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-hydroxyindole-acetic 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 during ACTH treatment. Beneficial effects of ACTH in OMS are not associated with normalization of HVA or 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.
Subject(s)
Adrenocorticotropic Hormone/administration & dosage , Myoclonus/drug therapy , Neurotransmitter Agents/cerebrospinal fluid , Ocular Motility Disorders/drug therapy , 3,4-Dihydroxyphenylacetic Acid/cerebrospinal fluid , Catecholamines/cerebrospinal fluid , Child, Preschool , Dihydroxyphenylalanine/cerebrospinal fluid , Female , Homovanillic Acid/cerebrospinal fluid , Humans , Hydroxyindoleacetic Acid/cerebrospinal fluid , Infant , Male , Myoclonus/cerebrospinal fluid , Ocular Motility Disorders/cerebrospinal fluid , Reference ValuesABSTRACT
The authors report a 7-year-old girl who developed neck pain and stiffness over a four-day period. There was no fever, trauma, systemic illness or headache. Physical examination demonstrated subtle neurologic deficits indicative of cervical cord compression. CAT scan and subsequent Magnetic Resonance Imaging (MRI) of the cervical spine demonstrated a spinal epidural hematoma, which was evacuated surgically. Post-operative angiography failed to demonstrate a vascular abnormality. The child recovered without neurologic deficit. MRI proved to be a sensitive tool in identifying the nature and extent of this lesion, and may be considered in lieu of myelography.
