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Cerebrospinal Fluid Cyclic Nucleotide Alterations in the Lesch-Nyhan Syndrome

  • N. Lawrence Edwards
  • Michael V. Johnston
  • Faye S. Silverstein
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 253A)

Abstract

Attempts to explain the mechanisms responsible for the various neurologic manifestations of the Lesch-Nyhan syndrome have been hampered by an incomplete understanding of the neurochemical and purinergic derangements that exist in the central nervous systems of these boys. We have serially monitored cerebrospinal fluid (CSF) in 4 subjects with the Lesch-Nyhan syndrome to investigate the correlation of clinical neurologic manifestations with either excesses or deficiencies of monoamine transmitter or purine metabolites. We have previously reported our findings of altered monoamine metabolites in the Lesch-Nyhan spinal fluids (1) as well as the correlation of these abnormalities with elevations of purine bases in the CSF (2). Other spinal fluid studies in these patients addressed the effect of allopurinol therapy on spinal fluid levels of hypoxanthine and xanthine (3). We have extended our inquiry into the abnormalities of Lesch-Nyhan spinal fluid by measuring cyclic nucleotide (cyclic AMP and cyclic GMP) levels.

Keywords

Cyclic Nucleotide Spinal Fluid Level Cyclic Nucleotide Level Purine Metabolite HPRT Deficiency 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Plenum Press, New York 1989

Authors and Affiliations

  • N. Lawrence Edwards
    • 1
    • 2
  • Michael V. Johnston
    • 3
  • Faye S. Silverstein
    • 3
  1. 1.Department of MedicineUniversity of FloridaGainesvilleUSA
  2. 2.The Veterans Administration Medical CenterGainesvilleUSA
  3. 3.Departments of Neurology and PediatricsUniversity of MichiganAnn ArborUSA

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