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MCID: LMB024
MIFTS: 42

Limbic Encephalitis

Categories: Cancer diseases, Immune diseases, Neuronal diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Limbic Encephalitis

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MalaCards integrated aliases for Limbic Encephalitis:

Name: Limbic Encephalitis 53 55 73

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Neuronal diseases Immune diseases
See all MalaCards categories (disease lists)


External Ids:

UMLS 73 C0338430
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Summaries for Limbic Encephalitis

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NIH Rare Diseases : 53 Limbic encephalitis represents a group of autoimmune conditions characterized by inflammation of the limbic system and other parts of the brain. The cardinal sign of limbic encephalitis is a severe impairment of short-term memory; however, symptoms may also include confusion, psychiatric symptoms, and seizures. The symptoms typically develop over a few weeks or months, but they may evolve over a few days. Limbic encephalitis is often associated with an underlying neoplasm (paraneoplastic limbic encephalitis); however some cases never have a neoplasm identified (non-paraneoplastic limbic encephalitis). Delayed diagnosis is common, but improvements are being made to assist in early detection. Various tests including imaging studies (MRI, PET) laboratory tests (CSF analysis), and tests that measure the electrical activity of the brain (EEG) may be utilized to confirm a diagnosis. Treatment includes removal of the neoplasm (if identified) and immunotherapy.

MalaCards based summary : Limbic Encephalitis is related to epilepsy and status epilepticus. An important gene associated with Limbic Encephalitis is GAD1 (Glutamate Decarboxylase 1), and among its related pathways/superpathways are Glucose / Energy Metabolism and Long-term potentiation. The drugs Antibodies and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include brain, lung and testes, and related phenotypes are behavior/neurological and nervous system

Wikipedia : 76 Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain.... more...

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Related Diseases for Limbic Encephalitis

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

(show top 50) (show all 138)
# Related Disease Score Top Affiliating Genes
1 epilepsy 30.4 CNTN2 GAD1 GRIN2B GSR LGI1
2 status epilepticus 30.4 GRIA1 GRIN2B LGI1
3 stiff-person syndrome 30.3 AMPH GAD1 GAD2
4 paraneoplastic cerebellar degeneration 30.2 AMPH ELAVL3
5 hashimoto encephalopathy 29.7 ENO1 TPO
6 limbic encephalitis with lgi1 antibodies 12.7
7 non-herpetic acute limbic encephalitis 12.4
8 posttransplant acute limbic encephalitis 12.3
9 limbic encephalitis with dpp6 antibodies 12.3
10 classic paraneoplastic limbic encephalitis 12.3
11 limbic encephalitis with ncmags antibodies 12.3
12 limbic encephalitis with nmda receptor antibodies 12.3
13 limbic encephalitis with caspr2 antibodies 12.2
14 limbic encephalitis with neurexin-3 antibodies 12.2
15 encephalitis 11.3
16 neuropathy, hereditary sensory and autonomic, type iia 11.2
17 thymoma 10.5
18 lung cancer 10.5
19 teratoma 10.5
20 glioma 10.4
21 small cell cancer of the lung 10.4
22 lymphoma 10.4
23 ovarian germ cell teratoma 10.4
24 relapsing polychondritis 10.3
25 lymphoma, hodgkin, classic 10.3
26 temporal lobe epilepsy 10.3
27 encephalopathy 10.3
28 dementia 10.2
29 adenocarcinoma 10.2
30 neurosyphilis 10.2
31 breast cancer 10.2
32 aceruloplasminemia 10.2
33 testicular cancer 10.2
34 cerebellar degeneration 10.2
35 autoimmune polyendocrine syndrome, type ii 10.2 GAD1 GAD2
36 epilepsy, familial temporal lobe, 1 10.1 ADAM22 LGI1
37 spastic cerebral palsy 10.1 GAD1 GAD2
38 creutzfeldt-jakob disease 10.1
39 multiple sclerosis 10.1
40 systemic lupus erythematosus 10.1
41 neuroblastoma 10.1
42 thymoma, familial 10.1
43 b-cell lymphomas 10.1
44 lupus erythematosus 10.1
45 myoclonus 10.1
46 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 10.1 GAD1 GAD2
47 diabetes mellitus, ketosis-prone 10.1 GAD1 GAD2
48 von economo's disease 10.0 GRIN2B GSR
49 alzheimer disease 10.0
50 renal cell carcinoma, nonpapillary 10.0

