Diagnosis and treatment of mitochondrial myopathies

Ann Med. 2013 Feb;45(1):4-16. doi: 10.3109/07853890.2011.605389. Epub 2011 Aug 25.

Abstract

Mitochondrial disorders are a heterogeneous group of disorders resulting from primary dysfunction of the respiratory chain. Muscle tissue is highly metabolically active, and therefore myopathy is a common element of the clinical presentation of these disorders, although this may be overshadowed by central neurological features. This review is aimed at a general medical and neurologist readership and provides a clinical approach to the recognition, investigation, and treatment of mitochondrial myopathies. Emphasis is placed on practical management considerations while including some recent updates in the field.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Biopsy
  • Cytochrome-c Oxidase Deficiency / complications
  • Deglutition Disorders / complications
  • Dietary Supplements
  • Endocrine System Diseases / complications
  • Endocrine System Diseases / drug therapy
  • Exercise Test
  • Exercise Therapy
  • Hearing Disorders / complications
  • Heart Diseases / complications
  • Heart Diseases / diagnosis
  • Heart Diseases / drug therapy
  • Humans
  • Mitochondrial Myopathies / complications
  • Mitochondrial Myopathies / diagnosis*
  • Mitochondrial Myopathies / enzymology
  • Mitochondrial Myopathies / therapy*
  • Muscle, Skeletal / enzymology
  • Muscle, Skeletal / pathology*
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / deficiency
  • Ubiquinone / therapeutic use
  • Vision Disorders / complications
  • Vitamins / therapeutic use

Substances

  • Vitamins
  • Ubiquinone
  • coenzyme Q10