Background: The coexistence of neuromyelitis optica spectrum disorders (NMOSD) with other autoimmune diseases (AID) has been increasingly reported. The prevalence and significance of this association are not fully understood.
Objectives: This study aimed to compare the clinical and laboratory characteristics in NMOSD patients with and without AID.
Methods: Retrospective cross-sectional observational study was conducted involving adults meeting NMOSD criteria followed in a neuroimmunology clinic at a tertiary center. Descriptive analysis of clinical/paraclinical/treatment/outcome data collected from the medical records was compared between NMOSD patients with AID (polyautoimmunity) and those without AID.
Results: From a cohort of 46 NMOSD patients, 16 (34.8 %) patients, mostly women around 40 years of age, presented with polyautoimmunity: 10 anti-AQP4 positive, 4 anti-MOG positive, and 2 seronegative. Five different organ -specific AID, and six systemic AID were identified in the polyautoimmunity patients group, in addition to 6 cases of multiple autoimmune syndrome. The AID manifestation preceded NMOSD in 10 (62.5 %) patients, with a median interval of 7 years. The NMOSD with polyautoimmunity and NMOSD without AID groups had similar initial clinical manifestations with optic neuritis and/or myelitis being most frequent. Inflammatory CSF, namely elevated proteins, was more common in the polyautoimmunity group (13.0 % in NMOSD vs. 31.3 % in NMOSD+AID, p = 0.003). After a 10±6 years follow-up period, more patients with polyautoimmunity had a relapsing disease (75.0 % in NMOSD vs. 46.7 % in NMOSD+AID, p = 0.012) but no difference in the functional outcome evaluated by the EDSS score was identified.
Conclusions: Polyautoimmunity was common in AQP4 positive NMOSD patients leading to a significantly higher risk of disease recorrence. The presence of polyautoimmunity and multiple autoimmune syndrome in NMOSD patients suggests the existence of common susceptibility factors or pathophysiological mechanisms, emphasizing the importance of a multidisciplinary approach to those patients.
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http://dx.doi.org/10.1016/j.msard.2024.105848 | DOI Listing |
Mult Scler Relat Disord
December 2024
Department of Neurology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
Introduction: Longitudinally extensive spinal cord lesions (LESCL) are characterized by T2-hyperintense signals spanning at least three vertebral body segments, with neuromyelitis optica spectrum disorders (NMOSD) being a significant cause. This study aimed to characterize the clinical, radiological, serological, and cerebrospinal fluid (CSF) features of LESCL and to compare NMOSD and non-NMOSD cases.
Methods: We conducted a retrospective cross-sectional study of adult patients diagnosed with LESCL at our center over a twelve-year period collecting data on demographics, clinical presentations, MRI findings, CSF analysis, and serological testing for AQP4-IgG and MOG-IgG antibodies.
J Neurol Sci
December 2024
Center for Advanced Neurological Research, Nitte University, Mangalore,India.
Background: Among white populations, a poly-specific antibody response against measles (M), rubella (R) and varicella zoster(Z) otherwise known as MRZR is seen in ∼70 % of MS and rarely in other demyelinating disorders. While the basis for MRZR is unclear, vaccination exposure / community acquired infections may have an influence on its frequency.
Objective: To determine the frequency and specificity of MRZR in MS and related disorders in a non- white population with historically low vaccinations and to contrast against oligoclonal bands (OCB).
Front Immunol
December 2024
Department of Neurology, University of Virginia, Charlottesville, VA, United States.
Background: We evaluated comprehension and application of the 2015 neuromyelitis optica spectrum disorder (NMOSD) criteria core elements by neurologists in Latin America (LATAM) who routinely diagnose and care for NMOSD patients by (i) identifying typical/suggestive NMOSD syndromes, (ii) detecting typical MRI NMOSD lesions and meeting MRI dissemination in space (DIS) criteria, and (iii) evaluating historical symptoms suggestive of NMOSD.
Methods: We conducted an anonymous, voluntary, self-administered web- and case-based survey cross-sectional study from October 2023 to January 2024 of neurologists identified through the LACTRIMS database. Questions were presented first through iterative clinical cases or imaging, followed by questions directly evaluating comprehension of definitions.
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Yaroslavl State Medical University, Yaroslavl, Russia.
Objective: To analyze the subjective sleep assessment in patients with neuromyelitis optica spectrum diseases (NMOSD) according to the current disease criteria of 2015.
Material And Methods: Twenty patients (17 women and 3 men), median age 44.5 years [Q:Q=27.
J Neurol Neurosurg Psychiatry
December 2024
Department of Neurology and Institute of Neuroimmunology and MS (INIMS), University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Background: Recurrent attacks in neuromyelitis optica spectrum disorders (NMOSDs) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can lead to severe disability. We aimed to analyse the real-world use of immunotherapies in patients with NMOSD and MOGAD, focusing on changes in treatment strategies, effects on attack rates (ARR) and risk factors for attacks.
Methods: This longitudinal registry-based cohort study included 493 patients (320 with aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive NMOSD (65%), 44 with AQP4-IgG seronegative NMOSD (9%) and 129 MOGAD (26%)) with 1247 treatments from 19 German and one Austrian centre from the registry of the neuromyelitis optica study group (NEMOS).
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