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



Diseases related to Limbic Encephalitis
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Symptoms & Phenotypes for Limbic Encephalitis

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MGI Mouse Phenotypes related to Limbic Encephalitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.7 ADAM22 AMPH BRSK2 CNTN2 ELAVL3 GAD1
2 nervous system MP:0003631 9.36 ADAM22 AMPH BRSK2 CNTN2 ELAVL3 GAD1
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Drugs & Therapeutics for Limbic Encephalitis

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Drugs for Limbic Encephalitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antibodies Phase 2,Not Applicable
2 Immunoglobulins Phase 2,Not Applicable
3 Immunologic Factors Phase 2,Not Applicable
4 gamma-Globulins Phase 2
5 Immunoglobulins, Intravenous Phase 2
6 Rho(D) Immune Globulin Phase 2
7 Autoantibodies Phase 2
8
Tacrolimus Approved, Investigational Not Applicable 104987-11-3 445643 439492
9
Prednisone Approved, Vet_approved Not Applicable 53-03-2 5865
10 Anticonvulsants Not Applicable
11 Calcineurin Inhibitors Not Applicable
12 Immunosuppressive Agents Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 IVIG Treatment for Refractory Immune-Related Adult Epilepsy Terminated NCT01545518 Phase 2 IVIG
2 Interest Balance Auto-immune Population in Patients With Focal Epilepsy of Unknown Cause, Not Structural, Not Genetics, Newly Diagnosed Unknown status NCT02885207 Not Applicable
3 Immunotherapy of the Paraneoplastic Syndromes Completed NCT00378326 Not Applicable Tacrolimus
4 Antibodies Causing Epilepsy Syndromes: The ACES Study. Recruiting NCT02802475

Search NIH Clinical Center for Limbic Encephalitis

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Genetic Tests for Limbic Encephalitis

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Anatomical Context for Limbic Encephalitis

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MalaCards organs/tissues related to Limbic Encephalitis:

41
Brain, Lung, Testes, Temporal Lobe, B Cells, Thyroid, T Cells
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Publications for Limbic Encephalitis

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Articles related to Limbic Encephalitis:

