MCID: LMB053
MIFTS: 25

Limbic Encephalitis with Lgi1 Antibodies

Categories: Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Limbic Encephalitis with Lgi1 Antibodies

MalaCards integrated aliases for Limbic Encephalitis with Lgi1 Antibodies:

Name: Limbic Encephalitis with Lgi1 Antibodies 52 58
Limbic Encephalitis with Leucine-Rich Glioma-Inactivated 1 Antibodies 52 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 33 G13.1
Orphanet 58 ORPHA163908

Summaries for Limbic Encephalitis with Lgi1 Antibodies

NIH Rare Diseases : 52 Limbic encephalitis with LGI1 antibodies is a relatively new disease characterized by progressive memory loss, confusion, sleep disturbances, and problems with behaviors and spatial orientation. Seizures are common and frequently precede other symptoms. About half of people with this diagnosis exhibit a specific type of seizure called faciobrachial dystonic seizures, which resemble brief jerks of the face arm. Hyponatremia (low levels of sodium in the blood) may also be present. The disease occurs when antibodies mistakenly attack LGI1 (leucine-rich glioma inactivated 1), a protein found in the brain that binds together two epilepsy -related proteins called ADAM22 and ADAM23. Some people with limbic encephalitis with LGI1 antibodies (5 to 10%) also have a cancer diagnosis (most commonly thymoma and lung cancer), but most do not. The disease is diagnosed through testing of blood or spinal fluid for LGI1 antibodies. If treatment is started when seizures are first noticed, limbic encephalitis may be prevented. Treatment is intravenous or oral corticosteroids , intravenous immunoglobulin (IVIG), plasmapheresis (a process where antibodies are removed from the blood), or a combination of these. Long-term outcome is mostly favorable, although some people are left with some degree of residual impairment. Relapse occurs in up to one third of patients.

MalaCards based summary : Limbic Encephalitis with Lgi1 Antibodies, also known as limbic encephalitis with leucine-rich glioma-inactivated 1 antibodies, is related to autoimmune disease and encephalitis. An important gene associated with Limbic Encephalitis with Lgi1 Antibodies is HLA-DQB1 (Major Histocompatibility Complex, Class II, DQ Beta 1), and among its related pathways/superpathways are Allograft rejection and Measles. Affiliated tissues include brain, lung and testes.

