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Pediatric inflammatory leukoencephalopathies

Omar Abdel-Mannan1, Yael Hacohen1

  • 1Department of Neuroinflammation, Queen Square MS Centre, UCL Queen Square Institute of Neurology, Faculty of Brain Sciences, University College London, London, United Kingdom; Department of Neurology, Great Ormond Street Hospital, London, United Kingdom.

Handbook of Clinical Neurology
|September 25, 2024
PubMed

Insights

Acquired demyelinating syndromes (ADS) in children, including multiple sclerosis (MS), are distinct from myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and aquaporin 4-antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD). Differentiating these conditions is crucial for effective treatment strategies.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Demyelinating Diseases

Background:

  • Acquired demyelinating syndromes (ADS) are acute neurological conditions affecting the optic nerve, brain, or spinal cord in children.
  • While multiple sclerosis (MS) is a common relapsing form of ADS, other distinct entities like myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and aquaporin 4-antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) have been identified.
  • Historically considered variants of MS, MOGAD and AQP4-NMOSD are now recognized as separate conditions based on cumulative evidence.

Purpose of the Study:

  • To delineate distinct subtypes of acquired demyelinating syndromes (ADS) in pediatric patients.
  • To highlight the differences between multiple sclerosis (MS), MOGAD, and AQP4-NMOSD despite clinical overlaps.
  • To emphasize the importance of accurate diagnosis for guiding treatment in pediatric ADS.

Main Methods:

  • Review of clinical, biologic, and pathologic evidence differentiating MS, MOGAD, and AQP4-NMOSD.
  • Analysis of diagnostic criteria and phenotypic characteristics of pediatric ADS.
  • Examination of current and emerging therapeutic approaches for these distinct conditions.

Main Results:

  • Biologic, clinical, and pathologic data confirm MOGAD and AQP4-NMOSD as distinct entities from MS.
  • Phenotypic overlaps exist but do not preclude differentiation between MS, MOGAD, and AQP4-NMOSD.
  • Numerous inflammatory and non-inflammatory conditions can mimic ADS in children, necessitating careful diagnosis.

Conclusions:

  • Accurate discrimination between pediatric MS, MOGAD, and AQP4-NMOSD is essential for appropriate patient management.
  • Advances in disease-modifying therapies for MS and emerging treatments for MOGAD and AQP4-NMOSD underscore the need for precise diagnosis.
  • Recognizing mimics of ADS is critical for effective treatment planning in pediatric neurological care.

Keywords:
Aquaporin-4 antibodiesDisease-modifying therapiesMimicsMyelin oligodendrocyte glycoprotein antibody-associated diseaseNeuromyelitis optica spectrum disorderPediatric multiple sclerosis

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