Abstract Details

Title Neuromyelitis Optica Spectrum Disorder with Overlapping Lupus Cerebritis: A Case Report

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (2:45 PM-3:45 PM)

Poster/Presentation
Number 025

Objective We present a unique case of demyelinating disease with +AQP4-IgG antibodies and rapidly progressive dementia with +ANA, +SS-A/SS-B, +anti-dsDNA, and hypocomplementemia supporting the diagnosis of NMOSD concomitantly with lupus cerebritis within 6 months.

Background

NMOSD has been reported as the first manifestation of SLE or coexisting with other autoimmune disorders. Systemic literature review finds 46 cases of overlapping SLE and NMOSD with nearly half the cases having onset of NMOSD after lupus with median 5 years between diagnoses.

Design/Methods Case-report

Results A 58-year-old female with thyroid disease presented with headache, imbalance, and dysarthria. MRI brain showed restricted diffusion in periventricular distribution and corpus callosum with patchy meningeal and right thalamic enhancement. MRI C spine also showed multifocal abnormalities. Elevated serum AQP4-IgG, ESR, CRP, and VGKC antibody with normal ANA, ANCA, DS-DNA, MOG, HTRA1 gene. Fluoroscopy guided LP unsuccessful. Treated for NMOSD. Six months later, patient presented with rapidly progressive dementia and personality changes. MRI now with progressive diffusion abnormalities in brain, enhancing lesion in the T spine and cauda equina. Now also with positive ANA, SS-A/SS-B, dsDNA, and hypocomplementemia. Patient improved with dexamethasone and PLEX x 5 treatments. She underwent induction therapy for lupus cerebritis and NMOSD with rituximab and cyclophosphamide. Follow-up imaging at 3 months with new white matter lesions, no enhancing lesions and patient clinically improved. Patient started on mycophenolate mofetil and hydroxychloroquine.

Conclusions

Our case adds to the paucity of literature and highlights the importance of recognizing NMOSD and concomitant autoimmune encephalitis such as lupus cerebritis in patients presenting with neuropsychiatric manifestations.

Authors/Disclosures
Samantha Plum, MD (Prisma Health Upstate) PRESENTER	Dr. Scoville has nothing to disclose.
Saher Choudhary, MD (Prisma Health–Upstate/USC–SOM Greenville)	Dr. Choudhary has nothing to disclose.

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