J Korean Rheum Assoc.  2007 Sep;14(3):263-267. 10.4078/jkra.2007.14.3.263.

A Case of Systemic Lupus Erythematous Associated with Neuromyelitis Optica (Devic's Syndrome)

• Oh PC
• Kim GH
• Jin CH
• Baek HJ

• KMID: 2202176
• DOI: http://doi.org/10.4078/jkra.2007.14.3.263

Abstract

Neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating disease, characterized by optic neuritis and myelitis. NMO is a very uncommon and serious neurologic manifestation of systemic lupus erythematous (SLE). We report a 28-year-old man with NMO as neuropsychiatric manifestation of SLE. He was diagnosed as lupus nephritis at 16-year-old. He had optic neuritis at three years and seven months ago. Oral prednisolone was tapered off according to the improved eye symptoms. Two months later, he visited rheumatology clinics for urinary disturbance and paresthesia on both feet. A spinal magnetic resonance imaging revealed increased signal intensity in T2-weighted images from second to sixth cervical level and from eleventh to twelfth thoracic level. We diagnosed neuromyelitis optica and treated with intravenous cyclophosphamide therapy monthly for three times. He was discharged without any neurological deficits and has been followed up.

Keyword

Systemic lupus erythematous; Neuromyelitis optica; Devic's syndrome

MeSH Terms

Adolescent
Adult
Cyclophosphamide
Demyelinating Diseases
Foot
Humans
Lupus Nephritis
Magnetic Resonance Imaging
Myelitis
Neurologic Manifestations
Neuromyelitis Optica*
Optic Neuritis
Paresthesia
Prednisolone
Rheumatology
Cyclophosphamide
Prednisolone

Figure

• 

  Fig. 1. (A) Fundus photography as the frist episode of optic neuritis. Left fundus photography shows the hyperemia, edematous change and blurring around optic disc and right fundus photography shows normal optic disc. (B) Fundus photography as the second episode of optic neuritis. Right fundus photography shows the hyperemia around optic disc and left fundus photography shows the pale optic disc.

• 

  Fig. 2. Sagittal T2-weighted magnetic resonance image of the cervical and thoracic spine. (A) High signal intensity from C2 to C6, (B) high signal intensity and cord swelling from T11 to T12.

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