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Lesch-Nyhan syndrome: CSF neurotransmitter abnormalities.

Abstract
Serial determinations of spinal fluid homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) were made in four patients with the Lesch-Nyhan syndrome over a 5-year period. Control spinal fluids for age-matched comparison were obtained from 194 neurologic and nonneurologic pediatric patients. A rapid decline in control spinal fluid HVA and 5-HIAA occurs over the first 3 years of life (50 and 60%, respectively), and a more gradual decline persists throughout adolescence. The Lesch-Nyhan subjects have similar age-related changes in their spinal fluid neurotransmitter levels. Sequential 5-HIAA determinations from the four Lesch-Nyhan boys fall within the control range. The Lesch-Nyhan HVA levels are lower than the mean value for the age-matched control group in 18 of 19 samples. Ten of 19 determinations fell below the control range. Our findings provide evidence for altered CNS dopamine metabolism in the Lesch-Nyhan syndrome.
AuthorsF S Silverstein, M V Johnston, R J Hutchinson, N L Edwards
JournalNeurology (Neurology) Vol. 35 Issue 6 Pg. 907-11 (Jun 1985) ISSN: 0028-3878 [Print] United States
PMID2582310 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Phenylacetates
  • Serotonin
  • Hydroxyindoleacetic Acid
  • Dopamine
  • Homovanillic Acid
Topics
  • Adolescent
  • Child
  • Child, Preschool
  • Chromatography, High Pressure Liquid
  • Dopamine (metabolism)
  • Homovanillic Acid (cerebrospinal fluid)
  • Humans
  • Hydroxyindoleacetic Acid (cerebrospinal fluid)
  • Infant
  • Lesch-Nyhan Syndrome (cerebrospinal fluid)
  • Male
  • Phenylacetates (cerebrospinal fluid)
  • Serotonin (metabolism)
  • Spinal Puncture

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