MCID: LMB024
MIFTS: 42

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 53 59 55 72

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

MESH via Orphanet 45 D020363
UMLS via Orphanet 73 C0338430
Orphanet 59 ORPHA163892
UMLS 72 C0338430

Summaries for Limbic Encephalitis

NIH Rare Diseases : 53 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 status epilepticus and stiff-person syndrome. 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 : 75 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 327)
# Related Disease Score Top Affiliating Genes
1 status epilepticus 30.8 LGI1 GRIN2B GRIA1
2 stiff-person syndrome 30.7 GAD2 GAD1 AMPH
3 paraneoplastic cerebellar degeneration 30.6 ELAVL3 AMPH
4 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 30.4 TPO GAD2 GAD1
5 hashimoto encephalopathy 30.0 TPO ENO1
6 autoimmune disease 29.6 TPO GAD2 ENO1
7 epilepsy 29.4 LGI1 GSR GRIN2B GAD1 CNTN2
8 limbic encephalitis with lgi1 antibodies 12.9
9 paraneoplastic limbic encephalitis 12.6
10 non-herpetic acute limbic encephalitis 12.5
11 non-paraneoplastic limbic encephalitis 12.5
12 posttransplant acute limbic encephalitis 12.5
13 limbic encephalitis with dpp6 antibodies 12.5
14 classic paraneoplastic limbic encephalitis 12.4
15 obsolete: limbic encephalitis with ncmags antibodies 12.4
16 limbic encephalitis with nmda receptor antibodies 12.4
17 obsolete: other forms of non-paraneoplastic limbic encephalitis 12.4
18 limbic encephalitis associated with antibodies to cell membrane antigens 12.4
19 limbic encephalitis with caspr2 antibodies 12.4
20 limbic encephalitis with neurexin-3 antibodies 12.4
21 neuropathy, hereditary sensory and autonomic, type iia 11.4
22 encephalitis 11.3
23 amnestic disorder 10.8
24 visual epilepsy 10.7
25 seizure disorder 10.7
26 teratoma 10.7
27 thymoma, familial 10.7
28 thymoma 10.7
29 temporal lobe epilepsy 10.7
30 glioma 10.7
31 glial tumor 10.7
32 autoimmune encephalitis 10.6
33 teratoma, ovarian 10.6
34 ovarian germ cell teratoma 10.6
35 anterograde amnesia 10.5
36 encephalopathy 10.5
37 ataxia and polyneuropathy, adult-onset 10.5
38 herpes simplex 10.5
39 small cell cancer of the lung 10.5
40 rapidly involuting congenital hemangioma 10.5
41 paraneoplastic syndromes 10.4
42 epilepsy, familial temporal lobe, 1 10.4 LGI1 ADAM22
43 lymphoma, hodgkin, classic 10.4
44 aceruloplasminemia 10.4
45 relapsing polychondritis 10.4
46 retrograde amnesia 10.4
47 herpes simplex encephalitis 10.4
48 myoclonus 10.4
49 autoimmune polyendocrine syndrome, type ii 10.4 GAD2 GAD1
50 lung cancer 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 9.7 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 9)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antibodies Phase 2
2 Immunoglobulins Phase 2
3 Immunologic Factors Phase 2
4 gamma-Globulins Phase 2
5 Immunoglobulins, Intravenous Phase 2
6 Rho(D) Immune Globulin Phase 2
7 Autoantibodies Phase 2
8 Anticonvulsants
9 leucine

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

Search NIH Clinical Center for Limbic Encephalitis

Genetic Tests for Limbic Encephalitis

Anatomical Context for Limbic Encephalitis

MalaCards organs/tissues related to Limbic Encephalitis:

41
Brain, Lung, Testes, Temporal Lobe, Breast, T Cells, Bone

Publications for Limbic Encephalitis

Articles related to Limbic Encephalitis:

