Disorders of Glycogen Metabolism

  • Salvatore DiMauro
  • Darryl C. De Vivo


The concentration of glycogen in the central nervous system (CNS) is approximately 0.1 g/100 g fresh tissue as compared to 1.0 g/100 g in muscle and 6–10 g/100 g in liver. Because of this low content and its rapid disappearance after death, the very presence of glycogen in the brain remained controversial until the late 1930s, when Kerr documented the presence of a polysaccharide indistinguishable from liver glycogen.1 Major fluctuations of tissue glycogen content are accompanied by even greater changes of water content (and volume).2 The advantages afforded the brain by the protection of the bony skull and the fibrous dura mater are attenuated by the metabolic vulnerability of the brain. The low brain tissue glycogen content renders this organ vulnerable to insult within several minutes of onset of hypoglycemia or hypoxia.


Glycogen Storage Disease Pompe Disease Glycogen Metabolism Neuronal Perikaryon Lafora Disease 
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Copyright information

© Plenum Press, New York 1985

Authors and Affiliations

  • Salvatore DiMauro
    • 1
  • Darryl C. De Vivo
    • 1
  1. 1.Department of Neurology, and Division of Pediatric NeurologyColumbia University College of Physicians and SurgeonsNew YorkUSA

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