Diagnostic algorithm for relapsing acquired demyelinating syndromes in children

Yael Hacohen, Kshitij Mankad, W K Chong, Frederik Barkhof, Angela Vincent, Ming Lim, Evangeline Wassmer, Olga Ciccarelli, Cheryl Hemingway

Research output: Contribution to journalArticleAcademicpeer-review

133 Citations (Scopus)


OBJECTIVE: To establish whether children with relapsing acquired demyelinating syndromes (RDS) and myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) show distinctive clinical and radiologic features and to generate a diagnostic algorithm for the main RDS for clinical use.

METHODS: A panel reviewed the clinical characteristics, MOG-Ab and aquaporin-4 (AQP4) Ab, intrathecal oligoclonal bands, and Epstein-Barr virus serology results of 110 children with RDS. A neuroradiologist blinded to the diagnosis scored the MRI scans. Clinical, radiologic, and serologic tests results were compared.

RESULTS: The findings showed that 56.4% of children were diagnosed with multiple sclerosis (MS), 25.4% with neuromyelitis optica spectrum disorder (NMOSD), 12.7% with multiphasic disseminated encephalomyelitis (MDEM), and 5.5% with relapsing optic neuritis (RON). Blinded analysis defined baseline MRI as typical of MS in 93.5% of children with MS. Acute disseminated encephalomyelitis presentation was seen only in the non-MS group. Of NMOSD cases, 30.7% were AQP4-Ab positive. MOG-Ab were found in 83.3% of AQP4-Ab-negative NMOSD, 100% of MDEM, and 33.3% of RON. Children with MOG-Ab were younger, were less likely to present with area postrema syndrome, and had lower disability, longer time to relapse, and more cerebellar peduncle lesions than children with AQP4-Ab NMOSD. A diagnostic algorithm applicable to any episode of CNS demyelination leads to 4 main phenotypes: MS, AQP4-Ab NMOSD, MOG-Ab-associated disease, and antibody-negative RDS.

CONCLUSIONS: Children with MS and AQP4-Ab NMOSD showed features typical of adult cases. Because MOG-Ab-positive children showed notable and distinctive clinical and MRI features, they were grouped into a unified phenotype (MOG-Ab-associated disease), included in a new diagnostic algorithm.

Original languageEnglish
Pages (from-to)269-278
Number of pages10
Issue number3
Publication statusPublished - 18 Jul 2017


  • Adolescent
  • Algorithms
  • Aquaporin 4
  • Autoantibodies
  • Brain
  • Child
  • Child, Preschool
  • Clinical Decision-Making
  • Encephalomyelitis
  • Female
  • Follow-Up Studies
  • Herpesvirus 4, Human
  • Humans
  • Journal Article
  • Magnetic Resonance Imaging
  • Male
  • Multiple Sclerosis
  • Myelin-Oligodendrocyte Glycoprotein
  • Neuromyelitis Optica
  • Oligoclonal Bands
  • Optic Neuritis
  • Recurrence
  • Retrospective Studies
  • Viral Load

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