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Critical Care Management of Motor Neuron Disease
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Critical Care Management of Motor Neuron Disease

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    • 1. Critical Care Management of Motor Neuron Disease PJ Papadakos MD FCCM Director CCM Professor Anesthesiology, Surgery and Neurosurgery Rochester NY USA
    • 2. June 8, 2009
    • 3. June 8, 2009
    • 4. ICU-acquired Weakness June 8, 2009
    • 5. Basic Anatomy
    • 6.  
    • 7.  
    • 8. Pathogenesis? June 8, 2009 Axonopathy UMN lesion Neuropathy NMJ Myopathy
    • 9. Critical Iliness Neuropathy
    • 10. Incidence Rate
      • ~2% of mechanically ventilated patients
      • ~10% of mechanical ventilation  7 days
      • ~60-85% of patients with severe sepsis
    • 11. Definition
      • Critical illness polyneuropathy (CIP)
        • Acute axonal sensory-motor polyneuropathy
        • Lower limb of critically ill patients
        • Gold diagnostic standard: Electroneurography
    • 12. Critical illness myopathy and neuropathy Current Opinion in Critical Care 2005; 11:126-132 Fig. Major electroneurographic features in axonal and demyelinating neuropathy
    • 13.
      • Critical illness myopathy (CIM)
        • Acute primary myopathy
        • Spectrum from pure functional impairment with normal histology to muscle atrophy and necrosis
        • Diagnosis: Electrophysiological investigation or muscle biopsy
    • 14. Short-term Outcome
      • Approach to the comatose patient
      • Acute neuromuscular respiratory failure
    • 15. Approach to the comatose patient
      • GCS– motor reflex
      • Disappearance of motor reflexes  Extensive CNS damage  Inappropriate pessimistic prognoses
      • Predicted outcome alters treatment
    • 16. Acute neuromuscular respiratory failure
      • Difficult weaning from ventilator
      • Duration of weaning from mechanical ventilation is 2-7 times greater in patients with CIP
        • De Jonghe et al. : 6 days vs 3 days
        • Garnacho-Montero et al. : 15 days vs 2 days
    • 17. Long-term Outcome
      • 263 patients, mean duration of follow-up: 3-6 months (2 days to 8 years)
      • Profound muscle weakness
        • Improvement: upper limbs and proximal lower limbs  respiratory system  distal lower limbs
        • CIP is a lesion of terminal motor axons
    • 18.
      • Complete functional recovery: 68.4% (180/263)
        • Regaining the ability to breathe spontaneously and to walk independently
      • Severe disability: 28.1% (74/263)
        • Tetraparesis, tetraplegia, or paraplegia
    • 19.
      • Persisting milder disabilities
        • Reduced or absent DTR
        • Stocking and glove sensory loss
        • Muscle atrophy
        • Painful hyperesthesia
        • Foot drop
    • 20. Prevention and Treatment
      • Damage muscle
        • Several electrolyte abnormalities
          • Hypokalemia
          • Hyperkalemia
          • Hypophosphatemia
    • 21.
      • Damage muscle
        • Propofol: propofol infusion syndrome (when >5 mg/kg/h for >48 hrs)
          • Severe metabolic acidosis
          • Rhabdomyolysis
          • Renal failure
          • Fatal cardiac failure
        • Catecholamines
        • Chronic corticosteroid use
    • 22.
      • No specific treatments exist for CIP and CIM
    • 23. Conclusion
      • Only limited evidence is available suggesting that CIP increases ICU and hospital mortality in critically ill patients.
      • Data are insufficient to demonstrate any association with long-term mortality.
    • 24. Spinal Trauma
    • 25.