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1ClinicalDatapatient, aged 45, 150 cm tall, weighing 40 kg, admitted to hospital for motor neurone disease (Motor disease, MND), the proposedgastricanoral surgeryPast history: Patients 30 years ago appeared lower limb muscle weakness, nearly a month of limb weakness combined eating dysfunction, 1 month ago walking fell, head bruising, 2 weeks ago appeared difficult to swallowPreoperative examination: laboratory examination and electrocardiogram did not see obvious abnormalitiesThere were no obvious abnormalities in the chest examinationDue to the combination of motor neurone disease patients with breathing difficulties, the original plan to do respiratory function measurement, but because the patient felt fatigue respiratory function measurement was suspendedpreoperative anaesthetic monitoring: blood pressure monitoring with noninvasive cuffs, SpO2, ECG, BIS, PETCO2Patient Admission SpO2 92%, HR:80bpm, BP:145/76mmHg, BIS value 87-95 fluctuatesEpisdural anesthesia is considered among them, but due to the patient's excessive tension, refused to do epidural anesthesia, we choose the whole hemp induced larynx cover placed in the way of anesthesiaanaesthetic induction: inhalation of 8% heptafluoroetherande and oxygen flow 8ml/min anaesthetic induction, after 4min BIS value dropped to 45-60, blood pressure down to 88/45mmHg, heart rate 63 times/min to give ephedrine Alkaline 6mg, with blood pressure stable at 100/60mmHg, a variety of reflections disappear immediately to give 3.5 - throat cover into the throat cavity, intubation process smoothly, throat cover after the blood pressure rose to 109/70mmHgAnaesthetic maintenance uses 4% heptafluoroetherande and gives the right metamine 0.4mg/minDuring the operation, the patient's blood pressure was stable between 110-120/60-70mmHg, the heart rate was about 70, PETCO2 30 fluctuated, the BIS fluctuated between 40-60, the operation was 50min, and the procedure was smooth infusion colloid 300ml, crystal fluid 500ml, bleeding volume 100ml, urine volume 100ml, surgical give Parisib sodium 40mg Intraoperative lysing pills and opioid analgesics were not given After the operation 20min patients breathing function, consciousness recovery, deoxygenation oxygen maintenance of more than 90%, pull out the larynx, hemodynamics did not see significant fluctuations, oxygen absorption observation 1h, good breathing patients without obvious discomfort, slight pain At this time blood pressure 138/82mmHg, HR is 76bpm The next day visit patients, patients generally in good condition, blood oxygen in more than 98%, blood pressure stable at 120/80mmHg or so, 7 days after discharge, without any anaesthetic-related complications 2 Discussion motor neurone disease is a relatively rare disease, male and female, the disease rate is 1.2:1-2.5:1 The annual incidence rate is 1.5/100,000-2.7/100,000, and the prevalence rate is about 2.7/100,000-7.4/100,000 The etiology and pathogenesis are not clear, and are currently related to genetic mechanisms, oxidative stress, excitatory toxicity, neurotrophic factor disorders, auto
immune mechanisms, virus infection and environmental factors Mainly for muscle weakness, muscle atrophy and cone beam signs and other manifestations There are few clinical manifestations of damage to intelligence, sensation, vertebral in vitro and autonomic nervous system
clinical manifestations The sole damage to the front foot of the spinal cord, characterized by muscle weakness and muscle atrophy, but without cone beam signs, is the performance of the amortic muscle atrophy (PMA) damage is limited to the elongated motor nuclei of the myelin and the symptoms of the throat muscle and tongue muscle weakness and cone beam signs for primary lateral sclerosis (PLS) There are small percentages of MND patients outside the motor system, such as: dementia, vertebral symptoms, sensory abnormalities and rectum dysfunction At this point, it is possible that MND is accompanied by other diseases or MND fatigue and other systems, called atypical MND patients with motor neurone disease have many difficulties in stomach anoral surgery First of all, because the incidence of motor neurone disease is very low, there are various disputes in the choice of anesthesia, although there are also cases of motor neurone disease in the lumbar-hard combination of successful cases, but because the patient's proposed gastric surgery, the requirements of a higher plane, and the patient is overstretched, we consider the use of general anesthesia Motor neurone disease often produces spinal palsy in the late stages, and a few can be the first symptoms The tongue muscle is first affected, and then appears the palate, pharynx, throat, chewing muscle atrophy, resulting in unclear composition, difficulty swallowing, chewing muscle weakness Late stage to the whole body muscle atrophy, powerless, life can not take care of themselves, and eventually often because of the lungs infection death So the assessment of lung function before anesthesia is very necessary, as there is often muscle atrophy, open ness is sometimes limited, and it is essential to establish a reliable airway we chose to place the larynx cover because of the short operating time and the patient's not full stomach Although we choose to place the larynx cover, and did not produce adverse consequences, but after a large number of literature reading motor neurone disease will develop to the weakness of the respiratory muscle will produce low ventilation, hypercarbonemia and secretion removal capacity decreased, so the possibility of accidental inhalation is also greatly increased, so the trachea intubation with sacs should be a safer choice The risk of the cardiovascular system during the perination period is not common However, there have been reports of long-term trachea intubation increase the risk of secondary intubation after surgery, because the lung function of motor neurone disease patients itself is worse than normal people, long-term trachea intubation will likely cause patients to increase the rate of lung infection after surgery, so the patient's secondary intubation rate is increased the use of narcotic drugs is not clear lying as an absolute contraindication, but most narcotic drugs should be used with caution A trachea intubation without muscle loosening is a good choice The depolarized myaminic amber choline taboo is used in patients with motor neurone disease because of the risk of transverse muscle dissolution and hyperkalemia, which can lead to arrhythmia and fibromyalfia Although there is no clear report ingestion of non-depolarized myocardine, and in patients with motor neurone disease, the action time of non-depolarized muscle pine medicine is prolonged and uncertain, the operation time is relatively short, the requirements of surgery for muscle loosening are not very high, and the application of myaminic and opioid analgesics will increase the risk of delayed respiratory muscle function recovery, we choose muscle-free inhalation anesthesia induction and maintenance Heptifluorite is a good alternative, its average end-of-breath concentration before trachea intubation is 4.2%, that is, when the end-of-breath concentration reaches 2MAC, the intubation can not produce intubation reflection And we have to apply the larynx, so it has a smaller impact on the patient and increases the patient's comfort when awake Since opioids also have the potential to increase respiratory muscle inhibition in patients, we choose to give nonsteroidal analgesics (Parisib sodium), which is a selective COX-2 inhibitor, which plays an important role in the synthesis of prosthesis-like precursors associated with pain, inflammatory response and fever, without affecting the patient's breathing and interfering with the transfer of neuromuscular joints , the prevalence of motor neurone disease is low, and most patients in advanced stages need gastric fistula in order to improve their quality of life In this case, patients choose heptafluorein anaesthetic induced to combine right metoric to maintain and combine nonsteroidal analgesic analgesics, in short surgery can produce adequate sedative and analgesic effects and hemodynamic stability And the larynx intubation reduces the patient's inwellness during the awakening period, and can maintain good ventilation function The effect of anesthesia is satisfactory, which provides a reference for general anesthesia in the future of motor neurone disease patients However, if such patients perform a long period of major surgery anaesthetic, the process of anesthesia management , the choice of narcotic drugs, still need our continuous exploration and research