title: Effect of Increased Immunosuppression on Developmental Outcome of Opsoclonus Myoclonus Syndrome (OMS).
abstract: Opsoclonus myoclonus syndrome (OMS) produces long-term cognitive, behavioral, and motor deficits. Objective was to see if more aggressive treatment improved outcome. Assessment included opsoclonus myoclonus syndrome rating, developmental/cognitive and motor assessment, and adaptive behavior. Fourteen subjects completed testing. Nine had neuroblastoma. Onset was at 10 to 35 months; onset to diagnosis: 2 days to 14 months, and onset to first treatment: 5 days to 15 months. Initial treatment was corticotropin (12), oral steroids (3), plus intravenous immunoglobulin in all. Ten received rituximab, 5 cyclophosphamide. Age at testing ranged from 2.5 to 10.3 years. Adaptive Behavior Score (11 subjects), mean 93.5; estimated Intelligence Quotient/Developmental Quotient mean 93.5; Motor: mean 92.8. Residual opsoclonus myoclonus syndrome symptoms at the time of the evaluation were generally minor; opsoclonus myoclonus syndrome scores ranged from 0 to 6. Comparison to previously reported opsoclonus myoclonus syndrome subjects showed improved outcomes: Adaptive behavior, cognitive and motor scores were significantly higher (P < .001) in new subjects. Outcomes have improved with more aggressive immunosuppression, with most opsoclonus myoclonus syndrome survivors now functioning at or near normal.
authors.affiliation: Neurology Division, Children's Hospital Los Angeles, Los Angeles, CA, USA Department of Neurology, Keck School of Medicine of University of Southern California, Los Angeles, CA, USA wmitchell@chla.usc.edu.
authors.forename: Wendy G
authors.initials: WG
authors.lastname: Mitchell
authors.affiliation: Neurology Division, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Amelia A
authors.initials: AA
authors.lastname: Wooten
authors.affiliation: Clinical Translational Science Institute, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Sharon H
authors.initials: SH
authors.lastname: O'Neil
authors.affiliation: Neurology Division, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Jenny G
authors.initials: JG
authors.lastname: Rodriguez
authors.affiliation: Neurology Division, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Rosa E
authors.initials: RE
authors.lastname: Cruz
authors.affiliation: Clinical Translational Science Institute, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Rachael
authors.initials: R
authors.lastname: Wittern
mesh_terms: D000223:Adaptation, Psychological / Q000187:drug effects; D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D017668:Age of Onset; D003072:Cognition Disorders / Q000209:etiology / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007165:Immunosuppression Therapy / Q000379:methods*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D009069:Movement Disorders / Q000209:etiology / Q000628:therapy; D009460:Neurologic Examination; D053578:Opsoclonus-Myoclonus Syndrome / Q000150:complications / Q000628:therapy*; D012720:Severity of Illness Index; D013256:Steroids / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007136:Immunoglobulins; D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide; D000324:Adrenocorticotropic Hormone
keywords: ACTH; cognitive development; corticotropin; immunosuppression; neuroblastoma; opsoclonus myoclonus syndrome; outcome; rituximab