(show top 50) (show all 588)
# Title Authors Year
1
Limbic Encephalitis in Association with Systemic Lupus Erythematosus. ( 29780149 )
2018
2
Anti-Hu antibody-mediated limbic encephalitis associated with cervical cancer: A case report. ( 29607578 )
2018
3
Limbic Encephalitis Manifesting as Selective Amnesia and Seizure-like Activity: A Case Report. ( 29397673 )
2018
4
Development of moyamoya disease after non-herpetic acute limbic encephalitis: A case report. ( 29731274 )
2018
5
Simultaneous 18F- FDG PET/MRI in Autoimmune Limbic Encephalitis. ( 29643688 )
2018
6
Paraneoplastic limbic encephalitis associated with lung cancer. ( 29717222 )
2018
7
Occupation-Associated Fatal Limbic Encephalitis Caused by Variegated Squirrel Bornavirus 1, Germany, 2013. ( 29774846 )
2018
8
Syndrome and outcome of antibody-negative limbic encephalitis. ( 29667271 )
2018
9
A case report of limbic encephalitis in a metastatic colon cancer patient during first-line bevacizumab-combined chemotherapy. ( 29489644 )
2018
10
Ondine's curse and temporal lobe seizures: anti-Hu- and Zic4-associated paraneoplastic brainstem and limbic encephalitis. ( 29667352 )
2018
11
Anti-Hu-Associated Autoimmune Limbic Encephalitis in a Patient with PD-1 Inhibitor-Responsive Myxoid Chondrosarcoma. ( 29158368 )
2018
12
Nivolumab-induced Limbic Encephalitis with Anti-Hu Antibody in a Patient With Advanced Pleomorphic Carcinoma of the Lung. ( 29857970 )
2018
13
Accelerated long-term forgetting in focal epilepsies with special consideration given to patients with diagnosed and suspected limbic encephalitis. ( 29475601 )
2018
14
Possible autoantibody-negative limbic encephalitis after anterior temporal lobectomy for hippocampal sclerosis. ( 29053037 )
2018
15
Paraneoplastic limbic encephalitis associated with mixed olfactory neuroblastoma and craniopharyngioma: A case report and literature review. ( 29901583 )
2018
16
Possible anti-VGKC autoimmune limbic encephalitis associated with SIADH. ( 29514833 )
2018
17
Anesthesia management in a case of Turner syndrome with anti-NMDA limbic encephalitis and multiple co-morbidities for repair of fracture femur. ( 29739177 )
2018
18
Pre- and long-term postoperative courses of hippocampus-associated memory impairment in epilepsy patients with antibody-associated limbic encephalitis and selective amygdalohippocampectomy. ( 29253681 )
2018
19
Paraneoplastic limbic encephalitis associated with mixed olfactory neuroblastoma and craniopharyngioma: A case report and literature review: Erratum. ( 29995829 )
2018
20
Cancer frequency detected by positron emission tomography-computed tomography in limbic encephalitis. ( 30408704 )
2018
21
Limbic encephalitis with relapsing polychondritis: persistent white matter lesions and brain atrophy. ( 30426814 )
2018
22
Natalizumab may control immune checkpoint inhibitor-induced limbic encephalitis. ( 30465016 )
2018
23
Striatum Involvement in LGI1 Limbic Encephalitis. ( 30466226 )
2018
24
Limbic encephalitis: Experience of a moroccan center. ( 30474361 )
2018
25
The role of cognitive rehabilitation in limbic encephalitis: A case report. ( 30508904 )
2018
26
Optic neuritis as the initial clinical presentation of limbic encephalitis: a case report. ( 30509326 )
2018
27
Toxic Epidermal Necrolysis in a Patient with Autoimmune Limbic Encephalitis with Anti-Glutamate Receptor Antibodies. ( 30186144 )
2018
28
Atypical development of neurosyphilis mimicking limbic encephalitis. ( 30253732 )
2018
29
Human herpesvirus 6 associated post-transplant acute limbic encephalitis: Clinical observations of biomarkers for risk of seizure in a pediatric population. ( 30256500 )
2018
30
Thymoma and Limbic Encephalitis: A Dangerous Liaison. ( 30277673 )
2018
31
A Survival Case of Super-refractory Status Epilepticus due to Glutamic Acid Decarboxylase Antibodies-associated Limbic Encephalitis. ( 30345184 )
2018
32
Pre- and postsynaptic effects of LGI1 autoantibodies in a murine model of limbic encephalitis. ( 30364979 )
2018
33
SUDEP following the second seizure in new-onset epilepsy due to limbic encephalitis. ( 30368169 )
2018
34
Limbic encephalitis associated with systemic sclerosis. ( 30005357 )
2018
35
Limbic Encephalitis in Patients with Epilepsy-is Quantitative MRI Diagnostic? ( 30014154 )
2018
36
Anesthesia management in a case of Turner syndrome with anti-NMDA limbic encephalitis and multiple co-morbidities for repair of fracture femur. ( 30071714 )
2018
37
Neurocardiac prodrome in LGI1-antibody-negative non-paraneoplastic limbic encephalitis. ( 30103960 )
2018
38
Three cases of antibody-LGI1 limbic encephalitis and review of literature. ( 30112956 )
2018
39
Autoimmune limbic encephalitis in association with acute stroke. ( 30140588 )
2018
40
A Patient with Limbic Encephalitis Associated with Anti-leucine-rich Glioma-inactivated 1 (LGI1) Antibody Presenting with Slowly Progressive Cognitive Impairment and Fluctuating Striatal Lesions. ( 30146575 )
2018
41
Primary B Cell Lymphoma of the CNS Mimicking Anti-LGI1 Limbic Encephalitis. ( 30147672 )
2018
42
Limbic encephalitis associated with antibodies against the α-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic acid receptor: a case report. ( 30183201 )
2018
43
Limbic encephalitis as a relapse of Whipple's disease with digestive involvement and spondylodiscitis. ( 29987509 )
2018
44
Emergence of new-onset psychotic disorder following recovery from LGI1 antibody-associated limbic encephalitis. ( 28363946 )
2017
45
Bilateral Hippocampal Infarction Mimicking Limbic Encephalitis. ( 29151536 )
2017
46
Limbic encephalitis - aA report of four cases. ( 28860940 )
2017
47
Limbic Encephalitis Associated with Human Herpesvirus-7 (HHV-7) in an Immunocompetent Adult: The First Reported Case in Japan. ( 28717094 )
2017
48
Dissociation findings between short-term and long-term memory in autoimmune limbic encephalitis. ( 28991662 )
2017
49
Semi-quantitative analysis of cerebral FDG-PET reveals striatal hypermetabolism and normal cortical metabolism in a case of VGKCC limbic encephalitis. ( 28424014 )
2017
50
Peripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review. ( 28948076 )
2017
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Variations for Limbic Encephalitis

