Neuromyelitis optica spectrum disorders with a benign course. Analysis of 544 patients

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory autoimmune disease of the central nervous system (CNS) most frequently characterized by recurring attacks of longitudinal transverse myelitis and optic neuritis. Other core manifestations of the disease include area postrema syndrome (intractable nausea, vomiting and hiccups) brainstem symptoms, and more rarely, symptoms of diencephalic and cerebral involvement (Wingerchuk et al., 2015). About three fourths of the NMOSD patients carry serum aquaporin-4 IgG (AQP4-IgG) (Hoftberger et al., 2013; Ketelslegers et al., 2011; Melamed et al., 2015; Waters et al., 2014) whereas 7–42% of the AQP4-IgG seronegative patients are myelin oligodendrocyte glycoprotein (MOG)-IgG seropositive (Hamid et al., 2017; Mader et al., 2011). Historically, NMOSD was considered a form of multiple sclerosis (MS) which may also cause recurring attacks of optic nerve and spinal cord inflammation. However, a number of immunopathogenetic (Lucchinetti et al., 2002; Misu et al., 2007; Roemer et al., 2007), demographic(Kim et al., 2017; Wingerchuk and Lucchinetti, 2007), clinical (Merle et al., 2007; Nakajima et al., 2010; Wingerchuk, 2004; Wingerchuk et al., 2007) and imaging (Ito et al., 2009; Kim et al., 2015; Nakamura et al., 2009; Yonezu et al., 2014) evidence support distinction between the two conditions and have granted NMOSD the status of being a unique nosological entity on its own right. Aquaporin 4-IgG positive NMOSD is an immune-mediated astrocytopathy with secondary demyelination, probably as a result of secondary oligodendrocyte dysfunction whereas MOG-IgG NMOSD result from direct attack of the antibody on the myelin sheath with relative preservation of oligodendrocytes (Jarius et al., 2020). Outcome is one of the most prominent differential characteristics in the clinical course of MS and NMOSD. Whereas MS patients typically have a long progressive course with increasing disability mainly due to a continuous neurodegeneration process, NMOSD patients usually accumulate more severe disability shortly following disease onset because of recurring inflammatory attacks (Akaishi et al., 2020; Ontaneda et al., 2015; Weinshenker et al., 1989; Wingerchuk et al., 2007; Wingerchuk and Weinshenker, 2003).

The term “benign MS” has been used to name a form of the disease characterized by a lenient course in which patients remain fully functional in all neurological systems, or with Expanded Disability Status Scale (EDSS) score ≤ 3.0 for 10 or 15 years after disease onset (Calabrese et al., 2013; Lublin and Reingold, 1996; Sartori et al., 2017). Many studies have addressed the frequency, clinical characteristics, and predictive factors of benign MS (Bueno et al., 2015; Correale et al., 2012; Crielaard et al., 2019; Gajofatto et al., 2016; Leray et al., 2013; Maarouf et al., 2022; Mathey et al., 2021; Niiranen et al., 2021; Ramsaransing and De Keyser, 2006; Reynders et al., 2017; Sayao et al., 2007).

On the other hand, a search of PubMed yielded few case reports (Bergamaschi et al., 2009; Koller et al., 2006; Pandit and Mustafa, 2017) and two studies describing NMOSD patients with EDSS ≤ 3.0 after a minimal 10-year disease duration (Collongues et al., 2011; Uzawa et al., 2015). These studies used the 2006 criteria for neuromyelitis optica diagnosis, and a 10-year course after disease onset for definition of benign disease.

The aim of the present study is to analyze the demographic, clinical characteristics, and predictive factors of benign NMOSD in a large cohort of patients. Differently from previous studies, we used the 2015 International Panel for NMO Diagnosis (IPND) criteria for NMOSD (Wingerchuk et al., 2015), and disease duration of at least 15 years with an EDSS score of ≤ 3.0 as a more stringent marker of benign disease.

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