
(A) Drug treatment:
1. Anticholinesterase drugs are temporarily effective drug treatment of MG, but the drugs only suppress the symptoms but not cure, only temporary improvement in symptoms can not suppress the immune. Common are:
(1) neostigmine tablets: Each 15 ~ 30mg, 2 to 4 times a day; injection every 0.5mg ~ 1.0mg, 30 minutes after injection of effective, best after 1 hour, generally used for first aid or study drug.
(2) pyridostigmine tablets: Each 60 ~ 120mg, 3 to 6 times a day, 1 hour after taking the blood concentration, 1.5 to 2 hours of the peak, half-life of 4.25 hours, the drug has role in a long time, fewer side effects features.
(3) U.S. neostigmine (enzyme inhibition Ning): Every 5 ~ 10mg, 2 to 4 times a day, is the most potent anti-cholinesterase drugs, neostigmine about 2 to 4 times, the role of a long time, can maintain 6 to 8 hours. Drug side effects, has recently found to promote insulin secretion, can lead to hypokalemia, clinical application to note. In the application of anti-cholinesterase drugs, they should be individual differences in specific dosage used, usually start small dose and gradually increase to maintain the best performance and ability to maintain consumption standards. Side effects are miosis, salivation, abdominal pain, diarrhea, sweating, muscle beating.
2. Immunosuppressant
(1) adrenal cortex hormones: ① the mechanism of hormone can inhibit the synthesis of AchR antibodies, the postsynaptic membrane AchR or less from the destruction caused by autoimmune attack; ② easy to make the presynaptic release of Ach; ③ can regeneration of the endplate, increasing the number of postsynaptic AchR. Suitable for adults, especially after the age of 40 systemic onset myasthenia, anti-cholinesterase drugs were not satisfied; or thymoma or thymic hyperplasia before and after surgery; or thymus immune function before radiation therapy were more active. A method for the reduced dose approach to begin the selection of high-dose prednisone, the daily 60 ~ 100mg, 8 to 12 weeks maintenance medication, after the gradual reduction of the condition of patients, 15 ~ 30mg medication until the next day, the subsequent slow reduction the amount of speed for several years (high dose → → decreasing the maintenance dose), this method is that the patient reactions, drug defects, the early treatment of exacerbations can occur because of hormones on neuromuscular transmission in joints caused by direct inhibition. Symptoms increase in the rejection by the hormone increased pyridostigmine appropriate dose and frequency, or the complement of potassium, calcium, potassium can improve the membrane potential, calcium can promote the release of Ach, ease symptoms increase the response. Another method is incremental, that is start small dose of oral prednisone daily 10mg, after the 20mg, 30mg gradually increased to 60 ~ 100mg, MG symptoms improved, the maintenance of 8 to 12 weeks, then gradually reduce, to every other day for 15 ~ 30mg, a few years (cumulative dose → → → dose → decreasing the maintenance dose). Side effects of this method is suitable for outpatient treatment. Recent adrenal corticosteroid therapy high efficacy, the effective rate of 96%, 89% efficiency of mitigation and significant, but the drug can not clear the initiating antigen in the thymus, it is easy to relapse after drug withdrawal. It was proposed to reduce the recurrence: ① hormone reduction is not too fast, at least 3 to 6 months; ② the amount too little to maintain, at least 10 ~ 20mg / day; ③ stopping too early, at least one year treatment period , to improve the efficacy and used with other drugs. Hing's side effects of hormone expression library (moon face, obesity, hirsutism), as well as hypertension, diabetes, cataracts, ulcers, gastrointestinal bleeding, psychiatric symptoms, osteoporosis, bone stock head necrosis. In order to improve effectiveness and reduce the role of hormonal side effects and can be used with other drugs, such as potassium chloride given orally can improve the membrane potential; prevention of gastric ulcers and bleeding, oral administration of a cyanide m guanidine, inhibit gastric acid production. Ach may be given to promote the release of calcium gluconate; promote protein synthesis, inhibition of protein breakdown may be given Nandrolone phenylpropionate; prevention of osteoporosis and aseptic necrosis of the femoral head may be given vitamin D and calcium. MG associated with active tuberculosis, peptic ulcer, severe hypertension, heart disease and other infectious diseases, hormone therapy should be listed as a contraindication.
