MCID: LMB024
MIFTS: 41

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 52 58 54 71

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

MESH via Orphanet 44 D020363
UMLS via Orphanet 72 C0338430
Orphanet 58 ORPHA163892
UMLS 71 C0338430

Summaries for Limbic Encephalitis

NIH Rare Diseases : 52 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 paraneoplastic limbic encephalitis and encephalitis. An important gene associated with Limbic Encephalitis is AMPH (Amphiphysin), and among its related pathways/superpathways are Glucose / Energy Metabolism and Neuroscience. 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 : 74 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 343)
# Related Disease Score Top Affiliating Genes
1 paraneoplastic limbic encephalitis 33.9 GSR ELAVL3 BRSK2
2 encephalitis 32.4 PNMA2 LGI1 AMPH
3 amnestic disorder 30.8 LGI1 GRIN2B GRIA1 GAD1
4 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 30.7 TPO GAD2 GAD1
5 status epilepticus 30.7 LGI1 GRIN2B GRIA1
6 temporal lobe epilepsy 30.7 LGI1 GRIN2B GAD2 GAD1
7 retrograde amnesia 30.6 GRIN2B GRIA1
8 anterograde amnesia 30.4 PNMA2 LGI1 GRIN2B AMPH AK5
9 hashimoto encephalopathy 30.4 TPO ENO1
10 stiff-person syndrome 30.4 LGI1 GAD2 GAD1 AMPH
11 focal epilepsy 29.2 LGI1 GRIN2B GRIA1 GAD2 GAD1 ADAM22
12 epilepsy 29.1 LGI1 GSR GRIN2B GRIA1 GAD1 CNTN2
13 limbic encephalitis with lgi1 antibodies 12.9
14 non-herpetic acute limbic encephalitis 12.5
15 non-paraneoplastic limbic encephalitis 12.5
16 posttransplant acute limbic encephalitis 12.5
17 limbic encephalitis with dpp6 antibodies 12.5
18 classic paraneoplastic limbic encephalitis 12.4
19 limbic encephalitis with nmda receptor antibodies 12.4
20 limbic encephalitis associated with antibodies to cell membrane antigens 12.4
21 limbic encephalitis with caspr2 antibodies 12.4
22 limbic encephalitis with neurexin-3 antibodies 12.4
23 neuropathy, hereditary sensory and autonomic, type iia 11.4
24 teratoma 10.7
25 visual epilepsy 10.7
26 seizure disorder 10.7
27 thymoma, familial 10.7
28 thymoma 10.7
29 autoimmune encephalitis 10.7
30 glioma 10.7
31 glial tumor 10.7
32 teratoma, ovarian 10.6
33 ovarian germ cell teratoma 10.6
34 encephalopathy 10.6
35 ataxia and polyneuropathy, adult-onset 10.5
36 herpes simplex 10.5
37 rapidly involuting congenital hemangioma 10.5
38 small cell cancer of the lung 10.5
39 autoimmune disease 10.5
40 paraneoplastic syndromes 10.5
41 lymphoma, hodgkin, classic 10.4
42 lung cancer 10.4
43 relapsing polychondritis 10.4
44 cerebellar degeneration 10.4
45 herpes simplex encephalitis 10.4
46 myoclonus 10.4
47 cerebellopontine angle meningioma 10.4 LGI1 AMPH
48 ocular motor apraxia 10.4
49 small cell carcinoma 10.4
50 lymphoma 10.4

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.73 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 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulins Phase 2
2 Antibodies Phase 2
3 Immunoglobulins, Intravenous Phase 2
4 Immunologic Factors Phase 2
5 Autoantibodies Phase 2
6
Glutamic acid Approved, Nutraceutical 56-86-0 33032
7 Anticonvulsants
8
Leucine Investigational, Nutraceutical 61-90-5 6106

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
3 Antibodies Causing Epilepsy Syndromes: The ACES Study. Recruiting NCT02802475
4 Epidemiology and Prognosis of Encephalitis in Intensive Care Recruiting NCT02906631
5 Core Cerebrospinal Fluid Biomarker Profile in Leucine Rich Glioma Inactivated 1 (LGI1) Antibody Associated Encephalitis Not yet recruiting NCT04001270
6 Autoimmunity Family Background in Neurological Syndromes With Antibodies Against Glutamic-acid Decarboxylase Not yet recruiting NCT04104620

Search NIH Clinical Center for Limbic Encephalitis

Genetic Tests for Limbic Encephalitis

Anatomical Context for Limbic Encephalitis

MalaCards organs/tissues related to Limbic Encephalitis:

