TY - CHAP M1 - Book, Section TI - Leigh Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - It is a severe progressive mitochondrial disorder impeding oxidative phosphorylation with leads to necrotizing encephalopathy between the age of 3 months and 2 years. It has also been reported that the onset begins later during childhood. It causes brain, spinal cord, and optic nerves degeneration and clinically is characterized by acquired motor skills, muscle weakness and hypotonia, lactic acidosis associated with respiratory and kidney dysfunctions. The association with hypertrophic cardiomyopathy has been reported and represents a major anesthesia consideration. Although malignant hyperthermia (MH) has not been reported with Leigh disease, it may be wise to avoid triggering agents as other myopathic conditions with a propensity for MH have been misdiagnosed in the past in the presence of muscular disorders. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/10 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164076517 ER -