Movement Disorders (revue)

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Monoaminergic effects of high‐dose corticotropin in corticotropin‐responsive pediatric opsoclonus‐myoclonus

Identifieur interne : 004F54 ( Main/Exploration ); précédent : 004F53; suivant : 004F55

Monoaminergic effects of high‐dose corticotropin in corticotropin‐responsive pediatric opsoclonus‐myoclonus

Auteurs : Michael R. Pranzatelli [États-Unis] ; Yung-Yu Huang [États-Unis] ; Elizabeth Tate [États-Unis] ; David S. Goldstein [États-Unis] ; Courtney S. Holmes [États-Unis] ; Edward M. Goldstein [États-Unis] ; Karen Ketner [États-Unis] ; Morris Kinast [États-Unis] ; Bernadette M. Lange [États-Unis] ; Alfred Sanz [États-Unis] ; Michael I. Shevell ; Richard E. Stanford [États-Unis] ; Ingrid P. Taff [États-Unis]

Source :

RBID : ISTEX:F14D1F55132B7EAB82E8639682DFAC9E710628D7

Descripteurs français

English descriptors

Abstract

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.

Url:
DOI: 10.1002/mds.870130323


Affiliations:


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Le document en format XML

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<term>Adenohypophyseal hormone</term>
<term>Adrenocorticotropic Hormone (administration & dosage)</term>
<term>CSF 5‐HIAA</term>
<term>Catecholamine</term>
<term>Catecholamines (cerebrospinal fluid)</term>
<term>Cerebrospinal fluid</term>
<term>Chemotherapy</term>
<term>Child</term>
<term>Child, Preschool</term>
<term>Corticotropin</term>
<term>Dihydroxyphenylalanine (cerebrospinal fluid)</term>
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<term>High dose</term>
<term>Homovanillic Acid (cerebrospinal fluid)</term>
<term>Humans</term>
<term>Hydroxyindoleacetic Acid (cerebrospinal fluid)</term>
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<term>Male</term>
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<term>Myoclonus (cerebrospinal fluid)</term>
<term>Myoclonus (drug therapy)</term>
<term>Neurotransmitter Agents (cerebrospinal fluid)</term>
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<term>Ocular Motility Disorders (cerebrospinal fluid)</term>
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<term>Opsoclonus</term>
<term>Paraneoplastic</term>
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<term>Hormone adénohypophysaire</term>
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<div type="abstract" xml:lang="en">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.</div>
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