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070  What is seronegative neuromyelitis optica spectrum disorder?
  1. Emily Gibbons1,
  2. Giovanna Campgana1,
  3. Karen O’Connell2,
  4. Tianrong Yeo2,
  5. Daniel Whittam1,
  6. Venkatra- man Karthikeayan1,
  7. Maria Isabel Leite2,
  8. Jacqueline Palace2,
  9. Anu Jacob3,
  10. Saif Huda1
  1. 1The Walton Centre for Neurology and Neurosurgery, Liverpool
  2. 2Nuffield Department of Clinical Neu- rosciences, John Radcliffe Hospital, Oxford
  3. 3Cleveland Clinic, Abu Dhabi and The Walton Centre for Neurology and Neurosurgery, Liverpool

Abstract

Background Most but not all cases of Neuromyelitis Optica Spectrum Disorder (NMOSD) are associated with Aquaporin-4 (AQP4) or myelin oligodendrocyte glycoprotein (MOG) antibodies.

Objective To determine the clinical characteristics of seronegative NMOSD

Method Retrospective review of seronegative NMOSD in Liverpool and Oxford between January 2010–2020

Results Of NMOSD=727, 49(7%) were seronegative. The male to female ratio was 1:2.5 and median age at onset was 36(5–57) years. In 2/3 of patients the index presentation was myelitis=22 or myelitis+optic neuritis=11. In 26/33 (79%), longitudinally extensive myelitis was present. Optic neuritis=9 (4 bilateral) and brain involvement=7 were also seen. Relapsing disease was observed in 39/49(80%) of patients. The median annualised attack rate was 0.58 over a median disease duration of 78 (3–258) months. Unmatched CSF oligoclonal bands (CSF-OCBs) were detected in 4/38(11%) and 31/49(63%) fulfilled multiple sclerosis (MS) diagnostic criteria. Immunosuppression (typically Mycophenolate and Rituximab) was used in 34/49(69%). Median last EDSS was 4 (1–10) with death recorded in 5/49 (10%) patients.

Conclusion Seronegative NMOSD is uncommon. Longitudinal myelitis with/without optic neuritis is a common initial presentation. Similar to AQP4-IgG, NMOSD disability and mortality rates are high. Absence of unmatched CSF-OCB and typical brain lesions help to distinguish this disease from MS.

emily.gibbons@doctors.org.uk

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