Long-term follow-up of neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome

E. De Grandis, S. Parodi, M. Conte, P. Angelini, F. Battaglia, C. Gandolfo, A. Pessagno, V. Pistoia, W. G. Mitchell, M. Pike, R. Haupt, E. Veneselli

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: The aim of this study is to describe the long-term neurological, neuropsychological and neuroradiological sequelae and to determine prognostic factors for neurological outcome in children with neuroblastoma-associated opsoclonus-myoclonus-ataxia (OMA) syndrome. Methods: Data on medical history were collected for the study patients. Examinations with grading of neurological signs, neuropsychological tests and brain magnetic resonance imaging with spectroscopy were performed during a follow-up clinic. Results: Fourteen subjects entered the study. All had localized neuroblastoma and they were evaluated after a median of 7.8 years. Patients with a chronic/multiphasic neurological course received steroids combined with intravenous immunoglobulins in the majority of cases. 71% presented neurological sequelae and 62% had a full-scale IQ below the normal range. All patients showed at least some deficit in the neuropsychological functions assessed (language, visual-motor integration, memory, attention and motor ability). Long-term deficits were more frequently detected in patients with an interval of more than 2 months between OMA onset and its diagnosis, even if in most comparisons statistical significance was not reached. Cerebellar atrophy, observed in 36% of patients, was not associated with the neurological outcome. Conclusions: Persisting disability is present in most children with neuroblastoma-associated OMA. However, our results support the role of an early diagnosis of OMA in reducing sequelae and encourage the use of new immunosuppressive therapies.

Original languageEnglish
Pages (from-to)103-111
Number of pages9
JournalNeuropediatrics
Volume40
Issue number3
DOIs
Publication statusPublished - 2009

Keywords

  • ataxia
  • cerebellum
  • neuroblastoma
  • opsoclonus-myoclonus syndrome
  • paraneoplastic syndromes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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