MCID: LMB024
MIFTS: 42

Limbic Encephalitis

Categories: Rare diseases

Aliases & Classifications for Limbic Encephalitis

MalaCards integrated aliases for Limbic Encephalitis:

Name: Limbic Encephalitis 49 51 69

Classifications:



External Ids:

UMLS 69 C0338430

Summaries for Limbic Encephalitis

NIH Rare Diseases : 49 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. Last updated: 4/26/2016

MalaCards based summary : Limbic Encephalitis is related to alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 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 Immunoglobulins and Antibodies 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 : 72 Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain.... more...

Related Diseases for Limbic Encephalitis

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

(show top 50) (show all 130)
# Related Disease Score Top Affiliating Genes
1 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 30.5 GAD1 GAD2 TPO
2 status epilepticus 30.5 GRIA1 GRIN2B LGI1
3 temporal lobe epilepsy 30.4 GAD1 GRIN2B LGI1
4 stiff-person syndrome 30.1 AMPH GAD1 GAD2
5 paraneoplastic cerebellar degeneration 30.0 AMPH ELAVL3
6 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 29.8 GAD1 GAD2
7 limbic encephalitis with lgi1 antibodies 12.6
8 non-herpetic acute limbic encephalitis 12.2
9 posttransplant acute limbic encephalitis 12.2
10 limbic encephalitis with dpp6 antibodies 12.2
11 classic paraneoplastic limbic encephalitis 12.1
12 limbic encephalitis with ncmags antibodies 12.1
13 limbic encephalitis with nmda receptor antibodies 12.1
14 limbic encephalitis with caspr2 antibodies 12.1
15 limbic encephalitis with neurexin-3 antibodies 12.1
16 encephalitis 11.1
17 neuropathy, hereditary sensory and autonomic, type iia 11.1
18 epilepsy 10.3
19 lung cancer 10.3
20 neuronitis 10.3
21 autoimmune polyendocrine syndrome, type ii 10.3 GAD1 GAD2
22 thymoma 10.3
23 thymoma, familial 10.3
24 small cell cancer of the lung 10.3
25 epilepsy, familial temporal lobe, 1 10.3 ADAM22 LGI1
26 teratoma 10.3
27 glioma 10.3
28 lymphoma 10.2
29 ovarian germ cell teratoma 10.2
30 spastic cerebral palsy 10.2 GAD1 GAD2
31 relapsing polychondritis 10.2
32 von economo's disease 10.2 GRIN2B GSR
33 diabetes mellitus, ketosis-prone 10.2 GAD1 GAD2
34 lymphoma, hodgkin, classic 10.1
35 ocular dominance 10.1 GAD1 GRIN2B
36 dementia 10.1
37 cerebellar degeneration 10.0
38 adenocarcinoma 10.0
39 testicular cancer 10.0
40 neurosyphilis 10.0
41 encephalopathy 10.0
42 paraneoplastic syndromes 10.0
43 cerebritis 10.0
44 prostatitis 10.0
45 anterograde amnesia 9.9
46 lambert-eaton myasthenic syndrome 9.9
47 psychotic disorder 9.9
48 myoclonus 9.9
49 alzheimer disease 9.9
50 myasthenia gravis 9.9

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



Diseases related to Limbic Encephalitis

Symptoms & Phenotypes for Limbic Encephalitis

MGI Mouse Phenotypes related to Limbic Encephalitis:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.65 AMPH BRSK2 ELAVL3 GAD1 GAD2 GRIA1
2 nervous system MP:0003631 9.32 AMPH BRSK2 ELAVL3 GAD1 GAD2 GRIA1

Drugs & Therapeutics for Limbic Encephalitis

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

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulins Phase 2
2 Antibodies Phase 2
3 Rho(D) Immune Globulin Phase 2
4 gamma-Globulins Phase 2
5 Immunoglobulins, Intravenous Phase 2
6 Autoantibodies Phase 2
7
Tacrolimus Approved, Investigational 104987-11-3 445643 439492
8
Prednisone Approved, Vet_approved 53-03-2 5865
9 Immunosuppressive Agents
10 Calcineurin Inhibitors
11 Anticonvulsants

Interventional clinical trials:


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

Search NIH Clinical Center for Limbic Encephalitis

Genetic Tests for Limbic Encephalitis

Anatomical Context for Limbic Encephalitis

MalaCards organs/tissues related to Limbic Encephalitis:

38
Brain, Lung, Testes, Temporal Lobe, Prostate, Bone Marrow, B Cells

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 497)
# Title Authors Year
1
Possible autoantibody-negative limbic encephalitis after anterior temporal lobectomy for hippocampal sclerosis. ( 29053037 )
2018
2
Limbic Encephalitis Manifesting as Selective Amnesia and Seizure-like Activity: A Case Report. ( 29397673 )
2018
3
Pre- and long-term postoperative courses of hippocampus-associated memory impairment in epilepsy patients with antibody-associated limbic encephalitis and selective amygdalohippocampectomy. ( 29253681 )
2018
4
Anti-Hu-Associated Autoimmune Limbic Encephalitis in a Patient with PD-1 Inhibitor-Responsive Myxoid Chondrosarcoma. ( 29158368 )
2018
5
Peripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review. ( 28948076 )
2017
6
Clinical features of limbic encephalitis with LGI1 antibody. ( 28670128 )
2017
7
Limbic encephalitis - aA report of four cases. ( 28860940 )
2017
8
Autoimmune Limbic Encephalitis Associated With Type 1 Diabetes Mellitus. ( 28720552 )
2017
9
Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) with Limbic Encephalitis. ( 28824066 )
2017
10
Selective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies. ( 29093718 )
2017
11
Three case studies of nonparaneoplastic limbic encephalitis (NPLE) in young adult males: Onset, course, and recovery following rehabilitation services. ( 29185868 )
2017
12
Paraneoplastic limbic encephalitis with late-onset magnetic resonance imaging findings: A case report. ( 28781798 )
2017
13
Delayed LGI1 seropositivity in voltage-gated potassium channel (VGKC)-complex antibody limbic encephalitis. ( 28432047 )
2017
14
A Likely Case of Limbic Encephalitis in a Patient With Voltage-Gated Potassium Channel Complex Antibody, Without a Known Antigenic Target: A Review of the Disease State and Value of Antibody Titers. ( 28750836 )
2017
15
Paraneoplastic Lambert-Eaton Myasthenic Syndrome With Limbic Encephalitis: Clinical Correlation With the Coexistence of Anti-VGCC and Anti-GABAB Receptor Antibodies. ( 29189554 )
2017
16
A Case of Paraneoplastic Limbic Encephalitis in a Patient with Invasive Thymoma with Anti-Glutamate Receptor Antibody-Positive Cerebrospinal Fluid: A Case Report. ( 29269707 )
2017
17
Seronegative Paraneoplastic Limbic Encephalitis Associated with Thymoma. ( 28926420 )
2017
18
Paraneoplastic limbic encephalitis in a patient with extensive disease small-cell lung cancer. ( 28413671 )
2017
19
Gamma-aminobutyric acid-B limbic encephalitis and asystolic cardiac arrest: a case report. ( 29287596 )
2017
20
Semi-quantitative analysis of cerebral FDG-PET reveals striatal hypermetabolism and normal cortical metabolism in a case of VGKCC limbic encephalitis. ( 28424014 )
2017
21
Latent autoimmune diabetes and limbic encephalitis with antibodies against glutamic acid decarboxylase. ( 29154389 )
2017
22
Erratum: A case of neurosyphilis presenting with limbic encephalitis. ( 28458373 )
2017
23
A rare case of autoimmune limbic encephalitis: an uncharted territory! ( 29057855 )
2017
24
GAD antibody-associated limbic encephalitis in a young woman with APECED. ( 28567288 )
2017
25
Multiple paraneoplastic antibodies (anti-SOX1, anti-Hu, and anti-Amphiphysin) detected in a patient with limbic encephalitis and small cell lung cancer. ( 28879908 )
2017
26
AUTOIMMUNE LIMBIC ENCEPHALITIS (CASE REPORTS). ( 28628018 )
2017
27
Seizure semiology in leucine-rich glioma-inactivated protein 1 antibody-associated limbic encephalitis. ( 29050866 )
2017
28
Small-cell lung carcinoma and acute onset of antiglial nuclear antibody-positive limbic encephalitis. ( 28396782 )
2017
29
Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report. ( 28984777 )
2017
30
High-grade glioma mimicking limbic encephalitis on (18)F-FDG PET/CT scan. ( 28610872 )
2017
31
Tuberculous limbic encephalitis: A case report. ( 28606665 )
2017
32
Limbic Encephalitis Associated with Human Herpesvirus-7 (HHV-7) in an Immunocompetent Adult: The First Reported Case in Japan. ( 28717094 )
2017
33
Bilateral Hippocampal Infarction Mimicking Limbic Encephalitis. ( 29151536 )
2017
34
Limbic encephalitis with LGI1 antibodies in a 14-year-old boy. ( 28919330 )
2017
35
Characteristics in limbic encephalitis with anti-adenylate kinase 5 autoantibodies. ( 28062719 )
2017
36
Sarcoidosis limbic encephalitis: A case report. ( 29114371 )
2017
37
Emergence of new-onset psychotic disorder following recovery from LGI1 antibody-associated limbic encephalitis. ( 28363946 )
2017
38
Dissociation findings between short-term and long-term memory in autoimmune limbic encephalitis. ( 28991662 )
2017
39
Do we need to measure specific antibodies in patients with limbic encephalitis? ( 28062718 )
2017
40
Limbic Encephalitis Associated with SjAPgren's Syndrome: Report of Three Cases. ( 27523010 )
2016
41
Limbic encephalitis associated with glutamic acid decarboxylase antibodies in a young adolescent. ( 27117742 )
2016
42
Type 1 diabetes and GAD65 limbic encephalitis: a case report of a 10-year-old girl. ( 27115322 )
2016
43
Limbic encephalitis following immunotherapy against metastatic malignant melanoma. ( 27009198 )
2016
44
Paraneoplastic Limbic Encephalitis in a Human Epidermal Growth Factor Receptor-2-positive Gastric Cancer Patient Treated with Trastuzumab-combined Chemotherapy: A Case Report and Literature Review. ( 27629954 )
2016
45
Limbic encephalitis associated with anti-leucine-rich glioma-inactivated-1 protein antibodies in a child. ( 27841212 )
2016
46
Clinical characterization of autoimmune LGI1 antibody limbic encephalitis. ( 26896820 )
2016
47
Editorial: Limbic Encephalitis: Autoimmune Impact on Inhibitory GABAergic Neuronal Pathways in Temporal Lobe Epilepsy. ( 27014186 )
2016
48
Small-Cell Lung Cancer with Positive Anti-NMDAR and Anti-AMPAR Antibodies Paraneoplastic Limbic Encephalitis. ( 28070431 )
2016
49
Neuropsychological and FDG-PET profiles in VGKC autoimmune limbic encephalitis. ( 27566001 )
2016
50
Hyperfamiliarity for unknown faces after non-paraneoplastic limbic encephalitis: a case report. ( 27334285 )
2016

