MCID: LMB024
MIFTS: 38

Limbic Encephalitis malady

Categories: Rare diseases

Aliases & Classifications for Limbic Encephalitis

Aliases & Descriptions 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 non-herpetic acute limbic encephalitis and posttransplant 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 Immunoglobulins and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include brain, testes and lung, and related phenotypes are behavior/neurological and nervous system

Wikipedia : 71 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 97)
id Related Disease Score Top Affiliating Genes
1 non-herpetic acute limbic encephalitis 12.1
2 posttransplant acute limbic encephalitis 12.1
3 limbic encephalitis with dpp6 antibodies 12.0
4 classic paraneoplastic limbic encephalitis 12.0
5 limbic encephalitis with lgi1 antibodies 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 epilepsy 10.2
12 neuronitis 10.2
13 lung cancer 10.2
14 teratoma 10.2
15 thymoma 10.2
16 glioma 10.1
17 status epilepticus 10.1
18 relapsing polychondritis 10.1
19 lymphoma 10.1
20 cone dystrophy 4 10.1 ADAM22 LGI1
21 hemangioblastoma 10.1 GRIN2B GSR
22 triphalangeal thumbs and dislocation of patella 10.1 GAD1 GAD2
23 temporal lobe epilepsy 10.1
24 atrial tachyarrhythmia with short pr interval 10.0 AMPH GAD1 GAD2
25 hodgkin lymphoma 10.0
26 dementia 10.0
27 deafness, autosomal recessive 29 10.0 GAD1 GAD2
28 cerebellar degeneration 10.0
29 adenocarcinoma 10.0
30 testicular cancer 10.0
31 idiopathic generalized epilepsy 9.9 GRIA1 GRIN2B LGI1
32 paraneoplastic syndromes 9.9
33 breast cancer 9.9
34 neurosyphilis 9.9
35 paraneoplastic cerebellar degeneration 9.9
36 encephalopathy 9.9
37 prostatitis 9.9
38 squamous cell carcinoma 9.8
39 anterograde amnesia 9.8
40 prostate cancer 9.8
41 leukemia 9.8
42 ovarian cancer 9.8
43 myasthenia gravis 9.8
44 psychotic disorder 9.8
45 renal cell carcinoma 9.8
46 viral encephalitis 9.8
47 cerebellar ataxia 9.8
48 syndrome of inappropriate antidiuretic hormone 9.8
49 herpes simplex 9.8
50 ataxia 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 ADAM22 AMPH BRSK2 ELAVL3 GAD1 GAD2

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 Immunoglobulins Phase 2
2 Antibodies Phase 2
3 gamma-Globulins Phase 2
4 Immunoglobulins, Intravenous Phase 2
5 Rho(D) Immune Globulin 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:


id Name Status NCT ID Phase
1 IVIG Treatment for Refractory Immune-Related Adult Epilepsy Terminated NCT01545518 Phase 2
2 Immunotherapy of the Paraneoplastic Syndromes Completed NCT00378326
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, Testes, Lung, Temporal Lobe, Bone Marrow, Breast, Prostate

