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 12 20 58 54 15 70

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:0080741
MESH via Orphanet 45 D020363
UMLS via Orphanet 71 C0338430
Orphanet 58 ORPHA163892
UMLS 70 C0338430

Summaries for Limbic Encephalitis

GARD : 20 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 medial temporal lobes, temporal lobe and lung, and related phenotypes are behavior/neurological and nervous system

Disease Ontology : 12 An encephalitis that is characterized by subacute onset of short-term memory deficits, seizures or psychiatric symptoms located in the medial temporal lobes.

Wikipedia : 73 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 373)
# Related Disease Score Top Affiliating Genes
1 paraneoplastic limbic encephalitis 32.3 GSR ELAVL3 BRSK2
2 encephalitis 31.9 PNMA2 LGI1 CNTNAP2
3 autoimmune encephalitis 31.0 LGI1 CNTNAP2
4 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 31.0 TPO GAD2 GAD1
5 temporal lobe epilepsy 31.0 LGI1 GRIN2B GAD2 GAD1
6 status epilepticus 30.9 LGI1 GRIN2B GRIA1
7 retrograde amnesia 30.7 LGI1 GRIN2B GRIA1
8 amnestic disorder 30.5 LGI1 GRIN2B GRIA1 GAD1 CNTNAP2
9 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 30.1 GAD2 GAD1
10 hashimoto thyroiditis 30.1 TPO GAD2 ENO1
11 acute disseminated encephalomyelitis 30.0 PNMA2 LGI1 CNTNAP2
12 whipple disease 29.9 PNMA2 DPYSL5
13 dissociative amnesia 29.8 LGI1 CNTNAP2
14 focal epilepsy 29.8 LGI1 GRIN2B CNTNAP2
15 autosomal dominant epilepsy with auditory features 29.8 LGI1 CNTNAP2
16 anterograde amnesia 29.5 PNMA2 LGI1 GRIN2B DPYSL5 CNTNAP2 AMPH
17 speech and communication disorders 29.3 LGI1 GRIN2B CNTNAP2
18 movement disease 29.2 LGI1 GAD2 GAD1 DPYSL5 CNTNAP2 AMPH
19 stiff-person syndrome 29.2 PNMA2 LGI1 GAD2 GAD1 DPYSL5 CNTNAP2
20 epilepsy 29.2 LGI1 GSR GRIN2B GRIA1 GAD2 GAD1
21 choreatic disease 29.0 PNMA2 LGI1 GRIN2B DPYSL5 CNTNAP2 AMPH
22 limbic encephalitis with lgi1 antibodies 11.7
23 limbic encephalitis with dpp6 antibodies 11.4
24 classic paraneoplastic limbic encephalitis 11.3
25 limbic encephalitis with nmda receptor antibodies 11.3
26 non-herpetic acute limbic encephalitis 11.3
27 non-paraneoplastic limbic encephalitis 11.3
28 posttransplant acute limbic encephalitis 11.2
29 neuropathy, hereditary sensory and autonomic, type iia 11.1
30 limbic encephalitis associated with antibodies to cell membrane antigens 11.1
31 limbic encephalitis with caspr2 antibodies 11.1
32 limbic encephalitis with neurexin-3 antibodies 11.1
33 teratoma 10.7
34 thymoma, familial 10.6
35 thymoma 10.6
36 seizure disorder 10.6
37 glioma 10.6
38 glial tumor 10.6
39 teratoma, ovarian 10.6
40 ovarian germ cell teratoma 10.6
41 ataxia and polyneuropathy, adult-onset 10.5
42 rapidly involuting congenital hemangioma 10.5
43 encephalopathy 10.5
44 small cell cancer of the lung 10.5
45 herpes simplex 10.5
46 lung cancer 10.5
47 paraneoplastic syndromes 10.4
48 autoimmune disease 10.4
49 lymphoma, hodgkin, classic 10.4
50 relapsing polychondritis 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.8 ADAM22 AMPH BRSK2 CNTN2 CNTNAP2 DPYSL5
2 nervous system MP:0003631 9.44 ADAM22 AMPH BRSK2 CNTN2 CNTNAP2 DPYSL5

Drugs & Therapeutics for Limbic Encephalitis

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulins Phase 2
2 Antibodies Phase 2
3 Immunologic Factors Phase 2
4 Immunoglobulins, Intravenous Phase 2
5 Autoantibodies Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 IVIG Treatment for Refractory Immune-Related Adult Epilepsy Terminated NCT01545518 Phase 2 IVIG
2 Antibodies Causing Epilepsy Syndromes: The ACES Study. Unknown status NCT02802475

