Optic neuromyelitis (ONM) or Devic's syndrome is an inflammatory demyelinating central nervous system (CNS) disease, defined by the presence of optic neuritis, transverse myelitis affecting 3 or more spinal segments and seropositivity for antibodies directed against aquaporin 4 of astrocytes (NMO-IgG), in the absence of signs of brain lesions suggestive of multiple sclerosis (MS).1

We report the case of a 34-year-old woman diagnosed with lupus erythematosus (SLE) at age 18. During the course of the disease she presented malar rash, polyarthritis, serositis, positive antinuclear antibodies (ANA), anti-native DNA antibodies (anti-DNA) and several episodes of glomerulonephritis, for which she received pulse intravenous (iv) cyclophosphamide and maintenance therapy with azathioprine, suspended when she was 29 and remained stable.

Her initial chief complaint was dysphagia caused by esophageal diverticulum. At that time she received prednisone (7.5mg daily) and hydroxychloroquine (400mg daily). After 48h, she had an episode of acute low back pain and paraparesis and a few hours later it became paraplegia with areflexia, tactile and painful hypesthesia, abolished sense of position and a distended bladder. The exploration of the upper extremities was normal. To this a total blindness of the left eye (LE) and a significant decrease in visual acuity of the right eye (RE) was added. We performed magnetic resonance imaging (MRI) of brain and orbit which showed findings consistent with bilateral optic neuritis. The thoracolumbar spine MRI disclosed a lesion compatible with transverse myelitis in the segment between the D7 vertebral body and the conus medullaris. The cerebrospinal fluid analysis revealed 576leukocytes/mm3 with 95% polymorphonuclear and 0 erythrocytes/mm3. Proteins were 347.79mg/dl with normal glucose. Cultures, Gram stain, smear, Lowenstein culture and herpes simplex 1 and 2, varicella zoster and enterovirus PCR testing were negative. The ADA was 17.1U/l. Ophthalmologic examination revealed a bilateral afferent pupillary defect and a deficit in bilateral visual acuity (perception of large forms and hard colors in total amaurosis of the LE and RE). The fundus was normal. ANA titer was 1/1.280 and anti-DNA Ab 54U/ml. Extractable core antibodies were negative. Complement levels were low. Coagulation was normal and anticardiolipin, antiphospholipid antibodies and lupus anticoagulant were negative. We established a diagnosis of ONM and started treatment with methylprednisolone 1g iv/day for 5 consecutive days, 1g iv cyclophosphamide and 1g/kg iv immunoglobulin for 2 consecutive days. This was repeated a month later. Five weeks later, the patient was able to distinguish large forms with her LE and to distinguish colors and count fingers with the RE. There was no improvement in mobility of the lower extremities. Cervical, thoracic and lumbar spine MRI was performed, where 2 multisegmental intramedullary lesions were observed: the first one located from D7 to the conus medullaris and showed no changes from the previous MRI, and the second in the left posterolateral region of the conus medullaris at C4–C5, both compatible with transverse myelitis.

After 6 years with continued azathioprine treatment, the patient remained paraplegic and with a significant visual deficit (blurred RE vision and able to distinguish large forms with the LE). She has had no new episodes of ONM.

ONM is a rare and severe CNS demyelinating inflammatory condition characterized by the coexistence of optic neuritis and transverse myelitis. The description in 2004 of the IgG-NMO1 allowed the establishment, in 2006, of a new diagnostic criteria to distinguish this disease from MS.2 It is estimated that the annual incidence of ONM is 0.4 cases per 100000 persons/year and prevalence in Caucasians is 4.4 cases per 100000 population.3 The course can be a single episode or recurrent and, although it may be idiopathic, it is often associated with autoimmune diseases such as SLE or Sjögren's syndrome, presence of autoantibodies, infectious agents and exposure to drugs. The involvement of B lymphocytes with the participation of NMO-IgG in relapses4 has been proposed as contributing to its pathogenesis. MRI reveals hyperintense T2 images in 3 or more spinal cord segments and optic nerve damage that is enhanced after gadolinium administration. These findings differ from those observed in MS.5 The immediate start of aggressive treatment with high dose IV methylprednisolone and cyclophosphamide is essential to reduce the consequences of acute episodes.6 Azathioprine has been shown to reduce recurrence,7 although the prognosis is generally unfavorable.