重症疾病性神经肌肉病(英文课件)

,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,重症疾病性神经肌肉病(英文课件),16、云无心以出岫，鸟倦飞而知还。,17、童孺纵行歌，斑白欢游诣。,18、福不虚至，祸不易来。,19、久在樊笼里，复得返自然。,20、羁鸟恋旧林，池鱼思故渊。,重症疾病性神经肌肉病(英文课件)重症疾病性神经肌肉病(英文课件)16、云无心以出岫，鸟倦飞而知还。,17、童孺纵行歌，斑白欢游诣。,18、福不虚至，祸不易来。,19、久在樊笼里，复得返自然。,20、羁鸟恋旧林，池鱼思故渊。CRITICAL ILLNESS NEUROMYOPATHY Abbreviations,CIP critical illness polyneuropathy,CIM critical illness myopathy,CMAP compound muscl action potentials,SNAP sensory nerve action potential,EMG electromyogram,SIRS systematic inflammatory response syndrome,重症疾病性神经肌肉病(英文课件)16、云无心以出岫，鸟倦飞而,1,重症疾病性神经肌肉病(英文课件),2,重症疾病性神经肌肉病(英文课件),3,重症疾病性神经肌肉病(英文课件),4,重症疾病性神经肌肉病(英文课件),5,Studies about Aetiologyvariously,The various factors associated with the SIRS CIP and CIM(,Fig.,1,),A simplified depiction of theoretical mechanisms of dysfunction in CIP and CIM.(,Fig.2,),Disorder of microcirculation,（,Fig.3,),Studies about Aetiologyvari,6,Adapted with permission from Bolton.,Figure.1,Adapted with permission from,7,Adapted with permission fromBolton25.,Figure.,2,Adapted with permission fromBo,8,Figure.3,Schematic,theoretical presentation of disturbances in the microcirculation to various organs,including brain,peripheral nerve,and muscle,in SIRS.,Figure.3 Schematic,theor,9,重症疾病性神经肌肉病(英文课件),10,Incidence,50%70%SIRS,20%,50%ICU,重症疾病性神经肌肉病(英文课件),11,Weakness of limb and respiratory muscle,Tendon reflexes absent or decrease,Distal loss to pain,temperature,and vibration,Clinical Features,Clinical Features,12,The diagnostic criteria for CIP are shown in following Table,Diagnosis,Diagnosis,13,Diagnostic criteria for CIP,The patient is critically ill(sepsis and multiple organ failure,SIRS),Difficulty weaning patient from ventilator afternonneuromuscular causes such as heart and lung diseasehave been excluded,Possible limb weakness,Electrophysiologic evidence of axonal motor and sensory polyneuropathy,14,Decline in the CMAP amplitude firstly(,Fig.4,),Dcline in the SNAP amplitude,Motor unit potentials may be reduced in number,Single-fiber EMG indicate dysfunction of terminal motor axons,Electrophysiologic Features,Electrophysiologic Features,15,Measurement of compound thenar muscle action,potentials at the onset of sepsis,(A)and 3 weeks later(B).,FIG.4,Measurement of compound,16,Peripheral axonal degeneration.,Moderate loss of dorsal root ganglion cells,Central chromatolysis of anterior horn cells,No inflammation in the peripheral nervous system,Morphologic Features,Morphologic Features,17,Muscle biopsy,Acute and chronic denervation,Occasional myopathic changes,Muscle biopsy,18,Pathology of critical illness polyneuropathy.There is chromatolysis of anterior horn cells,(A);,severe axonal degeneration in this cross-section of superficial peripheral nerve,(B),and longitudinal section of deep peroneal nerve,(C);,and acute and chronic denervation of intercostal muscle,(D),Pathology of critical,19,Axonal variants of Guillain,Barre syndrome,Develop earlier,Often associated with,CJ,infection,Abnormal cerebral spinal fluid,Differential Diagnosis,Differential Diagnosis,20,Transient neuromuscular blockade,Repetitive nerve stimulation,Measurement of anti-MuSK(muscle specific receptor tyrosine kinase)antibodies,Transient neuromuscular block,21,Treatment of sepsis and multiple organ dysfunction syndrome,Management of difficulty