Abstract Details

Title A Case Report of Rapidly Fatal MOG Antibody-associated ADEM in a Pediatric Patient

Topic Child Neurology and Developmental Neurology

Presentation(s) P6 - Poster Session 6 (8:00 AM-9:00 AM)

Poster/Presentation
Number 8-008

Objective We report a case of a previously healthy 8-year-old male with incomplete vaccination status and recent immigration from Peru admitted with acute fever and encephalopathy. Symptoms began with headache and abdominal pain, followed by development of fever on day 3, then declining mental status on day 4 prompted magnetic resonance imaging (MRI) which showed acute demyelinating encephalomyelitis (ADEM) without herniation, which rapidly progressed to clinical evidence of herniation with brain death on day 5. Post-mortem lab results and autopsy were consistent with myelin oligodendrocyte glycoprotein (MOG) antibody-associated ADEM. In review of the literature, this represents the first reported case of rapidly fatal MOG antibody-associated ADEM in a pediatric patient.

Background There are rare reports of hospital mortality in adults with MOG antibody-associated ADEM, and one study demonstrated a <3% hospital mortality in children with ADEM, though antibody status was unconfirmed in the cohort. There is a case series of three MOG antibody-associated ADEM pediatric patients with brain herniation without fatality, but per our review, no prior reports of pediatric hospital mortality from MOG antibody-associated ADEM exist.

Design/Methods N/A

Results N/A

Conclusions This case describes a pediatric patient with rapid onset cerebral edema resulting in increased intracranial pressure (ICP), brainstem herniation, and brain death found to have MOG antibody-associated ADEM. Supportive findings include the 1:1000 MOG antibody titers with normal CSF infectious testing, normal Karius pathogen serum testing, and autopsy results consistent with early active demyelination and diffuse meningoencephalitis which have been previously reported in MOG antibody-associated ADEM. At this time, it is impossible to predict which patients presenting with ADEM will go on to develop brain herniation, thus clinicians may consider proactive ICP monitoring, early steroid administration, and neuroprotective strategies in this patient population.

Authors/Disclosures
Emily Allen PRESENTER	No disclosure on file
Charles Jake Sadle, DO (UofL Dept. of Neurology Neurology ��ɫ�� Office)	Dr. Sadle has nothing to disclose.
Amanda Rogers, MD (Pediatric Neurology, University of Louisville)	An immediate family member of Dr. Rogers has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Medpace.
Erin Owen	No disclosure on file

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