重症疾病性神经肌肉病英文课件

1、重症疾病性神经肌肉病英文课件 AbbreviationsCIP critical illness polyneuropathyCIM critical illness myopathyCMAP compound muscl action potentialsSNAP sensory nerve action potentialEMG electromyogramSIRS systematic inflammatory response syndromeHISTORICAL REVIEWIn 1955 observed a polyneuropathy after shock or cardia

2、c arrestIn 1961 described “coma-polyneuropathies” In 1971 described a polyneuropathy in patients with burnsin 1977 severe polyneuropathy about septic patients By 1983 the term “critical illness polyneuropathy”（CIP） was appliedRecently the termed “critical illness myopathy ”（CIM） was applied Studies

3、about AetiologyvariouslyThe 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) Adapted with permission from Bolton.Figure. 1Adapted with permission fromBolton25.Fig

4、ure. 2 Figure.3 Schematic, theoretical presentation of disturbances in the microcirculation to various organs, including brain, peripheral nerve, and muscle, in SIRS.Incidence 50%70% SIRS 20%50% ICU Weakness of limb and respiratory muscle Tendon reflexes absent or decrease Distal loss to pain, tempe

5、rature, and vibrationClinical FeaturesThe diagnostic criteria for CIP are shown in following TableDiagnosis Diagnostic criteria for CIPThe patient is critically ill (sepsis and multiple organ failure,SIRS)Difficulty weaning patient from ventilator afternonneuromuscular causes such as heart and lung

6、diseasehave been excludedPossible limb weaknessElectrophysiologic evidence of axonal motor and sensory polyneuropathyDecline in the CMAP amplitude firstly (Fig. 4)Dcline in the SNAP amplitudeMotor unit potentials may be reduced in numberSingle-fiber EMG indicate dysfunction of terminal motor axonsEl

7、ectrophysiologic FeaturesMeasurement of compound thenar muscle action potentials at the onset of sepsis (A) and 3 weeks later (B).FIG.4Peripheral axonal degeneration.Moderate loss of dorsal root ganglion cellsCentral chromatolysis of anterior horn cellsNo inflammation in the peripheral nervous syste

8、mMorphologic Features Muscle biopsy Acute and chronic denervation Occasional myopathic changes 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 sectio

9、n of deep peroneal nerve (C); and acute and chronic denervation of intercostal muscle (D)Axonal variants of GuillainBarre syndromeDevelop earlierOften associated with CJ infectionAbnormal cerebral spinal fluidDifferential Diagnosis Transient neuromuscular blockadeRepetitive nerve stimulationMeasurem

10、ent of anti-MuSK (muscle specific receptor tyrosine kinase) antibodiesTreatment 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 rehabilitationTreatment Two newer research

11、approaches are being exploredIntensive insulin therapyThe administration of recombinant human activated protein C Recovery depends on the distanceRecovery for weeks in mild cases and months in severe casesSlowing of nerve conduction may have a poor prognosisPrognosisIncidenceAt least one-third of IC

12、U patients（ treated for status asthmaticus）In 7% of patients after transplantation Clinical FeaturesMajor feature is flaccid weaknessTendon reflexes depressedOphthalmoplegia may be presentMyalgias are uncommon Diagnostic criteria of CIM SNAP amplitudes 80% of the lower limit of normal Needle EMG wit

13、h short-duration, low-amplitude MUPs with early or normal full recruitment, with or without fibrillation potentials Absence of a decremental response on repetitive nerve stimulationDiagnosis Muscle histopathologic findings of myopathy with myosin loss CMAP amplitudes 80% of the lower limit of normal

14、 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.Nerve conduction studiesLow-amplitude CMAPs Long duration CMAPsNormal

15、SNAPsPhrenic nerve conduction normal latencies diaphragm CMAP amplitudes reduceElectrophysiologic Features EMGFibrillation potentials and positive sharpMotor unit potentials low amplitude and short durationElectrical inexcitability by direct needle stimulationFeatures of the histopathology in thick

16、filament myosin loss (Fig. 5)Electron microscopy reveals selective loss of thick (myosin) filaments (Fig. 6)Inflammatory changes are conspicuously absent Morphologic Features Figure. 5 Muscle histopathology in a critically ill patient with thick filament myosin loss. (original magnification, 100) (c

17、ourtesy of Dr. Andrew Engel). Figure. 6 Electron microscopy of muscle in CIM. (original magnification, 44,000) (courtesy of Dr. Andrew Engel).6. Differential Diagnosis CIPDirect needle stimulation of the muscle Electrical inexcitability in CIMThere is a response in CIP (Fig. 7)Serum CKMuscle biopsy 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 (ri