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Monoaminergic effects of high‐dose corticotropin in corticotropin‐responsive pediatric opsoclonus‐myoclonus
Author(s) -
Pranzatelli Michael R.,
Huang YungYu,
Tate Elizabeth,
Goldstein David S.,
Holmes Courtney S.,
Goldstein Edward M.,
Ketner Karen,
Kinast Morris,
Lange Bernadette M.,
Sanz Alfred,
Shevell Michael I.,
Stanford Richard E.,
Taff Ingrid P.
Publication year - 1998
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870130323
Subject(s) - homovanillic acid , monoaminergic , medicine , endocrinology , adrenocorticotropic hormone , serotonergic , cerebrospinal fluid , serotonin , norepinephrine , dopamine , dopaminergic , catecholamine , chemistry , hormone , receptor
Children with the opsoclonus‐myoclonus syndrome (OMS) usually respond to corticotropin (adrenocorticotrophic hormone, ACTH) treatment but the mechanism of benefit is unknown. We previously showed that both cerebrospinal fluid (CSF) homovanillic acid (HVA) and 5‐hydroxyindoleacetic acid (5‐HIAA) concentrations are low in pediatric OMS. In this study, we measured levels of CSF Dopa, catecholamines, deaminated metabolites of catecholamines, as well as HVA and 5‐HIAA in eight patients before and during treatment with ACTH. All the children were ACTH‐responsive with 50‐70% improvement in multiple clinical features of OMS. ACTH treatment reduced the HVA concentration in every child by a mean of 21% (p < 0.001). Treatment with ACTH was associated with significant correlations between dopaminergic markers such as HVA, dihydroxyphenylacetic acid (DOPAC), and Dopa. There were no significant changes in the CSF concentrations of the noradrenergic markers norepinephrine (NE) and dihydroxyphenylglycol (DHPG), or the serotonergic marker 5‐HIAA. The only child with a marked inflammatory pattern in CSF, which was reversed by ACTH, was atypical for a large increase in NE and decrease in 5‐HIAA levels. The pattern of decreased HVA and unchanged DOPAC levels could reflect decreased extraneuronal uptake of catecholamines (which steroids inhibit) or decreased 0‐methylation of catecholamines in nonneuronal cells.