title: [Neuropsychological profile in opsoclonus-myoclonus-ataxia syndrome presenting as neuroblastic tumours].
abstract: BACKGROUND
  Sociosanitary improvements experienced in western society have significantly increased the survival of patients with opsoclonus-myoclonus-ataxia syndrome (OMAS). However, several studies have reported neurological, cognitive-behavioral and development persistent deficits in 70-80% of these patients.
  
  
  METHODS
  We reviewed cases of neuroblastic tumors diagnosed in a total period of 13 years and six months (from January 2000 to May 2013) and its association with OMAS in the pediatric service of a general hospital of a third level. Furthermore, it conducts a full neuropsychological study in three children diagnosed with OMAS.
  
  
  RESULTS
  We objectified deficits in intelligence, attention, processing speed, memory, language, visuospatial and visuoconstructive skills, fine motor skills and executive functions. In addition, we found alterations in the psychological profile.
  
  
  CONCLUSIONS
  Data emphasize the role of the cerebellum in complex cognitive processing in child population probably linked to neurodevelopmental deficits in this structure caused by deficiencies of the immune system. The results are interpreted in the framework of child neuropsychology and their interest in studying the brain-behavior relationships in the dynamic context of brain development.
authors.affiliation: Universidad de La Laguna, 38320 La Laguna, Espana.
authors.forename: Jezabel
authors.initials: J
authors.lastname: Bravo
authors.affiliation: Hospital de Cruces, Baracaldo, Espana.
authors.forename: Ricardo
authors.initials: R
authors.lastname: López-Almaraz
authors.affiliation: Hospital Universitario de Canarias, La Laguna, Espana.
authors.forename: Mercedes
authors.initials: M
authors.lastname: Mateos
authors.affiliation: Hospital Universitario de Canarias, La Laguna, Espana.
authors.forename: Leticia
authors.initials: L
authors.lastname: Díaz
authors.affiliation: Universidad de La Laguna, 38320 La Laguna, Espana.
authors.forename: Sergio
authors.initials: S
authors.lastname: Hernández-Expósito
mesh_terms: D001932:Brain Neoplasms / Q000209:etiology / Q000503:physiopathology* / Q000523:psychology*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D009447:Neuroblastoma / Q000209:etiology / Q000503:physiopathology* / Q000523:psychology*; D009483:Neuropsychological Tests; D053578:Opsoclonus-Myoclonus Syndrome / Q000150:complications / Q000503:physiopathology* / Q000523:psychology*