Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Oct 1;4(10):e2128871.
doi: 10.1001/jamanetworkopen.2021.28871.

Comparison of Spinal Cord Magnetic Resonance Imaging Features Among Children With Acquired Demyelinating Syndromes

Giulia Fadda  1 , Cesar A Alves  2 , Julia O'Mahony  3   4 , Denise A Castro  5 , E Ann Yeh  6 , Ruth Ann Marrie  3   4 , Douglas L Arnold  1 , Patrick Waters  7 , Amit Bar-Or  8 , Arastoo Vossough  2 , Brenda Banwell  9 ; Canadian Pediatric Demyelinating Disease Study Group
Collaborators, Affiliations

Comparison of Spinal Cord Magnetic Resonance Imaging Features Among Children With Acquired Demyelinating Syndromes

Giulia Fadda et al. JAMA Netw Open. .

Abstract

Importance: The recognition of magnetic resonance imaging (MRI) features associated with distinct causes of myelitis in children is essential to guide investigations and support diagnostic categorization.

Objective: To determine the clinical and MRI features and outcomes associated with spinal cord involvement in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), multiple sclerosis (MS), and seronegative monophasic myelitis.

Design, setting, and participants: In this cohort study, participants were recruited between 2004 and 2017 through the multicenter Canadian Pediatric Demyelinating Disease Study, which enrolled youth younger than 18 years presenting within 90 days of an acquired demyelinating syndrome. Of the 430 participants recruited, those with lesions on available spine MRI and anti-MOG testing performed on archived samples obtained close to clinical presentation were selected. Participants with poor-quality images and final diagnoses of nondemyelinating disease, anti-aquaporin 4 antibody positivity, and relapsing seronegative myelitis were excluded. Data analysis was performed from December 2019 to November 2020.

Main outcomes and measures: Spinal cord involvement was evaluated on 324 MRI sequences, with reviewers blinded to clinical, serological, and brain MRI findings. Associated clinical features and disability scores at 5 years of follow-up were retrieved. Results were compared between groups.

Results: A total of 107 participants (median [IQR] age at onset, 11.14 [5.59-13.39] years; 55 girls [51%]) were included in the analyses; 40 children had MOGAD, 21 had MS, and 46 had seronegative myelitis. Longitudinally extensive lesions were very common among children with MOGAD (30 of 40 children [75%]), less common among those with seronegative myelitis (20 of 46 children [43%]), and rare in children with MS (1 of 21 children [5%]). Axial gray matter T2-hyperintensity (ie, the H-sign) was observed in 22 of 35 children (63%) with MOGAD, in 14 of 42 children (33%) with seronegative myelitis, and in none of those with MS. The presence of leptomeningeal enhancement was highly suggestive for MOGAD (22 of 32 children [69%] with MOGAD vs 10 of 38 children [26%] with seronegative myelitis and 1 of 15 children [7%] with MS). Children with MOGAD were more likely to have complete lesion resolution on serial images (14 of 21 children [67%]) compared with those with MS (0 of 13 children).

Conclusions and relevance: These findings suggest that several features may help identify children at presentation who are more likely to have myelitis associated with MOGAD. Prominent involvement of gray matter and leptomeningeal enhancement are common in pediatric MOGAD, although the pathological underpinning of these observations requires further study.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest Disclosures: Dr Yeh reported receiving grants from the National MS Society, MS Society of Canada, Stem Cell Network, Canadian Institutes of Health Research, Peterson Foundation, Biogen, Ontario Institute of Regenerative Medicine, and Center for Brain and Mental Health; serving as a scientific advisor for Horizon and Biogen; and receiving speaker’s honoraria from F. Hoffman–La Roche, Canadian Rhematological Association, MS at the Limits, New York University, and the Saudi Epilepsy Society outside the submitted work. Dr Marrie reported receiving grants from Biogen Idec (funds to coinvestigators only) and Roche Canada (funds to coinvestigators only) outside the submitted work. Dr Waters reported being codirector of the University of Oxford Autoimmune Neurology Diagnostic Laboratory, which runs tests for MOG antibodies for patient diagnosis, during the conduct of the study; in addition, Dr Waters reported having a patent for neurological autoimmune disorders licensed to Euroimmun AG and a patent for GABA-A receptor autoimmunity issued. Dr Banwell reported receiving grants from MS Society of Canada during the conduct of the study and personal fees from Novartis, UCB, and Roche outside the submitted work. No other disclosures were reported.

Figures

Figure 1.
Figure 1.. Flowchart of Study Design and Patient Recruitment
ADEM indicates acute disseminated encephalomyelitis; ADS, acquired demyelinating syndrome; CPDDS, Canadian Pediatric Demyelinating Disease Study; MOG-IgG, myelin oligodendrocyte glycoprotein antibodies; MOGAD, myelin oligodendrocyte glycoprotein antibody–associated disease; MRI, magnetic resonance imaging; MS, multiple sclerosis; ON, optic neuritis; QC, quality control; TM, transverse myelitis.
Figure 2.
Figure 2.. Characteristic Imaging Features of Spinal Cord Involvement in Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease
A, Sagittal (left) and axial (right) T2-weighted magnetic resonance imaging (MRI) of the spine shows a spinal cord lesion spanning over more than 10 vertebral segments, with H-sign appearance most evident in the cranial and caudal extremities of the lesion and hazy involvement of most of the cross-sectional spinal cord area in the central portion of the lesion. B, Sagittal (left) and axial (right) T2-weighted MRI of the spine shows a longitudinally extensive lesion with pencil-sign appearance on sagittal view and corresponding anterior-horns hyperintensity on axial image. C, Sagittal (left) and axial (right) gadolinium-enhanced T1-weighted MRI of the spine shows leptomeningeal and diffuse roots enhancement.
See this image and copyright information in PMC

References

    1. Fadda G, Armangue T, Hacohen Y, Chitnis T, Banwell B. Paediatric multiple sclerosis and antibody-associated demyelination: clinical, imaging, and biological considerations for diagnosis and care. Lancet Neurol. 2021;20(2):136-149. doi:10.1016/S1474-4422(20)30432-4 - DOI - PubMed
    1. Waters P, Fadda G, Woodhall M, et al. ; Canadian Pediatric Demyelinating Disease Network . Serial anti-myelin oligodendrocyte glycoprotein antibody analyses and outcomes in children with demyelinating syndromes. JAMA Neurol. 2020;77(1):82-93. doi:10.1001/jamaneurol.2019.2940 - DOI - PMC - PubMed
    1. Armangue T, Olivé-Cirera G, Martínez-Hernandez E, et al. ; Spanish Pediatric Anti-MOG Study Group . Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol. 2020;19(3):234-246. doi:10.1016/S1474-4422(19)30488-0 - DOI - PubMed
    1. Ramanathan S, Mohammad S, Tantsis E, et al. ; Australasian and New Zealand MOG Study Group . Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry. 2018;89(2):127-137. doi:10.1136/jnnp-2017-316880 - DOI - PMC - PubMed
    1. Baumann M, Grams A, Djurdjevic T, et al. . MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein. J Neurol. 2018;265(4):845-855. doi:10.1007/s00415-018-8781-3 - DOI - PubMed

Publication types