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Expression for Limbic Encephalitis

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Search GEO for disease gene expression data for Limbic Encephalitis.
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Pathways for Limbic Encephalitis

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Pathways related to Limbic Encephalitis according to GeneCards Suite gene sharing:

(show all 12)
# Super pathways Score Top Affiliating Genes
1
Glucose / Energy Metabolism 4
12.05 BRSK2 ENO1 GAD1 GAD2
2
Long-term potentiation 37
Show member pathways
 11.89 GRIA1 GRIN2B PPP1R9B
3
Neuroscience 4
11.7 BRSK2 CNTN2 GAD1 GAD2 GRIA1 GRIN2B
4
beta-Alanine metabolism (KEGG) 37
Show member pathways
 11.04 GAD1 GAD2
5
Synthesis and interconversion of nucleotide di- and triphosphates 66
10.88 AK5 GSR
6
Biogenic Amine Synthesis 1
Show member pathways
 10.8 GAD1 GAD2
7
SALM protein interactions at the synapses 66
10.76 GRIA1 GRIN2B
8
Beta-alanine metabolism (TR) 1
Show member pathways
 10.66 GAD1 GAD2
9
Synaptic Neurotransmission Pathways: GABAergic Inhibition 65
10.6 GAD1 GAD2
10
LGI-ADAM interactions 66
10.5 ADAM22 LGI1
11
Alanine and aspartate metabolism 1
Show member pathways
 10.27 GAD1 GAD2
12
Taurine and hypotaurine metabolism 37
10 GAD1 GAD2
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GO Terms for Limbic Encephalitis

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

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.85 AMPH GAD2 GRIA1 GRIN2B LGI1
2 neuron projection GO:0043005 9.73 CNTN2 GRIA1 GRIN2B PPP1R9B
3 axon GO:0030424 9.67 ADAM22 CNTN2 GAD1 GAD2
4 cell surface GO:0009986 9.55 CNTN2 ENO1 GRIA1 GRIN2B TPO
5 synaptic membrane GO:0097060 9.48 GRIA1 GRIN2B
6 postsynaptic density membrane GO:0098839 9.4 GRIA1 GRIN2B
7 inhibitory synapse GO:0060077 9.32 GAD1 GAD2
8 dendritic spine membrane GO:0032591 9.26 GRIA1 PPP1R9B
9 synapse GO:0045202 9.17 AMPH CNTN2 GAD1 GAD2 GRIA1 GRIN2B
10 clathrin-sculpted gamma-aminobutyric acid transport vesicle membrane GO:0061202 8.96 GAD1 GAD2

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

# Name GO ID Score Top Affiliating Genes
1 neuron projection development GO:0031175 9.5 CNTN2 LGI1 PPP1R9B
2 nucleobase-containing small molecule interconversion GO:0015949 9.4 AK5 GSR
3 ionotropic glutamate receptor signaling pathway GO:0035235 9.37 GRIA1 GRIN2B
4 carboxylic acid metabolic process GO:0019752 9.26 GAD1 GAD2
5 neurotransmitter biosynthetic process GO:0042136 9.16 GAD1 GAD2
6 chemical synaptic transmission GO:0007268 9.02 AMPH GAD1 GAD2 GRIA1 GRIN2B
7 glutamate decarboxylation to succinate GO:0006540 8.96 GAD1 GAD2

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

# Name GO ID Score Top Affiliating Genes
1 lyase activity GO:0016829 9.5 ENO1 GAD1 GAD2
2 carboxy-lyase activity GO:0016831 9.26 GAD1 GAD2
3 ionotropic glutamate receptor activity GO:0004970 9.16 GRIA1 GRIN2B
4 glutamate decarboxylase activity GO:0004351 8.96 GAD1 GAD2
5 glutamate binding GO:0016595 8.8 GAD1 GAD2 GRIN2B
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Sources for Limbic Encephalitis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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