Lesch-nyhan syndrome: CSF neurotransmitter abnormalities

Faye S. Silverstein, Michael V. Johnston, Raymond J. Hutchinson, N. Lawrence Edwards

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)907-911
Number of pages5
JournalNeurology
Volume35
Issue number6
DOIs
StatePublished - Jun 1985
Externally publishedYes

ASJC Scopus subject areas

  • Clinical Neurology

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