Cognitive Impairment is Associated with Longitudinal Disability in LGI-1-IgG Encephalitis (S22.007)

Abstract

<h3>Objective:</h3> To determine predictors of longitudinal clinical outcomes in leucine-rich glioma inactivated-1 immunoglobulin G (LGI-1)-IgG autoimmune encephalitis (AE). <h3>Background:</h3> Longitudinal studies prognosticating outcomes in LGI-1-IgG AE are needed to identify key drivers of disability. We examine disability utilizing modified Rankin Scale (mRS) and an AE specific scale, the clinical assessment scale in AE (CASE). <h3>Design/Methods:</h3> A retrospective observational study of seropositive LGI-1-IgG AE patients was conducted between 2013–2022. Clinical predictors included demographics, clinical and paraclinical data, magnetic resonance imaging (MRI), and Montreal Cognitive Assessment (MoCA). Clinical outcomes included mRS and CASE scores. Logistic and linear regressions were used for modeling mRS and CASE, respectively. Baseline clinical characteristics were included as independent variables in the regression models. <h3>Results:</h3> Thirty patients (60% male, median age=69.5; IQR=63.0–76.0) were included, with a median follow-up time of 8.7 months (IQR=4.3–24.1). Seizures (29, [96.7%]), and cognitive impairment (CI) (30, [100%]) were present in the majority. Median initial MoCA was 23/30 (IQR=21.0–25.0). The majority received acute immunotherapy (27, [90%]), and maintenance immunotherapy, (26 [86.7%]). Baseline mRS (median=2.0, IQR=2.0–3.0) and CASE (mean=4.3, SD=3.7) correlated with each other (<i>r</i>=0.58, <i>P</i>&lt;.001) and with initial MoCA score (mRS <i>r</i>=−0.60, <i>P</i>=.009, CASE <i>r</i>=−0.51, <i>P</i>=.031). After 12 months from symptom onset, mRS (OR=0.88, [CI=0.82–0.94], <i>P</i>&lt;.001) and CASE (β=−0.03, [SE=0.01], <i>P</i>&lt;.001) decreased significantly. Temporal lobe(s) Fluid Attenuation Inversion Recovery (FLAIR) hyperintensity correlated with higher mRS longitudinally (OR=17.49, [CI=2.37, 129.05], <i>P</i>=.005). Higher initial MOCA was associated with lower mRS (OR=0.66, [CI=0.45–0.98], <i>P</i>=.04) and CASE (β=−0.24, [SE=0.13], <i>P</i>=.061) longitudinally. At last follow-up, most patients had ongoing cognitive symptoms (25, [83.3%]), while few had ongoing seizure activity (3, [10%]). <h3>Conclusions:</h3> CI is associated with disability at baseline and long-term follow up, underscoring cognition as an important determinant of disability outcomes in LGI-1-IgG AE. Severity of CI on baseline MoCA may offer prognostic information on long-term disability. <b>Disclosure:</b> Dr. Aboseif has a non-compensated relationship as a AAN Autoimmune Neurology Section -- Education Workgroup with AAN that is relevant to AAN interests or activities. Ms. Li has nothing to disclose. Dr. Amin has nothing to disclose. Brittany Lapin has received personal compensation for serving as an employee of Cleveland Clinic. Brittany Lapin has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Quality of Life Research. Brittany Lapin has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Anesthesia and Analgeisa. Dr. Abbatemarco 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. Abbatemarco has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech . The institution of Dr. Abbatemarco has received research support from Horizon. Dr. Rae-Grant has received publishing royalties from a publication relating to health care. Dr. Rae-Grant has received publishing royalties from a publication relating to health care. Dr. Punia has nothing to disclose. Rachel Galioto has nothing to disclose. Dr. Kunchok has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Kunchok 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 . Dr. Kunchok has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology.