Critical Care Management of Motor Neuron Disease


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Critical Care Management of Motor Neuron Disease

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