MCID: LMB024
MIFTS: 37

Limbic Encephalitis

Categories: Rare diseases

Aliases & Classifications for Limbic Encephalitis

MalaCards integrated aliases for Limbic Encephalitis:

Name: Limbic Encephalitis 50 52 69

Classifications:



Summaries for Limbic Encephalitis

NIH Rare Diseases : 50 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 limbic encephalitis with lgi1 antibodies and non-herpetic acute limbic encephalitis. An important gene associated with Limbic Encephalitis is GAD1 (Glutamate Decarboxylase 1), and among its related pathways/superpathways are Long-term potentiation and Neuroscience. 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 : 72 Limbic encephalitis is a form of encephalitis, a disease characterised 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 100)
id Related Disease Score Top Affiliating Genes
1 limbic encephalitis with lgi1 antibodies 12.1
2 non-herpetic acute limbic encephalitis 12.1
3 posttransplant acute limbic encephalitis 12.1
4 limbic encephalitis with dpp6 antibodies 12.0
5 classic paraneoplastic limbic encephalitis 12.0
6 limbic encephalitis with ncmags antibodies 12.0
7 limbic encephalitis with nmda receptor antibodies 12.0
8 limbic encephalitis with caspr2 antibodies 11.9
9 encephalitis 11.0
10 neuropathy, hereditary sensory and autonomic, type ii 11.0
11 satoyoshi syndrome 10.4 GAD1 GAD2
12 immunodeficiency, common variable, 10 10.4 ADAM22 LGI1
13 dyskinetic cerebral palsy 10.3 GAD1 GAD2
14 atrial tachyarrhythmia with short pr interval 10.3 AMPH GAD1 GAD2
15 lung cancer 10.2
16 epilepsy 10.2
17 neuronitis 10.2
18 subungual glomus tumor 10.2 GRIN2B GSR
19 thymoma 10.2
20 teratoma 10.2
21 testicular lymphoma 10.2 GAD1 GRIN2B LGI1
22 glioma 10.2
23 biliary cirrhosis, primary, 4 10.2 GAD1 GAD2
24 idiopathic generalized epilepsy 10.1 GRIA1 GRIN2B LGI1
25 limb defects, distal transverse, with mental retardation and spasticity 10.1 GRIN2B GSR
26 lymphoma 10.1
27 status epilepticus 10.1
28 relapsing polychondritis 10.1
29 parathyroid cancer, childhood 10.1 AMPH ELAVL3
30 temporal lobe epilepsy 10.1
31 hodgkin lymphoma 10.0
32 dementia 10.0
33 adenocarcinoma 10.0
34 testicular cancer 10.0
35 cerebellar degeneration 10.0
36 neurosyphilis 10.0
37 breast cancer 9.9
38 prostatitis 9.9
39 cerebritis 9.9
40 paraneoplastic cerebellar degeneration 9.9
41 encephalopathy 9.9
42 paraneoplastic syndromes 9.9
43 prostate cancer 9.8
44 renal cell carcinoma 9.8
45 cerebellar ataxia 9.8
46 creutzfeldt-jakob disease 9.8
47 stiff-person syndrome 9.8
48 leukemia 9.8
49 myasthenia gravis 9.8
50 squamous cell carcinoma 9.8

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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.65 ADAM22 AMPH BRSK2 ELAVL3 GAD1 GAD2
2 nervous system MP:0003631 9.32 ELAVL3 GAD1 GAD2 GRIA1 GRIN2B LGI1

Drugs & Therapeutics for Limbic Encephalitis

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

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

Interventional clinical trials:


id 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:

39
Brain, Lung, Testes, Temporal Lobe, Bone Marrow, Prostate, Breast

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 480)
id Title Authors Year
1
Clinical features of limbic encephalitis with LGI1 antibody. ( 28670128 )
2017
2
Do we need to measure specific antibodies in patients with limbic encephalitis? ( 28062718 )
2017
3
Emergence of new-onset psychotic disorder following recovery from LGI1 antibody-associated limbic encephalitis. ( 28363946 )
2017
4
GAD antibody-associated limbic encephalitis in a young woman with APECED. ( 28567288 )
2017
5
Limbic encephalitis - aA report of four cases. ( 28860940 )
2017
6
Seronegative Paraneoplastic Limbic Encephalitis Associated with Thymoma. ( 28926420 )
2017
7
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
8
Autoimmune Limbic Encephalitis Associated With Type 1 Diabetes Mellitus. ( 28720552 )
2017
9
Paraneoplastic limbic encephalitis with late-onset magnetic resonance imaging findings: A case report. ( 28781798 )
2017
10
Limbic encephalitis with LGI1 antibodies in a 14-year-old boy. ( 28919330 )
2017
11
High-grade glioma mimicking limbic encephalitis on (18)F-FDG PET/CT scan. ( 28610872 )
2017
12
AUTOIMMUNE LIMBIC ENCEPHALITIS (CASE REPORTS). ( 28628018 )
2017
13
Semi-quantitative analysis of cerebral FDG-PET reveals striatal hypermetabolism and normal cortical metabolism in a case of VGKCC limbic encephalitis. ( 28424014 )
2017
14
Erratum: A case of neurosyphilis presenting with limbic encephalitis. ( 28458373 )
2017
15
Delayed LGI1 seropositivity in voltage-gated potassium channel (VGKC)-complex antibody limbic encephalitis. ( 28432047 )
2017
16
Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) with Limbic Encephalitis. ( 28824066 )
2017
17
Small-cell lung carcinoma and acute onset of antiglial nuclear antibody-positive limbic encephalitis. ( 28396782 )
2017
18
Peripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review. ( 28948076 )
2017
19
Multiple paraneoplastic antibodies (anti-SOX1, anti-Hu, and anti-Amphiphysin) detected in a patient with limbic encephalitis and small cell lung cancer. ( 28879908 )
2017
20
Characteristics in limbic encephalitis with anti-adenylate kinase 5 autoantibodies. ( 28062719 )
2017
21
Paraneoplastic limbic encephalitis in a patient with extensive disease small-cell lung cancer. ( 28413671 )
2017
22
Tuberculous limbic encephalitis: A case report. ( 28606665 )
2017
23
Limbic Encephalitis Associated with Human Herpesvirus-7 (HHV-7) in an Immunocompetent Adult: The First Reported Case in Japan. ( 28717094 )
2017
24
Evidence of a pathogenic role for CD8(+) T cells in anti-GABAB receptor limbic encephalitis. ( 27213174 )
2016
25
A unique combination of autoimmune limbic encephalitis, type 1 diabetes, and Stiff person syndrome associated with GAD-65 antibody. ( 27011652 )
2016
26
Autoimmune neurological syndromes associated limbic encephalitis and paraneoplastic cerebellar degeneration. ( 27592059 )
2016
27
Non-paraneoplastic limbic encephalitis and central nervous HHV-6B reactivation: Causality or coincidence? ( 27431532 )
2016
28
Anti-leucine-rich glioma-inactivated 1 limbic encephalitis: A case report and literature review. ( 26889260 )
2016
29
Anti NMDA receptor encephalitis( limbic encephalitis) with corticospinal involvement in a middle aged Indian female. ( 27728547 )
2016
30
Alternating faciobrachial dystonic seizures in LGI1-antibody limbic encephalitis. ( 26984950 )
2016
31
Clinical characterization of autoimmune LGI1 antibody limbic encephalitis. ( 26896820 )
2016
32
The clinical diagnosis and treatment about 22 cases of limbic encephalitis were retrospectively analyzed. ( 27113304 )
2016
33
Clinical analysis of leucine-rich glioma inactivated-1 protein antibody associated with limbic encephalitis onset with seizures. ( 27428233 )
2016
34
A case of neurosyphilis presenting with limbic encephalitis. ( 27980287 )
2016
35
Limbic encephalitis following immunotherapy against metastatic malignant melanoma. ( 27009198 )
2016
36
Hyperfamiliarity for unknown faces after non-paraneoplastic limbic encephalitis: a case report. ( 27334285 )
2016
37
Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort. ( 27037228 )
2016
38
Small-Cell Lung Cancer with Positive Anti-NMDAR and Anti-AMPAR Antibodies Paraneoplastic Limbic Encephalitis. ( 28070431 )
2016
39
Limbic encephalitis associated with anti-leucine-rich glioma-inactivated-1 protein antibodies in a child. ( 27841212 )
2016
40
Limbic encephalitis: The great unknown. ( 27448690 )
2016
41
Paraneoplastic Limbic Encephalitis in a Male with Nasopharyngeal Carcinoma. ( 27174340 )
2016
42
Cholangiocarcinoma associated with limbic encephalitis and early cerebral abnormalities detected by 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography-positron emission tomography: a case report. ( 27439460 )
2016
43
Limbic Encephalitis Associated with SjAPgren's Syndrome: Report of Three Cases. ( 27523010 )
2016
44
Correlations of Clusters of Non-Convulsive Seizure and Magnetic Resonance Imaging in a Case With GAD65-Positive Autoimmune Limbic Encephalitis. ( 27429684 )
2016
45
Neuropsychological and FDG-PET profiles in VGKC autoimmune limbic encephalitis. ( 27566001 )
2016
46
Voltage-gated potassium channel antibody limbic encephalitis: a case illustrating the neuropsychiatric and PET/CT features with clinical and imaging follow-up. ( 27590077 )
2016
47
Limbic encephalitis associated with glutamic acid decarboxylase antibodies in a young adolescent. ( 27117742 )
2016
48
Limbic Encephalitis Associated With GAD65 Antibodies: Brief Review of the Relevant literature. ( 27030381 )
2016
49
Autoimmune Limbic Encephalitis and Syndrome of Inappropriate Antidiuretic Hormone Secretion Associated with Lamotrigine-induced Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome. ( 27181555 )
2016
50
Seizure control and cognitive improvement via immunotherapy in late onset epilepsy patients with paraneoplastic versus GAD65 autoantibody-associated limbic encephalitis. ( 27855355 )
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:

id 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:

id 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:

id 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 extracellular-glutamate-gated ion channel activity GO:0005234 9.26 GRIA1 GRIN2B
4 ionotropic glutamate receptor activity GO:0004970 9.16 GRIA1 GRIN2B
5 glutamate binding GO:0016595 8.96 GAD1 GAD2
6 glutamate decarboxylase activity GO:0004351 8.62 GAD1 GAD2

Sources for Limbic Encephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 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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