Variations for Limbic Encephalitis

Expression for Limbic Encephalitis

Search GEO for disease gene expression data for Limbic Encephalitis.

Pathways for Limbic Encephalitis

GO Terms for Limbic Encephalitis

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

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.72 AMPH GAD2 GRIA1 GRIN2B LGI1
2 inhibitory synapse GO:0060077 9.26 GAD1 GAD2
3 dendritic spine membrane GO:0032591 9.16 GRIA1 PPP1R9B
4 synapse GO:0045202 9.1 AMPH GAD1 GAD2 GRIA1 GRIN2B LGI1
5 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 nucleobase-containing small molecule interconversion GO:0015949 9.37 AK5 GSR
2 ionotropic glutamate receptor signaling pathway GO:0035235 9.32 GRIA1 GRIN2B
3 carboxylic acid metabolic process GO:0019752 9.26 GAD1 GAD2
4 neurotransmitter biosynthetic process GO:0042136 9.16 GAD1 GAD2
5 chemical synaptic transmission GO:0007268 9.02 AMPH GAD1 GAD2 GRIA1 GRIN2B
6 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 9.37 GAD1 GAD2
3 extracellularly glutamate-gated ion channel activity GO:0005234 9.26 GRIA1 GRIN2B
4 ionotropic glutamate receptor activity GO:0004970 9.16 GRIA1 GRIN2B
5 glutamate decarboxylase activity GO:0004351 8.96 GAD1 GAD2
6 glutamate binding GO:0016595 8.8 GAD1 GAD2 GRIN2B

Sources for Limbic Encephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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