Alexander Ankar1, Jonathan Yarimi1, Meagan Newell1, Varun Kannan1, Daniel Davila-Williams1, Nikita Shukla1, Sarah Risen1
1Baylor College of Medicine/Texas Children's Hospital
Objective:
To describe two rare cases of MOG-Ab Disease presenting with status epilepticus in pediatric patients.
Background:
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is associated with conditions such as optic neuritis and acute disseminated encephalomyelitis. A newer entity of fluid-attenuated inversion recovery (FLAIR) imaging with hyperintense cortical lesions in MOG associated encephalitis with seizures (FLAMES) has also been described. These patients typically present with fevers, headaches and seizures in adulthood. Here, we describe two pediatric patients presenting in status epilepticus with diagnoses of FLAMES.
Design/Methods:
Retrospective analyses of two cases were performed.
Results:
A 5-year-old male presented with three days of fevers, headaches, and vomiting with new onset seizures. MRI showed left hemispheric leptomeningeal enhancement without diffusion restriction. Cerebrospinal fluid (CSF) analysis was unremarkable and infectious testing was negative. He subsequently developed nonconvulsive focal status epilepticus. Repeat MRI showed FLAIR hyperintense lesions with diffusion restriction involving the left hemispheric cortex. Serum MOG-IgG titers were elevated. He was treated with high-dose steroids and improved.
Similarly, a 2-year-old male presented with four days of fevers, headaches, and vomiting with new onset seizures. He also developed nonconvulsive focal status epilepticus. His seizures were refractory to midazolam infusion and required pentobarbital. CSF analysis showed an elevated white blood cell count with a lymphocytic predominance and negative infectious testing. MRI showed bilateral cortical FLAIR hyperintensities with diffusion restriction. He was treated with high-dose steroids given concerns for an inflammatory etiology. Serum MOG-IgG titers were elevated, after which he was treated with another course of steroids and intravenous immune globulin (IVIg) with improvement.
Conclusions:
FLAMES is a rare entity with core clinical features that typically occurs in adulthood. However, pediatric FLAMES can have fulminant presentations, including status epilepticus and refractory status epilepticus. Awareness of pediatric FLAMES and early MOG testing can lead to faster recognition and quicker treatment times with immunotherapies.