(show top 50) (show all 1251)
# Title Authors PMID Year
1
Antibodies and neuronal autoimmune disorders of the CNS. 9 38
20035430 2010
2
[Limbic encephalitis with antibodies against intracellular antigens]. 9 38
20420174 2010
3
Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. 9 38
20437582 2010
4
Plasma exchange for anti GAD associated non paraneoplastic limbic encephalitis. 9 38
18955013 2008
5
Antibodies to voltage-gated potassium and calcium channels in epilepsy. 9 38
16870397 2006
6
Identification of adenylate kinase 5 antibodies during routine diagnostics in a tissue-based assay: Three new cases and a review of the literature. 38
31177032 2019
7
"Limbic encephalitis with acute onset and Hu antibodies treated with rituximab: Paraneoplastic or non-paraneoplastic disorder?" 38
31330415 2019
8
A 9-year neuropsychological report of a patient with LGI1-associated limbic encephalitis. 38
31142216 2019
9
Hippocampal network abnormalities explain amnesia after VGKCC-Ab related autoimmune limbic encephalitis. 38
31072956 2019
10
Inflammatory Reaction Secondary to Immune Checkpoint Inhibitor Therapy Mimicking a Post-Operative Brain Abscess. 38
31203078 2019
11
Clinical features of patients with anti-leucine-rich glioma inactivated-1 protein associated encephalitis: a Chinese case series. 38
30621547 2019
12
Insights into the mechanisms of epilepsy from structural biology of LGI1-ADAM22. 38
31432233 2019
13
GABABR-Mediated Paraneoplastic Limbic Encephalitis Due To Thymic Small Cell Carcinoma. 38
31062226 2019
14
Clinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series. 38
31434821 2019
15
Limbic encephalitis as a relapse of Whipple's disease with digestive involvement and spondylodiscitis. 38
29987509 2019
16
Conventional brain MRI features distinguishing limbic encephalitis from mesial temporal glioma. 38
31028423 2019
17
A case of anti-leucine-rich glioma-inactivated 1 antibody-mediated limbic encephalitis. 38
31295779 2019
18
Anti-LGI1 limbic encephalitis presenting as an expanding insular lesion. 38
31342888 2019
19
A case study: Therapeutic plasma exchange in voltage-gated potassium channel autoimmune encephalitis. 38
31378682 2019
20
Olfactory stimulus-induced temporal lobe seizures in limbic encephalitis. 38
31082694 2019
21
Seronegative limbic encephalitis. 38
31321626 2019
22
Pure topographical disorientation in novel environments without anterograde amnesia: a case study. 38
31298073 2019
23
Network-wide abnormalities explain memory variability in hippocampal amnesia. 38
31282861 2019
24
Three cases of antibody-LGI1 limbic encephalitis and review of literature. 38
30112956 2019
25
[A case of paraneoplastic limbic encephalitis associated with neck neuroendocrine carcinoma]. 38
31243254 2019
26
The neurological syndromes associated with glutamic acid decarboxylase antibodies. 38
31000408 2019
27
Impact of anti-CASPR2 autoantibodies from patients with autoimmune encephalitis on CASPR2/TAG-1 interaction and Kv1 expression. 38
31176559 2019
28
Central Nervous System Opportunistic Infections. 38
31378873 2019
29
[AUTOIMMUNE ENCEPHALITIS: A BRIDGE BETWEEN NEUROLOGY AND PSYCHIATRY]. 38
31215187 2019
30
The expanded clinical spectrum of anti-GABABR encephalitis and added value of KCTD16 autoantibodies. 38
31009048 2019
31
Deleted in colorectal cancer (netrin-1 receptor) antibodies and limbic encephalitis in a cat with hippocampal necrosis. 38
30942925 2019
32
Autoimmune Encephalitides. 38
30952414 2019
33
Motor neuron disease as a treatment responsive paraneoplastic neurological syndrome in patient with small cell lung cancer, anti-Hu antibodies and limbic encephalitis. 38
30953906 2019
34
Diagnosing autoimmune limbic encephalitis. 38
31085562 2019
35
A Case Report of Seronegative Limbic Encephalitis. 38
31328072 2019
36
A Case Report of Neurosyphilis Limbic Encephalitis With Reversible Geschwind Syndrome and Mood Disorder. 38
31083037 2019
37
Rare Case of Anti-LGI1 Limbic Encephalitis with New Onset Epilepsy: A Case Report. 38
31309031 2019
38
Neurological Disorders Associated with Glutamic Acid Decarboxylase Antibodies. 38
31355097 2019
39
Paraneoplastic Limbic Encephalitis Complicated with Small Cell Lung Cancer at the Time of Recurrence. 38
31320869 2019
40
Autoimmune Encephalitis as an Extra-articular Manifestation of Rheumatoid Arthritis. 38
30449780 2019
41
Adenylate Kinase: A Ubiquitous Enzyme Correlated with Medical Conditions. 38
30666486 2019
42
Encephalitis on deployment in Kenya: think beyond the infections. 38
30992337 2019
43
Alzheimer's Disease Including Focal Presentations. 38
30925614 2019
44
Anterior cingulate cortex involvement in non-paraneoplastic limbic encephalitis. 38
31005361 2019
45
Severe anti-GAD antibody-associated encephalitis after stem cell transplantation. 38
30381136 2019
46
Limbic encephalitis-like presentation in neuromyelitis optica spectrum disorder: a case report. 38
30835052 2019
47
Atypical Limbic Encephalitis and its Complex Psychiatric Presentations: Implications for Diagnosis and Management. 38
30983671 2019
48
Disrupted AMPA Receptor Function upon Genetic- or Antibody-Mediated Loss of Autism-Associated CASPR2. 38
30843029 2019
49
[Analysis of Small Cell Lung Cancer with Paraneoplastic Limbic Encephalitis]. 38
30909991 2019
50
Anti-Amphiphysin-associated limbic encephalitis in a 72-year-old patient with aortic angiosarcoma. 38
30872337 2019

