MCID: LMB024
MIFTS: 41

Limbic Encephalitis

Categories: Rare diseases

Aliases & Classifications for Limbic Encephalitis

MalaCards integrated aliases for Limbic Encephalitis:

Name: Limbic Encephalitis 53 55 73

Classifications:



External Ids:

UMLS 73 C0338430

Summaries for Limbic Encephalitis

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 status epilepticus and stiff-person syndrome. 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...

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 136)
# Related Disease Score Top Affiliating Genes
1 status epilepticus 30.5 GRIA1 GRIN2B LGI1
2 stiff-person syndrome 30.2 AMPH GAD1 GAD2
3 paraneoplastic cerebellar degeneration 30.0 AMPH ELAVL3
4 limbic encephalitis with lgi1 antibodies 12.6
5 non-herpetic acute limbic encephalitis 12.2
6 posttransplant acute limbic encephalitis 12.2
7 limbic encephalitis with dpp6 antibodies 12.2
8 limbic encephalitis with nmda receptor antibodies 12.1
9 classic paraneoplastic limbic encephalitis 12.1
10 limbic encephalitis with ncmags antibodies 12.1
11 limbic encephalitis with caspr2 antibodies 12.1
12 limbic encephalitis with neurexin-3 antibodies 12.1
13 encephalitis 11.1
14 neuropathy, hereditary sensory and autonomic, type iia 11.1
15 autoimmune polyendocrine syndrome, type ii 10.5 GAD1 GAD2
16 epilepsy, familial temporal lobe, 1 10.5 ADAM22 LGI1
17 spastic cerebral palsy 10.4 GAD1 GAD2
18 epilepsy 10.4
19 lung cancer 10.3
20 neuronitis 10.3
21 teratoma 10.3
22 von economo's disease 10.3 GRIN2B GSR
23 small cell cancer of the lung 10.3
24 thymoma 10.3
25 diabetes mellitus, ketosis-prone 10.3 GAD1 GAD2
26 ovarian germ cell teratoma 10.3
27 glioma 10.3
28 lymphoma 10.2
29 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 10.2 GAD1 GAD2
30 relapsing polychondritis 10.2
31 ocular dominance 10.2 GAD1 GRIN2B
32 temporal lobe epilepsy 10.1
33 lymphoma, hodgkin, classic 10.1
34 dementia 10.1
35 neurosyphilis 10.0
36 adenocarcinoma 10.0
37 testicular cancer 10.0
38 cerebellar degeneration 10.0
39 encephalopathy 10.0
40 breast cancer 10.0
41 prostatitis 10.0
42 cerebritis 10.0
43 paraneoplastic syndromes 10.0
44 systemic lupus erythematosus 9.9
45 ovarian cancer 9.9
46 aceruloplasminemia 9.9
47 psychotic disorder 9.9
48 myasthenia gravis congenital 9.9
49 prostate cancer 9.9
50 lymphoma, non-hodgkin, familial 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:

46
# 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 Antibodies Phase 2,Not Applicable
2 Immunoglobulins Phase 2,Not Applicable
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 Not Applicable 104987-11-3 445643 439492
8
Prednisone Approved, Vet_approved Not Applicable 53-03-2 5865
9 Immunosuppressive Agents Not Applicable
10 Calcineurin Inhibitors Not Applicable
11 Anticonvulsants 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 Immunotherapy of the Paraneoplastic Syndromes Completed NCT00378326 Not Applicable 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 Not Applicable

Search NIH Clinical Center for Limbic Encephalitis

Genetic Tests for Limbic Encephalitis

Anatomical Context for Limbic Encephalitis

MalaCards organs/tissues related to Limbic Encephalitis:

