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.