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 LGI1 GRIN2B GRIA1 GAD2 AMPH
2 axon GO:0030424 9.67 GAD2 GAD1 CNTN2 ADAM22
3 neuron projection GO:0043005 9.61 GRIN2B GRIA1 CNTN2
4 cell surface GO:0009986 9.55 TPO GRIN2B GRIA1 ENO1 CNTN2
5 synaptic membrane GO:0097060 9.48 GRIN2B GRIA1
6 postsynaptic density membrane GO:0098839 9.4 GRIN2B GRIA1
7 inhibitory synapse GO:0060077 9.32 GAD2 GAD1
8 synapse GO:0045202 9.17 LGI1 GRIN2B GRIA1 GAD2 GAD1 CNTN2
9 dendritic spine membrane GO:0032591 8.96 GRIA1
10 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:

# Name GO ID Score Top Affiliating Genes
1 neuron projection development GO:0031175 9.5 PPP1R9B LGI1 CNTN2
2 nucleobase-containing small molecule interconversion GO:0015949 9.4 GSR AK5
3 ionotropic glutamate receptor signaling pathway GO:0035235 9.37 GRIN2B GRIA1
4 carboxylic acid metabolic process GO:0019752 9.26 GAD2 GAD1
5 neurotransmitter biosynthetic process GO:0042136 9.16 GAD2 GAD1
6 chemical synaptic transmission GO:0007268 9.02 GRIN2B GRIA1 GAD2 GAD1 AMPH
7 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 9.5 GAD2 GAD1 ENO1
2 carboxy-lyase activity GO:0016831 9.26 GAD2 GAD1
3 ionotropic glutamate receptor activity GO:0004970 9.16 GRIN2B GRIA1
4 glutamate decarboxylase activity GO:0004351 8.96 GAD2 GAD1
5 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 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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