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.
Get full access to this article
View all access and purchase options for this article.
References
1. Kinsbourne M. Myoclonic encephalopathy of infants. J Neurol Neurosurg Psychiatr. 1962;25:271–276.
2. Brunklaus A, Pohl K, Zuberi SM, et al. Investigating neuroblastoma in childhood opsoclonus-myoclonus syndrome. Arch Dis Child. 2012;97:461–463.
3. Papero P, Pranzatelli MR, Margolis LJ, Tate E, Wilson LA, Glass P. Neurobehavioral and psychosocial functioning of children with opsoclonus-myoclonus syndrome. Dev Med Child Neurol. 1995;37:915–932.
4. Pohl KR, Pritchard J, Wilson J. Neurological sequelae of the dancing eye syndrome. Eur J Pediatr. 1996;155:237–244.
5. Russo C, Cohn SL, Petruzzi MJ, de Alarcon PA. Long-term neurologic outcome in children with opsoclonus-myoclonus associated with neuroblastoma: a report from the Pediatric Oncology Group. Med Pediatr Oncol. 1997;28:284–288.
6. Rudnick E, Khakoo Y, Antunes NL, et al. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: clinical outcome and antineuronal antibodies—a report from the Children’s Cancer Group Study. Med Pediatr Oncol. 2001;36:612–622.
7. Mitchell WG, Davalos-Gonzalez Y, Brumm VL, et al. Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae. Pediatrics. 2002;109:86–98.
8. Mitchell WG, Brumm VL, Azen CG, Patterson KE, Aller SK, Rodriguez J. Longitudinal neurodevelopmental evaluation of children with opsoclonus-ataxia. Pediatrics. 2005;116:901–907.
9. Klein A, Schmitt B, Boltshauser E. Long-term outcome of ten children with opsoclonus-myoclonus syndrome. Eur J Pediatr. 2007;166:359–363.
10. De Grandis E, Parodi S, Conte M, et al. Long-term follow-up of neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome. Neuropediatrics. 2009;40:103–111.
11. Kaulfersch W, Urban C, Fritsch G, Maurer G. Myoclonic encephalopathy (Kinsbourne syndrome) [in German]. Padiatr Padol. 1984;19:279–285.
12. Turkel SB, Brumm VL, Mitchell WG, Tavare CJ. Mood and behavioral dysfunction with opsoclonus-myoclonus ataxia. J Neuropsychiatr Clin Neurosci. 2006;18:239–241.
13. Altman AJ, Baehner RL. Favorable prognosis for survival in children with coincident opso-myoclonus and neuroblastoma. Cancer. 1976;37:846–852.
14. Borgna-Pignatti C, Balter R, Marradi P, Colamaria V. Treatment with intravenously administered immunoglobulins of the neuroblastoma-associated opsoclonus-myoclonus. J Pediatr. 1996;129:179–180.
15. Matthay KK, Blaes F, Hero B, et al. Opsoclonus myoclonus syndrome in neuroblastoma a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004. Cancer Lett. 2005;228:275–282.
16. Pless M, Ronthal M. Treatment of opsoclonus-myoclonus with high-dose intravenous immunoglobulin. Neurology. 1996;46:583–584.
17. Pranzatelli MR, Tate ED, Travelstead AL, et al. Rituximab (anti-CD20) adjunctive therapy for opsoclonus-myoclonus syndrome. J Pediatr Hematol Oncol. 2006;28:585–593.
18. Pranzatelli MR, Tate ED, Travelstead AL, et al. Insights on chronic-relapsing opsoclonus-myoclonus from a pilot study of mycophenolate mofetil. J Child Neurol. 2009;24:316–322.
19. Pranzatelli MR, Tate ED, Travelstead AL, Longee D. Immunologic and clinical responses to rituximab in a child with opsoclonus-myoclonus syndrome. Pediatrics. 2005;115:e115–e119.
20. Rostasy K, Wilken B, Baumann M, et al. High dose pulsatile dexamethasone therapy in children with opsoclonus-myoclonus syndrome. Neuropediatrics. 2006;37:291–295.
21. Sugie H, Sugie Y, Akimoto H, Endo K, Shirai M, Ito M. High-dose i.v. human immunoglobulin in a case with infantile opsoclonus polymyoclonia syndrome. Acta Paediatr. 1992;81:371–372.
22. Veneselli E, Conte M, Biancheri R, Acquaviva A, De Bernardi B. Effect of steroid and high-dose immunoglobulin therapy on opsoclonus-myoclonus syndrome occurring in neuroblastoma. Med Pediatr Oncol. 1998;30:15–17.
23. Pranzatelli MR, Tate ED, Travelstead AL, et al. B- and T-cell markers in opsoclonus-myoclonus syndrome: immunophenotyping of CSF lymphocytes. Neurology. 2004;62:1526–1532.
24. Pranzatelli MR, Tate ED, Travelstead AL, Allison TJ, Verhulst SJ. CSF B-cell expansion in opsoclonus-myoclonus syndrome: a biomarker of disease activity. Mov Disord. 2004;19:770–777.
25. Adaptive Behavior Assessment System, Second Edition (ABAS-II). 2000, Torrance: WPS.
26. Bayley N. Bayley Scales of Infant and Toddler Development®, Third edition. San Antonio: Pearson; 2005.
27. Wechsler Preschool and Primary Scale of Intelligence™–Fourth Edition. San Antonio: Pearson; 2012.
28. Wechsler Intelligence Scale for Children–Fourth Edition. Torrance: WPS; 2006.
29. Tate ED, Pranzatelli MR, Verhulst SJ, et al. Active comparator-controlled, rater-blinded study of corticotropin-based immunotherapies for opsoclonus-myoclonus syndrome. J Child Neurol. 2012;27:875–884.
