To report an atypical case of neuromyelitis optica (NMO) with Sjögren syndrome that presented only ileus as a neurologic finding.
A thirty two-year-old woman was hospitalized for abnormal abdominal symptoms, and was diagnosed as having paralytic ileus. Dry eye symptoms due to Sjogren syndrome were also observed, and visual acuity OU decreased suddenly 10 days after hospitalization. The best corrected visual acuity was 0.04 OD and 0.6 OS, and the optic discs OU were swollen and red. Although no neurologic signs were found, pleocytosis and abnormal MRI findings suggested NMO. Steroid pulse therapy was performed, and after three cycles, the ileus symptoms disappeared, and the disturbed visual acuity OU returned to normal. Because the anti-aquaporin 4 antibody was positive and no abnormal abdominal findings that might have caused ileus were found, we diagnosed NMO presenting only paralytic ileus as a neurologic finding.
Differential diagnosis is important for NMO initiated by ileus as the only neurologic sign.