# Autoimmune encephalitis associated with anti-LGI1 antibody: a potential cause of neuropsychiatric systemic lupus erythematosus

**Authors:** Sixian Chen, Haitao Ren, Siyuan Fan, Shangzhu Zhang, Mengtao Li, Hongzhi Guan

PMC · DOI: 10.1136/lupus-2024-001429 · 2025-02-18

## TL;DR

This study identifies anti-LGI1 antibodies as a potential cause of neuropsychiatric symptoms in lupus patients, emphasizing the importance of early detection for better treatment outcomes.

## Contribution

The study links anti-LGI1 encephalitis to neuropsychiatric systemic lupus erythematosus, highlighting its clinical significance and treatment response.

## Key findings

- Four SLE patients tested positive for anti-LGI1 antibodies and showed limbic encephalitis symptoms.
- Three patients improved significantly with immunotherapy and had no relapses over 33–60 months.
- MRI revealed hyperintense basal ganglia lesions in two patients with anti-LGI1 encephalitis.

## Abstract

To investigate the clinical features and treatment outcomes of anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis in patients with SLE.

Between October 2014 and April 2024, serum or cerebrospinal fluid samples were collected from 332 patients with SLE suspected of autoimmune encephalitis. Cell-based assays were used to detect autoimmune antibodies, including anti-LGI1 antibodies. Four patients tested positive for anti-LGI1 antibodies, and their clinical, radiological and treatment data were analysed.

All four patients exhibited signs of limbic encephalitis, including short-term memory deficits, seizures and psychiatric disturbances. Two cases also presented with faciobrachial dystonic seizures. MRI findings revealed hyperintense basal ganglia lesions in two patients. Treatment with corticosteroids, intravenous immunoglobulin and mycophenolate mofetil led to significant improvement in three patients, with no relapses during a follow-up period ranging from 33 to 60 months. One patient succumbed to pneumonia despite initial improvement of neurological function.

Screening for anti-LGI1 antibodies in patients with neuropsychiatric systemic lupus erythematosus (NPSLE) is crucial when limbic encephalitis presents, as it enables timely and effective treatment, potentially improving patients’ outcomes. Additional basic and clinical research is required to clarify the pathogenic role of these antibodies in NPSLE.

## Linked entities

- **Proteins:** LGI1 (leucine rich glioma inactivated 1)
- **Chemicals:** mycophenolate mofetil (PubChem CID 5281078)
- **Diseases:** systemic lupus erythematosus (MONDO:0007915), neuropsychiatric systemic lupus erythematosus (MONDO:0043985), pneumonia (MONDO:0005249), encephalitis (MONDO:0019956)

## Full-text entities

- **Genes:** LGI1 (leucine rich glioma inactivated 1) [NCBI Gene 9211] {aka ADLTE, ADPAEF, ADPEAF, DEE121, EPITEMPIN, EPT}
- **Diseases:** basal ganglia lesions (MESH:D001480), encephalitis (MESH:D004660), SLE (MESH:D008180), faciobrachial dystonic seizures (MESH:D012640), autoimmune antibodies (MESH:D001327), Autoimmune encephalitis (MESH:D020274), NPSLE (MESH:D020945), memory deficits (MESH:D008569), psychiatric disturbances (MESH:D001523), limbic encephalitis (MESH:D020363), pneumonia (MESH:D011014)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11836784/full.md

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Source: https://tomesphere.com/paper/PMC11836784