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Título

NEUROMYELITIS OPTICA SPECTRUM DISORDER (NMOSD) ASSOCIATED WITH PRIMARY ANTIPHOSPHOLIPID SYNDROME: A CASE REPORT

RESUMO

Case presentation: A previously healthy woman aged 25 years reported that one and a half years ago, left-sided paresthesia up to her breast evolving to the right side in a few days, followed by painful sensations in both legs, urinary retention, and bilateral weakness limiting her to walk. She was diagnosed with a deep vein thrombosis in the left leg and received oral anticoagulation for six months. She was able to walk after 30 days without treatment. One month later, she presented a sudden visual loss in the right eye with progressive improvement after 15 days. After five months, a mild episode of paresthesia and weakness in both legs, with spontaneous improved after a few days. On neurologic exam during a new severe episode revealed clinical signs of acute transverse thoracic myelitis with no muscle strength in both legs and anesthesia up to T4 spinal level. Intermittent bladder catheterization was required (high urinary residue). Cranial nerves were normal, including visual function. Spinal cord images showed signs of myelitis affecting C7-T1 and T8-T9 levels with slight contrast enhancement. The cerebrospinal fluid analysis showed mildly elevated cell count and protein level with no signs of infections. Oligoclonal bands were not done. There was a positive aquaporin-4 antibodies test (1:80). Oral anticoagulation was indicated again due to lupus anticoagulant positivity. Visual evoked potential showed signs of visual pathway dysfunction in both eyes. She received five days of intravenous methylprednisolone followed by seven sections of plasmapheresis. Muscle strength improved to grade 3 in both legs, and she recovered truncal and bladder control. She is currently receiving initial doses of rituximab. Discussion: Recurrent transverse myelitis, optic neuropathy, and positive aquaporin-4 antibody indicate NMOSD in this patient. Previous deep venous thrombosis in association with a lupus anticoagulant are features of the antiphospholipid syndrome (APS). The association between NMOSD and primary APS is rare, although autoantibodies related to APS had been often reported in patients with NMOSD. Pathophysiological mechanisms are complex, and both diseases may overlap, affecting the nervous system. Cooccurrence of both conditions increases morbidity and unfavorable outcomes. Final comments: NMOSD is a severe disease, and comorbidity with rheumatological diseases may occur and worsen the prognosis. Further studies are necessary to understand this relationship.

Palavras Chave

Neuromyelitis Optica Spectrum Disorder, Transverse Myelitis, Antiphospholipid Syndrome, Case Report