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@ARTICLE{Baumgartner:283118,
author = {Baumgartner, Tobias and Freyberg, Moritz and Campetella,
Lucia and Crijnen, Yvette and Dargvainiene, Justina and
Behning, Charlotte and Bien, Christian G and Rada, Anna and
Prüss, Harald and Rössling, Rosa and Kovac, Stjepana and
Strippel, Christine and Thaler, Franziska S and Eisenhut,
Katharina and Lewerenz, Jan and Becker, Felicitas and
Reinecke, Raphael and Malter, Michael Peter and Sühs,
Kurt-Wolfram and Tauber, Simone C and Von Podewils, Felix
and Melzer, Nico and Wandinger, Klaus-Peter and Fernandez
Ceballos, Romina-Anna-Maria and Kuhle, Jens and Berger,
Klaus and Bauer, Tobias and Rüber, Theodor and Racz, Attila
and Becker, Albert J and Pitsch, Julika and Kuhlenbäumer,
Gregor and Muñiz-Castrillo, Sergio and Honnorat, Jerome and
Titulaer, Maarten J and Leypoldt, Frank and Surges, Rainer},
collaboration = {group, and the GENERATE study},
title = {{R}isk of {E}pilepsy and {F}actors {A}ssociated {W}ith
{T}ime to {S}eizure {R}emission in {A}nti-{LGI}1
{E}ncephalitis: {L}ong-{T}erm {O}utcome in 236 {P}atients.},
journal = {Neurology: Neuroimmunology $\&$ Neuroinflammation ;
official journal of the American Academy of Neurology},
volume = {12},
number = {6},
issn = {2332-7812},
address = {Philadelphia, Pa.},
publisher = {Wolters Kluwer},
reportid = {DZNE-2026-00014},
pages = {e200469},
year = {2025},
abstract = {Autoimmune encephalitis (AIE) with anti-leucine-rich
glioma-inactivated 1 (LGI1) antibodies typically manifests
with subacute cognitive deficits, seizures, and psychiatric
symptoms, mostly in older adults. Immunotherapy (IT) leads
to the cessation of seizures in most patients, yet some
develop AIE-associated epilepsy (AEAE) and persistent
cognitive deficits. The aim of this large multicentric
retrospective observational cohort study was to assess
long-term outcomes of patients with anti-LGI1 encephalitis
regarding seizures and AEAE and to identify associated
factors.We included patients with anti-LGI1 encephalitis
from 3 national referral centers/consortia meeting the
following inclusion criteria: (I) definite LGI1 limbic
encephalitis (Graus criteria); (II) occurrence of seizures;
and (III) follow-up period ≥24 months. We aimed to (1)
determine the risk of seizure recurrence (ROSR) on
remission, (2) investigate clinical and paraclinical
biomarkers for an effect on time to seizure remission using
Cox proportional hazard modeling (n = 188), and (3) assess
the risk of AEAE and determine associated factors (n =
236).AEAE was observed in $5.9\%$ (16/271) of the full
cohort. Both AEAE (16/16 vs 129/215, p = 0.001) and longer
time to seizure remission (OR 1.36 per year, p = 0.025) were
associated with persistent cognitive impairment. Patients
with pilomotor seizures had a lower rate of seizure
remission (hazard ratio [HR] 0.58, $95\%$ CI 0.55-0.60, p <
0.001) while patients under IT administration had a higher
rate of seizure remission over time (HR 12.4, $95\%$ CI
9.67-16.0, p < 0.001). In addition, patients receiving
second-line IT tended to achieve earlier seizure remission
(log-rank test, p = 0.019). The ROSR at 12, 60, and 120
months on seizure remission was $9\%$ $(95\%$ CI
$4.5\%-13\%),$ $20\%$ $(95\%$ CI $11\%-28\%),$ and $53\%$
$(95\%$ CI $14\%-74\%),$ respectively.In conclusion, our
results demonstrate that AEAE in anti-LGI1 encephalitis is
rare and suggest that the diagnosis of epilepsy is
inappropriate in patients reaching seizure remission because
of a relatively low ROSR. Accordingly, on seizure remission,
the diagnosis of acute symptomatic seizures would be
appropriate. Moreover, we validate and quantify the
importance of IT for seizure remission and identify
biomarkers associated with lower rates of seizure remission.
Late remission of seizures and AEAE were associated with
persistent cognitive impairment.},
keywords = {Humans / Female / Male / Middle Aged / Retrospective
Studies / Adult / Aged / Epilepsy: etiology / Epilepsy:
epidemiology / Autoantibodies: blood / Seizures: etiology /
Encephalitis: complications / Encephalitis: immunology /
Autoimmune Diseases of the Nervous System: complications /
Autoimmune Diseases of the Nervous System: immunology /
Autoimmune Diseases of the Nervous System: therapy / Limbic
Encephalitis: complications / Limbic Encephalitis:
immunology / Follow-Up Studies / Intracellular Signaling
Peptides and Proteins: immunology / Young Adult /
Autoantibodies (NLM Chemicals) / LGI1 protein, human (NLM
Chemicals) / anti-leucine-rich glioma-inactivated 1
autoantibody (NLM Chemicals) / Intracellular Signaling
Peptides and Proteins (NLM Chemicals)},
cin = {AG Prüß / AG Stöcker},
ddc = {610},
cid = {I:(DE-2719)1810003 / I:(DE-2719)1013026},
pnm = {353 - Clinical and Health Care Research (POF4-353) / 354 -
Disease Prevention and Healthy Aging (POF4-354)},
pid = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-354},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40953325},
pmc = {pmc:PMC12440303},
doi = {10.1212/NXI.0000000000200469},
url = {https://pub.dzne.de/record/283118},
}