1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-2-22-a 10.15844/pedneurbriefs-2-3-10 Degenerative and Metabolic Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Monoamine Metabolites in Rett Syndrome http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 03 1988 01 08 2016 2 3 22 22 Copyright: © 1988 The Author(s) 1988 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Cerebrospinal fluid values for monoamine metabolites, gamma-aminobutyric acid, and other amino compounds in Rett syndrome

Cerebral metabolites of noradrenaline, dopamine and serotonin, y-aminobutyric acid, and 23 amino acids were present in normal concentrations in the CSF of 5 girls with Rett syndrome studied in the Depts of Pharmacology and Therapeutics, and Dept of Paediatrics, University of British Columbia, Vancouver, Canada.

 Cerebral Metabolites Y-Aminobutyric Acid Rett Syndrome

Cerebral metabolites of noradrenaline, dopamine and serotonin, y-aminobutyric acid, and 23 amino acids were present in normal concentrations in the CSF of 5 girls with Rett syndrome studied in the Depts of Pharmacology and Therapeutics, and Dept of Paediatrics, University of British Columbia, Vancouver, Canada. The authors doubt that any biochemical abnormalities have been clearly established as characteristic of the syndrome. [1]

COMMENT. A previous report of low CSF levels of monoamine metabolites in patients with Rett syndrome [2] is not supported by the present study. Hyperammonemia reported originally by Rett is another suggested biochemical basis for the syndrome unconfirmed in subsequent reports. The lack of uniformity of these findings suggests that Rett syndrome is a nonspecific entity with more than one etiology.

 Perry TL Dunn HG Ho HH Crichton JU Cerebrospinal fluid values for monoamine metabolites, gamma-aminobutyric acid, and other amino compounds in Rett syndrome J Pediatr 1988 Feb 112 2 234 8 10.1016/S0022-3476(88)80060-X 2448442 Zoghbi HY Percy AK Glaze DG Butler IJ Riccardi VM Reduction of biogenic amine levels in the Rett syndrome N Engl J Med 1985 Oct 10 313 15 921 4 10.1056/NEJM198510103131504 2412119