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Neuropediatrics 2008; 39(3): 164-171
DOI: 10.1055/s-0028-1085466
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Neurometabolic Effects of ACTH on Free Amino Compounds in Opsoclonus-myoclonus Syndrome

M. R. Pranzatelli 1 , E. D. Tate 1 , J. M. Crowley 2 , B. Toennies 3 , M. Creer 3 , 4
  • 1Department of Neurology, Division of Child and Adolescent Neurology, Southern Illinois University School of Medicine, Springfield, Illinois, USA
  • 2Department of Internal Medicine, Division of Endocrinology, Diabetics and Metabolism, Mass Spectrometry Resource, Washington University, St. Louis, Missouri, USA
  • 3Biochemical Genetics Diagnostic Laboratory, Cardinal Glennon Children's Medical Center, St. Louis, Missouri, USA
  • 4Department of Pathology, St. Louis University Hospital, St. Louis, Missouri, USA
Weitere Informationen

Publikationsverlauf

received 06.03.2008 revised 13.06.2008

accepted 07.07.2008

Publikationsdatum:
07. November 2008 (online)

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Abstract

To evaluate the possible role of central free amino compounds in pediatric opsoclonus-myoclonus syndrome (OMS), 21 cerebrospinal fluid (CSF) amino compounds were measured by an amino acid analyzer or mass spectroscopy in 74 anesthetized children, 54 with OMS and 20 age-matched neurological controls. In OMS, only phosphoethanolamine was increased compared to controls; OMS severity and duration had significant converse effects on alanine and phosphoethanolamine. In contrast, corticotropin (ACTH) treatment was associated with increased alanine and phenylalanine, and decreased taurine compared to controls and untreated OMS, and increased glutamine, lysine, ornithine, and tyrosine compared to untreated OMS. Other than low taurine, these effects were not found with corticosteroid treatment, and non-steroidogenic immunotherapy had no effect. The ACTH dose-association was most apparent for alanine and phosphoethanolamine, but lysine and ornithine were also higher in the high-dose ACTH group. There were no significant disease- or treatment-associated perturbations in GABA, glycine, or other amino acids. These data suggest a unique pattern of ACTH effects on non-neurotransmitter CSF amino compounds, for the most part not shared by steroids.

Key words

paraneoplastic syndrome - immunotherapy - ataxia - dancing eyes - ACTH - neuroblastoma

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Correspondence

Prof. M. R. Pranzatelli

National Pediatric Myoclonus Center

Southern Illinois University School of Medicine

PO Box 19643

Springfield

IL 62794-9643

USA

Telefon: +1/217/545 76 35

Fax: +1/217/545 19 03

eMail: mpranzatelli@siumed.edu