Amyotrophic Lateral Sclerosis (ALS)
Background
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Progressive degenerative disease of upper & lower motor neurons leading to denervation & atrophy of skeletal muscle with resultant weakness & eventual death
Considerations
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Aspiration risk due to bulbar dysfunction
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Perioperative respiratory failure due to weakness & aspiration
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Autonomic dysfunction
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Altered response to neuromuscular blockers:
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Succinylcholine contraindicated due to hyperkalemia risk
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NdMR (nondepolarizing muscle relaxant) sensitivity
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Chronic pain (often girdle area)
Goals
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Minimize aspiration risk (prophylaxis, consider RSI)
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Maximize respiratory function (muscle strength, secretion & pain management)
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Anticipate potential need for postop ventilation/ICU
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Maintain stable hemodynamics
Conflicts
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RSI vs. avoid succinylcholine/sensitive to NdMRs
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RSI vs. maintain hemodynamic stability
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Regional vs. existing neurologic deficits:
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Epidural documented in case reports but be cautious of high block
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Peripheral nerve blocks not documented because disease so rare but definitely a consideration given respiratory muscle weakness
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Avoid interscalene so as to not compromise phrenic nerve
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General anesthetic vs. respiratory depression (neuraxial is safe)