Poster abstracts

104 Overlapping autoimmunity: a case of concomitant aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) antibody positivity in neuromyelitis optica spectrum disorder

Abstract

Objectives To describe a rare case of double antibody positive Neuromyelitis Optica Spectrum Disorder (NMOSD) with both Aquaporin-4 and MOG antibodies, occurring following a Pertussis infection in a patient with a history of auto-immunity.

Methods Retrospective review of clinical records.

Results A 41-year-old Chinese woman with a history of Systemic Lupus Erythematosus presented with a sub-acute onset of progressive gait ataxia and urinary retention occurring seven days after a confirmed Bordetella pertussis infection. Magnetic resonance imaging revealed extensive subcortical and thalamic T2/FLAIR hyperintensities with subtle enhancement, and a longitudinally extensive non-enhancing spinal cord lesion (T1-T7), without optic nerve involvement. Cerebrospinal fluid protein was raised (0.55 g/L) with 7 mononuclear cells and matched oligoclonal bands. Viral PCRs were negative including JC virus and Pertussis. Established live cell-based immunoassays revealed positivity for both Aquaporin-4 antibodies (in CSF and serum) and MOG antibodies in high titres. Our patient meets the 2015 Consensus Diagnostic Criteria for NMOSD.1 Treatment with high dose corticosteroids and rituximab lead to clinical and radiological improvement, but she had a clinical relapse 10 months later with new LETM (T3-T7), necessitating increased immunosuppression with more rigorous rituximab dosing of 1000mg every 6 months.

Conclusions Double positivity for both Aquaporin-4 and MOG antibodies in NMOSD is rare. We describe a case of double-positive NMOSD occurring following an infective illness. This case demonstrates that NMOSD may occasionally masquerade as post-infectious Acute Disseminated Encephalomyelitis and highlights the importance of checking antibodies in these patients, given the treatment strategies and risk of relapse differs considerably.

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