40
Brain, Lung, Testes, Temporal Lobe, T Cells, Breast, B Cells

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 1347)
# Title Authors PMID Year
1
Antibodies and neuronal autoimmune disorders of the CNS. 54 61
20035430 2010
2
[Limbic encephalitis with antibodies against intracellular antigens]. 54 61
20420174 2010
3
Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. 61 54
20437582 2010
4
Plasma exchange for anti GAD associated non paraneoplastic limbic encephalitis. 54 61
18955013 2008
5
Antibodies to voltage-gated potassium and calcium channels in epilepsy. 61 54
16870397 2006
6
Long lasting trigeminal neuropathy, limbic encephalitis and abdominal ganglionitis without primary cancer: An atypical case of Hu-antibody syndrome. 61
32388246 2020
7
A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases. 61
31982129 2020
8
Central nervous system complications associated with immune checkpoint inhibitors. 61
32312871 2020
9
Clinical features and outcomes of Chinese patients with anti-γ-aminobutyric acid B receptor encephalitis. 61
32509023 2020
10
Cryptococcus Meningitis Can Co-occur with Anti-NMDA Receptor Encephalitis. 61
32522922 2020
11
Expanding clinical spectrum of Caspr2 antibody-associated disease: warning on brainstem involvement and respiratory failure. 61
32371280 2020
12
Psychiatric Manifestation of Anti-LGI1 Encephalitis. 61
32560097 2020
13
Possible link of genetic variants to autoimmunity in GAD-antibody-associated neurological disorders. 61
32388243 2020
14
Nivolumab-induced autoantibody negative limbic encephalitis in a patient with Hodgkin lymphoma. 61
32048537 2020
15
Limbic Encephalitis with Anti-myelin Oligodendrocyte Glycoprotein Antibody. 61
32132338 2020
16
Acute Limbic Encephalitis in a 16-year-old Boy. 61
31895880 2020
17
Diagnosis and treatment of limbic encephalitis in the cancer patient. 61
32511017 2020
18
[Ictal EEG pattern of transient epileptic amnesia in acute phase of non-herpetic limbic encephalitis]. 61
32435050 2020
19
[A Case of Anterior Cranial Fossa Dural Arteriovenous Fistula Involving Uncontrolled Seizures Diagnosed Based on Characteristic Findings on Arterial Spin-labeling Imaging]. 61
32572008 2020
20
Anti-leucine-rich glioma-inactivated 1-positive limbic encephalitis presenting hypothalamic symptoms. 61
31865478 2020
21
Limbic encephalitis in a neuroscientist: CASPR 2 antibody-associated disease after antigen exposure. 61
32302793 2020
22
Distinctive binding properties of human monoclonal LGI1 autoantibodies determine pathogenic mechanisms. 61
32437528 2020
23
CD19+ B-cells in autoantibody-negative limbic encephalitis. 61
32199348 2020
24
Anti-Hu limbic encephalitis preceding the appearance of mediastinal germinoma by 9 years. 61
32086300 2020
25
Neurologic syndromes related to anti-GAD65: Clinical and serologic response to treatment. 61
32123047 2020
26
Neurological Syndromes Associated with Anti-GAD Antibodies. 61
32456344 2020
27
Clinical characteristics of GAD 65-associated autoimmune encephalitis. 61
32416610 2020
28
Clinical significance of Kelch-like protein 11 antibodies. 61
31953318 2020
29
The neuropsychological spectrum of anti-LGI1 antibody mediated autoimmune encephalitis. 61
32480239 2020
30
A case of limbic encephalitis evolving into a frontotemporal dementia-like picture. 61
31881112 2020
31
Mild Encephalitis/Encephalopathy with reversible splenial lesion syndrome: An unusual presentation of anti-GFAP astrocytopathy. 61
32340854 2020
32
Use of PET/CT with 18F-FDG in the diagnosis of paraneoplastic limbic encephalitis. 61
31836566 2020
33
Systematic review of the clinical spectrum of CASPR2 antibody syndrome. 61
31912210 2020
34
Primary HIV infection presenting with Kaposi sarcoma and limbic encephalitis. 61
31768888 2020
35
AMPA-R Limbic Encephalitis Associated with Systemic Lupus Erythematosus. 61
32279661 2020
36
Immune checkpoint inhibitor induced anti-glutamic acid decarboxylase 65 (Anti-GAD 65) limbic encephalitis responsive to intravenous immunoglobulin and plasma exchange. 61
31832829 2020
37
Immune-mediated epilepsy with GAD65 antibodies. 61
32087461 2020
38
Does hippocampal atrophy explain anterograde and retrograde amnesia following autoimmune limbic encephalitis? 61
32320116 2020
39
[Episodes with goose bumps caused by anti-LGI-1 encephalitis]. 61
32293017 2020
40
Subgroup comparison according to clinical phenotype and serostatus in autoimmune encephalitis: a multicenter retrospective study. 61
31814224 2020
41
Glioblastoma as an autoimmune limbic encephalitis mimic: A case and review of the literature. 61
32182452 2020
42
Differential diagnosis of temporal lobe lesions with hyperintense signal on T2-weighted and FLAIR sequences: pictorial essay. 61
32336830 2020
43
Limbic encephalitis with antibodies to N-methyl-D-aspartate (NMDA)-type glutamate receptor after allogeneic transplantation. 61
32200528 2020
44
Limbic encephalitis associated with AMPA receptor and CRMP5 antibodies: A case report and literature review. 61
31991060 2020
45
Pathologic tearfulness after limbic encephalitis: A novel disorder and its neural basis. 61
31980582 2020
46
Caspr2 antibody-associated limbic encephalitis: contribution of visual aided analysis of 18F-FDG PET images using normal database comparison. 61
31784408 2020
47
Antibody-Negative Paraneoplastic Limbic Encephalitis, Parkinsonism, Hypothermia, and Narcolepsy Associated with Endometrial Carcinoma. 61
32071946 2020
48
Anti-glutamic acid decarboxylase antibody paraneoplastic limbic encephalitis associated with Acute Myeloid Leukemia. 61
31786428 2020
49
Limbic encephalitis associated with tuberculous mediastinal lymphadenitis. 61
31956698 2020
50
Anti-myelin oligodendrocyte glycoprotein antibody-positive acute disseminated encephalomyelitis mimicking limbic encephalitis: A case report. 61
31733424 2020