41
Brain, Lung, Testes, Temporal Lobe, Colon, Bone Marrow, B Cells

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 513)
# 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
Emergence of new-onset psychotic disorder following recovery from LGI1 antibody-associated limbic encephalitis. ( 28363946 )
2017
20
Bilateral Hippocampal Infarction Mimicking Limbic Encephalitis. ( 29151536 )
2017
21
Limbic encephalitis - aA report of four cases. ( 28860940 )
2017
22
Limbic Encephalitis Associated with Human Herpesvirus-7 (HHV-7) in an Immunocompetent Adult: The First Reported Case in Japan. ( 28717094 )
2017
23
Dissociation findings between short-term and long-term memory in autoimmune limbic encephalitis. ( 28991662 )
2017
24
Semi-quantitative analysis of cerebral FDG-PET reveals striatal hypermetabolism and normal cortical metabolism in a case of VGKCC limbic encephalitis. ( 28424014 )
2017
25
Peripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review. ( 28948076 )
2017
26
Erratum: A case of neurosyphilis presenting with limbic encephalitis. ( 28458373 )
2017
27
Limbic encephalitis with LGI1 antibodies in a 14-year-old boy. ( 28919330 )
2017
28
Small-cell lung carcinoma and acute onset of antiglial nuclear antibody-positive limbic encephalitis. ( 28396782 )
2017
29
Do we need to measure specific antibodies in patients with limbic encephalitis? ( 28062718 )
2017
30
Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report. ( 28984777 )
2017
31
Gamma-aminobutyric acid-B limbic encephalitis and asystolic cardiac arrest: a case report. ( 29287596 )
2017
32
A rare case of autoimmune limbic encephalitis: an uncharted territory! ( 29057855 )
2017
33
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
34
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
35
Sarcoidosis limbic encephalitis: A case report. ( 29114371 )
2017
36
Paraneoplastic Lambert-Eaton Myasthenic Syndrome With Limbic Encephalitis: Clinical Correlation With the Coexistence of Anti-VGCC and Anti-GABAB Receptor Antibodies. ( 29189554 )
2017
37
Selective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies. ( 29093718 )
2017
38
Autoimmune Limbic Encephalitis Associated With Type 1 Diabetes Mellitus. ( 28720552 )
2017
39
Characteristics in limbic encephalitis with anti-adenylate kinase 5 autoantibodies. ( 28062719 )
2017
40
GAD antibody-associated limbic encephalitis in a young woman with APECED. ( 28567288 )
2017
41
Latent autoimmune diabetes and limbic encephalitis with antibodies against glutamic acid decarboxylase. ( 29154389 )
2017
42
Paraneoplastic limbic encephalitis in a patient with extensive disease small-cell lung cancer. ( 28413671 )
2017
43
AUTOIMMUNE LIMBIC ENCEPHALITIS (CASE REPORTS). ( 28628018 )
2017
44
Seronegative Paraneoplastic Limbic Encephalitis Associated with Thymoma. ( 28926420 )
2017
45
Paraneoplastic limbic encephalitis with late-onset magnetic resonance imaging findings: A case report. ( 28781798 )
2017
46
Tuberculous limbic encephalitis: A case report. ( 28606665 )
2017
47
Delayed LGI1 seropositivity in voltage-gated potassium channel (VGKC)-complex antibody limbic encephalitis. ( 28432047 )
2017
48
Three case studies of nonparaneoplastic limbic encephalitis (NPLE) in young adult males: Onset, course, and recovery following rehabilitation services. ( 29185868 )
2017
49
High-grade glioma mimicking limbic encephalitis on (18)F-FDG PET/CT scan. ( 28610872 )
2017
50
Clinical features of limbic encephalitis with LGI1 antibody. ( 28670128 )
2017

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 postsynaptic density membrane GO:0098839 9.37 GRIA1 GRIN2B
3 dendritic spine membrane GO:0032591 9.26 GRIA1 PPP1R9B
4 inhibitory synapse GO:0060077 9.16 GAD1 GAD2
5 synapse GO:0045202 9.1 AMPH GAD1 GAD2 GRIA1 GRIN2B LGI1
6 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 ionotropic glutamate receptor activity GO:0004970 9.26 GRIA1 GRIN2B
4 extracellularly glutamate-gated ion channel activity GO:0005234 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
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
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