Amyotrophic Lateral Sclerosis (ALS) 

 

 

Background

 

  • Progressive degenerative disease of upper & lower motor neurons leading to denervation & atrophy of skeletal muscle with resultant weakness & eventual death

 

 

Considerations 

 

  • Aspiration risk due to bulbar dysfunction

  • Perioperative respiratory failure due to weakness & aspiration 

  • Autonomic dysfunction

  • Altered response to neuromuscular blockers:

    • Succinylcholine contraindicated due to hyperkalemia risk

    • NdMR (nondepolarizing muscle relaxant) sensitivity

  • Chronic pain (often girdle area)

 

 

Goals 

 

  • Minimize aspiration risk (prophylaxis, consider RSI)

  • Maximize respiratory function (muscle strength, secretion & pain management)

  • Anticipate potential need for postop ventilation/ICU

  • Maintain stable hemodynamics

 

 

Conflicts 

 

  • RSI vs. avoid succinylcholine/sensitive to NdMRs

  • RSI vs. maintain hemodynamic stability

  • Regional vs. existing neurologic deficits:

    • Epidural documented in case reports but be cautious of high block 

    • Peripheral nerve blocks not documented because disease so rare but definitely a consideration given respiratory muscle weakness

      • Avoid interscalene so as to not compromise phrenic nerve

  • General anesthetic vs. respiratory depression (neuraxial is safe)

 

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