P2.C058: Rapid Neurological Deterioration in an Adolescent Female due to Myelin Oligodendrocyte Glycoprotein Antibody Disease, a Case Report

Introduction: Patient is a 20 year old previously healthy and 7 month postpartum female who presented with and continued to have rapid neurological deterioration during her admission in the PICU. Extensive workup was performed, resulting in a diagnosis of Myelin Oligodendrocyte Glycoprotein Antibody Disease.

Case Description: Patent presented for acute onset neurological symptoms including right facial, arm, and leg weakness, diplopia, emesis, headache, and abducens nerve palsy. The patient was admitted to the Pediatric Intensive Care Unit (PICU) for comprehensive evaluation and management. Initial examination revealed right hemiplegia and right lateral rectus palsy. CT and CTA of the head and neck were unremarkable for hemorrhage or large vessel occlusion. Cerebrospinal fluid studies revealed elevated protein and leukocytosis, however there was no growth on bacterial or viral cultures and the meningoencephalitis PCR panel was unremarkable. MRI of the brain showed a non-enhancing mass in the left thalamus extending to the midbrain.
Approximately 24 hours after admission, the patient deteriorated further, exhibiting slurred speech, word-finding difficulties, altered mental status, lethargy, bilateral clonus, and fixed pupils, necessitating urgent intubation to prevent respiratory failure. External ventricular drain was placed at the bedside due to concern for elevated intracranial pressure.

Discussion: Repear MRI after intubation revealed rapid progression of the lesion, now with bilateral thalamic lesions extending to the pons and a new
lesion in the left parietal subcortex. Additional diagnostic tests revealed positive titers for Epstein-Barr virus and Lyme IgM, as well as microbial cell-free DNA sequencing (Karius Panel) positive for H. pylori and CMV. Empiric treatment targeting bacterial, fungal, and viral pathogens, alongside IVIg therapy, failed to yield neurological improvement.
Subsequent brain biopsy demonstrated a macrophage-rich lesion with recent hemorrhage and reactive changes, prompting consultation with neurology, neurosurgery, infectious disease, and rheumatology specialists. Positive anti-MOG IgG titer at 1:80 suggested a diagnosis of
Myelin Oligodendrocyte Glycoprotein Antibody Disease (MOGAD). Despite steroid administration, the patient's neurological status did not significantly improve, leading to transfer to an external center for further specialty consultation.

Conclusion: We present a case of an adolescent female who experienced rapid neurological deterioration and underwent extensive workup resulting in a diagnosis of Myelin Oligodendrocyte Glycoprotein Antibody Disease. Patient's rapid onset of symptoms and lack of improvement with standard treatment prompted a broad differential diagnosis.