Long-term neurocognitive outcome and quality of life in pediatric acute disseminated encephalomyelitis

Agnese Suppiej; Elisa Cainelli; Giulia Casara; Ambra Cappellari; Margherita Nosadini; Stefano Sartori

doi:10.1016/j.pediatrneurol.2013.12.006

Long-term neurocognitive outcome and quality of life in pediatric acute disseminated encephalomyelitis

Pediatr Neurol. 2014 Apr;50(4):363-7. doi: 10.1016/j.pediatrneurol.2013.12.006. Epub 2013 Dec 12.

Authors

Agnese Suppiej¹, Elisa Cainelli², Giulia Casara³, Ambra Cappellari³, Margherita Nosadini³, Stefano Sartori³

Affiliations

¹ Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy. Electronic address: suppiej@pediatria.unipd.it.
² Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy; Lifespan Cognitive Neuroscience Laboratory, Department of General Psychology, University of Padua, Padua, Italy.
³ Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy.

PMID: 24630282
DOI: 10.1016/j.pediatrneurol.2013.12.006

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.

Aim: 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.

Conclusion: 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.

Keywords: ADEM; cognitive; follow-up; impairments; multiple sclerosis; neuropsychological; sequelae.

MeSH terms

Adolescent
Adult
Age of Onset
Attention
Brain / pathology
Child
Child, Preschool
Cognition*
Encephalomyelitis, Acute Disseminated / complications
Encephalomyelitis, Acute Disseminated / pathology
Encephalomyelitis, Acute Disseminated / psychology*
Encephalomyelitis, Acute Disseminated / therapy
Executive Function
Female
Follow-Up Studies
Humans
Language Development
Male
Memory
Multiple Sclerosis / complications
Quality of Life*
Recovery of Function
Time Factors
Treatment Outcome
Young Adult