Rituximab in Churg-Strauss syndrome
Authors: M Koukoulaki
Journal: Annals of the Rheumatic Diseases (2006)

<h3>Objective:</h3> To elucidate the prevalence and correlates of cognitive impairment in patients with Neuromyelitis Optica Spectrum Disorder (NMOSD). <h3>Background:</h3> Neuromyelitis optica spectrum disorder (NMOSD) is a chronic central nervous system autoimmune, demyelinating disorder targeting self-antigens including aquaporin-4 and myelin oligodendrocyte glycoprotein. Cognitive impairment (CI) is an under-recognized and poorly understood in patients with NMSOD. <h3>Design/Methods:</h3> Montreal Cognitive Assessment (MoCA) were performed on 219 NMOSD patients in CIRCLES (Collaborative International Research in Clinical and Longitudinal Experience Study), a multicenter, longitudinal biorepository. The comparator group included 19 individuals, predominately patients with multiple sclerosis and healthy controls. MoCA were obtained at 6-month intervals from 2017–2020. Descriptive statistics, correlation analyses, and regression models were employed at baseline and longitudinally. <h3>Results:</h3> 29% (64/219) of NMOSD patients demonstrated mild CI (MoCA 20–26/30) and 5% (11/219) demonstrated moderate CI (MoCA&lt;20/30). Expanded Disability Status Scale (p&lt;0.0001), educational level (p&lt;0.001), immune therapy (p&lt;0.029), and pain scores (p=0.002) significantly correlated with CI. Annual relapse rate, age, gender, disease duration, AQP4 serostatus or presence of brain lesions were not correlated. CI among NMOSD patients was more pronounced among African American than White patients (p&lt;0.0002). Age adjusted degree of change in MoCA over time did not differ between NMOSD patients and comparator. Serial MoCA analysis within the NMOSD group revealed that 30% (45/150) had consistently decreasing MoCA scores, with most impacted attention and language functions. <h3>Conclusions:</h3> To our knowledge, this is the largest and most diverse patient sample study of CI in NMOSD and the first to evaluate potential CI progression. Our results indicate that 34% of NMOSD patients experience mild to moderate cognitive impairment, a third of whom have cumulative CI. They highlight the potential for relapse-independent disease activity and the importance of assessing neuropsychological outcomes in NMOSD. Correlations of CI with clinical and demographic characteristics may provide the potential for targeted evaluation and intervention. <b>Disclosure:</b> Dr. Vlahovic has nothing to disclose. Dr. McDonald has nothing to disclose. Mrs. Hinman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Epi Excellence. Miss Tomczak has nothing to disclose. Dr. Lock has received personal compensation for serving as an employee of InterX Inc. Dr. Lock has received personal compensation for serving as an employee of Diagnose Early . Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi . Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono . Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squib. Dr. Lock has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen. Dr. Lock has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for OGLE, WORM &amp; TRAVIS, PLLP. Gayle Deutsch has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Nelson has nothing to disclose. Dr. Han has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Han has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arena Pharmaceuticals.