(2) cyclophosphamide: 1000mg dosage of intravenous injection, every 5 1; or 200mg intravenously 2 to 3 times per week; or oral daily 100mg, until it reached a total of 10 ~ 30g. Child day 3 ~ 5mg/kg, twice used, improves reduction, 2mg/kg daily maintenance. The greater the total treatment with cyclophosphamide, the better its efficacy the longer course of treatment, reached a total of more than 10g, 90% effective, reaching more than 30g or 30g is 100% effective. Treatment up to 33 months can make 100% of patients symptoms disappeared completely, reaching a stable remission. Applicable, ① adrenal cortex hormone therapy can not be tolerated by: ② adrenal cortex hormone therapy were ineffective or slow effect; ③ thymectomy were ineffective; ④ reduction of the adrenal cortex hormones relapse. Reduce the number of side effects can cause white blood cells (white blood cell count <4 × 109 / L reduction, <3 × 109 / L discontinued), thrombocytopenia (platelet count <100 × 109 / L reduction, <60 × 109 / L stop drugs), hair loss, bleeding bladder.
(3) azathioprine: often with the combination of prednisone or other immunosuppressive agents. Adult dosage of 150 ~ 300mg / day, children's day 1 ~ 3mg/kg, orally in two long-term. Side effects: may cause leukopenia (white blood cell count <2.5 × 109 / L reduction, <2.0 × 109 / L discontinued), thrombocytopenia, and alopecia.
3. Gamma globulin therapy with high dose intravenous gamma globulin treatment of severe systemic MG interest may obtain a significant effect. It was reported intravenous gamma globulin in patients with MG Ts can increase. Speculated that intravenous injection of gamma globulin can be against the AchR Ab or from one site to replace their AchR antibodies to protect against the AchR antibodies damage. Dosage: daily 400mg/kg, occasional side effects include headache, swollen feet.
4. VEP therapy is the vincristine, cyclophosphamide, prednisolone (vincristine enoloxan prednisolone, VEP) therapy for thymoma associated with MG patients but not suitable for surgery.
5. Disable and wise use of medication curare, succinylcholine, quinine, quinidine, chloroform, streptomycin, neomycin, kanamycin, colistin, polymyxin, vancomycin, and other aminoglycoside antibiotics have neuromuscular blocking effect and should be disabled. Morphine, pethidine, which are inhibiting respiration, should be disabled or used with caution; tetracycline family of antibiotics, tetracyclines and that magnesium ions are added to inhibit the release of Ach, should be used with caution.
(B) non-drug therapy
1, surgery can remove the MG thymus produces its own immune response in patients initiating antigen, immune activity can reduce the generation of T-lymphocytes, reducing the synthesis of acetylcholine receptor antibodies, now that the MG is the most basic treatment.
Indications: ① various types of thymoma with MG; ② associated with thymus hypertrophy and high-AchR antibody titers young female body type MG; ③ response to anti-cholinesterase drugs were not satisfied, regardless of whether the thymoma or thymic hyperplasia , can be done thymectomy. Remission rate increased year by year after the first 1 year of 30%, 50% for 2 years, 3 years was 65%, 6 years to 80%. Surgery: ① women than men; ② illness is more light, the shorter the better course; ③ germinal centers within the thymus more epithelial cells, the more obvious the better operation; ④ and used immediately after surgery and effect of adrenal cortex hormones; ⑤ with thymoma were less effective.
2, â‘  thymus radiotherapy radiotherapy: suppress immune function in the thymus, the blood T lymphocytes and B lymphocytes decreased, AchR antibody reduced to thymic atrophy. Methods for the depth of X ray or 60 Co irradiation, thymectomy similar efficacy and side effects are local pain, ulceration of red, white blood cell count decreased, malaise, headache, loss of appetite and so on. â‘¡ spleen radiation therapy: inhibition of B lymphocyte function, thereby inhibiting the production of AchR antibodies is to reduce or alleviate the symptoms of MG. Method of daily exposure IGy, 10 days for treatment, interval of 3 weeks and then the second line treatment, or per 2Gy, 2 to 3 times per week, amounting to 20 ~ 30Gy. Side effects include mild diarrhea, bowel and so on. â‘¢ systemic low-dose radiation therapy: make lymphocytes, AchR antibody reduced, so that Ts cells back to normal. Side effects are general malaise, fatigue, insomnia, loss of appetite and so on. â‘£ local irradiation: MG patients with partial neuromuscular postsynaptic membrane at the accumulation of excessive AchR antibodies, play through the inhibition of local radiotherapy.
