Demyelinating Syndrome in Systemic Sclerosis and Neuromyelitis Optica

Abstract INTRODUCTION: The objective of this study is to report a case of a 44-year-old female who presented with intractable hiccups, vomiting, and later complicated with paraplegia. Imaging and sero/immunological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity and transverse myelitis on MRI. Further investigation revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes, namely systemic sclerosis and neuromyelitis optica, and their underlying clinical manifestations and therapeutic interventions, are seldom reported in literature and are worth reporting. CASE REPORT: The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified as steroid induced scleroderma renal crisis on renal biopsy. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received consent forms from the participant in our study, and we have them on file in case they are requested. We have also received patient’s consent for the data presented in this article and Figure 1. CONCLUSION: This report expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.