Abstract
Introduction
While monophasic and relapsing forms of myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) are increasingly diagnosed world-wide, consensus on management is yet to be developed.
Objective:
To survey the current global clinical practice of clinicians treating MOGAD.
Method
Neurologists worldwide with expertise in treating MOGAD participated in an online survey (February–April 2019).
Results
Fifty-two responses were received (response rate 60.5%) from 86 invited experts, comprising adult (78.8%, 41/52) and pædiatric (21.2%, 11/52) neurologists in 22 countries. All treat acute attacks with high dose corticosteroids. If recovery is incomplete, 71.2% (37/52) proceed next to plasma exchange (PE). 45.5% (5/11) of paediatric neurologists use IV immunoglobulin (IVIg) in preference to PE. Following an acute attack, 55.8% (29/52) of respondents typically continue corticosteroids for ≤ 3 months; though less commonly when treating children. After an index event, 60% (31/51) usually start steroid-sparing maintenance therapy (MT); after ≤ 2 attacks 92.3% (48/52) would start MT. Repeat MOG antibody status is used by 52.9% (27/51) to help decide on MT initiation. Commonly used first line MTs in adults are azathioprine (30.8%, 16/52), mycophenolate mofetil (25.0%, 13/52) and rituximab (17.3%, 9/52). In children, IVIg is the preferred first line MT (54.5%; 6/11). Treatment response is monitored by MRI (53.8%; 28/52), optical coherence tomography (23.1%; 12/52) and MOG antibody titres (36.5%; 19/52). Regardless of monitoring results, 25.0% (13/52) would not stop MT.
Conclusion
Current treatment of MOGAD is highly variable, indicating a need for consensus-based treatment guidelines, while awaiting definitive clinical trials.
Introduction
Myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) have been widely recognised as a distinct clinical entity only in the last decade, following the development of reliable cell-based assays using full-length human MOG as the target antigen [1, 2]. They encompass monophasic and relapsing presentations of central demyelination. Within the ‘neuromyelitis optica’ phenotype, optic neuritis is more common than transverse myelitis [3–8]. The clinical spectrum has since expanded to include brainstem and cortical encephalitis [9–12]. The most frequent presentation in young children is acute disseminated encephalomyelitis (ADEM) [8, 12, 13].
In comparison to aquaporin-4 antibody positive neuromyelitis optica spectrum disorders (AQP4-Ab NMOSD), relapse is less common. Approximately half of MOGAD patients may have monophasic disease, but some experience frequent relapses despite immunosuppressive therapy [14–17]. The value of antibody titres in predicting relapse is not yet fully understood. Overall, motor and visual disability outcomes seem better in MOGAD than in AQP4-Ab NMOSD [5, 14, 17], but the impact of relapses on long-term disability is unclear.
The unpredictability of MOGAD presents a challenge when developing treatment paradigms. Retrospective studies suggest that both acute and maintenance immunotherapy improve outcomes. However, there are no randomised controlled trials (RCTs) in MOGAD and an international, evidence-based consensus on management is yet to be developed. The objective of this survey is to describe the current clinical practice of neurologists treating adults and children with MOGAD internationally, to identify common themes, divergent practices and unanswered questions, which could inform the planning of collaborative studies and clinical trials.
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