Treatment responsive relapsing MOG-Ab associated demyelination

E.U. paediatric MOG consortium consensus: Part 5 – Treatment of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders

“…Similar effects were observed in paediatric MOGAD [3,9]. However, only temporary improvement with following relapse (defined by the consensus group as a new clinical episode accompanied by radiological evidence depending on the subtype of MOGAD, appearing at least one month subsequently to the last acute attack) or "flare-up" (defined by the consensus group as re-occurrence of symptoms within one month after start of acute treatment and not meeting definition of a relapse) were also reported [3,22]. In a multicentre retrospective cohort with relapsing MOGAD patients in half of all children a high risk of relapse was observed (1) at oral prednisone doses <0.5 mg/kg/day (2), within the first two months after IVMP pulse or (3) in cases with rapid oral prednisone taper (mean 1.5 months in relapsing vs. 5 months in monophasic patients) [23].…”

“…The treatment response of the acute phase has been analysed in a large retrospective cohort with primarily adult NMOSD patients with MOG-abs: in 50% of patients, IVMP treatment was followed by complete or almost complete recovery measured by visual acuity (VA) and Expanded Disability Status Scale (EDSS), and in 44% by partial recovery [22]. Similar effects were observed in paediatric MOGAD [3,9]. However, only temporary improvement with following relapse (defined by the consensus group as a new clinical episode accompanied by radiological evidence depending on the subtype of MOGAD, appearing at least one month subsequently to the last acute attack) or "flare-up" (defined by the consensus group as re-occurrence of symptoms within one month after start of acute treatment and not meeting definition of a relapse) were also reported [3,22].…”

“…Although the acute treatment appears to have no effect on the following disease course in MOGAD [9,15], efficient treatment in the acute phase is mandatory in order to prevent residual symptoms in the long-term [3,9,16,17]. In recent cohort studies regarding children with ADS and MOG-abs, acute treatment protocols included intravenous methylprednisolone (IVMP), with or without oral prednisone taper, intravenous immunoglobulins (IVIG) and plasma exchange (PLEX) [1,3,9,15,18].…”

“…The vast majority of paediatric MOGAD patients were treated with IVMP at acute presentation (first episode or relapse), with dosages between 20 and 30 mg/kg/day over 3e5 days [1,3,9,15,18]. Short-term high-dose steroids are associated with mainly mild side effects, including hyperglycemia, electrolyte abnormalities, hypertension or neuropsychological alterations [19].…”

“…Due to limited data regarding oral steroid tapering with studies describing heterogeneous prednisone doses and duration [23,24], the effectiveness in relapse prevention remains uncertain. Interestingly, IVMP seems to have no or only partial effect in selected patients, in whom treatment escalation via IVIG or PLEX was necessary [3,9,18,22,25]. However, timing issues, differences in MOG-abs titres, the dosage of IVMP given, and previous or concomitant treatments might play a role [22].…”