Abstract Details

Title Cognitive Impairment is Associated with Longitudinal Disability in LGI-1-IgG Encephalitis

Topic Autoimmune Neurology

Presentation(s) S22 - Autoimmune Neurology: Autoimmune Encephalitis and Other Antibody-associated Syndromes (4:42 PM-4:54 PM)

Poster/Presentation
Number 007

Background

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).

Objective To determine predictors of longitudinal clinical outcomes in leucine-rich glioma inactivated-1 immunoglobulin G (LGI-1)-IgG autoimmune encephalitis (AE).

Design/Methods

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.

Results

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 (r=0.58, P<.001) and with initial MoCA score (mRS r=-0.60, P=.009, CASE r=-0.51, P=.031). After 12 months from symptom onset, mRS (OR=0.88, [CI=0.82-0.94], P<.001) and CASE (β=-0.03, [SE=0.01], P<.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], P=.005). Higher initial MOCA was associated with lower mRS (OR=0.66, [CI=0.45-0.98], P=.04) and CASE (β=-0.24, [SE=0.13], P=.061) longitudinally. At last follow-up, most patients had ongoing cognitive symptoms (25, [83.3%]), while few had ongoing seizure activity (3, [10%]).

Conclusions

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.

Authors/Disclosures
Albert Aboseif, DO (Mayo Clinic Rochester) PRESENTER	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.
Yadi Li	No disclosure on file
Moein Amin, MD (Cleveland Clinic)	Dr. Amin has nothing to disclose.
Brittany Lapin	No disclosure on file
Justin Abbatemarco, MD (Cleveland Clinic Foundation)	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 EMD Serono. 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 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 . 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 TG Therapeutics, Inc.. The institution of Dr. Abbatemarco has received research support from Horizon.
Alexander D. Rae-Grant, MD, FAAN (Ebsco Information Services)	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.
Vineet Punia, MD (Cleveland Clinic)	Dr. Punia has nothing to disclose.
Rachel Galioto	Rachel Galioto has nothing to disclose.
Amy Kunchok, MBBS (Cleveland Clinic - Mellen Centre)	Dr. Kunchok has received personal compensation in the range of $0-$499 for serving as a Consultant for EMD Serono. 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 $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. 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.