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

A Case of Coexisting Neuromyelitis Optica in Systemic Lupus Erythematosus

• Ahn SM
• Kim SS

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 neuritis, 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 myelitis, suggesting multiple sclerosis. Fifteen months later, the patient was hospitalized due to decreased visual acuity and ocular 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 plasmaphereses, 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.

Keyword

Devic's syndrome; Multiple sclerosis; Neuromyelitis optica; Optic neuritis; Systemic lupus erythematosus

MeSH Terms

Abdomen
Adult
Antibodies, Monoclonal, Murine-Derived
Autoimmune Diseases
Azathioprine
Eye
Female
Herpes Zoster
Humans
Lower Extremity
Lupus Erythematosus, Systemic
Multiple Sclerosis
Myelitis
Myelitis, Transverse
Neuromyelitis Optica
Optic Neuritis
Paraplegia
Paresthesia
Plasmapheresis
Steroids
Stomach Ulcer
Vision, Ocular
Visual Acuity
Rituximab
Antibodies, Monoclonal, Murine-Derived
Azathioprine
Steroids

Figure

• 

  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.

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  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.

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  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.

Reference

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