Abstract Details

Title Relapse Burden and Long-term Recovery in LGI1-IgG Autoimmune Encephalitis: Predictors and Treatment Responses

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (11:30 AM-12:30 PM)

Poster/Presentation
Number 1-018

Objective

To characterize long-term outcomes, relapse burden, and treatment responses in Leucine-rich glioma inactivated 1 (LGI1)-IgG Autoimmune Encephalitis (AE).

Background

Factors that influence relapses and long-term outcomes in LGI1–IgG AE are poorly defined.

Design/Methods

Retrospective longitudinal study of 171 adults with LGI1-IgG AE evaluated at Mayo Clinic (January 1, 2000–December 31, 2023), with external validation in 64 adults from Seoul National University Hospital. Poor functional (modified Rankin Scale >2), cognitive (Clinical Dementia Rating >0.5), and relapse outcomes were assessed at short-term (3–6 months) and last follow-up. Associations were examined using multivariable logistic regression, Cox proportional hazards models with time-varying covariates, and mixed-effects ordinal regression.

Results

Among 171 Mayo Clinic patients (65% male; median age 66 years), 145 completed 6-month follow-up; 53 (37%) experienced 69 relapses. Corticosteroid use at presentation was associated with favorable short-term functional, cognitive, and seizure outcomes. Poor baseline CDR, treatment delay, and cumulative relapses predicted poor long-term functional and cognitive outcomes. Male sex (OR 3.71; 95% CI, 1.29–10.72; p=0.015) and persistent LGI1-IgG seropositivity (OR 2.94; 95% CI, 1.10–7.86; p=0.032) were associated with relapse. Chronic immunotherapy reduced relapse risk, particularly rituximab (HR 0.19; 95% CI, 0.05–0.76) and chronic corticosteroids (HR 0.32; 95% CI, 0.14–0.73). Higher cumulative relapse burden limited long-term functional recovery (OR 2.94; 95% CI, 1.44–6.01; p=0.003).

Validation in the SNUH cohort (64 patients; 18 relapses) confirmed these associations, including relapse risk with persistent LGI1-IgG seropositivity (OR 6.90; 95% CI, 1.40–33.92), poor cognition with baseline CDR, treatment delay, and relapses; and reduced relapse hazard with rituximab (HR 0.06; 95% CI, 0.00–0.89).

Conclusions

In LGI1-IgG AE, baseline cognitive deficits, treatment delay and cumulative relapses are strongly associated with poor long-term functional and cognitive outcomes. Persistent LGI1-IgG seropositivity identifies patients at increased risk of relapse. Strategies that prevent relapses, particularly rituximab and chronic corticosteroid therapy appear to be essential for optimizing sustained recovery.

Authors/Disclosures
Naveen K. Paramasivan, MD (Mayo Clinic) PRESENTER	Dr. Paramasivan has nothing to disclose.
Soo Hyun Ahn, MD (Seoul National University Hospital)	Dr. Ahn has nothing to disclose.
Andreu Vilaseca-Jolonch, MD	Dr. Vilaseca-Jolonch has received research support from Fundación Martín Escudero.
Felipe Jones, MD	Dr. Jones has nothing to disclose.
Andrea Stabile, MD	Dr. Stabile has nothing to disclose.
Surendra Dasari	Surendra Dasari has nothing to disclose.
Kelsey M. Smith, MD (Mayo Clinic)	The institution of Dr. Smith has received research support from UCB Pharmaceuticals.
Jeffrey W. Britton, MD, FAAN (Mayo Graduate School of Medicine)	Dr. Britton has received personal compensation in the range of $0-$499 for serving as a Online course with American Clinical Neurophysiology Society.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Merck. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received intellectual property interests from a discovery or technology relating to health care. Dr. Flanagan has received intellectual property interests from a discovery or technology relating to health care. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology that is relevant to AAN interests or activities.
Andrew McKeon, MD (Mayo Clinic)	The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
Anastasia Zekeridou, MD, PhD, FAAN (Neuroimmunology Laboratory, Mayo Clinic)	The institution of Dr. Zekeridou has received research support from Roche/Genentech. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology)	Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.
Stephen L. Yates, PhD	Dr. Yates has received personal compensation for serving as an employee of UCB Biosciences, Inc.. Dr. Yates has stock in UCB Biosciences, Inc..
Panayotes Demakakos	Panayotes Demakakos has nothing to disclose.
Soon-Tae Lee, MD, PhD (Department of Neurology, Seoul National University Hospital)	Prof. Lee has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Advanced Neural Technologies. Prof. Lee has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Piehealthcare. Prof. Lee has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Salted. Prof. Lee has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. Prof. Lee has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Prof. Lee has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. The institution of Prof. Lee has received research support from Roche. Prof. Lee has received intellectual property interests from a discovery or technology relating to health care.
Divyanshu Dubey, MD, FAAN (Mayo Clinic)	The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.

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