Anesthetic implications of Leigh's syndrome

Leigh's syndrome (LS) is a mitochondrial disorder characterized by progressive neurodegenerative changes with loss of developmental milestones, abnormalities of central control of respiration, and metabolic derangements. The primary genetic defect involves the respiratory chain complex and pyruvate dehydrogenase complex resulting in abnormal mitochondrial function and defective oxidative phosphorylation. While most patients have respiratory and neurological impairment, involvement of the cardiovascular and musculoskeletal systems may also occur. The authors present the use of spinal anesthesia for muscle biopsy in a 19 month old with LS and review the potential anesthetic implications of this syndrome.