Metabolic Disorders Affecting the Nervous System

  • Roy O. Weller
  • Michael Swash
  • D. Lindsay McLellan
  • Carl L. Scholtz


Inborn errors of metabolism make a considerable contribution to the morbidity and mortality of children in a Western population (Raine 1974), since there are so many different disorders, albeit each with a low incidence. Inherited biochemical defects have been reported for many groups of compounds including carbohydrates, amino acids, purines and pyrimidines, trace metals, lipids and mucopolysaccharides. The onset of clinical symptoms may not become apparent until adult life, but this is unusual, the majority appearing during childhood and frequently in the first few days of life.


Branch Chain Amino Acid Congenital Hypothyroidism Maple Syrup Urine Disease Urea Cycle Disorder Neonatal Hypothyroidism 
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Copyright information

© Springer-Verlag Berlin Heidelberg 1983

Authors and Affiliations

  • Roy O. Weller
    • 1
    • 2
  • Michael Swash
    • 3
    • 4
  • D. Lindsay McLellan
    • 1
    • 2
  • Carl L. Scholtz
    • 5
    • 6
  1. 1.University of SouthamptonEngland
  2. 2.Wessex Regional Neurological CentreSouthamptonEngland
  3. 3.The London Hospital and St Mark’s HospitalLondonEngland
  4. 4.the London Hospital Medical CollegeLondonEngland
  5. 5.The London Hospital Medical CollegeEngland
  6. 6.The London HospitalLondonEngland

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