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Boesen, Magnus S; Blinkenberg, Morten; Koch‐Henriksen, Nils; Thygesen, Lau C; Uldall, Peter V; Magyari, Melinda; Born, Alfred P

Implications of the International Paediatric Multiple Sclerosis Study Group consensus criteria for paediatric acute disseminated encephalomyelitis: a nationwide validation study

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  • Media type: E-Article
  • Title: Implications of the International Paediatric Multiple Sclerosis Study Group consensus criteria for paediatric acute disseminated encephalomyelitis: a nationwide validation study
  • Contributor: Boesen, Magnus S; Blinkenberg, Morten; Koch‐Henriksen, Nils; Thygesen, Lau C; Uldall, Peter V; Magyari, Melinda; Born, Alfred P
  • imprint: Wiley, 2018
  • Published in: Developmental Medicine & Child Neurology
  • Language: English
  • DOI: 10.1111/dmcn.13798
  • ISSN: 0012-1622; 1469-8749
  • Keywords: Neurology (clinical) ; Developmental Neuroscience ; Pediatrics, Perinatology and Child Health
  • Origination:
  • Footnote:
  • Description: <jats:sec><jats:title>Aim</jats:title><jats:p>The International Paediatric Multiple Sclerosis Study Group (<jats:styled-content style="fixed-case">IPMSSG</jats:styled-content>) has proposed criteria for acute disseminated encephalomyelitis (<jats:styled-content style="fixed-case">ADEM</jats:styled-content>) not evaluated in clinical practice. Our objective was to assess epidemiological implications of the <jats:styled-content style="fixed-case">IPMSSG</jats:styled-content> criteria for <jats:styled-content style="fixed-case">ADEM</jats:styled-content> in a cohort study using prospectively collected data.</jats:p></jats:sec><jats:sec><jats:title>Method</jats:title><jats:p>We identified all diagnosed cases of <jats:styled-content style="fixed-case">ADEM</jats:styled-content> in Denmark between 2008 and 2015 from the Danish National Patient Register by International Classification of Diseases 10 codes assigned to acute demyelinating episodes, and we reviewed all medical records to validate <jats:styled-content style="fixed-case">ADEM</jats:styled-content>.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>We found 52 children up to the age of 18 years with a verified clinical diagnosis of <jats:styled-content style="fixed-case">ADEM</jats:styled-content> (incidence rate 0.54/100 000 person‐years; all had abnormal brain magnetic resonance imaging). Only 18 (35%) fulfilled the <jats:styled-content style="fixed-case">IPMSSG</jats:styled-content> criteria regarding encephalopathy and polyfocal neurological deficits. Among all 52 children with <jats:styled-content style="fixed-case">ADEM</jats:styled-content>, 33 per cent had clinical sequelae after a median follow‐up of 4 years 6 months (range: 10mo–8y 3mo). Surprisingly, none progressed to multiphasic <jats:styled-content style="fixed-case">ADEM</jats:styled-content> or multiple sclerosis, but median age at end of follow‐up was only 10 years 9 months (range: 2y–24y 3mo).</jats:p></jats:sec><jats:sec><jats:title>Interpretation</jats:title><jats:p>Among 52 children with <jats:styled-content style="fixed-case">ADEM</jats:styled-content>, none converted to multiphasic <jats:styled-content style="fixed-case">ADEM</jats:styled-content> or multiple sclerosis (median follow‐up: 4y 6mo; range: 10mo–8y 3mo). Applying the <jats:styled-content style="fixed-case">IPMSSG</jats:styled-content> criteria to all children with a diagnosis of <jats:styled-content style="fixed-case">ADEM</jats:styled-content> leaves 65 per cent of the cases without a diagnosis and lowers the incidence rate of paediatric <jats:styled-content style="fixed-case">ADEM</jats:styled-content>.</jats:p></jats:sec><jats:sec><jats:title>What this paper adds</jats:title><jats:p> <jats:list list-type="bullet"> <jats:list-item><jats:p>The incidence of paediatric acute disseminated encephalomyelitis (ADEM) was 0.54 per 100 000 person‐years in children younger than 18 years.</jats:p></jats:list-item> <jats:list-item><jats:p>Only 35 per cent of children with ADEM fulfilled the International Paediatric Study Group consensus criteria.</jats:p></jats:list-item> <jats:list-item><jats:p>ADEM in clinical practice was primarily based on magnetic resonance imaging findings.</jats:p></jats:list-item> <jats:list-item><jats:p>Paediatric neurologists diagnosed ADEM in the absence of encephalopathy.</jats:p></jats:list-item> <jats:list-item><jats:p>None of the children with ADEM progressed to multiple sclerosis/multiphasic ADEM during follow‐up.</jats:p></jats:list-item> </jats:list> </jats:p></jats:sec>