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CASE REPORT
Seronegative neuromyelitis optica spectrum disorder: severe polysymptomatic presentation with successful treatment response
  1. Gilberto Pires da Rosa1,
  2. Francisca Costa2,
  3. Joana Guimarães3,4 and
  4. Fernando Friões1,5
  1. 1 Intermediate Care Unit, Department of Internal Medicine, Centro Hospitalar Universitário de São João, Porto, Portugal
  2. 2 Department of Neuroradiology, Centro Hospitalar Universitário de São João, Porto, Portugal
  3. 3 Department of Neurology, Centro Hospitalar Universitário de São João, Porto, Portugal
  4. 4 Neurology and Neurosurgery Unit of Clinical Neurosciences and Mental Health Department, Faculty of Medicine, University of Porto, Porto, Portugal
  5. 5 Cardiovascular Research Centre, University of Porto Medical School, Porto, Portugal
  1. Correspondence to Dr Gilberto Pires da Rosa, gilbertopiresrosa{at}gmail.com

Abstract

We report the case of a 50-year-old caucasian man presenting with lumbar pain, bilateral ataxia, central facial palsy, ophthalmoparesis and urinary retention. Cerebral MRI hinted a hyperintensity of the medulla oblongata and cervical medulla suggestive of myelitis. Cerebrospinal fluid displayed lymphocytic pleocytosis and elevated protein concentration. Without the possibility to rule out an infectious or inflammatory aetiology, antibiotics and corticosteroids were started. Nevertheless, neurological status deteriorated with loss of muscle strength and left eye amaurosis. A neuroaxis MRI exhibited encephalomyelitis with signal abnormalities involving the pons, medulla oblongata, left optic nerve and cervicodorsal medulla. Although negative for aquaporin-4-IgG antibodies, the patient fulfilled criteria for seronegative neuromyelitis optica spectrum disorder with the presence of multiple core clinical characteristics. Through early institution of corticosteroids, plasma exchange and rituximab, good functional recovery was achieved (Expanded Disability Status Scale score of 2). However, left eye amaurosis persisted despite salvage therapy with intravenous immunoglobulin.

  • immunology
  • neurology
  • brain stem / cerebellum
  • neuroimaging
  • spinal cord
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Footnotes

  • Contributors GPdR: active role in the hospitalisation of the patient (diagnosis and therapeutic decisions), review of literature and elaboration of the manuscript. FC: active role in the hospitalisation of the patient (diagnosis—neuroimaging), selection and labeling of the images used in the case report, review and completion of the manuscript. JG: active role in the hospitalisation of the patient (diagnosis and therapeutic decisions), review and completion of the manuscript, follow-up of the patient (consultation). FF: active role in the hospitalisation of the patient (diagnosis and therapeutic decisions), review of literature, elaboration and review of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.

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