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 surface GO:0009986 9.77 TPO GRIN2B GRIA1 ENO1 CNTN2
2 axon GO:0030424 9.71 GAD2 GAD1 CNTN2 ADAM22
3 cell junction GO:0030054 9.63 PPP1R9B LGI1 GRIN2B GRIA1 GAD2 AMPH
4 inhibitory synapse GO:0060077 9.32 GAD2 GAD1
5 dendritic spine membrane GO:0032591 9.26 PPP1R9B GRIA1
6 synapse GO:0045202 9.23 PPP1R9B LGI1 GRIN2B GRIA1 GAD2 GAD1
7 clathrin-sculpted gamma-aminobutyric acid transport vesicle membrane GO:0061202 8.96 GAD2 GAD1

Biological processes related to Limbic Encephalitis according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 nervous system development GO:0007399 9.65 PPP1R9B LGI1 ELAVL3 CNTN2 BRSK2
2 neuron projection development GO:0031175 9.61 PPP1R9B LGI1 CNTN2
3 modulation of chemical synaptic transmission GO:0050804 9.5 PPP1R9B GRIN2B GRIA1
4 regulation of NMDA receptor activity GO:2000310 9.49 GRIN2B GRIA1
5 nucleobase-containing small molecule interconversion GO:0015949 9.48 GSR AK5
6 ionotropic glutamate receptor signaling pathway GO:0035235 9.46 GRIN2B GRIA1
7 carboxylic acid metabolic process GO:0019752 9.4 GAD2 GAD1
8 neurotransmitter biosynthetic process GO:0042136 9.37 GAD2 GAD1
9 neurotransmitter receptor localization to postsynaptic specialization membrane GO:0099645 9.16 LGI1 ADAM22
10 chemical synaptic transmission GO:0007268 9.02 GRIN2B GRIA1 GAD2 GAD1 AMPH
11 glutamate decarboxylation to succinate GO:0006540 8.96 GAD2 GAD1

Molecular functions related to Limbic Encephalitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 lyase activity GO:0016829 9.54 GAD2 GAD1 ENO1
2 carboxy-lyase activity GO:0016831 9.32 GAD2 GAD1
3 glutamate receptor activity GO:0008066 9.26 GRIN2B GRIA1
4 ionotropic glutamate receptor activity GO:0004970 9.16 GRIN2B GRIA1
5 glutamate decarboxylase activity GO:0004351 8.96 GAD2 GAD1
6 glutamate binding GO:0016595 8.8 GRIN2B GAD2 GAD1

Sources for Limbic Encephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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