Search NIH Clinical Center for Limbic Encephalitis

Genetic Tests for Limbic Encephalitis

Anatomical Context for Limbic Encephalitis

The Foundational Model of Anatomy Ontology organs/tissues related to Limbic Encephalitis:

19
Medial Temporal Lobes

MalaCards organs/tissues related to Limbic Encephalitis:

40
Temporal Lobe, Lung, Bone Marrow, Thymus, Spinal Cord, Myeloid, Amygdala

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 1421)
# 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. 54 61
20437582 2010
4
Plasma exchange for anti GAD associated non paraneoplastic limbic encephalitis. 61 54
18955013 2008
5
Antibodies to voltage-gated potassium and calcium channels in epilepsy. 54 61
16870397 2006
6
CD8+ T-Lymphocyte-Driven Limbic Encephalitis Results in Temporal Lobe Epilepsy. 61
33368582 2021
7
Anti-contactin-associated protein-like 2 antibody-associated cerebellar ataxia: A case report and literature review. 61
33640718 2021
8
Human herpesvirus 6 and central nervous system disease in oncology patients: A retrospective case series and literature review. 61
33548682 2021
9
Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management. 61
33649021 2021
10
Continuous EEG Findings in Autoimmune Encephalitis. 61
31800465 2021
11
Acute psychotic onset in LGI1-related limbic encephalitis. 61
33675002 2021
12
Seronegative limbic encephalitis manifesting as subacute amnestic syndrome: a case report and review of the literature. 61
33757596 2021
13
ASL MRI and 18F-FDG-PET in autoimmune limbic encephalitis: clues from two paradigmatic cases. 61
33763811 2021
14
Water drinking behavior: a clinical clue for the diagnosis of limbic encephalitis in an elderly patient. 61
33683632 2021
15
Autoimmune limbic encephalitis related to SARS-CoV-2 infection: Case-report and review of the literature. 61
33521691 2021
16
18F-FDG PET/CT in Anti-Leucine-Rich Glioma-Inactivated 1 Antibody Encephalitis: Typical Pattern and Follow-up. 61
33323741 2021
17
Takotsubo syndrome associated with autoimmune limbic encephalitis: a case report. 61
33573602 2021
18
Specific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis. 61
32816110 2021
19
Clinical spectrum of high-titre GAD65 antibodies. 61
33563803 2021
20
Paraneoplastic limbic encephalitis as initial presentation of testicular neoplasm: A case report. 61
33556762 2021
21
Leucine Zipper 4 Autoantibody: A Novel Germ Cell Tumor and Paraneoplastic Biomarker. 61
33583072 2021
22
Familial autoimmunity in neurological patients with GAD65 antibodies: an interview-based study. 61
33544221 2021
23
Seizures in steroid-responsive encephalopathy. 61
33219869 2021
24
Paraneoplastic Encephalitis Associated with Locally Advanced Human Epidermal Growth Factor Receptor 2-Positive Breast Cancer. 61
33634625 2021
25
Limbic encephalitis in a patient with systemic lupus erythematosus successfully treated with high-dose glucocorticoids and intravenous cyclophosphamide therapy: the potential pathogenicity of anti-glutamate receptor antibodies. 61
33441041 2021
26
[GABAB receptor autoimmune encephalitis presenting as transient epileptic amnesia]. 61
33328416 2021
27
[Pediatric autoimmune encephalitis associated with anti-glutamic acid decarboxylase 65 antibody: two cases report and literature review]. 61
33397004 2021
28
All that glitters is not gold: A limbic encephalitis due to neurosyphilis. 61
32631679 2021
29
Neurological manifestations associated with SARS-CoV-2 and other coronaviruses: A narrative review for clinicians. 61
33446327 2021
30
Neuronal Surface Antibody-Medicated Autoimmune Encephalitis (Limbic Encephalitis) in China: A Multiple-Center, Retrospective Study. 61
33679765 2021
31
Metabolic Imaging Patterns on 18F-FDG PET in Acute and Subacute LGI1 Autoimmune Limbic Encephalitis. 61
32910055 2021
32
New-Onset Seizure With Possible Limbic Encephalitis in a Patient With COVID-19 Infection: A Case Report and Review. 61
33648382 2021
33
Voltage-gated potassium channel blanket testing in first-episode psychosis: Diagnostic nihilism? 61
33423505 2021
34
Clinical Presentation and Outcomes of Severe Acute Respiratory Syndrome Coronavirus 2-Related Encephalitis: The ENCOVID Multicenter Study. 61
32986824 2021
35
LGI1 downregulation increases neuronal circuit excitability. 61
33104247 2020
36
Imaging Review of Paraneoplastic Neurologic Syndromes. 61
33093137 2020
37
Correction to: ESNR 2020. 61
33104850 2020
38
Psychiatric symptoms in anti glutamic acid decarboxylase associated limbic encephalitis in adults: a systematic review. 61
33022299 2020
39
Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect. 61
33396564 2020
40
Voltage-gated potassium channel limbic encephalitis presenting as functional cognitive impairment. 61
33318259 2020
41
Limbic Encephalitis Brain Damage Induced by Cocal Virus in Adult Mice Is Reduced by Environmental Enrichment: Neuropathological and Behavioral Studies. 61
33396704 2020
42
Double filtration plasmapheresis - 10-year pediatric experience as an alternative to plasma exchange. 61
32919882 2020
43
[Paraneoplastic anti-gamma aminobutyric acid (GABA)B receptor antibody limbic encephalitis associated with small cell lung cancer presenting as new-onset status epilepticus: a case report]. 61
33229837 2020
44
Stiff-Person Syndrome Associated with Anti-Glutamic Acid Decarboxylase Autoimmune Encephalitis in a Young Woman: A Case Report. 61
33413757 2020
45
Expanding the Catatonia Tent: Recognizing Electroconvulsive Therapy Responsive Syndromes. 61
33122500 2020
46
An uncommon presentation of paraneoplastic limbic encephalitis. 61
33197844 2020
47
A case of limbic encephalitis associated with asymptomatic COVID-19 infection. 61
32661082 2020
48
Use of diffusion-weighted imaging to distinguish seizure-related change from limbic encephalitis. 61
32583056 2020
49
Autoimmune encephalitis and gastrointestinal dysmotility: achalasia, gastroparesis, and slow transit constipation. 61
33036051 2020
50
A Case of Anti-LGI1 Encephalitis Developing Immunoglobulin Responsive Orthostatic Hypotension after Remission. 61
33055478 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 axon GO:0030424 9.72 GAD2 GAD1 CNTNAP2 CNTN2 ADAM22
2 neuronal cell body GO:0043025 9.65 PPP1R9B GRIA1 DPYSL5 CNTNAP2 CNTN2
3 cell surface GO:0009986 9.63 TPO GRIN2B GRIA1 ENO1 CNTNAP2 CNTN2
4 synaptic vesicle membrane GO:0030672 9.61 GRIA1 GAD2 AMPH
5 cell junction GO:0030054 9.5 PPP1R9B LGI1 GRIN2B GRIA1 GAD2 CNTNAP2
6 dendritic spine membrane GO:0032591 9.48 PPP1R9B GRIA1
7 inhibitory synapse GO:0060077 9.46 GAD2 GAD1
8 juxtaparanode region of axon GO:0044224 9.4 CNTNAP2 CNTN2
9 clathrin-sculpted gamma-aminobutyric acid transport vesicle membrane GO:0061202 9.37 GAD2 GAD1
10 synapse GO:0045202 9.23 PPP1R9B LGI1 GRIN2B GRIA1 GAD2 GAD1

