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 paediatric (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 consensusbased treatment guidelines, while awaiting definitive clinical trials.
Aim: To analyse the efficacy of single dose rituximab (RTX) as induction therapy followed by conventional oral steroid-sparing agents (azathioprine, mycophenolate mofetil or methotrexate) in a cohort of patients with aggressive neuromyelitis optica spectrum disorder (NMOSD) without CD19, 20 and 27 biomarker testing. Methods: A retrospective analysis of clinical outcomes in eight patients with aggressive NMOSD treated with one course of RTX induction therapy in the Neurology Department at Kuala Lumpur Hospital from 2005 to 2018 was performed. The effectiveness of the treatment was determined by the number of relapses, expanded disability status scale, annualized relapsed rates, and modified Rankin Scale both before and after treatment. B cell enumeration testing was done instead of CD19, 20 and 27 biomarker testing. Results: There was a reduction in the mean annualized relapse rate from 4.7 to 0.5 attacks per year after treatment (P = 0.011). Mean expanded disability status scale and modified Rankin Scale values improved from 5.4 to 3.6 (P = 0.018) and 3.6 to 2.6 (P = 0.023), respectively. No patient developed any adverse effect. Conclusion: Single-course RTX induction therapy regime may be an alternative therapeutic option in resource limited hospitals to suppress NMOSD disease activity in the short term as pulse induction therapy whilst awaiting the effectiveness of conventional steroid-sparing agents. Further prospectively designed studies are required to prove efficacy.
Ankylosing spondylitis (AS) and multiple sclerosis (MS) are two different chronic inflammatory autoimmune conditions with unclear etiopathogenesis. One is a rheumatological disorder while the other is a neurological one. Current literature on an association between these two conditions is limited. Furthermore, early recognition of potential cases with both conditions co-existing is important as there have been very rare reports on the occurrence of demyelinating diseases or unmasking of latent MS in patients with ankylosing spondylitis treated with anti-TNFα. This has important therapeutic implications for patients at such risk.
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