Anesthetic implications of Leigh's syndrome

Paediatric Anaesthesia
Torin Shear, Joseph D Tobias

Abstract

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.

References

May 1, 1975·Anesthesia and Analgesia·E MelmanJ Marrufo
Apr 1, 1990·Journal of Child Neurology·P J Grattan-SmithK J Collins
Jul 1, 1989·Canadian Journal of Anaesthesia = Journal Canadien D'anesthésie·U T LarsenB de Fine Olivarius
Jun 1, 1988·Pediatric Emergency Care·R C Woody, S M Laney
Nov 1, 1994·Movement Disorders : Official Journal of the Movement Disorder Society·G LeraC D Marsden
Mar 1, 1996·Annals of Neurology·S RahmanD R Thorburn
Mar 1, 1996·British Journal of Anaesthesia·A R WolfF W Davies
Dec 4, 1998·Paediatric Anaesthesia·R J Bray
Jan 13, 2000·Paediatric Anaesthesia·J D Tobias
May 18, 2001·Current Opinion in Cardiology·M HiranoS DiMauro
Aug 1, 1951·Journal of Neurology, Neurosurgery, and Psychiatry·D LEIGH

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Citations

Jul 4, 2012·Saudi Journal of Anaesthesia·Abdullah Sulieman TerkawiJoseph D Tobias
Feb 17, 2021·Paediatric Anaesthesia·Stephanie CungMelissa M Masaracchia

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