Dados do Trabalho

Título

IS IT MOGAD OR PEDIATRIC MS?

Apresentação do caso

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. 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 has no evidence of disease activity and no treatment adverse reactions.

Discussão

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. 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.

Comentários finais

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.

Palavras Chave

Autoimmune disease; demyelinating disease; pediatric multiple sclerosis; MOGAD.

Declaração de conflito de interesses de TODOS os autores

Os autores declaram que não há conflito de interesses.