212 - Treatment Refractory Acute Disseminated Encephalomyelitis in a Pregnant Woman

32 year-old G3P2 female (~4 weeks pregnant) with PMHx of PCOS presented for 5 days of worsening AMS, aphasia, fatigue, and blurred vision. Patient was afebrile without antecedent immunizations, infectious prodrome, or prior neurologic episodes. Brain MRI demonstrated multifocal T2 signal abnormalities concerning for demyelination, later confirmed with brain biopsy. Spinal imaging was unremarkable and extensive evaluation with LPs and serologies was negative for malignancy, granulomatous disease, paraneoplastic etiology, NMO/AQP4, cysticercosis, fungal/bacterial infection, toxoplasmosis, JC virus, MOG, St. Louis encephalitis. With suspicion for ADEM, patient received IV steroids, IVIG, then PLEX without clinical improvement. Given persistent clinical deterioration, decision was made to terminate pregnancy and escalate treatment to Cytoxan and Tocilizumab followed by Rituxan. Patient improved with chemotherapy and prednisone taper and was then admitted to IPR for severe cognitive impairment, severe mixed aphasia, and dysphagia. Upon discharge from IPR, patient had significantly improved, but still required 24-hour direct supervision.

Discussions:

ADEM is a monophasic autoimmune demyelinating disorder of the CNS caused by an inflammatory reaction in the brain or spinal cord. In the absence of a clear antecedent infection, recent immunization, otherwise negative workup, and the temporal response/improvement to chemotherapy following pregnancy termination, there is consideration for pregnancy being the stimulus that precipitated the autoimmune demyelinating response.

Conclusions:

As a diagnosis of exclusion, more research is needed to identify specific biomarkers associated with ADEM for expedited diagnosis and to determine whether earlier detection and aggressive treatment would improve functional outcomes, especially given the substantial impact of the potential deficits. Additionally, with limited documentation in literature, further investigation is needed on whether pregnancy can precipitate ADEM or if it is associated with treatment refractory ADEM.