Steroid‐responsive aseptic meningitis with raised intracranial pressure syndrome associated with myelin oligodendrocyte glycoprotein autoantibodies

Wong, Wui‐Kwan; Troedson, Christopher; Peacock, Ken; Brilot-Turville, Fabienne; Menezes, Manoj P.; Dale, Russell C.; Webster, Richard

doi:10.1111/jpc.16189

Cited by 2 publications

(1 citation statement)

References 8 publications

(24 reference statements)

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“…MOG-ON with bilateral optic disc swelling may be mistaken for idiopathic intracranial hypertension, which affects a similar age group and is more common, though does not usually cause the rapid and substantial vision loss or pain on eye movement seen in MOG-ON [53]. MOGAD can also cause papilloedema secondary to raised intracranial pressure, in association with an aseptic meningoencephalitis presentation [54,55].…”

Section: Optic Disc Swellingmentioning

confidence: 99%

MOG antibody-associated optic neuritis

Jeyakumar,

Lerch,

Dale

et al. 2024

Eye

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Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a demyelinating disorder, distinct from multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). MOGAD most frequently presents with optic neuritis (MOG-ON), often with characteristic clinical and radiological features. Bilateral involvement, disc swelling clinically and radiologically, and longitudinally extensive optic nerve hyperintensity with associated optic perineuritis on MRI are key characteristics that can help distinguish MOG-ON from optic neuritis due to other aetiologies. The detection of serum MOG immunoglobulin G utilising a live cell-based assay in a patient with a compatible clinical phenotype is highly specific for the diagnosis of MOGAD. This review will highlight the key clinical and radiological features which expedite diagnosis, as well as ancillary investigations such as visual fields, visual evoked potentials and cerebrospinal fluid analysis, which may be less discriminatory. Optical coherence tomography can identify optic nerve swelling acutely, and atrophy chronically, and may transpire to have utility as a diagnostic and prognostic biomarker. MOG-ON appears to be largely responsive to corticosteroids, which are often the mainstay of acute management. However, relapses are common in patients in whom follow-up is prolonged, often in the context of early or rapid corticosteroid tapering. Establishing optimal acute therapy, the role of maintenance steroid-sparing immunotherapy for long-term relapse prevention, and identifying predictors of relapsing disease remain key research priorities in MOG-ON.

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Section: Optic Disc Swellingmentioning

confidence: 99%

MOG antibody-associated optic neuritis

Jeyakumar,

Lerch,

Dale

et al. 2024

Eye

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Increased Intracranial Pressure in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease

Nguyen,

Miles,

Harder

et al. 2024

Neurol Neuroimmunol Neuroinflamm

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Background and ObjectivesElevated intracranial pressure (ICP) in myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) has been largely unexplored. The objectives of this study were to determine the frequency of increased ICP in MOGAD and its association with disease course and outcomes and to highlight cases requiring medical and/or surgical management of increased ICP.MethodsIn this retrospective, single-center cohort study, we examined the clinical and paraclinical data from the initial presentation and follow-up data of children diagnosed with MOGAD. In those with opening pressure (OP) measurements, univariate analyses were used to evaluate factors associated with increased ICP, which was defined as OP > 28 cm H2O. We also present a case series of patients with or without OP measurement who required medical and/or surgical management of increased ICP.ResultsOf 86 children with MOGAD, 43 (50.0%) had an OP recorded and 7 (8.1%) required ICP management. In those with OP recorded, the median (interquartile range) OP for the different MOGAD phenotypes were: 30.0 (22.8–41.6) (acute disseminated encephalomyelitis, ADEM), 20.5 (16.1–23.6) (optic neuritis), 17.0 (17.0–22.5) (myelitis), and 19.5 (16.5–29.3) (other) cm H20. Overall, 20.9% had increased ICP based on an OP > 28 cm H2O, of whom 77.8% presented with ADEM. In a subgroup analysis of those presenting with ADEM, those with an elevated ICP had longer hospital stay (p= 0.007) and neurologic disability (defined as modified Rankin Scale >1) (p= 0.049). In those with or without OP recorded, 7 (6 with ADEM, one with cerebral cortical encephalitis) required ICP-directed therapies. Findings on brain MRI in these 7 children revealed extensive disease burden with bilateral cerebral involvement and evidence of restricted diffusion. While neuropsychological data in this small subset revealed significant variability, all sustained identifiable deficits after discharge, including attention-deficit hyperactivity disorders and language and learning disorders.DiscussionIn pediatric MOGAD, increased OP and ADEM at initial presentation were associated with longer hospital stays and greater long-term morbidity. Although invasive ICP monitoring has not been specifically advocated in the management of MOGAD, it is important to recognize signs and symptoms of increased ICP in these patients and consider ICP monitoring and management strategies based on clinical and radiologic findings, especially in those presenting with ADEM and with OP > 28 cm H2O.

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Steroid‐responsive aseptic meningitis with raised intracranial pressure syndrome associated with myelin oligodendrocyte glycoprotein autoantibodies

Cited by 2 publications

References 8 publications

MOG antibody-associated optic neuritis

MOG antibody-associated optic neuritis

Increased Intracranial Pressure in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease

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