in weaning from the ventilator,Attempts at direct treatment of CIP(still unproven),Physiotherapy and rehabilitation,Treatment,Treatment of sepsis and multip,22,Two newer research approaches are being explored,Intensive insulin therapy,The administration of recombinant human activated protein C,23,Recovery depends on the distance,Recovery for weeks in mild cases and months in severe cases,Slowing of nerve conduction may have a poor prognosis,Prognosis,Recovery depends on the distan,24,重症疾病性神经肌肉病(英文课件),25,Incidence,At least one-third of ICU patients,（,treated for status asthmaticus,）,In 7%of patients after transplantation,Incidence,26,Clinical Features,Major feature is flaccid weakness,Tendon reflexes depressed,Ophthalmoplegia may be present,Myalgias are uncommon,Clinical Features,27,Diagnostic criteria of CIM,SNAP amplitudes 80%of the lower limit of normal,Needle EMG with short-duration,low-amplitude MUPs with early or normal full recruitment,with or without fibrillation potentials,Absence of a decremental response on repetitive nerve stimulation,Diagnosis,Diagnosis,28,Muscle histopathologic findings of myopathy with myosin loss,CMAP amplitudes 80%of the lower limit of normal in two or more nerves without conduction block,Elevated serum creatine kinase(CK,）,Demonstration of muscle inexcitability,*For a definite diagnosis of critical illness myopathy,patients should have all of the first five features.,29,Nerve conduction studies,Low-amplitude CMAPs,Long duration CMAPs,Normal SNAPs,Phrenic nerve conduction normal latencies diaphragm CMAP amplitudes reduce,Electrophysiologic Features,Nerve conduction studiesElectr,30,EMG,Fibrillation potentials and positive sharp,Motor unit potentials low amplitude and short duration,Electrical inexcitability by direct needle stimulation,31,Features of the histopathology in thick filament myosin loss(,Fig.5,),Electron microscopy reveals selective loss of thick(myosin)filaments(,Fig.6,),Inflammatory changes are conspicuously absent,Morphologic Features,Features of the histopathology,32,Figure.5,Muscle histopathology in a critically ill patient with thick filament myosin loss.(original magnification,100)(courtesy of Dr.Andrew Engel).,33,Figure.6,Electron microscopy of muscle in CIM.(original magnification,44,000)(courtesy of Dr.Andrew Engel).,34,6.Differential Diagnosis,CIP,Direct needle stimulation of the muscle,Electrical inexcitability in CIM,There is a response in CIP,(,Fig.7,),Serum CK,Muscle biopsy,6.Differential Diagnosis CIP,35,FIGURE.7,Results of direct and indirect muscle stimulation.CMAPs from the anterior tibial muscles of a patient with critical illness polyneuropathy(left)and critical illness myopathy(right).,FIGURE.7 Results of direct,36,Subtypes of CIM,Muscle m,orphologic,Differentiating feature of neuromuscular disorders in critical illness Table 1.,37,重症疾病性神经肌肉病(英文课件),38,No specific therapy available as to now,Positioning to avoid additional nerve damage by pressure,Avoid administering muscle relaxants and corticosteroids,Treatment,No specific therapy available,39,Thank you!,Thank you!,40,谢谢你的阅读,知识就是财富,丰富你的人生,71,、既然我已经踏上这条道路，那么，任何东西都不应妨碍我沿着这条路走下去。,康德,72,、家庭成为快乐的种子在外也不致成为障碍物但在旅行之际却是夜间的伴侣。,西塞罗,73,、坚持意志伟大的事业需要始终不渝的精神。,伏尔泰,74,、路漫漫其修道远，吾将上下而求索。,屈原,75,、内外相应，言行相称。,韩非,谢谢你的阅读知识就是财富71、既然我已经踏上这条道路，那么，,41,