E.U. paediatric MOG consortium consensus: Part 4 – Outcome of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders

Abstract

There is increasing knowledge on the role of antibodies against myelin oligodendrocyte glycoprotein (MOG-abs) in acquired demyelinating syndromes and autoimmune encephalitis in children. Better understanding and prediction of outcome is essential to guide treatment protocol decisions. Therefore, this part of the Paediatric European Collaborative Consensus provides an oversight of existing knowledge of clinical outcome assessment in paediatric MOG-ab-associated disorders (MOGAD). The large heterogeneity in disease phenotype, disease course, treatment and follow-up protocols is a major obstacle for reliable prediction of outcome. However, the clinical phenotype of MOGAD appears to be the main determinant of outcome. Patients with a transverse myelitis phenotype in particular are at high risk of accruing neurological disability (motor and autonomic), which is frequently severe. In contrast, having a single episode of optic neuritis any time during disease course is broadly associated with a lower risk of persistent disability. Furthermore, MOG-ab-associated optic neuritis often results in good functional visual recovery, although retinal axonal loss may be severe. The field of cognitive and behavioural outcome and epilepsy following demyelinating episodes has not been extensively explored, but in recent studies acute disseminated encephalomyelitis (-like) phenotype in the young children was associated with cognitive problems and epilepsy in long-term follow-up. In conclusion, main domains of importance in determining clinical outcome in paediatric MOGAD are visual, motor, autonomic and cognitive function. A standardised evaluation of these outcome domains in all children is of importance to allow adequate rehabilitation and follow-up.

E.U. paediatric MOG consortium consensus: Part 1 – Classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders

“…Regardless of the disease severity at onset, most of these MOG-TM patients showed a good motor recovery [60], which may be better than the recovery in TM patients without MOG-abs, as previous paediatric TM cohorts with unknown MOG-ab serostatus reported worse outcomes [63,64]. However, bowel, bladder and/or erectile residual symptoms are common [60,123]. A proportion of MOG-TM patients have recurrent episodes during follow-up, described in around 17% of adult [27,39], and 0e14% of paediatric MOG-TM patients [14,22,33].…”

“…The relapsing clinical phenotypes following ON are discussed later in more detail. Importantly, although in general the functional VA of MOG-ON patients recovers well, optical coherence tomography (OCT) scans showed signs of severe axonal damage, which was as severe as observed in AQP4-ab-positive patients [35,49,59], and was not or only partly correlated to the number of ON episodes [35,49,123].…”

“…Although ADEM patients generally have a favourable long-term prognosis, mainly based on recovery of motor function, there is a risk of long-term cognitive impairment [45,46,123], and a higher risk of post-ADEM epilepsy in MOG-ab-positive compared to MOGab-negative patients [47]. While ADEM patients typically have a monophasic disease course, relapses can occur in the MOG-abpositive ADEM patients [31].…”

“…Patients who remain seropositive during follow-up appear at higher risk for relapse compared to the patients who convert to a seronegative status (38% vs. 13%, respectively) [14]. Additionally, older age has been found to be strongly associated with a relapsing disease course [14,123].…”

Editorial on EJPN focus section on E.U. paediatric MOG consortium consensus statements

“…The outcome of paediatric MOGAD for which only limited relevant data exists, is discussed in part 4. The working group addressing this important aspect suggests a detailed set of outcome assessment tools that can be used in the spectrum of clinical entities and age-groups within paediatric MOGAD [4]. The final part of this focus section deals with the important but also complex issue of acute and maintenance treatment which has been discussed in depth within the group.…”