A score that predicts aquaporin-4 IgG positivity in patients with longitudinally extensive transverse myelitis.

Abstract

BACKGROUND AND PURPOSE:

Longitudinally extensive transverse myelitis (LETM) associated with aquaporin-4 autoantibodies (AQP4-IgG) can cause severe disability. Early diagnosis and prompt treatment are critical to prevent relapses. A novel score is described based on clinical and neuroimaging characteristics that predicts AQP4-IgG positivity in patients with LETM.

METHODS:

Patients were enrolled both retrospectively and prospectively from multiple Italian centers. Clinical and neuroimaging characteristics of AQP4-IgG positive and negative patients were compared through univariate and multivariate analysis.

RESULTS:

Sixty-six patients were included. Twenty-seven (41%) were AQP4-IgG positive and median age at onset was 45.5 years (range 19-81, interquartile range 24). Female sex (odds ratio [OR] 17.9, 95% confidence interval [CI] 2.6-381.9; p = 0.014), tonic spasms (OR 45.6, 95% CI 3.1-2197; p = 0.017) and lesion hypointensity on T1-weighted images (OR 52.9, 95% CI 6.8-1375; p = 0.002) were independently associated with AQP4-IgG positivity. The AQP4-IgG positivity in myelitis (AIM) score predicted AQP4-IgG positivity with 85% sensitivity and 95% specificity. Positive and negative likelihood ratios were 16.6 and 0.2 respectively. The inter-rater and intra-rater agreement in the score application were both excellent.

CONCLUSIONS:

The AIM score predicts AQP4-IgG positivity with good sensitivity and specificity in patients with a first episode of LETM. The score may assist clinicians in early diagnosis and treatment of AQP4-IgG positive LETM.

• Authors: Lucia Campetella, Claudia Papi, Gregorio Spagni, Eleonora Sabatelli, Sara Mariotto, Matteo Gastaldi, Gianvito Masi, Sara Carta, Lara Ahmad, Francesca Rossi, Giorgia Teresa Maniscalco, Giovanna De Luca, Raffaele Iorio
• Journal: European journal of neurology (Eur J Neurol) Vol. 30 Issue 8 Pg. 2534-2538 (08 2023) ISSN: 1468-1331 [Electronic] England
• PMID: 37170839 (Publication Type: Journal Article)
• Copyright: © 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

Chemical References

• Aquaporin 4
• Immunoglobulin G
• Autoantibodies

Topics

• Humans
• Female
• Young Adult
• Adult
• Middle Aged
• Aged
• Aged, 80 and over
• Myelitis, Transverse (diagnostic imaging)
• Neuromyelitis Optica (diagnostic imaging)
• Retrospective Studies
• Neoplasm Recurrence, Local
• Aquaporin 4
• Immunoglobulin G
• Autoantibodies