Frequency and characteristics of short versus longitudinally extensive myelitis in adults with MOG antibodies: A retrospective multicentric study

Ciron, Jonathan; Cobo‐Calvo, Álvaro; Audoin, Bertrand; Bourre, Bertrand; Brassat, David; Cohen, M.; Collongues, Nicolas; Deschamps, Romain; Durand‐Dubief, Françoise; Laplaud, David‐Axel; Maillart, Élisabeth; Papeix, Caroline; Zephir, Hélène; Béreau, Matthieu; Brochet, Bruno; Carra‐Dallière, Clarisse; Derache, Nathalie; Gagou-Scherer, Clarisse; Henry, Carole; Kerschen, Philippe; Mathey, Guillaume; Maubeuge, Nicolas; Maurousset, Aude; Montcuquet, Alexis; Moreau, Thibault; Prat, C; Taithe, F.; Thouvenot, Éric; Tourbah, Ayman; Rollot, Fabien; Vukusic, Sandra; Marignier, Romain

doi:10.1177/1352458519849511

Cited by 50 publications

(73 citation statements)

References 27 publications

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“…The role of follow-up MRI in MOGAD remains to be determined [16,17]. However, it is noteworthy that MRI at last assessment was often unremarkable: 80% of patients had normal or almost normal brain MRI, and 70% had negative MRI of the spinal cord, including even some patients with a history of myelitis.…”

Section: Discussionmentioning

confidence: 99%

The long‐term outcome of MOGAD: An observational national cohort study of 61 patients

Deschamps

Pique

Ayrignac

et al. 2021

Euro J of Neurology

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Background and objective The prognosis in myelin oligodendrocyte glycoprotein (MOG) antibody‐associated disease (MOGAD) is a matter of debate. Our aim was to assess the long‐term outcomes of patients with MOGAD. Methods We retrospectively analysed the clinical and paraclinical data of patients from the French nationwide observatory study NOMADMUS who tested positive for MOG antibodies (MOG‐IgG) and who had clinical follow‐up of at least 8 years from their first episode. Results Sixty‐one patients (median [range] age at onset 27 [3–69] years), with a median (mean; range) follow‐up of 177 (212.8; 98–657) months, were included. Among 58 patients with a relapsing course, 26.3% relapsed in the first year after onset. Of the 61 patients, 90.2% experienced at least one episode of optic neuritis. At last visit, the median (mean; range) Expanded Disability Status Scale (EDSS) score was 1 (2.12; 0–7.5), 12.5% had an EDSS score ≥6 and 37.5% had an EDSS score ≥3. Of 51 patients with final visual acuity (VA) data available, 15.7% had VA ≤0.1 in at least one eye and 25.5% had VA ≤0.5 in at least one eye. Bilateral blindness (VA ≤0.1) was present in 5.9% of patients. Finally, 12.5% of patients presented bladder dysfunction requiring long‐term urinary catheterization. No factor associated significantly with a final EDSS score ≥3 or with final VA ≤0.1 was found. Conclusion Overall long‐term favourable outcomes were achieved in a majority of our patients, but severe impairment, in particular visual damage, was not uncommon.

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Section: Discussionmentioning

confidence: 99%

The long‐term outcome of MOGAD: An observational national cohort study of 61 patients

Deschamps

Pique

Ayrignac

et al. 2021

Euro J of Neurology

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“…25,26 In addition, testing for MOG-IgG in SSM patients is also recommended, since previous studies have found 38.4% of SSM to be the initial presentation in MOG-IgG-associated diseases. 27…”

Section: Discussionmentioning

confidence: 99%

Short segment myelitis as the initial and only manifestation of aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorders

Fang

Zheng

Yang

et al. 2020

Ther Adv Neurol Disord

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Background: Short segment myelitis (SSM, < 3 vertebral segments) is an under-recognized initial manifestation of neuromyelitis optica spectrum disorders (NMOSD). Though infrequent, failure to recognize SSM in patients with NMOSD would lead to incorrect diagnosis and treatment. Therefore, delineation of features of NMOSD-associated SSM is of paramount importance. Objective: Our study aimed to determine the demographic, clinical and radiological features of NMOSD-associated SSM, and compare those with NMOSD-associated longitudinally extensive transverse myelitis (LETM) and multiple sclerosis (MS)-associated SSM, respectively. Methods: Chinese patients presenting initially only with acute myelitis and diagnosed with NMOSD ( n = 46) and MS ( n = 11) were included. Clinical, serological, imaging and disability data were collected. Mann–Whitney U test or two-tailed Fisher’s exact tests were used to analyse the data. Results: Of the 46 enrolled NMOSD patients, 34 (74%) collectively had 38 LETM lesions, while 12 (26%) had 14 SSM lesions. When compared with LETM, NMOSD presenting with SSM were more likely to have a delayed diagnosis and a lower level of disability at nadir during the first attack. T1-weighted imaging hypointensity was more prominent in NMOSD-associated LETM lesions than NMOSD-associated SSM lesions. When compared with MS-associated SSM, NMOSD-associated SSM lesions were more likely to be centrally located, grey matter involving and transversally extensive on axial imaging and spanned no less than 2 vertebral segments on sagittal imaging. Conclusion: These findings suggest that SSM does not preclude the possibility of a NMOSD diagnosis. Testing for serum aquaporin-4 immunoglobulin G (AQP4-IgG) and careful study of lesions on spinal cord magnetic resonance imaging could aid in an earlier and correct diagnosis.

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“…The presence of short-segment myelitis or intrathecally restricted OCBs should not exclude a diagnosis of MOGAD. Further, serum MOG antibody testing is warranted in patients with the following features, even if the McDonald criteria were fulfilled: 1) recurrent myelitis with or without brain lesions; 2) conus medullaris involvement, especially if present at onset; 3) unsatisfied disease control with steroid or DMT, i.e., frequent flare-ups during steroid tapering or after steroid cessation, or relapses even during DMT use (7,9,10). Notably, the clinical clues become more important with a lowtiter MOG antibody testing result.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder Masquerading as Multiple Sclerosis: An Under-Recognized Entity?

Zheng

Cai

et al. 2021

Front. Immunol.

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Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) covers a wide spectrum of manifestations and is defined by the presence of MOG seropositivity. However, in a proportion of patients, there may be an overlap in some of the clinical and radiological manifestations between MOGAD and multiple sclerosis (MS). Being wary of this entity is critical to ensure appropriate therapy. Herein, we present a case with recurrent episodes of short-segment myelitis typical for multiple sclerosis, but later diagnosed as MOGAD by MOG antibody seropositivity. This case, along with previous reports, highlights an increasingly recognized subgroup in MOGAD with initial clinical phenotypes suggestive of MS, but later showing a disease course and therapeutic response compatible with MOGAD. Given the potential overlap of some clinical phenotypes in patients with MS and those with MOGAD, we recommend MOG antibody testing in all patients with recurrent short-segment myelitis, conus medullaris involvement, and those who demonstrated steroid dependence.

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Frequency and characteristics of short versus longitudinally extensive myelitis in adults with MOG antibodies: A retrospective multicentric study

Cited by 50 publications

References 27 publications

The long‐term outcome of MOGAD: An observational national cohort study of 61 patients

The long‐term outcome of MOGAD: An observational national cohort study of 61 patients

Short segment myelitis as the initial and only manifestation of aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorders

Case Report: Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder Masquerading as Multiple Sclerosis: An Under-Recognized Entity?

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