title: Long-term neurocognitive outcome and quality of life in pediatric acute disseminated encephalomyelitis.
abstract: BACKGROUND
  Acute disseminated encephalomyelitis is an inflammatory-demyelinating disorder of the central nervous system usually with a monophasic course and a favorable neurological outcome. Long-term neurocognitive sequelae and quality of life have not yet been fully investigated.
  
  
  OBJECTIVE
  To examine neurocognitive outcome and quality of life in pediatric monophasic acute disseminated encephalomyelitis.
  
  
  METHODS
  Of the 36 patients diagnosed with acute disseminated encephalomyelitis at our institution, six were lost to follow-up and eight relapsed (two with multiphasic forms and six with multiple sclerosis). The outcome of the 22 remaining patients was evaluated using four subscales of the Wechsler Intelligence Scales for estimation of IQ, a battery of neuropsychological tests, and semistructured and PedsQL questionnaires for quality of life. The effect of age at onset, neuroradiological recovery, and time elapsed from the acute event on outcome was also investigated.
  
  
  RESULTS
  Estimated IQ, neuropsychological mean group scores, and quality of life at follow-up were within the normal range, but 23% of the patients had pathological scores in various neuropsychological functions, among which attention was the most clearly affected. The neuroradiological recovery was not correlated with the result of the neuropsychological tests. Age at onset correlated with linguistic skills, whereas the time elapsed from the acute event had a significant effect on attention tasks: scores were worse in the group of patients with a follow-up shorter than 7 years.
  
  
  CONCLUSIONS
  Our results suggest that pediatric monophasic acute disseminated encephalomyelitis has a favorable neurocognitive outcome. Patients with longer follow-up had a better outcome, suggesting a neurocognitive course that is different from that of multiple sclerosis and a potential for long-term recovery of affected functions.
authors.affiliation: Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy. Electronic address: suppiej@pediatria.unipd.it.
authors.forename: Agnese
authors.initials: A
authors.lastname: Suppiej
authors.affiliation: Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy; Lifespan Cognitive Neuroscience Laboratory, Department of General Psychology, University of Padua, Padua, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Cainelli
authors.affiliation: Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Casara
authors.affiliation: Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy.
authors.forename: Ambra
authors.initials: A
authors.lastname: Cappellari
authors.affiliation: Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Nosadini
authors.affiliation: Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Sartori
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001288:Attention; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003071:Cognition; D004673:Encephalomyelitis, Acute Disseminated / Q000150:complications / Q000473:pathology / Q000523:psychology* / Q000628:therapy; D056344:Executive Function; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007804:Language Development; D008297:Male; D008568:Memory; D009103:Multiple Sclerosis / Q000150:complications; D011788:Quality of Life; D020127:Recovery of Function; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: ADEM; cognitive; follow-up; impairments; multiple sclerosis; neuropsychological; sequelae