Effect of Increased Immunosuppression on Developmental Outcome of Opsoclonus Myoclonus Syndrome (OMS)

Wendy G Mitchell; Amelia A Wooten; Sharon H O'Neil; Jenny G Rodriguez; Rosa E Cruz; Rachael Wittern

doi:10.1177/0883073814549581

Effect of Increased Immunosuppression on Developmental Outcome of Opsoclonus Myoclonus Syndrome (OMS)

J Child Neurol. 2015 Jul;30(8):976-82. doi: 10.1177/0883073814549581. Epub 2014 Oct 23.

Authors

Wendy G Mitchell¹, Amelia A Wooten², Sharon H O'Neil³, Jenny G Rodriguez², Rosa E Cruz², Rachael Wittern³

Affiliations

¹ 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.
² Neurology Division, Children's Hospital Los Angeles, Los Angeles, CA, USA.
³ Clinical Translational Science Institute, Children's Hospital Los Angeles, Los Angeles, CA, USA.

PMID: 25342308
DOI: 10.1177/0883073814549581

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.

Keywords: ACTH; cognitive development; corticotropin; immunosuppression; neuroblastoma; opsoclonus myoclonus syndrome; outcome; rituximab.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adaptation, Psychological / drug effects
Adolescent
Adrenocorticotropic Hormone / therapeutic use
Age of Onset
Cognition Disorders / etiology
Cognition Disorders / therapy
Cyclophosphamide / therapeutic use
Female
Follow-Up Studies
Humans
Immunoglobulins / therapeutic use
Immunosuppression Therapy / methods*
Immunosuppressive Agents / therapeutic use*
Male
Movement Disorders / etiology
Movement Disorders / therapy
Neurologic Examination
Opsoclonus-Myoclonus Syndrome / complications
Opsoclonus-Myoclonus Syndrome / therapy*
Severity of Illness Index
Steroids / therapeutic use
Time Factors
Treatment Outcome*

Substances

Immunoglobulins
Immunosuppressive Agents
Steroids
Cyclophosphamide
Adrenocorticotropic Hormone