Abstract Details

Title Late-onset Neuromyelitis Optica Spectrum Disorder: The Importance of the Serostatus

Topic Autoimmune Neurology

Presentation(s) S11 - Autoimmune CNS Inflammatory Disorders: Clinical Advances (4:14 PM-4:25 PM)

Poster/Presentation
Number 005

Background The information about clinical features and outcomes of patients with LO-NMOSD (age at onset ≥ 50 years) is limited and mostly based on AQP4-IgG-positive patients.

Objective To (1) describe the clinical features associated with late-onset neuromyelitis optica spectrum disorder (LO-NMOSD) according to the serostatus, and (2) to identify predictors of disability.

Design/Methods This was a retrospective, multicenter study of 238 patients who fulfilled the 2015 NMOSD criteria. Data were collected from January 2013 to 2018 using medical records and a specific questionnaire for NMOSD. Patients with an early-onset NMOSD (EO-NMOSD, age at onset < 50 years) were compared to those with LO-NMOSD according to their serostatus. Samples were tested for AQP4-IgG and MOG-IgG using cell-based assays.

Results Sixty-nine (29%) patients had LO-NMOSD: 60 (87%) had AQP4-IgG, 5 (7.2%) had MOG-IgG and 4 (5.8%) were double seronegative. Compared to EO-NMOSD-AQP4-IgG-positive patients, those with LO-NMOSD-AQP4-IgG-positive had a significant lower female:male ratio (11:1 vs 4:14), and a higher risk to reach an Expanded Disability Status Scale (EDSS) of 6.0 (hazard ratio, HR, 2.05, 95% CI 1.2-3.3) but similar annualized relapse rate. No clinical or prognostic differences were found between EO-NMOSD and LO-NMOSD associated with MOG-IgG. LO-NMOSD-double-seronegative patients had a higher risk to reach an EDSS of 6.0 (HR, 12, 95% CI 4.1-34.7) than EO-NMOSD-double-seronegative patients. In patients with LO-NMOSD, double seronegativity increased 5-fold the risk of reaching an EDSS of 6.0 as compared to those with AQP4-IgG-seropositivity.

Conclusions Up to 30% of patients with NMOSD have a late onset, most of them associated with AQP4-IgG, and have a worse disability outcome compared to patients with an early onset. In NMOSD, a late-onset associated with double seronegativity confers the worst prognosis.

Authors/Disclosures
PRESENTER	No disclosure on file
Maria Sepúlveda	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Irene Pulido Valdeolivas	No disclosure on file
Eugenia Martinez-Hernandez, MD (Hospital Clinic Barcelona)	Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. The institution of Dr. Martinez-Hernandez has received research support from Instituto de Salud Carlos III. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with Sanofi. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings and speaker honoraria with UCB. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a speaker honoraria with Alexion. Dr. Martinez-Hernandez has received personal compensation in the range of $0-$499 for serving as a speaker honoraria with Terumo. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with Janssen. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with CSL Behring.
	No disclosure on file
Sara Llufriu	Sara Llufriu has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Sara Llufriu has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Sara Llufriu has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merci. Sara Llufriu has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Abbot. Sara Llufriu has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi.
Francesc R. Graus, MD (IDIBAPS)	Dr. Graus has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for MedLink. Dr. Graus has received intellectual property interests from a discovery or technology relating to health care.
Albert Saiz (Hospital Clinico De Barcelona)	Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janseen. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for novartis. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen.

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