What is the best course of action for a patient with complex I deficiency and Leigh-like syndrome who experiences acute worsening of choreoathetosis after anesthesia?

Management of Acute Ballism Exacerbation in Complex I Deficiency and Leigh-like Syndrome Post-Anesthesia

For patients with complex I deficiency and Leigh-like syndrome experiencing acute worsening of choreoathetosis (ballism) after anesthesia, immediate supportive care with close monitoring of respiratory function and metabolic status is essential, as postoperative inflammatory mediators may trigger mitochondrial decompensation regardless of anesthetic agents used. 1

Immediate Management

• Initiate close monitoring in an intensive care setting to detect early signs of respiratory failure or metabolic acidosis, which can develop within 24 hours post-anesthesia 1
• Start lactate-free IV fluids (5% dextrose-0.9% saline) to maintain normoglycemia and prevent lactic acidosis exacerbation 1
• Monitor plasma lactate levels frequently to detect metabolic decompensation 1
• Assess respiratory function continuously, as respiratory failure is a common complication in Leigh syndrome patients after anesthesia 1

Management of Movement Disorder Exacerbation

• Avoid sedatives if possible in early stages of neurological deterioration to allow better neurological assessment 1
• If seizure activity is suspected or present, treat with phenytoin and low-dose benzodiazepines 1
• Consider neurology consultation for management of acute movement disorder exacerbation 1
• Evaluate for signs of intracranial hypertension, which may accompany metabolic decompensation 1

Metabolic Support

• Maintain normoglycemia to prevent additional metabolic stress 1
• Avoid prolonged fasting periods which can exacerbate mitochondrial dysfunction 1
• Consider supplemental oxygen to optimize tissue oxygenation and reduce metabolic demands 1
• Monitor electrolytes and acid-base status closely, correcting abnormalities promptly 1

Prevention of Further Complications

• Avoid medications that may worsen mitochondrial function, particularly prolonged use of propofol 1
• Use neuromuscular blocking agents judiciously if required 1
• Consider the possibility that inflammatory mediators from surgical stress may be contributing to mitochondrial decompensation rather than specific anesthetic agents 1

Special Considerations

• Patients with Leigh syndrome, particularly those with complex I deficiency, are at higher risk for post-anesthetic complications including respiratory failure and metabolic acidosis 1
• The temporal relationship between anesthesia and clinical deterioration is well-documented, with symptoms typically appearing within 24 hours after an apparently uneventful anesthetic recovery 1
• Patients with pre-existing respiratory abnormalities appear to be at particularly high risk for post-anesthetic complications 1
• The postoperative inflammatory response may inhibit the mitochondrial electron transport chain, potentially leading to critical mitochondrial failure, particularly in the central nervous system 1

Long-term Considerations

• For future anesthetic needs, there is no evidence to avoid any particular anesthetic agent, but careful individualized assessment is crucial 1
• Meticulous attention to fluid management is essential in any future surgical procedures 1
• Consider genetic testing to identify specific mutations (such as in NDUFS4, NDUFS8) which may help guide management 2, 3, 4
• Complex I deficiency is frequently associated with Leigh syndrome and may present with predominant brainstem involvement, which can affect respiratory control 2, 5