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 nervous system development GO:0007399 9.63 PPP1R9B LGI1 ELAVL3 DPYSL5 CNTN2 BRSK2
2 neuron projection development GO:0031175 9.62 PPP1R9B LGI1 CNTNAP2 CNTN2
3 learning GO:0007612 9.61 PPP1R9B CNTNAP2 CNTN2
4 nucleobase-containing small molecule interconversion GO:0015949 9.51 GSR AK5
5 ionotropic glutamate receptor signaling pathway GO:0035235 9.49 GRIN2B GRIA1
6 carboxylic acid metabolic process GO:0019752 9.48 GAD2 GAD1
7 neurotransmitter biosynthetic process GO:0042136 9.46 GAD2 GAD1
8 neurotransmitter receptor localization to postsynaptic specialization membrane GO:0099645 9.4 LGI1 ADAM22
9 protein localization to juxtaparanode region of axon GO:0071205 9.37 CNTNAP2 CNTN2
10 clustering of voltage-gated potassium channels GO:0045163 9.26 CNTNAP2 CNTN2
11 chemical synaptic transmission GO:0007268 9.02 GRIN2B GRIA1 GAD2 GAD1 AMPH
12 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 carboxy-lyase activity GO:0016831 9.26 GAD2 GAD1
2 ionotropic glutamate receptor activity GO:0004970 9.16 GRIN2B GRIA1
3 glutamate decarboxylase activity GO:0004351 8.96 GAD2 GAD1
4 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
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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