Related Diseases for Limbic Encephalitis with Lgi1 Antibodies

Diseases related to Limbic Encephalitis with Lgi1 Antibodies via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 72)
# Related Disease Score Top Affiliating Genes
1 autoimmune disease 30.0 HLA-DRB1 HLA-DQB1
2 encephalitis 10.5
3 limbic encephalitis 10.5
4 sleep disorder 10.3
5 autoimmune encephalitis 10.3
6 pseudobulbar affect 10.3
7 myoclonus 10.3
8 autoimmune polyglandular syndrome type 3 9.8 HLA-DRB1 HLA-DQB1
9 hypersomnia 9.8 HLA-DRB1 HLA-DQB1
10 narcolepsy 2 9.8 HLA-DRB1 HLA-DQB1
11 narcolepsy 9.8 HLA-DRB1 HLA-DQB1
12 primary adrenal insufficiency 9.8 HLA-DRB1 HLA-DQB1
13 type ii mixed cryoglobulinemia 9.8 HLA-DRB1 HLA-DQB1
14 moyamoya angiopathy 9.8 HLA-DRB1 HLA-DQB1
15 recurrent respiratory papillomatosis 9.8 HLA-DRB1 HLA-DQB1
16 beryllium disease 9.8 HLA-DRB1 HLA-DQB1
17 apple allergy 9.8 HLA-DRB1 HLA-DQB1
18 chronic beryllium disease 9.8 HLA-DRB1 HLA-DQB1
19 autoimmune hepatitis 9.8 HLA-DRB1 HLA-DQB1
20 autoimmune hepatitis type 1 9.8 HLA-DRB1 HLA-DQB1
21 focal epithelial hyperplasia 9.8 HLA-DRB1 HLA-DQB1
22 pediatric multiple sclerosis 9.8 HLA-DRB1 HLA-DQB1
23 metal allergy 9.8 HLA-DRB1 HLA-DQB1
24 narcolepsy 1 9.8 HLA-DRB1 HLA-DQB1
25 idiopathic bronchiectasis 9.8 HLA-DRB1 HLA-DQB1
26 osteonecrosis of the jaw 9.8 HLA-DRB1 HLA-DQB1
27 pityriasis rosea 9.8 HLA-DRB1 HLA-DQB1
28 red cell aplasia 9.8 HLA-DRB1 HLA-DQB1
29 oligoarticular juvenile idiopathic arthritis 9.8 HLA-DRB1 HLA-DQB1
30 arthritis 9.8 HLA-DRB1 HLA-DQB1
31 ocular cicatricial pemphigoid 9.8 HLA-DRB1 HLA-DQB1
32 cicatricial pemphigoid 9.8 HLA-DRB1 HLA-DQB1
33 whipple disease 9.8 HLA-DRB1 HLA-DQB1
34 paraneoplastic pemphigus 9.8 HLA-DRB1 HLA-DQB1
35 pemphigus 9.8 HLA-DRB1 HLA-DQB1
36 alveolar echinococcosis 9.8 HLA-DRB1 HLA-DQB1
37 pure red-cell aplasia 9.8 HLA-DRB1 HLA-DQB1
38 cytomegalovirus retinitis 9.8 HLA-DRB1 HLA-DQB1
39 microscopic colitis 9.8 HLA-DRB1 HLA-DQB1
40 cystic echinococcosis 9.8 HLA-DRB1 HLA-DQB1
41 acute disseminated encephalomyelitis 9.8 HLA-DRB1 HLA-DQB1
42 peanut allergy 9.8 HLA-DRB1 HLA-DQB1
43 panuveitis 9.8 HLA-DRB1 HLA-DQB1
44 allergic bronchopulmonary aspergillosis 9.8 HLA-DRB1 HLA-DQB1
45 guillain-barre syndrome 9.8 HLA-DRB1 HLA-DQB1
46 rheumatic fever 9.8 HLA-DRB1 HLA-DQB1
47 rheumatic heart disease 9.8 HLA-DRB1 HLA-DQB1
48 graft-versus-host disease 9.7 HLA-DRB1 HLA-DQB1
49 vogt-koyanagi-harada disease 9.7 HLA-DRB1 HLA-DQB1
50 autoimmune disease of gastrointestinal tract 9.7 HLA-DRB1 HLA-DQB1

Graphical network of the top 20 diseases related to Limbic Encephalitis with Lgi1 Antibodies:



Diseases related to Limbic Encephalitis with Lgi1 Antibodies

Symptoms & Phenotypes for Limbic Encephalitis with Lgi1 Antibodies

Drugs & Therapeutics for Limbic Encephalitis with Lgi1 Antibodies

Search Clinical Trials , NIH Clinical Center for Limbic Encephalitis with Lgi1 Antibodies

Genetic Tests for Limbic Encephalitis with Lgi1 Antibodies

Anatomical Context for Limbic Encephalitis with Lgi1 Antibodies

MalaCards organs/tissues related to Limbic Encephalitis with Lgi1 Antibodies:

40
Brain, Lung, Testes

Publications for Limbic Encephalitis with Lgi1 Antibodies

Articles related to Limbic Encephalitis with Lgi1 Antibodies:

# Title Authors PMID Year
1
Seizure semiology of anti-LGI1 antibody encephalitis. 52
29171405 2017
2
Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up. 52
27590293 2016
3
The Treatment and Follow-Up of Anti-LGI1 Limbic Encephalitis. 52
26694143 2016
4
Limbic encephalitis with LGI1 antibodies in a 14-year-old boy. 61
28919330 2018
5
Selective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies. 61
29093718 2017
6
A rare case of limbic encephalitis with anti leucine-rich glioma inactivated-1 (LGI1) antibodies. 61
24878970 2014
7
Surgical control of limbic encephalitis associated with LGI1 antibodies. 61
22940785 2012

Variations for Limbic Encephalitis with Lgi1 Antibodies

Expression for Limbic Encephalitis with Lgi1 Antibodies

Search GEO for disease gene expression data for Limbic Encephalitis with Lgi1 Antibodies.

