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Abstract Details

Epstein–Barr Virus as an Underrecognized Trigger of Acute Disseminated Encephalomyelitis: A Case Report
Autoimmune Neurology
P3 - Poster Session 3 (11:30 AM-12:30 PM)
1-096
To present Epstein-Barr Virus (EBV) as a less common trigger for Acute Disseminated Encephalomyelitis (ADEM).
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder of the central nervous system of children, which is typically preceded by an infectious illness or vaccination. Frequently, no specific pathogen is identified despite a clear infectious prodrome. When identified, common infectious triggers in vaccinated patients include Influenza A/B, Mycoplasma Pneumoniae, and Varicella Zoster Virus.
Not applicable.
 A 6-year-old fully vaccinated female presented with 3 weeks of progressively worsening headaches, followed by gait instability, dysarthria and emesis. Her parent reported no recent illness, vaccination, or travel history. Neurologic examination was notable for truncal ataxia, dysmetria, scanning dysarthria, and ten cafe au lait spots. MRI of the brain and spine demonstrated diffuse asymmetric supratentorial, infratentorial, cervical, and thoracic white matter FLAIR hyperintensities and cerebellar grey matter lesions. Cerebrospinal fluid analysis (CSF) revealed mild pleocytosis (16 cells/µL) with lymphocytic predominance and elevated protein (69 mg/dL). Infectious evaluation was remarkable for serum Epstein-Barr virus titer indicative of recent infection (IgG >1:160, IgM <1:10). CSF PCR panel, culture, and urine cytomegalovirus were negative. Myelin oligodendrocyte glycoprotein antibodies, aquaporin-4 antibodies, autoimmune encephalitis panel, and genetic neurofibromatosis panel were negative. Ophthalmic evaluation did not show evidence of optic neuritis. The patient subsequently became encephalopathic over the course of the admission.  The patient was treated with intravenous methylprednisolone (30mg/kg for 5 days) and intravenous immunoglobulin (2g/kg divided over 2 days), with improvements in cognition, gait, and speech. She was discharged on an oral prednisone taper, and a neurology follow-up was scheduled in four weeks. 

This case highlights EBV as an infrequent trigger of ADEM. EBV serology should be considered even without a clear infectious prodrome. Timely diagnosis of ADEM guides initiation of early immunotherapy and may optimize the potential for neurologic recovery. 

Authors/Disclosures
Cole M. Cimoch
PRESENTER
Mr. Cimoch has nothing to disclose.
Sarang Perumal, MD Dr. Perumal has nothing to disclose.
Jay Desai, BS Mr. Desai has nothing to disclose.
Natanya Mishal, MD (Neuro Network Partners) Dr. Mishal has nothing to disclose.