Is it MOGAD or pediatric MS?

*Correspondence: [e-mail].

Abstract

Case Presentation: A previously healthy 10-year-old boy presented with subacute right upper and lower limb tremor, slurred speech and unsteady gait. Brain MRI revealed a T2 hyperintense lesion in left pons with DWI restriction and one right ovoid lesion in the cerebellar peduncle, 2 periventricular lesions, one lesion in left inferior temporal lobe, and supratentorial unspecific T2-hyperintense white matter lesions. Cerebrospinal fluid showed exclusive positive oligoclonal bands (OCBs). Serum workup revealed positive IgG for cytomegalovirus (CMV), Epstein-Baar (EBV) and positive myelin oligodendrocyte glycoprotein (MOG) antibodies through cell-based assay (1:100). He was negative for anti-aquaporin-4 (AQP4-IgG). He received intravenous methylprednisolone (IVMP) and oral steroid taper with symptom resolution. A follow-up MRI 6 months later demonstrated increased lesion volume and new hyperintense periventricular lesions. A new lumbar puncture revealed persistence of OCBs. A single dose of intravenous immunoglobulin (IVIg) 2g/kg was prescribed until rituximab was available. Rituximab was initiated at 1g every 6 months. One week after the first dose of rituximab he presented a flare of right superior limb weakness with a new frontoparietal juxtacortical brain MRI lesion with contrast enhancement. He was treated with IVMP with improvement. At 8-month follow-up he had no evidence of disease activity and no treatment adverse reactions.

Discussion: This patient met space and time criteria for pediatric multiple sclerosis (POMS), but also had clearly positive serum MOG-IgG antibodies with a clinical syndrome suggestive of MOGAD fulfilling criteria for MOG-IgG associated disease (MOGAD). The presence of new periventricular and juxtacortical lesions, the positivity for Epstein-Baar, and the persistence of OCBs after the acute attack favor the POMS diagnosis. However, the initial brainstem lesion and clearly positive MOG-IgG titers favor the diagnosis of MOGAD. The transient increase of antibodies was described in AQP4-IgG positive neuromyelitis optica but not in MOGAD or MS. Positivity for CMV is associated with non-POMS disease course. Considering that neither POMS nor MOGAD could be completed excluded, we chose rituximab as maintenance treatment.

Final Comments: MOGAD and POMS are considered different conditions and there are only anecdotical reports of patients meeting criteria for both diseases. In these rare challenging cases, anti-CD20 medications are safe treatment options.

Publikationsverlauf

Artikel online veröffentlicht:
12. Mai 2025