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

Title: A score that predicts aquaporin-4 IgG positivity in patients with longitudinally extensive transverse myelitis
Authors: Campetella, Lucia; Papi, Claudia; Spagni, Gregorio; Sabatelli, Eleonora; Iorio, Raffaele
Contributors: Campetella, Lucia; Papi, Claudia; Spagni, Gregorio; Sabatelli, Eleonora; Mariotto, Sara; Gastaldi, Matteo; Masi, Gianvito; Carta, Sara; Ahmad, Lara; Rossi, Francesca; Maniscalco, Giorgia Teresa; De Luca, Giovanna; Iorio, Raffaele
Publication Year: 2023
Collection: Università Cattolica del Sacro Cuore: PubliCatt
Subject Terms: aquaporin 4; myelitis; transverse; neuromyelitis optica; Settore MED/26 - NEUROLOGIA
Description: 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.
Document Type: article in journal/newspaper
Language: English
Relation: info:eu-repo/semantics/altIdentifier/pmid/37170839; info:eu-repo/semantics/altIdentifier/wos/WOS:000995290800001; volume:30; issue:8; firstpage:2534; lastpage:2538; numberofpages:5; issueyear:2023; journal:EUROPEAN JOURNAL OF NEUROLOGY; https://hdl.handle.net/10807/240297
DOI: 10.1111/ene.15863
Availability: https://hdl.handle.net/10807/240297; https://doi.org/10.1111/ene.15863
Rights: info:eu-repo/semantics/openAccess ; license:Creative commons ; license uri:http://creativecommons.org/licenses/by-nc-nd/4.0/
Accession Number: edsbas.8238372E
Database: BASE