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Abstract

Neuromyelitis optica (sNMO) or Devic's disease, is a chronic, demyelinating, autoimmune and inflammatory disease of the central nervous system (CNS), that affects predominantly the optic nerve and the spinal cord. Recently unified under a broader term, NMOSD (NMO spectrum disorders), 80% of patients with this disease present detectable serum antibodies, very specific and pathogenomic, that target the water channels aquaporin-4, called AQP4-IgG. Classified as a rare genetic disease with multifactorial origin, NMO affects predominantly females and young adults. The case we present in this study represents an exception. Our patient was diagnosed with NMO at 72, having been previously hospitalized three times, two with transverse cervical myelitis and one with binocular diplopia. These episodes did not leave sequealae and where considered of ischemic origin considering a past cerebro vascular accident. However, the patient presented four more episodes of NMO after his definitive diagnose. In the first two of these attacks, AQP4-IgG testing was requested and positive, with values of 5.31 and 1.62 respectively. No testing for antibodies were requested during his other two attacks. In total, this patient suffered eight attacks in 10 years, which included five attacks with transverse spinal cord myelitis, two with binocular diplopia, and one with dorsal transverse myelitis. The purpose of our paper is to describe this case in detail and to discuss the clinical and neurophysiological testing, genetics, immunology and treatment options available for NMOSD, with the intention of give it more visibility and improve early diagnosis, and considering the long-term debilitating effects that it can have in the patients.