Email updates

Keep up to date with the latest news and content from Journal of Medical Case Reports and BioMed Central.

Open Access Highly Accessed Case report

Severe optic neuritis in a patient with combined neuromyelitis optica spectrum disease and primary Sjögren’s syndrome: a case report

Petrina Tan1*, Wai Yung Yu2, Thirugnanam Umapathi3 and Su-Ann Lim1

Author Affiliations

1 National Healthcare Group Eye Institute, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore

2 Department of Neuroradiology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore

3 Department of Neurology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore

For all author emails, please log on.

Journal of Medical Case Reports 2012, 6:401  doi:10.1186/1752-1947-6-401

Published: 24 November 2012

Abstract

Introduction

Optic neuritis, although uncommon, can be the initial presentation of Sjögren’s syndrome. Coexisting Sjögren’s syndrome has also been reported with neuromyelitis optica spectrum disorder. This case report highlights the association between the two diseases and the importance of rheumatological and neurological evaluations in patients with such diagnoses. Distinction of neuromyelitis optica with coexisting connective tissue disease has both prognostic and therapeutic significance for the patient.

Case presentation

We report a case of a 56-year-old Chinese woman who presented with bilateral asymmetric visual loss secondary to optic neuritis. She was subsequently found to be seropositive for neuromyelitis optica immunoglobulin G (NMO-IgG) (anti-aquaporin-4 antibody) and was diagnosed with neuromyelitis optica spectrum disorder. She also fulfilled the international criteria for Sjögren’s syndrome. Despite initial high dose immunosuppressive therapy, she failed to regain vision in one eye.

Conclusion

Patients presenting with optic neuritis and severe visual loss should be screened for neuromyelitis optica and treated appropriately. Neuromyelitis optica has been associated with systemic autoimmune diseases, in particular Sjögren’s syndrome, and current evidence indicates that they are two distinct entities. We recommend that both diagnoses be considered in cases of optic neuritis with severe visual loss.