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Related Concept Videos

Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...

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Diagnostic algorithm for relapsing acquired demyelinating syndromes in children.

Yael Hacohen1, Kshitij Mankad2, W K Chong2

  • 1From the Department of Paediatric Neurology (Y.H., C.H.), Great Ormond Street Hospital for Children; Department of Neuroinflammation (Y.H., O.C.), Queen Square MS Centre, UCL Institute of Neurology; Department of Clinical Neuroscience (Y.H.), UCL Institute of Child Health; Paediatric Neuroradiology (K.M., W.K.C.), Great Ormond Street Hospital; Institutes of Neurology and Biomedical Engineering (F.B.), University College London, UK; Department of Radiology and Nuclear Medicine (F.B.), VU University Medical Centre, Amsterdam, the Netherlands; Nuffield Department of Clinical Neurosciences (A.V.), John Radcliffe Hospital, University of Oxford; Children's Neurosciences (M.L.), Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London; and Department of Paediatric Neurology (E.W.), Birmingham Children's Hospital, Birmingham, UK. yael.hacohen@gosh.nhs.uk.

Neurology
|June 16, 2017
PubMed
Summary

Children with myelin oligodendrocyte glycoprotein antibody (MOG-Ab) associated disease show distinct features and are now a unified phenotype. A new diagnostic algorithm aids in classifying pediatric acquired demyelinating syndromes.

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Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Demyelinating Diseases of the Central Nervous System

Background:

  • Acquired demyelinating syndromes (RDS) in children encompass various conditions, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and others.
  • Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) are implicated in some pediatric RDS, but their distinct clinical and radiological profiles require further elucidation.
  • Differentiating between these RDS phenotypes is crucial for accurate diagnosis and management.

Purpose of the Study:

  • To determine if children with MOG-Ab and RDS exhibit unique clinical and radiological characteristics.
  • To develop a diagnostic algorithm for classifying major pediatric RDS for practical clinical application.

Main Methods:

  • A panel analyzed clinical data, MOG-Ab, aquaporin-4 antibody (AQP4-Ab), oligoclonal bands, and Epstein-Barr virus serology in 110 children with RDS.
  • A neuroradiologist, blinded to diagnoses, evaluated MRI scans.
  • Clinical, radiological, and serological findings were compared across different RDS groups.

Main Results:

  • The study identified multiple sclerosis (MS) in 56.4%, NMOSD in 25.4%, multiphasic disseminated encephalomyelitis (MDEM) in 12.7%, and relapsing optic neuritis (RON) in 5.5% of cases.
  • MOG-Ab were prevalent in AQP4-Ab-negative NMOSD (83.3%), MDEM (100%), and RON (33.3%).
  • Children with MOG-Ab were younger, had less severe disability, longer relapse times, and distinct MRI features (e.g., cerebellar peduncle lesions) compared to AQP4-Ab NMOSD patients.
  • A diagnostic algorithm was proposed, categorizing RDS into MS, AQP4-Ab NMOSD, MOG-Ab-associated disease, and antibody-negative RDS.

Conclusions:

  • Pediatric MS and AQP4-Ab NMOSD mirror adult presentations.
  • MOG-Ab-positive pediatric RDS cases demonstrate distinct clinical and MRI features, warranting classification as a unified phenotype: MOG-Ab-associated disease.
  • The proposed diagnostic algorithm integrates these findings for improved classification of pediatric CNS demyelinating disorders.