MCID: LMB024
MIFTS: 43

Limbic Encephalitis

Categories: Cancer diseases, Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Limbic Encephalitis

MalaCards integrated aliases for Limbic Encephalitis:

Name: Limbic Encephalitis 54 56 74

Classifications:



External Ids:

UMLS 74 C0338430

Summaries for Limbic Encephalitis

NIH Rare Diseases : 54 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 stiff-person syndrome 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 : 77 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 164)
# Related Disease Score Top Affiliating Genes
1 stiff-person syndrome 30.5 AMPH GAD1 GAD2
2 status epilepticus 30.3 GRIA1 GRIN2B LGI1
3 paraneoplastic cerebellar degeneration 30.2 AMPH ELAVL3
4 epilepsy 30.1 CNTN2 GAD1 GRIN2B GSR LGI1
5 hashimoto encephalopathy 29.7 ENO1 TPO
6 limbic encephalitis with lgi1 antibodies 12.8
7 non-herpetic acute limbic encephalitis 12.4
8 posttransplant acute limbic encephalitis 12.4
9 limbic encephalitis with dpp6 antibodies 12.4
10 classic paraneoplastic limbic encephalitis 12.3
11 limbic encephalitis with nmda receptor antibodies 12.3
12 limbic encephalitis with caspr2 antibodies 12.3
13 limbic encephalitis with neurexin-3 antibodies 12.3
14 neuropathy, hereditary sensory and autonomic, type iia 11.3
15 encephalitis 11.3
16 thymoma, familial 10.5
17 thymoma 10.5
18 lung cancer 10.5
19 glioma 10.5
20 teratoma 10.5
21 small cell cancer of the lung 10.5
22 lymphoma 10.4
23 ovarian germ cell teratoma 10.4
24 amnestic disorder 10.4
25 relapsing polychondritis 10.4
26 lymphoma, hodgkin, classic 10.3
27 encephalopathy 10.3
28 autoimmune polyendocrine syndrome, type ii 10.3 GAD1 GAD2
29 temporal lobe epilepsy 10.3
30 adenocarcinoma 10.3
31 neurosyphilis 10.3
32 seizure disorder 10.3
33 spastic cerebral palsy 10.3 GAD1 GAD2
34 epilepsy, familial temporal lobe, 1 10.3 ADAM22 LGI1
35 dementia 10.2
36 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 10.2 GAD1 GAD2
37 diabetes mellitus, ketosis-prone 10.2 GAD1 GAD2
38 breast cancer 10.2
39 systemic lupus erythematosus 10.2
40 aceruloplasminemia 10.2
41 asymptomatic neurosyphilis 10.2
42 testicular cancer 10.2
43 lupus erythematosus 10.2
44 cerebellar degeneration 10.2
45 creutzfeldt-jakob disease 10.1
46 multiple sclerosis 10.1
47 myasthenia gravis 10.1
48 sjogren syndrome 10.1
49 small cell carcinoma 10.1
50 myasthenia gravis congenital 10.1

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:

47
# 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

Drugs & Therapeutics for Limbic Encephalitis

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 Immunoglobulins, Intravenous Phase 2
5 Rho(D) Immune Globulin Phase 2
6 Autoantibodies Phase 2
7 gamma-Globulins Phase 2
8
Prednisone Approved, Vet_approved Not Applicable 53-03-2 5865
9
Tacrolimus Approved, Investigational Not Applicable 104987-11-3 6473866 445643 439492
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

Genetic Tests for Limbic Encephalitis

Anatomical Context for Limbic Encephalitis

MalaCards organs/tissues related to Limbic Encephalitis:

