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.
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Authors | F S Silverstein, M V Johnston, R J Hutchinson, N L Edwards |
Journal | Neurology
(Neurology)
Vol. 35
Issue 6
Pg. 907-11
(Jun 1985)
ISSN: 0028-3878 [Print] United States |
PMID | 2582310
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- Phenylacetates
- Serotonin
- Hydroxyindoleacetic Acid
- Dopamine
- Homovanillic Acid
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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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