1、重癥疾病性神經肌肉病(英文)重癥疾病性神經肌肉病(英文)CIP critical illness polyneuropathyCIM critical illness myopathyCMAP compound muscl action potentialsSNAP sensory nerve action potentialEMG electromyogramSIRS systematic inflammatory response syndrome重癥疾病性神經肌肉病(英文)In 1955 observed a polyneuropathy after shock or cardiac a

2、rrestIn 1961 described “coma-polyneuropathies” In 1971 described a polyneuropathy in patients with burnsin 1977 severe polyneuropathy about septic patients重癥疾病性神經肌肉病(英文)By 1983 the term “critical illness polyneuropathy”（CIP） was appliedRecently the termed “critical illness myopathy ”（CIM） was applie

3、d重癥疾病性神經肌肉病(英文)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)重癥疾病性神經肌肉病(英文) Adapted with permission from Bolton.Figure. 1重癥疾病性神經肌肉病(英文)Adapted with permissi

4、on fromBolton25.Figure. 2重癥疾病性神經肌肉病(英文) Figure.3 Schematic, theoretical presentation of disturbances in the microcirculation to various organs, including brain, peripheral nerve, and muscle, in SIRS.重癥疾病性神經肌肉病(英文)重癥疾病性神經肌肉病(英文)Incidence 50%70% SIRS 20%50% ICU重癥疾病性神經肌肉病(英文) Weakness of limb and respi

5、ratory muscle Tendon reflexes absent or decrease Distal loss to pain, temperature, and vibrationClinical Features重癥疾病性神經肌肉病(英文)The diagnostic criteria for CIP are shown in following TableDiagnosis重癥疾病性神經肌肉病(英文)Diagnostic criteria for CIPThe patient is critically ill (sepsis and multiple organ failur

6、e,SIRS)Difficulty weaning patient from ventilator afternonneuromuscular causes such as heart and lung diseasehave been excludedPossible limb weaknessElectrophysiologic evidence of axonal motor and sensory polyneuropathy重癥疾病性神經肌肉病(英文)Decline in the CMAP amplitude firstly (Fig. 4)Dcline in the SNAP am

7、plitudeMotor unit potentials may be reduced in numberSingle-fiber EMG indicate dysfunction of terminal motor axonsElectrophysiologic Features重癥疾病性神經肌肉病(英文)FIG.4重癥疾病性神經肌肉病(英文)Peripheral axonal degeneration.Moderate loss of dorsal root ganglion cellsCentral chromatolysis of anterior horn cellsNo infla

8、mmation in the peripheral nervous systemMorphologic Features重癥疾病性神經肌肉病(英文)Muscle biopsy Acute and chronic denervation Occasional myopathic changes重癥疾病性神經肌肉病(英文) Pathology of critical illness polyneuropathy. There is chromatolysis of anterior horn cells (A); (A); severe axonal degeneration in this cr

9、oss-section of superficial peripheral nerve (B) (B) and longitudinal section of deep peroneal nerve (C); (C); and acute and chronic denervation of intercostal muscle (D)(D)重癥疾病性神經肌肉病(英文)Axonal variants of GuillainBarre syndromeDevelop earlierOften associated with CJ infectionAbnormal cerebral spinal

10、 fluidDifferential Diagnosis重癥疾病性神經肌肉病(英文)Transient neuromuscular blockade Repetitive nerve stimulation Measurement of anti-MuSK (muscle specific receptor tyrosine kinase) antibodies重癥疾病性神經肌肉病(英文)Treatment of sepsis and multiple organ dysfunction syndrome Management of difficulty in weaning from the

11、 ventilator Attempts at direct treatment of CIP (still unproven) Physiotherapy and rehabilitationTreatment重癥疾病性神經肌肉病(英文) Two newer research approaches are being exploredIntensive insulin therapyThe administration of recombinant human activated protein C 重癥疾病性神經肌肉病(英文)Recovery depends on the distance

12、Recovery for weeks in mild cases and months in severe casesSlowing of nerve conduction may have a poor prognosisPrognosis重癥疾病性神經肌肉病(英文)重癥疾病性神經肌肉病(英文)IncidenceAt least one-third of ICU patients（ treated for status asthmaticus）In 7% of patients after transplantation重癥疾病性神經肌肉病(英文)Clinical FeaturesClini

13、cal FeaturesMajor feature is flaccid weaknessTendon reflexes depressedOphthalmoplegia may be presentMyalgias are uncommon重癥疾病性神經肌肉病(英文) 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

14、, with or without fibrillation potentials Absence of a decremental response on repetitive nerve stimulationDiagnosis重癥疾病性神經肌肉病(英文) 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 ser

15、um creatine kinase (CK） Demonstration of muscle inexcitability*For a definite diagnosis of critical illness myopathy, patients should have all of the first five features.重癥疾病性神經肌肉病(英文)Nerve conduction studiesLow-amplitude CMAPs Long duration CMAPsNormal SNAPsPhrenic nerve conduction normal latencies

16、 diaphragm CMAP amplitudes reduceElectrophysiologic Features重癥疾病性神經肌肉病(英文)EMGFibrillation potentials and positive sharpMotor unit potentials low amplitude and short durationElectrical inexcitability by direct needle stimulation重癥疾病性神經肌肉病(英文)Features of the histopathology in thick filament myosin los

17、s (Fig. 5)Electron microscopy reveals selective loss of thick (myosin) filaments (Fig. 6)Inflammatory changes are conspicuously absent Morphologic Features重癥疾病性神經肌肉病(英文) Figure. 5 Muscle histopathology in a critically ill patient with thick filament myosin loss. (original magnification, 100) (courte

18、sy of Dr. Andrew Engel).重癥疾病性神經肌肉病(英文) Figure. 6 Electron microscopy of muscle in CIM. (original magnification, 44,000) (courtesy of Dr. Andrew Engel).重癥疾病性神經肌肉病(英文)CIPDirect needle stimulation of the muscle Electrical inexcitability in CIMThere is a response in CIP (Fig. 7)Serum CKMuscle biopsy重癥疾病性神經肌肉病(英文)FIGURE. 7FIGURE. 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).重癥疾病性神經肌肉病(英文)Subtypes of CIMMuscle morph