42
Brain, Lung, Testes, Temporal Lobe, Prostate, B Cells, Colon

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 608)
# Title Authors Year
1
Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Limbic Encephalitis in a Patient with Adenocarcinoma of Prostate: A Case Report. ( 30692776 )
2019
2
Anti-Amphiphysin-associated limbic encephalitis in a 72-year-old patient with aortic angiosarcoma. ( 30872337 )
2019
3
Fulminant thymomatous AMPAR-antibody limbic encephalitis with hypertonic coma, bruxism, an isoelectric electroencephalogram and temporal cortical atrophy, with recovery. ( 30796080 )
2019
4
Deleted in colorectal cancer (netrin-1 receptor) antibodies and limbic encephalitis in a cat with hippocampal necrosis. ( 30942925 )
2019
5
Clinical variants of limbic encephalitis. ( 31105945 )
2019
6
Motor neuron disease as a treatment responsive paraneoplastic neurological syndrome in patient with small cell lung cancer, anti-Hu antibodies and limbic encephalitis. ( 30953906 )
2019
7
Hashimoto's Encephalopathy Presenting with Smoldering Limbic Encephalitis. ( 30982805 )
2019
8
Atypical Limbic Encephalitis and its Complex Psychiatric Presentations: Implications for Diagnosis and Management. ( 30983671 )
2019
9
Anterior cingulate cortex involvement in non-paraneoplastic limbic encephalitis. ( 31005361 )
2019
10
Conventional brain MRI features distinguishing limbic encephalitis from mesial temporal glioma. ( 31028423 )
2019
11
GABABR-Mediated Paraneoplastic Limbic Encephalitis Due To Thymic Small Cell Carcinoma. ( 31062226 )
2019
12
Hippocampal network abnormalities explain amnesia after VGKCC-Ab related autoimmune limbic encephalitis. ( 31072956 )
2019
13
Long-Term Memory Dysfunction in Limbic Encephalitis. ( 31080433 )
2019
14
Olfactory stimulus-induced temporal lobe seizures in limbic encephalitis. ( 31082694 )
2019
15
A Case Report of Neurosyphilis Limbic Encephalitis With Reversible Geschwind Syndrome and Mood Disorder. ( 31083037 )
2019
16
Diagnosing autoimmune limbic encephalitis. ( 31085562 )
2019
17
Limbic encephalitis-like presentation in neuromyelitis optica spectrum disorder: a case report. ( 30835052 )
2019
18
Paraneoplastic limbic encephalitis revealing a small cell carcinoma of the lung. ( 30622890 )
2019
19
Limbic encephalitis: Experience of a moroccan center. ( 30474361 )
2019
20
Atypical development of neurosyphilis mimicking limbic encephalitis. ( 30253732 )
2019
21
Human herpesvirus 6 associated post-transplant acute limbic encephalitis: Clinical observations of biomarkers for risk of seizure in a pediatric population. ( 30256500 )
2019
22
Three cases of antibody-LGI1 limbic encephalitis and review of literature. ( 30112956 )
2019
23
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 )
2019
24
Accelerated long-term forgetting in focal epilepsies with special consideration given to patients with diagnosed and suspected limbic encephalitis. ( 29475601 )
2019
25
Three case studies of nonparaneoplastic limbic encephalitis (NPLE) in young adult males: Onset, course, and recovery following rehabilitation services. ( 29185868 )
2019
26
Anti-Hu antibody-mediated limbic encephalitis associated with cervical cancer: A case report. ( 29607578 )
2018
27
Anti-Hu-Associated Autoimmune Limbic Encephalitis in a Patient with PD-1 Inhibitor-Responsive Myxoid Chondrosarcoma. ( 29158368 )
2018
28
Paraneoplastic limbic encephalitis associated with mixed olfactory neuroblastoma and craniopharyngioma: A case report and literature review. ( 29901583 )
2018
29
Paraneoplastic limbic encephalitis associated with mixed olfactory neuroblastoma and craniopharyngioma: A case report and literature review: Erratum. ( 29995829 )
2018
30
Autoimmune Epilepsy and/or Limbic Encephalitis Can Lead to Changes in Sleep Spindles. ( 30622387 )
2018
31
Gastaut-Geschwind Syndrome, Faciobrachial Dystonic Seizure, and Autoimmune Limbic Encephalitis. ( 30631627 )
2018
32
Thymoma-associated Myasthenia Gravis in a Young Adult with Development of Paraneoplastic Limbic Encephalitis and Systemic Lupus Erythematosus Post-thymectomy: A Case Report. ( 30656085 )
2018
33
SUDEP following the second seizure in new-onset epilepsy due to limbic encephalitis. ( 30368169 )
2018
34
Cancer frequency detected by positron emission tomography-computed tomography in limbic encephalitis. ( 30408704 )
2018
35
Limbic encephalitis with relapsing polychondritis: persistent white matter lesions and brain atrophy. ( 30426814 )
2018
36
Natalizumab may control immune checkpoint inhibitor-induced limbic encephalitis. ( 30465016 )
2018
37
Striatum Involvement in LGI1 Limbic Encephalitis. ( 30466226 )
2018
38
The role of cognitive rehabilitation in limbic encephalitis: A case report. ( 30508904 )
2018
39
Optic neuritis as the initial clinical presentation of limbic encephalitis: a case report. ( 30509326 )
2018
40
Limbic encephalitis associated with antibodies against the α-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic acid receptor: a case report. ( 30183201 )
2018
41
Toxic Epidermal Necrolysis in a Patient with Autoimmune Limbic Encephalitis with Anti-Glutamate Receptor Antibodies. ( 30186144 )
2018
42
Thymoma and Limbic Encephalitis: A Dangerous Liaison. ( 30277673 )
2018
43
A Survival Case of Super-refractory Status Epilepticus due to Glutamic Acid Decarboxylase Antibodies-associated Limbic Encephalitis. ( 30345184 )
2018
44
Pre- and postsynaptic effects of LGI1 autoantibodies in a murine model of limbic encephalitis. ( 30364979 )
2018
45
Limbic encephalitis as a relapse of Whipple's disease with digestive involvement and spondylodiscitis. ( 29987509 )
2018
46
Limbic encephalitis associated with systemic sclerosis. ( 30005357 )
2018
47
Limbic Encephalitis in Patients with Epilepsy-is Quantitative MRI Diagnostic? ( 30014154 )
2018
48
Anesthesia management in a case of Turner syndrome with anti-NMDA limbic encephalitis and multiple co-morbidities for repair of fracture femur. ( 30071714 )
2018
49
Neurocardiac prodrome in LGI1-antibody-negative non-paraneoplastic limbic encephalitis. ( 30103960 )
2018
50
Autoimmune limbic encephalitis in association with acute stroke. ( 30140588 )
2018

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 9.85 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

Sources for Limbic Encephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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