This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. To evaluate the possible role of brain amino acid neurotransmitter derangements in pediatric opsoclonus-myoclonus syndrome (OMS), 23 cerebrospinal fluid (CSF) amino compounds were measured by amino acid analyzer or mass spectroscopy in 45 children with OMS and 35 age-matched controls. In untreated OMS, CSF aspartic acid, ornithine, and valine were decreased and phosphoethanolamine was increased compared to controls. Corticotropin (ACTH) treatment was associated with increased alanine, glutamine, lysine, and threonine, and decreased taurine compared both to controls and to untreated OMS. These effects were not found with corticosteroid treatment. In ACTH-treated OMS, aspartic acid and valine remained below control levels. Both OMS severity and duration had significant effects on alanine, arginine, aspartic acid, lysine, phosphoethanolamine, and valine. There were no significant changes in CSF glycine, GABA, or other amino acids. These data suggest central amino acid abnormalities in OMS and differential effects of ACTH and corticosteroid treatment. The amino acid perturbations related to OMS severity and duration may be the most biologically relevant.
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