Cite article
Cite article
Cite article
OR
Download to reference manager
If you have citation software installed, you can download article citation data to the citation manager of your choice
Information, rights and permissions
Information
Published In
Article first published online: October 23, 2014
Issue published: July 2015
Keywords
History
Manuscript received: May 6, 2014
Revision received: July 15, 2014
Manuscript accepted: August 8, 2014
Published online: October 23, 2014
Issue published: July 2015
PubMed: 25342308
Authors
Author Contributions
WM was the principal investigator, designed the study, identified subjects, examined each subject, and wrote the majority of the manuscript. AW contacted families, arranged scheduling of testing, obtained consents, performed initial chart reviews, accompanied families to the developmental testing, organized data and did initial data analyses and graphs, and wrote portions of the initial draft of the manuscript. She also assisted with required paperwork for IRB submission. SON performed the developmental testing, rescored developmental testing from the 2005 study for comparison, and edited the portions of the paper concerning developmental testing methods and results. JR and RC were study coordinators, assisting in contacting and consenting families, particularly those who were Spanish speaking, and helped organize the consents and data collection sheets. RW was research assistant to SON and assisted in scoring and collating the developmental and behavioral testing results.
Metrics and citations
Metrics
Article usage*
Total views and downloads: 884
*Article usage tracking started in December 2016
Articles citing this one
Web of Science: 51 view articles Opens in new tab
Crossref: 56
- Presence of identical B-cell clone in both cerebrospinal fluid and tum...
- Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, an...
- An international pediatric-onset opsoclonus myoclonus ataxia syndrome ...
- Five Years Follow-up of Opsoclonus–Myoclonus–Ataxia Syndrome-Associate...
- Neuroblastic Tumor Recurrence Associated With Opsoclonus Myoclonus Ata...
- Síndrome opsoclono-mioclono: características clínicas, aspectos terapé...
- Opsoclonus-myoclonus syndrome: Clinical characteristics, therapeutic c...
- “Pediatrician’s Approach to Recognizing Neuroblastoma With Opsoclonus-...
- Clinical features and outcomes of opsoclonus myoclonus ataxia syndrome
- Monocentric retrospective clinical outcome in a group of 13 patients w...
- Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Ch...
- Child with Ataxia
- Opsoclonus-Myoclonus Syndrome in Children and Adolescents: A Therapeut...
- Clinical Analysis of Pediatric Opsoclonus-Myoclonus Syndrome in One of...
- Pathogenesis, diagnosis and treatment of paraneoplastic neurologic syn...
- IQ predictors in pediatric opsoclonus myoclonus syndrome: a large inte...
- Opsoclonus-myoclonus syndrome: Experience in a tertiary hospital in th...
- Síndrome opsoclonus mioclonus. Experiencia en los últimos 12 años en u...
- Treatment and revaccination of children with paraneoplastic opsoclonus...
- The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblast...
- The Efficacy and Safety of Rituximab for the Treatment of Pediatric Au...
- Autoimmune and Autoantibody-Associated Encephalomyelopathies
- Paraneoplastic Neurologic Syndromes
- Movement Disorders in the Structure of the Different Mental Processes ...
- An upfront immunomodulatory therapy protocol for pediatric opsoclonus‐...
- Update on opsoclonus–myoclonus syndrome in adults
- Current therapies and therapeutic decision making for childhood‐onset ...
- Current behavioral assessments of movement disorders in children
- Multifactorial analysis of opsoclonus-myoclonus syndrome etiology (“Tu...
- Evaluation of Responsiveness to Reduced-Dose Rituximab in Corticotropi...
- Effect of low-dose cyclophosphamide, ACTH, and IVIG combination immuno...
- Autoimmune Movement Disorders in Children
- Principles and approaches to the treatment of immune-mediated movement...
- Therapeutic plasma exchange for a case of refractory opsoclonus myoclo...
- Enigmatic entities: opsoclonus myoclonus ataxia syndrome linked to neu...
- Intravenous immunoglobulin with prednisone and risk-adapted chemothera...
- Psychological development of children with opsoclonus myoclonus syndro...
- Demographic, Clinical, and Immunologic Features of 389 Children with O...
- Immune-Mediated CNS Diseases: a Review
- Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination I...
- Cancer and Autoimmunity: Paraneoplastic Neurological Disorders Associa...
- Opsoclonus-Myoclonus Syndrome: A New Era of Improved Prognosis?
- Systematic review of immunoglobulin use in paediatric neurological and...
- Rapid Resolution of Enterovirus 71-Associated Opsoclonus Myoclonus Syn...
- Opsoclonus Myoclonus Syndrome
- Neuroblastoma
- Opsoclonus-Myoclonus-Ataxia Syndrome in Children: Clinical Characteris...
- Movement Disorders Presenting in Childhood
- Sensory Processing Difficulties in Opsoclonus-Myoclonus Syndrome...
- The “neuro” of neuroblastoma: Neuroblastoma as a neurodevelopmental di...
- Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment
- Genetic features of neuroblastic tumors associated with opsoclonus-myo...
- Trends and tenets in relapsing and progressive opsoclonus-myoclonus sy...
- Rituximab treatment for relapsed opsoclonus–myoclonus syndrome
- Opsoclonus-Myoclonus Syndrome, Neuroblastoma, and Insulin-Dependent Di...
- Autoimmune neurologic disorders in children
Figures and tables
Figures & Media
Tables
View Options
Get access
Access options
If you have access to journal content via a personal subscription, university, library, employer or society, select from the options below:
loading institutional access options
Alternatively, view purchase options below:
Purchase 24 hour online access to view and download content.
Access journal content via a DeepDyve subscription or find out more about this option.