Pathways for Limbic Encephalitis with Lgi1 Antibodies

Pathways related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

(show all 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.35 HLA-DRB1 HLA-DQB1
2
Show member pathways
12.31 HLA-DRB1 HLA-DQB1
3 12.28 HLA-DRB1 HLA-DQB1
4
Show member pathways
12.14 HLA-DRB1 HLA-DQB1
5
Show member pathways
12.11 HLA-DRB1 HLA-DQB1
6
Show member pathways
12.06 HLA-DRB1 HLA-DQB1
7
Show member pathways
11.94 HLA-DRB1 HLA-DQB1
8
Show member pathways
11.9 HLA-DRB1 HLA-DQB1
9 11.88 HLA-DRB1 HLA-DQB1
10
Show member pathways
11.8 HLA-DRB1 HLA-DQB1
11 11.77 HLA-DRB1 HLA-DQB1
12
Show member pathways
11.73 HLA-DRB1 HLA-DQB1
13 11.66 HLA-DRB1 HLA-DQB1
14 11.6 HLA-DRB1 HLA-DQB1
15 11.44 HLA-DRB1 HLA-DQB1
16
Show member pathways
11.44 HLA-DRB1 HLA-DQB1
17 11.38 HLA-DRB1 HLA-DQB1
18 11.32 HLA-DRB1 HLA-DQB1
19 11.24 HLA-DRB1 HLA-DQB1
20 11.12 HLA-DRB1 HLA-DQB1
21 10.81 HLA-DRB1 HLA-DQB1
22
Show member pathways
10.58 HLA-DRB1 HLA-DQB1

GO Terms for Limbic Encephalitis with Lgi1 Antibodies

Cellular components related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 lysosomal membrane GO:0005765 9.46 HLA-DRB1 HLA-DQB1
2 endosome membrane GO:0010008 9.43 HLA-DRB1 HLA-DQB1
3 trans-Golgi network membrane GO:0032588 9.4 HLA-DRB1 HLA-DQB1
4 endocytic vesicle membrane GO:0030666 9.37 HLA-DRB1 HLA-DQB1
5 ER to Golgi transport vesicle membrane GO:0012507 9.32 HLA-DRB1 HLA-DQB1
6 transport vesicle membrane GO:0030658 9.26 HLA-DRB1 HLA-DQB1
7 clathrin-coated endocytic vesicle membrane GO:0030669 9.16 HLA-DRB1 HLA-DQB1
8 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DRB1 HLA-DQB1
9 MHC class II protein complex GO:0042613 8.62 HLA-DRB1 HLA-DQB1

Biological processes related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.4 HLA-DRB1 HLA-DQB1
2 T cell receptor signaling pathway GO:0050852 9.37 HLA-DRB1 HLA-DQB1
3 antigen processing and presentation of exogenous peptide antigen via MHC class II GO:0019886 9.32 HLA-DRB1 HLA-DQB1
4 interferon-gamma-mediated signaling pathway GO:0060333 9.26 HLA-DRB1 HLA-DQB1
5 antigen processing and presentation GO:0019882 9.16 HLA-DRB1 HLA-DQB1
6 humoral immune response mediated by circulating immunoglobulin GO:0002455 8.96 HLA-DRB1 HLA-DQB1
7 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 8.62 HLA-DRB1 HLA-DQB1

Molecular functions related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 8.96 HLA-DRB1 HLA-DQB1
2 MHC class II receptor activity GO:0032395 8.62 HLA-DRB1 HLA-DQB1

Sources for Limbic Encephalitis with Lgi1 Antibodies

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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