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 460)
id Title Authors Year
1
Emergence of new-onset psychotic disorder following recovery from LGI1 antibody-associated limbic encephalitis. ( 28363946 )
2017
2
Characteristics in limbic encephalitis with anti-adenylate kinase 5 autoantibodies. ( 28062719 )
2017
3
Do we need to measure specific antibodies in patients with limbic encephalitis? ( 28062718 )
2017
4
The major histocompatibility complex and antibody-mediated limbic encephalitis. ( 28026032 )
2016
5
Editorial: Limbic Encephalitis: Autoimmune Impact on Inhibitory GABAergic Neuronal Pathways in Temporal Lobe Epilepsy. ( 27014186 )
2016
6
Alternating faciobrachial dystonic seizures in LGI1-antibody limbic encephalitis. ( 26984950 )
2016
7
Anti-leucine-rich glioma-inactivated 1 limbic encephalitis: A case report and literature review. ( 26889260 )
2016
8
Limbic Encephalitis Associated With GAD65 Antibodies: Brief Review of the Relevant literature. ( 27030381 )
2016
9
Teaching NeuroImages: Limbic encephalitis associated with relapsing polychondritis. ( 27185902 )
2016
10
Suspected antibody negative autoimmune limbic encephalitis: outcome of immunotherapy. ( 26940288 )
2016
11
Limbic encephalitis associated with glutamic acid decarboxylase antibodies in a young adolescent. ( 27117742 )
2016
12
Limbic encephalitis: The great unknown. ( 27448690 )
2016
13
Limbic encephalitis associated with anti-leucine-rich glioma-inactivated-1 protein antibodies in a child. ( 27841212 )
2016
14
Seizure control and cognitive improvement via immunotherapy in late onset epilepsy patients with paraneoplastic versus GAD65 autoantibody-associated limbic encephalitis. ( 27855355 )
2016
15
Limbic encephalitis following immunotherapy against metastatic malignant melanoma. ( 27009198 )
2016
16
Paraneoplastic Limbic Encephalitis in a Male with Nasopharyngeal Carcinoma. ( 27174340 )
2016
17
A case of neurosyphilis presenting with limbic encephalitis. ( 27980287 )
2016
18
Paraneoplastic limbic encephalitis in B cell lymphoma unclassifiable with features intermediate between diffuse large B cell lymphoma and classical Hodgkin lymphoma. ( 26984106 )
2016
19
Evidence of a pathogenic role for CD8(+) T cells in anti-GABAB receptor limbic encephalitis. ( 27213174 )
2016
20
Correlations of Clusters of Non-Convulsive Seizure and Magnetic Resonance Imaging in a Case With GAD65-Positive Autoimmune Limbic Encephalitis. ( 27429684 )
2016
21
Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort. ( 27037228 )
2016
22
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
23
Autoimmune limbic encephalitis due to VGKC-Ab. Thanks for the memory. Leo Robin (Title of song, 1937). ( 26819188 )
2016
24
Non-paraneoplastic limbic encephalitis and central nervous HHV-6B reactivation: Causality or coincidence? ( 27431532 )
2016
25
Neuropsychological and FDG-PET profiles in VGKC autoimmune limbic encephalitis. ( 27566001 )
2016
26
Clinical characterization of autoimmune LGI1 antibody limbic encephalitis. ( 26896820 )
2016
27
A unique combination of autoimmune limbic encephalitis, type 1 diabetes, and Stiff person syndrome associated with GAD-65 antibody. ( 27011652 )
2016
28
Clinical analysis of leucine-rich glioma inactivated-1 protein antibody associated with limbic encephalitis onset with seizures. ( 27428233 )
2016
29
Type 1 diabetes and GAD65 limbic encephalitis: a case report of a 10-year-old girl. ( 27115322 )
2016
30
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
31
Limbic Encephalitis Associated with SjAPgren's Syndrome: Report of Three Cases. ( 27523010 )
2016
32
The clinical diagnosis and treatment about 22 cases of limbic encephalitis were retrospectively analyzed. ( 27113304 )
2016
33
Voltage-gated potassium channel antibody limbic encephalitis: a case illustrating the neuropsychiatric and PET/CT features with clinical and imaging follow-up. ( 27590077 )
2016
34
Anti NMDA receptor encephalitis( limbic encephalitis) with corticospinal involvement in a middle aged Indian female. ( 27728547 )
2016
35
Hyperfamiliarity for unknown faces after non-paraneoplastic limbic encephalitis: a case report. ( 27334285 )
2016
36
Cognitive Impairments Preceding and Outlasting Autoimmune Limbic Encephalitis. ( 26881156 )
2016
37
Autoimmune neurological syndromes associated limbic encephalitis and paraneoplastic cerebellar degeneration. ( 27592059 )
2016
38
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
39
Small-Cell Lung Cancer with Positive Anti-NMDAR and Anti-AMPAR Antibodies Paraneoplastic Limbic Encephalitis. ( 28070431 )
2016
40
Reversible dementia: two nursing home patients with voltage-gated potassium channel antibody-associated limbic encephalitis. ( 26170033 )
2015
41
Clinico-pathological correlation in adenylate kinase 5 autoimmune limbic encephalitis. ( 26439959 )
2015
42
Limbic Encephalitis: Potential Impact of Adaptive Autoimmune Inflammation on Neuronal Circuits of the Amygdala. ( 26284026 )
2015
43
Squamous Cell Lung Carcinoma Presenting With Refractory Status Epilepticus: A Case Report of Paraneoplastic Limbic Encephalitis. ( 25784771 )
2015
44
Differences in epileptic symptoms depending on the type of autoimmune-mediated limbic encephalitis. ( 26163176 )
2015
45
A Rare Case of Ovarian Cancer Presenting with Paraneoplastic Cerebellar Degeneration and Limbic Encephalitis. ( 26415811 )
2015
46
Limbic encephalitis associated with leucine-rich glioma-inactivated 1 antibodies. ( 26142944 )
2015
47
The Effects of Carbamazepine and Lithium Combination on Serious Aggression and Anger Outbursts in a Case of Seronegative Limbic Encephalitis. ( 26835170 )
2015
48
Treating a GAD65 Antibody-Associated Limbic Encephalitis with Basiliximab: A Case Study. ( 26284025 )
2015
49
Multicentric Castleman's disease with voltage-gated potassium channel antibody-positive limbic encephalitis: a case report. ( 25648431 )
2015
50
Autoimmune limbic encephalitis detected on FDG brain scan performed for the evaluation of dementia. ( 25608163 )
2015

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 ionotropic glutamate receptor activity GO:0004970 9.26 GRIA1 GRIN2B
4 extracellular-glutamate-gated ion channel activity GO:0005234 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 Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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