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Diagnostic algorithm for relapsing acquired demyelinating syndromes in children.
Neurology 2017; 89(3):269-278Neur

Abstract

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.

Authors+Show Affiliations

From 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.From 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.From 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.From 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.From 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.From 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.From 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.From 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.From 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.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

28615429

Citation

Hacohen, Yael, et al. "Diagnostic Algorithm for Relapsing Acquired Demyelinating Syndromes in Children." Neurology, vol. 89, no. 3, 2017, pp. 269-278.
Hacohen Y, Mankad K, Chong WK, et al. Diagnostic algorithm for relapsing acquired demyelinating syndromes in children. Neurology. 2017;89(3):269-278.
Hacohen, Y., Mankad, K., Chong, W. K., Barkhof, F., Vincent, A., Lim, M., ... Hemingway, C. (2017). Diagnostic algorithm for relapsing acquired demyelinating syndromes in children. Neurology, 89(3), pp. 269-278. doi:10.1212/WNL.0000000000004117.
Hacohen Y, et al. Diagnostic Algorithm for Relapsing Acquired Demyelinating Syndromes in Children. Neurology. 2017 Jul 18;89(3):269-278. PubMed PMID: 28615429.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Diagnostic algorithm for relapsing acquired demyelinating syndromes in children. AU - Hacohen,Yael, AU - Mankad,Kshitij, AU - Chong,W K, AU - Barkhof,Frederik, AU - Vincent,Angela, AU - Lim,Ming, AU - Wassmer,Evangeline, AU - Ciccarelli,Olga, AU - Hemingway,Cheryl, Y1 - 2017/06/14/ PY - 2017/01/06/received PY - 2017/04/21/accepted PY - 2017/6/16/pubmed PY - 2017/7/25/medline PY - 2017/6/16/entrez SP - 269 EP - 278 JF - Neurology JO - Neurology VL - 89 IS - 3 N2 - 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. SN - 1526-632X UR - https://www.unboundmedicine.com/medline/citation/28615429/Diagnostic_algorithm_for_relapsing_acquired_demyelinating_syndromes_in_children_ L2 - http://www.neurology.org/cgi/pmidlookup?view=long&pmid=28615429 DB - PRIME DP - Unbound Medicine ER -