Shivani Venkatesh1, Samuel Marcucci1, Timothy Nguyen2, W. Daniel Chapman1
1UC Health Department of Neurology and Rehabilitation Medicine, 2Child Neurology, Cincinnati Children's Hospital
Objective:
We report a case of relapsing MOGAD, previously diagnosed as POMS.
Background:
Myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD) is a demyelinating disease that may present across all age groups and has clinical, radiographic, and immunologic features distinguishing this disease from multiple sclerosis (MS).Up to 5% of suspected pediatric onset multiple sclerosis (POMS) cases are found to have MOGAD.
Results:
A 28-year-old female was initially hospitalized in 2004 at age 6 with afebrile focal onset status epilepticus. One month later she presented with lethargy and meningismus. MRI showed T2 FLAIR hyper-intensities in the bilateral temporal lobes, brainstem, and subcortical white and gray matter, with mild leptomeningeal enhancement. She had a negative infectious workup and was treated for ADEM with IVIG and IV methylprednisolone. Over the next three years, she presented with recurrent optic neuritis and behavioral disturbances. Repeat MRI imaging during this period showed involvement of the optic nerves, cerebellum, and cervical spinal cord. AQP4-IgG testing was negative. CSF studies showed a lymphocytic pleocytosis, with oligoclonal bands not drawn. A diagnosis of POMS was made at age 12 and she was started on glatiramer acetate. Disease activity ceased after age 15. On presentation to our clinic, she had evidence of severe retinal nerve fiber layer thinning bilaterally on optical coherence tomography. As her clinical history and neuroimaging were atypical of MS and more consistent with MOGAD, serum MOG-IgG was sent and found to be positive with 1:100 titer.
Conclusions:
This case emphasizes key features distinguishing MOGAD from MS including age of onset, ADEM presentation, and recurrent bilateral optic neuritis. Thus, patients with an atypical clinical presentation for MS may warrant reevaluation of the initial diagnosis. Future research should focus on the utility of routine screening and treatment guidelines for monophasic versus relapsing cases of MOGAD.
Generative AI Usage
No, did not use generative AI in the drafting or editing in this abstract.
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