The absence of antibodies in longitudinally extensive transverse myelitis may predict a more favourable prognosis

Mult Scler. 2024 Mar;30(3):345-356. doi: 10.1177/13524585231221664. Epub 2024 Jan 23.

Abstract

Background: Isolated first episodes of longitudinally extensive transverse myelitis (LETM) have typically been associated with neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). However, in some cases, serological testing and screening for other aetiologies are negative, a condition referred to as double seronegative longitudinally extensive transverse myelitis (dsLETM).

Objective: The objective of this study was to evaluate comparative outcomes of dsLETM, MOGAD-LETM and NMOSD-LETM.

Methods: Cohort study of LETM cases seen in the UK NMOSD Highly Specialised Service between January 2008 and March 2022.

Results: LETM = 87 cases were identified (median onset age = 46 years (15-85); median follow-up = 46 months (1-144); 47% NMOSD-LETM = 41 (aquaporin-4 antibodies (AQP4-IgG) positive = 36), 20% MOGAD-LETM = 17 and 33% dsLETM = 29). Despite similar Expanded Disability Status Scale (EDSS) at nadir, last EDSS was higher in AQP4-IgG and seronegative NMOSD-LETM (sNMOSD) (p = 0.006). Relapses were less common in dsLETM compared to AQP4-IgG NMOSD-LETM and sNMOSD-LETM (19% vs 60% vs 100%; p = 0.001). Poor prognosis could be predicted by AQP4-IgG (odds ratio (OR) = 38.86 (95% confidence interval (CI) = 1.36-1112.86); p = 0.03) and EDSS 3 months after onset (OR = 65.85 (95% CI = 3.65-1188.60); p = 0.005).

Conclusion: dsLETM remains clinically challenging and difficult to classify with existing nosological terminology. Despite a similar EDSS at nadir, patients with dsLETM relapsed less and had a better long-term prognosis than NMOSD-LETM.

Keywords: Expanded Disability Status Scale; Longitudinally extensive transverse myelitis; double seronegative transverse myelitis; myelin oligodendrocyte antibody‚Äďassociated disease; neuroimmunology; neuromyelitis optica spectrum disorders.

MeSH terms

  • Aquaporin 4
  • Autoantibodies
  • Cohort Studies
  • Humans
  • Immunoglobulin G
  • Middle Aged
  • Myelin-Oligodendrocyte Glycoprotein
  • Myelitis, Transverse*
  • Neoplasm Recurrence, Local / complications
  • Neuromyelitis Optica*
  • Prognosis
  • Retrospective Studies

Substances

  • Aquaporin 4
  • Autoantibodies
  • Immunoglobulin G
  • Myelin-Oligodendrocyte Glycoprotein