3, plasma exchange with normal plasma or plasma substitutes periodic replacement of plasma MG patients, thereby reducing a patient blood AchR Ab. Calculated blood volume of 5% by weight, each 1000ml ~ 2000ml plasma exchange, daily or every other day, 5 to 6 times for treatment. Rapid onset of the treatment, curative effect is good, but not last, so for the onset of crisis, or crisis-prone, or refractory MG patients. After plasma exchange treatment began to improve in 1 to 2 weeks, antibody decline, and later to rise slowly, the gradual emergence of symptoms and, therefore, to be in remission after the use of other immunosuppressive therapy or thymectomy or thymus radiotherapy. Side effects: hypotension, syncope, hypoproteinemia, limb numbness, bleeding, thrombosis, allergy. The high cost of this therapy is not easy to promote.
4, blood replacement therapy is intravenous blood, Zaishu equal the same type of allogeneic fresh blood. Replacement of blood volume per adult 800 ~ 1000ml, children 100 ~ 200ml, the elderly and the youths 600 ~ 800ml, according to condition 3 to 7 days 1, 3 to 4 times as a course of treatment, bleeding time monitoring of blood pressure, pulse, low blood pressure at 12kPa (90mmHg), the pulse of more than 120 beats / min, stop bleeding, or 706 on behalf of both plasma infusion to maintain blood pressure. Mechanism of action with plasma exchange or plasma cleaning treatment, blood replacement can partially remove pathogenic antibodies to the rescue of critically patients, urban and rural areas can be carried out.
(C) treatment of myasthenia gravis crisis:
MG crisis is the most critical condition, the mortality is high, there are reports of 15.4% to 50%. First, what kind of crisis is to clear and then proceed to actively rescue measures.
1. Keep the airway open when there is paralysis of respiratory muscles, mucus discharge difficulties, do endotracheal intubation or tracheostomy, artificial mechanical ventilation to ensure adequate ventilation to correct hypoxia, a timely elimination of respiratory secretions.
2, the active use of effective infection control adequate antibiotics to control infection, the available lincomycin, penicillin, erythromycin, cephalosporins, Fortum, Bristol and other bacteria.
3, hormone use methylprednisolone 500 ~ 2000mg / day, or dexamethasone 10 ~ 20mg / day sugar infusion, 3 to 5 days after the change in stable condition after oral prednisone.
4, cholinesterase inhibitor, cholinergic crisis to disable anti-cholinesterase drugs, and intramuscular or intravenous injection of atropine 0.5 ~ 2.0mg, 15 ~ 30 minutes after symptom improvement was not obvious reusable applications. Myasthenic crisis, increase anti-cholinesterase drugs, the effect is not obvious, then disable the anti-cholinesterase drugs, a large number of applications for a long time after, can AchR on the Ach sensitivity was significantly reduced. Tracheal intubation in the control of positive pressure breathing oxygen should immediately stop all cases of cholinesterase inhibitor (ChEl), will enable the AchR get enough rest, and gradually increase the sensitivity of AchR on Ach, generally disable 3 ~ 5 days after the respiratory muscle function began to recover, accordi
ng to the degree of recovery in patients with muscle re-start small dose application ChEl, gradually increase the dosage to the optimum dose, but also to achieve better therapeutic effect.
5. With plasma exchange by plasma exchange can be a blood Ach Ab clearance, so that functional recovery of AchR. Wait for his condition improved with other therapies to control weakness.
6. Intravenous infusion in time to correct water and electrolyte balance disorders.
7. Strengthening nursing pay attention to oral care, according to strict tracheotomy care, maintenance of respiratory humidification to prevent asphyxia. A Chinese medicine treatment, diagnosis and treatment:
1, spleen and stomach Qi:
Governing Law: Spleen Qi.
Recipe: Bu Zhong Yi Qi Decoction. Fang Shen Qi Qi of the spleen surgery; Cimicifuga, Bupleurum Shengqingjiangzhuo; Citrus qi; Angelica and blood; Jiang date to reconcile business health; barley, Poria Jianpihuashitang increase the effect.
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