Journal List > J Korean Ophthalmol Soc > v.54(9) > 1009492

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Ahn and Kim: A Case of Coexisting Neuromyelitis Optica in Systemic Lupus Erythematosus
Original Article

J Korean Ophthalmol Soc 2013;54(9):1469-1474.

Published online: 7 September 2013

DOI: https://doi.org/10.3341/jkos.2013.54.9.1469

A Case of Coexisting Neuromyelitis Optica in Systemic Lupus Erythematosus

Seong Min Ahn, MD, Sang Soo Kim, MD

Department of Ophthalmology, Maryknoll Medical Center, Busan, Korea

Address reprint requests to Sang Soo Kim, MD Department of Ophthalmology, Maryknoll Medical Center,#121 Junggu-ro, Jung-gu, Busan 600-730, Korea Tel: 82-51-461-2469, Fax: 82-51-462-3534 E-mail: eyerheu@hanafos.com

∗ This case was presented as an e-poster at the 27th Joint Congress of the APAO/SOE Busan 2012.

Received 6 April 20122 May 2013       Accepted 17 July 2013

Copyright © 2013 Korean Ophthalmological Society

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

We present a case of a patient with coexisting neuromyelitis optica and systemic lupus erythematosus (SLE).

Case summary

A 26-year-old female was hospitalized in our medical center due to decreased visual acuity in her left eye; she had a history of gastric ulcers and herpes zoster infection. Steroid treatment was started under suspicion of optic neu-ritis, and she was diagnosed with SLE. After treatment, her vision improved, but eleven months later she was hospitalized with paresthesia on the abdomen and left flank progressing to the lower extremities. Spinal MRI showed transverse myeli-tis, suggesting multiple sclerosis. Fifteen months later, the patient was hospitalized due to decreased visual acuity and oc-ular pain in the right eye. Her vision was improved by steroid therapy. However, optic neuritis recurred in the right eye after five weeks, thus azathioprine was added to the treatment. Anti-aquaporin-4 Ab test was conducted based on the suspicion of neuromyelitis optica, and the serum was positive for anti-aquaporin-4 Ab (NMO-IgG). The patient was hospitalized again due to paraplegia after three months. Coexistence of neuromyelitis optica was verified because spinal MRI showed longitudinally extensive transverse myelitis. The symptoms were improved by high doses of steroids, a series of plasma-phereses, and rituximab. Optic neuritis was repeated in the right eye and the symptoms were improved with high doses of steroids. Myelitis recurred later and the symptoms improved with high doses of steroids and a series of plasmaphereses.

Conclusions

Coexisting neuromyelitis optica should be considered in cases with relapsing events which have transverse myelitis without cranial lesions in autoimmune diseases such as SLE.

Keywords: Devic’, s syndrome, Multiple sclerosis, Neuromyelitis optica, Optic neuritis, Systemic lupus erythematosus

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Figure 1.
The picture shows the mild enhancement of left optic nerve (arrow). It suggests that the prominent finding may be left intraorbital optic neuritis.
jkos-54-1469f1.tif
Figure 2.
The picture shows that subtle high signal in cord of C2 and C3 level (arrows) on T2-weighted image. These finding is likely to acute transeverse myelitis in spinal cord of C2-C6 level. It suggests that these findings may be multiple slclerosis.
jkos-54-1469f2.tif
Figure 3.
T2-weighted sequences of spinal cord MR. (A) The picture shows high signal lesion in cord of C2 and C3 level and more extensive lesion in cord of C-spine below C4 and upper T-spine level (arrows). (B) The picture shows the long segment intra-medullary increased signal in spinal cord from C4 to T10 level (arrowheads). It suggests that the finding is likely to neuromyelitis optica.
jkos-54-1469f3.tif
Table 1.
Proposed diagnostic criteria for optic neuromyelitis2
Definite NMO
Optic neuritis
Acute myelitis
At least two of three supportive criteria
Contiguous spinal and MR lesion extending ≥3 vertebral segments Brain MR not meeting diagnostic criteria for multiple sclerosis NMO-IgG seropositive status
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