Sunday, December 20, 2020

MOG antibody disease differs for children and adults

Cobo-Calvo A, Ruiz A, Rollot F, Arrambide G, Deschamps R, Maillart E, Papeix C, Audoin B, Lépine AF, Maurey H, Zephir H, Biotti D, Ciron J, Durand-Dubief F, Collongues N, Ayrignac X, Labauge P, Meyer P, Thouvenot E, Bourre B, Montcuquet A, Cohen M, Horello P, Tintoré M, De Seze J, Vukusic S, Deiva K, Marignier R; NOMADMUS, KidBioSEP, and OFSEP study groups. Clinical Features and Risk of Relapse in Children and Adults with Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease. Ann Neurol. 2021 Jan;89(1):30-41. doi: 10.1002/ana.25909. Epub 2020 Oct 15. PMID: 32959427.


Objective: The main objective was to compare clinical features, disease course, and myelin oligodendrocyte glycoprotein (MOG) antibody (Ab) dynamics between children and adults with MOG-Ab-associated disease (MOGAD). 

Methods: This retrospective multicentric, national study included 98 children and 268 adults with MOGAD between January 2014 and September 2019. Cox regression model for recurrent time-to-event data and Kaplan-Meier curves for time to antibody negativity were performed for the objectives. 

Results: Isolated optic neuritis was the most frequent clinical presentation in both children (40.8%) and adults (55.9%, p = 0.013), and acute disseminated encephalomyelitis syndrome was more frequent in children (36.7% vs 5.6%, p < 0.001). Compared to adults, children displayed better recovery (Expanded Disability Status Scale ≥ 3.0 at last follow-up reached only by 10 of 97 [10.3%] vs 66/247 [26.7%], p < 0.001). In the multivariate analysis, adults were at higher risk of relapse than children (hazard ratio = 1.41, 95% confidence interval [CI] = 1.12-1.78, p = 0.003). At 2 years, 64.2% (95% CI = 40.9-86.5) of nonrelapsing children became MOG-Ab negative compared to 14.1% (95% CI = 4.7-38.3) of relapsing children (log-rank p < 0.001), with no differences observed in adults (log-rank p = 0.280). 

Interpretation: MOGAD patients differ in the clinical presentation at onset, showing an age-related shift in the clinical features across age groups. Compared to children, adults have a higher risk of relapse and worse functional recovery. Finally, children with monophasic disease become MOG-Ab negative earlier than relapsing children, but this is not true in adults. Considering these differences, management and treatment guidelines should be considered independently in children and adults. ___________________________________________________________________________

.“We used to think of this disease as having a single phenotype,” commented Michael Levy, MD, PhD, FAAN, director of the Neuromyelitis Optica Clinic and Research Laboratory and research director of the division of neuroimmunology and neuroinfectious disease at Massachusetts General Hospital, who was not involved in the study.

“But as we have been able to do antibody tests on more and more patients, we have come to recognize that it actually has a broader spectrum of features, and one of the biggest points of stratification is age.”

Children and adults “really do have different disease courses and different manifestations,” he said...

“Children were more likely to have a substantial recovery than adults among the whole cohort after a similar follow-up,” the authors noted. “Based on the different disease course, specific management and treatment guidelines should differentiate between children and adults in MOGAD.”...

Just over half of both adults and children experienced at least one relapse, but adults had a higher risk of relapse than children younger than 10 years of age (p=0.011). Women had a higher risk of relapse than men, and use of multiple sclerosis DMDs increased relapse risk as well...

“Neurologists can use this study to understand that there are differences between children and adults and that you don't just treat them as one patient type. If you encounter a patient with MOG antibody disease, you need to consider their age and clinical course before you decide which treatment to start and before you advise the patient on prognosis.”

“We know that children have a good chance of being monophasic, with only a single attack and never experiencing a relapse,” he said. “I think in this cohort it happened more often than we expected, but I would take that with a bit of caution because the follow-up period was not as long as we would like, to really be certain these patients are truly monophasic.”




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