LGI1 encephalitis: potentially complement-activating anti-LGI1-IgG subclasses 1/2/3 are associated with the development of hippocampal sclerosis Article Swipe
Christian G. Bien
,
Anna Rada
,
Markus Mertens
,
Corinna Bien
,
Jan Bauer
,
Anne Hagemann
,
Friedrich G. Woermann
·
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.1007/s00415-024-12594-9
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.1007/s00415-024-12594-9
Two-thirds of published patients with anti-leucine rich, glioma inactivated 1 (LGI1) encephalitis develop hippocampal sclerosis (HS). It is likely that this contributes to residual cognitive long-term deficits and the risk of epilepsy. Almost all patients harbor anti-LGI1-immunoglobulin G-(IgG-) subclass 4, which is considered a “benign”, non-destructive subclass. In contrast, neuropathological case studies have suggested that the classical complement cascade may contribute to mediotemporal cell death in patients with LGI1 antibodies. IgG subclasses 1, 2, or 3 are required to initiate this cascade. We hypothesized that patients with these anti-LGI1-IgG1/2/3 in addition to IgG4 have a higher risk of developing HS than patients with anti-LGI1-IgG4 alone. We retrospectively assessed all anti-LGI1 encephalitis patients from this center with anti-LGI1-IgG-subclass information and follow-up MRI available. Nine out of 20 patients had developed HS (45%). Volumetric FreeSurfer analysis confirmed the visual HS diagnoses. HS and a lower hippocampal volume were associated with anti-LGI1-IgG1/2/3. All six patients with this IgG subclass status developed HS. There was no association with older or younger age at onset, female sex, longer latency from disease onset to start of immunotherapy, less intense immunotherapy, higher serum titers of LGI1 antibodies, LGI1 antibodies in CSF or higher LGI1-specific antibody indices. There was no association between anti-LGI1-IgG1/2/3 status and neuropsychological performance, epilepsy, or general neurological performance. This confirms our hypothesis that anti-LGI1-IgG1/2/3 in serum puts patients at risk of developing HS. If these findings can be confirmed and clinically corroborated, patients with anti-LGI1-IgG1/2/3 might become candidates for anti-complement-directed immunological treatments.
Related Topics
Concepts
Hippocampal sclerosis
Subclass
Medicine
Encephalitis
Autoimmune encephalitis
Immunology
Epilepsy
Neurology
Antibody
Multiple sclerosis
Immunoglobulin G
Autoantibody
Temporal lobe
Virus
Psychiatry
Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1007/s00415-024-12594-9
- OA Status
- hybrid
- Cited By
- 5
- References
- 50
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4401367224
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W4401367224Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1007/s00415-024-12594-9Digital Object Identifier
- Title
-
LGI1 encephalitis: potentially complement-activating anti-LGI1-IgG subclasses 1/2/3 are associated with the development of hippocampal sclerosisWork title
- Type
-
articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2024Year of publication
- Publication date
-
2024-08-06Full publication date if available
- Authors
-
Christian G. Bien, Anna Rada, Markus Mertens, Corinna Bien, Jan Bauer, Anne Hagemann, Friedrich G. WoermannList of authors in order
- Landing page
-
https://doi.org/10.1007/s00415-024-12594-9Publisher landing page
- Open access
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YesWhether a free full text is available
- OA status
-
hybridOpen access status per OpenAlex
- OA URL
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https://doi.org/10.1007/s00415-024-12594-9Direct OA link when available
- Concepts
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Hippocampal sclerosis, Subclass, Medicine, Encephalitis, Autoimmune encephalitis, Immunology, Epilepsy, Neurology, Antibody, Multiple sclerosis, Immunoglobulin G, Autoantibody, Temporal lobe, Virus, PsychiatryTop concepts (fields/topics) attached by OpenAlex
- Cited by
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5Total citation count in OpenAlex
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2025: 5Per-year citation counts (last 5 years)
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50Number of works referenced by this work
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10Other works algorithmically related by OpenAlex
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| abstract_inverted_index.antibodies. | 70 |
| abstract_inverted_index.association | 163, 203 |
| abstract_inverted_index.contributes | 22 |
| abstract_inverted_index.hippocampal | 14, 144 |
| abstract_inverted_index.inactivated | 9 |
| abstract_inverted_index.information | 118 |
| abstract_inverted_index.treatments. | 248 |
| abstract_inverted_index.anti-leucine | 6 |
| abstract_inverted_index.encephalitis | 12, 111 |
| abstract_inverted_index.hypothesized | 84 |
| abstract_inverted_index.neurological | 213 |
| abstract_inverted_index.performance, | 209 |
| abstract_inverted_index.performance. | 214 |
| abstract_inverted_index.LGI1-specific | 197 |
| abstract_inverted_index.corroborated, | 238 |
| abstract_inverted_index.immunological | 247 |
| abstract_inverted_index.mediotemporal | 63 |
| abstract_inverted_index.“benign”, | 45 |
| abstract_inverted_index.anti-LGI1-IgG4 | 104 |
| abstract_inverted_index.immunotherapy, | 181, 184 |
| abstract_inverted_index.non-destructive | 46 |
| abstract_inverted_index.retrospectively | 107 |
| abstract_inverted_index.neuropathological | 50 |
| abstract_inverted_index.anti-LGI1-IgG1/2/3 | 89, 205, 220, 241 |
| abstract_inverted_index.neuropsychological | 208 |
| abstract_inverted_index.anti-LGI1-IgG1/2/3. | 149 |
| abstract_inverted_index.anti-LGI1-IgG-subclass | 117 |
| abstract_inverted_index.anti-LGI1-immunoglobulin | 37 |
| abstract_inverted_index.anti-complement-directed | 246 |
| cited_by_percentile_year.max | 98 |
| cited_by_percentile_year.min | 97 |
| corresponding_author_ids | https://openalex.org/A5074695558 |
| countries_distinct_count | 2 |
| institutions_distinct_count | 7 |
| corresponding_institution_ids | https://openalex.org/I20121455, https://openalex.org/I4210104779 |
| sustainable_development_goals[0].id | https://metadata.un.org/sdg/3 |
| sustainable_development_goals[0].score | 0.8199999928474426 |
| sustainable_development_goals[0].display_name | Good health and well-being |
| citation_normalized_percentile.value | 0.89429731 |
| citation_normalized_percentile.is_in_top_1_percent | False |
| citation_normalized_percentile.is_in_top_10_percent | True |