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Last update 08 May 2025

LGI1

Last update 08 May 2025

Basic Info

Synonyms

EPITEMPIN, Epitempin-1, EPT

+ [5]

Introduction

Regulates voltage-gated potassium channels assembled from KCNA1, KCNA4 and KCNAB1. It slows down channel inactivation by precluding channel closure mediated by the KCNAB1 subunit. Ligand for ADAM22 that positively regulates synaptic transmission mediated by AMPA-type glutamate receptors (By similarity). Plays a role in suppressing the production of MMP1/3 through the phosphatidylinositol 3-kinase/ERK pathway. May play a role in the control of neuroblastoma cell survival.

Drugs associated with LGI1

S52.006

Target

GAD1 x LGI1 x NMDA receptor

Mechanism

GAD inhibitors [+2]

Active Org.

Heinrich-Heine-Universität Düsseldorf

Originator Org.

Heinrich-Heine-Universität Düsseldorf

Active Indication

Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Inactive Indication-

Drug Highest PhaseDiscovery

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

100 Clinical Results associated with LGI1

100 Translational Medicine associated with LGI1

0 Patents (Medical) associated with LGI1

856

Literatures (Medical) associated with LGI1

01 May 2025·Journal of the Neurological Sciences

Prevalence, clinical profiles, and prognosis of Isaacs syndrome: A nationwide survey study in Japan

Article

Author: Ishida, Mitsuyo ; Matsui, Naoko ; Osaki, Yusuke ; Tanaka, Keiko ; Watanabe, Takeshi ; Izumi, Yuishin ; Watanabe, Osamu ; Takashima, Hiroshi ; Kuwabara, Satoshi ; Sato, Yasunori ; Matsuura, Eiji ; Kokubun, Norito ; Hatanaka, Yuki

OBJECTIVESTo elucidate current epidemiological, clinical, and immunological profiles, and the treatment of Isaacs syndrome in Japan.METHODSWe conducted a nationwide survey using established methods. Questionnaires were sent to neurological facilities throughout Japan to identify Isaacs syndrome patients seen between April 2018 and March 2021.RESULTSThe estimated total number of Isaacs syndrome patients was 114 (95 % confidence interval [CI]: 89.63-138.91), and the estimated prevalence was 0.091 per 100,000 population. Detailed clinical data were available for 44 patients. The median age at onset was 40 (range, 17-78 years), and 55 % were female. The median time from symptom onset to diagnosis was 24 months (range, 1-372 months). Electrodiagnostic studies showed evidence of nerve hyperexcitability in 90 % (myokymic discharges in 50 % and stimulus-induced repetitive discharges in 32 %). Of the 28 patients examined in the cell-based assay, 22 % tested positive (11 % for both leucine-rich glioma-inactivated 1 [LGI1] and contactin-associated protein-like 2 [CASPR2] antibodies and 11 % for LGI1 antibodies only). The median modified Rankin Scale (mRS) score was 2 at diagnosis and 1.5 at the last visit. A high mRS score (mRS ≥4) at baseline was an independent risk factor for poor outcomes (mRS ≥3) (Odds ratio, 20.7; 95 % CI, 2.90-209.18; p < 0.001).CONCLUSIONWe elucidated the current epidemiological and clinical characteristics of Isaacs syndrome in Japan. Isaacs syndrome is a rare neuromuscular disorder. Electrophysiologic abnormalities were frequent, and serum antibodies were not detectable in the majority of patients. A high mRS score before treatment was a risk factor for poor outcomes.

01 May 2025·Journal of Neurology

Successful treatment of morvan syndrome with efgartigimod: report of two cases

Article

Author: Wang, Peng ; Li, Pengpeng ; Yao, Xiaoli ; He, Ruojie

OBJECTIVEMorvan Syndrome is a rare autoimmune-mediated clinical condition. This retrospective study aims at assessing the effectiveness and safety of intravenous Efgartigimod in 2 cases of the Morvan Syndrome with positive LGI1 and CASPR2 antibodies.METHODSWe reviewed the clinical manifestations, autoantibodies detection, electromyography (EMG) characteristics, treatments and follow-up outcomes in 2 patients of Morvan Syndrome with positive LGI1 and CASPR2 antibodies. Especially, we evaluated the therapeutic effectiveness and safety of intravenous Efgartigimod based on the improvement of symptoms, relapse conditions and adverse reactions.RESULTSThe patient 1 was a 35-year-old female who presented with muscle tremors and pain in lower limbs. The serum VGKC, LGI1 and CASPR2 antibodies were positive. M-wave and muscle tremor discharges were observed in both gastrocnemius muscles with EMG examination. She was treated with efgartigimod (800 mg, once a week for 4 times), and the symptoms were almost alleviated at 6 months follow-up. The patient 2 was a 31 year-old female who presented with muscle soreness and weakness in four limbs. The serum VGKC, LGI1 and CASPR2 antibodies also were positive. M-wave discharges were observed with EMG examination. She was treated with efgartigimod (400 mg, once a week for 4 times), and follow-up at 6 months also showed recovery of muscle strength.CONCLUSIONMorvan Syndrome is an antibody-related autoimmune syndrome with complex clinical manifestations. Our findings showed that the treatments of intravenous Efgartigimod could be effective and well tolerant in patients of Morvan Syndrome with LGI1 and CASPR2 antibodies.

01 May 2025·Journal of Neurology

Neurodegeneration and the immune system: lessons from autoimmune encephalitis

Review

Author: Farina, Antonio ; Muñiz-Castrillo, Sergio ; Campetella, Lucia ; Joubert, Bastien ; Smolik, Krzysztof ; Desestret, Virginie ; Honnorat, Jérôme

AbstractThe spectrum of autoimmune encephalitis (AE) is expanding to atypical clinical presentations that can mimic neurodegenerative disorders. Among the autoantibodies most frequently associated with manifestations mimicking neurodegenerative disorders—such as dementia, parkinsonism, ataxia and motor neuron disease—IgLON5-, LGI1- and CASPR2-antibodies, predominantly of the IgG4 subclass and associated with specific HLA haplotypes, are the most common. Since these forms of autoimmune encephalitis often lack inflammatory findings in cerebrospinal fluid or magnetic resonance imaging, recognizing clinical ‘red flags’ suggestive of an autoimmune etiology is crucial for accurate diagnosis and timely initiation of immunotherapy. Interestingly, in these forms of autoimmune encephalitis, both inflammatory and neurodegenerative disease mechanisms may be involved. The neurodegenerative component may result directly from antibody effects (e.g., tau deposition in IgLON5-antibody disease) or arise through other mechanisms (e.g., seizures or exacerbation of pre-existing pathology). Moreover, neuroinflammation has recently emerged as a key contributor to primary neurodegenerative disorders. For instance, microglial activation promotes tau pathology propagation, as observed in Alzheimer’s disease and other primary neurodegenerative disorders. While the precise mechanisms linking inflammation and neurodegeneration remain to be fully understood, further research into the interplay between autoimmunity and neurodegeneration may enhance our understanding of disease mechanisms and expand therapeutic opportunities in both autoimmune and neurodegenerative neurological disorders.

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LGI1

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