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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.
Insights
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.
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.
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.
