Fulminant demyelinating diseases include acute disseminated encephalomyelitis (ADEM), myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD), multiple sclerosis (MS) and neuromyelitis optica-spectrum disorders (NMOSD), among others. The purpose of this case report is to present the evolving MRI/MRS findings in a patient with fulminant MS. A seven year old female presented with a 3 day history of progressive encephalopathy, gait disturbance and urinary incontinence. She had a recent viral upper respiratory tract infection. Interestingly, family history revealed the diagnosis of multiple sclerosis in the patient’s mother. Brain MRI revealed extensive T2/FLAIR hyperintense lesions, predominantly related to the cerebral and cerebellar white matter, with associated incomplete rim and central enhancement and marginal diffusion restriction. MRS showed decreased NAA, elevated choline and a prominent lactate peak. Accompanying bilateral anterior optic neuritis and papillitis was noted with T2 hyperintensity, enhancement and diffusion restriction. Immune suppressive treatment was initiated. Short interval serial follow up demonstrated progressive confluence of the lesions with increasing edema. While overall enhancement and diffusion restriction decreased, there was a new leading edge of diffusion restriction and enhancement, mainly in the bifrontal region. A persistent inflammatory signature was demonstrated on MRS. Subsequent follow up demonstrated some cerebral volume loss with resolving edema, enhancement and diffusion restriction. An extensive autoimmune panel was negative, including aquaporin 4 antibodies, NMO and MOG antibodies. Although CSF was sent for oligoclonal bands (OCBs), the sample volume was inadequate for testing on the first lumbar puncture (LP). OCBs were not identified on subsequent LP obtained after initiation of treatment. Pediatric MS is defined when the onset is before the age of 16 with less than 1% of patients presenting under the age of 10. Marburg variant is a rare, rapidly progressive, fulminant form of multiple sclerosis with high morbidity and mortality rates. Fulminant ADEM typically presents as a monophasic demyelinating disease and is often preceded by a viral or bacterial infection. In this case, the patient fulfilled Mc Donald diagnostic criteria 2024 for MS with initial typical MS lesions in ≥4 central nervous system topographies. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Lara Nunez Denny, Harder Sheri, Tshuma Makabongwe, Azma Roxana

Keywords: Brain MRI, Pediatric Neuroradiology