Skeletal muscle disorders and associated factors that limit exercise performance.

S. F. Lewis, R. G. Haller

Research output: Contribution to journalArticle

41 Citations (Scopus)

Abstract

The study of skeletal muscle disorders is providing potentially important insights into regulatory mechanisms in human exercise and fatigue and information useful for diagnostic and treatment purposes. This review primarily concerned the general metabolic and physiological factors which set upper limits to performance of various types of exercise in patients with a variety of muscle disorders. From the standpoint of exercise performance, skeletal muscle diseases can be classified into three major groups. One group consists of primary disorders of muscle energy metabolism, including defects in muscle carbohydrate and lipid metabolism, disorders of mitochondrial electron transport, and abnormalities of purine nucleotide metabolism. Exercise performance largely reflects the capacity for ATP resynthesis. Oxidative phosphorylation is the dominant quantitative source of energy for ATP resynthesis under most exercise conditions. Consequently, patients with disordered oxidative metabolism (i.e., patients with defects in the availability or utilization of oxidizable substrate, such as those with phosphorylase or PFK deficiency or those with defects in mitochondrial electron transport) typically demonstrate severely impaired exercise performance. Intolerance to sustained exercise and premature fatigability are salient features of muscle oxidative disorders. Maximal oxygen uptake and maximal a-v O2 difference are markedly subnormal related to an attenuated muscle oxygen extraction. Muscle weakness and atrophy are less common. Anaerobic muscle performance is dramatically limited in patients with virtually complete defects of glycogenolysis/glycolysis but appears relatively normal in those with electron transport defects. A second major group of disorders includes patients with decreased muscle mass due to muscle necrosis, atrophy, and replacement of muscle by fat and connective tissue. These disorders are exemplified by the various muscular dystrophies (Duchenne's dystrophy, Becker's dystrophy, LG dystrophy, FSH dystrophy, and myotonic dystrophy) in which exercise performance is severely impaired due to muscle wasting and weakness in spite of largely normal pathways for muscle ATP resynthesis. In muscular dystrophy patients, the degree to which maximal oxygen uptake and anaerobic muscle performance are impaired appears to be a function of the severity of muscle weakness and atrophy. A third group of disorders includes patients with impaired activation of muscle contraction or relaxation. These disorders may be considered in two subcategories. In the first, impaired activation or relaxation of contractile activity is due to intrinsic muscle dysfunction (e.g., diseases associated with myotonia or periodic paralysis). In the second subcategory, there is impaired muscle activation due to a primary abnormality in the central nervous system, motor nerves, or neuromuscular junction.(ABSTRACT TRUNCATED AT 400 WORDS)

Original languageEnglish (US)
Pages (from-to)67-113
Number of pages47
JournalExercise and Sport Sciences Reviews
Volume17
StatePublished - 1989

Fingerprint

Muscular Diseases
Skeletal Muscle
Exercise
Muscles
Muscular Atrophy
Muscle Weakness
Electron Transport
Adenosine Triphosphate
Oxygen
Lipid Metabolism Disorders
Myotonia
Purine Nucleotides
Glycogenolysis
Phosphorylases
Myotonic Dystrophy
Muscle Relaxation
Duchenne Muscular Dystrophy
Muscular Dystrophies
Neuromuscular Junction
Oxidative Phosphorylation

ASJC Scopus subject areas

  • Endocrinology
  • Orthopedics and Sports Medicine
  • Public Health, Environmental and Occupational Health
  • Physical Therapy, Sports Therapy and Rehabilitation

Cite this

Skeletal muscle disorders and associated factors that limit exercise performance. / Lewis, S. F.; Haller, R. G.

In: Exercise and Sport Sciences Reviews, Vol. 17, 1989, p. 67-113.

Research output: Contribution to journalArticle

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