MCID: ATM075
MIFTS: 39

Autoimmune Encephalitis

Categories: Immune diseases, Infectious diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Autoimmune Encephalitis

MalaCards integrated aliases for Autoimmune Encephalitis:

Name: Autoimmune Encephalitis 53
Hashimoto's Encephalitis 72

Classifications:



External Ids:

UMLS 72 C0393639

Summaries for Autoimmune Encephalitis

NIH Rare Diseases : 53 Autoimmune encephalitis refers to a group of conditions that occur when the body's immune system mistakenly attacks healthy brain cells, leading to inflammation of the brain. People with autoimmune encephalitis may have various neurologic and/or psychiatric symptoms. Neurologic symptoms may include impaired memory and cognition, abnormal movements, seizures, and/or problems with balance, speech, or vision. Psychiatric symptoms may include psychosis, aggression, inappropriate sexual behaviors, panic attacks, compulsive behaviors, euphoria or fear. Symptoms may fluctuate, but often progress over days to a few weeks. Symptoms can progress to loss of consciousness or even coma. Autoimmune encephalitis may be associated with antibodies to proteins on the surface of nerve cells, or within nerve cells. Some of these proteins are involved in passing signals between nerve cells. In some cases it occurs in association with cancer (a paraneoplastic syndrome). Research regarding why specific antibodies attack the body's healthy cells is ongoing. Autoimmune encephalitis generally occurs sporadically, in people with no family history of the condition. Treatment may involve intravenous immunosuppressive therapy, and tumor removal when necessary. Early treatment decreases the likelihood for long-term complications, speeds recovery, and reduces the risk of recurrence (relapse). If not treated, the condition can lead to progressive neurologic deterioration and loss of life.

MalaCards based summary : Autoimmune Encephalitis, also known as hashimoto's encephalitis, is related to amnestic disorder and migraine with or without aura 1. An important gene associated with Autoimmune Encephalitis is CNR1 (Cannabinoid Receptor 1), and among its related pathways/superpathways are Neuroscience and Eicosanoid ligand-binding receptors. The drugs Prednisolone phosphate and Methylprednisolone have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and b cells, and related phenotypes are behavior/neurological and growth/size/body region

Wikipedia : 75 Autoimmune encephalitis is a type of encephalitis that can result from a number of autoimmune diseases... more...

Related Diseases for Autoimmune Encephalitis

Diseases in the Encephalitis family:

Viral Encephalitis Autoimmune Encephalitis
Chronic Encephalitis Infectious Encephalitis

Diseases related to Autoimmune Encephalitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 164)
# Related Disease Score Top Affiliating Genes
1 amnestic disorder 30.1 LGI1 CNR1
2 migraine with or without aura 1 29.6 TRPV1 HCRT CNR1
3 central nervous system disease 28.7 TRPV1 IFNG HCRT CNR1
4 encephalitis 11.0
5 status epilepticus 10.5
6 limbic encephalitis 10.5
7 encephalopathy 10.4
8 allergic encephalomyelitis 10.4
9 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.3
10 hallucinogen dependence 10.3 FAAH CNR1
11 autoimmune disease 10.3
12 polysubstance abuse 10.3 FAAH CNR1
13 visual epilepsy 10.3
14 teratoma 10.3
15 movement disease 10.3
16 seizure disorder 10.3
17 ataxia and polyneuropathy, adult-onset 10.2
18 teratoma, ovarian 10.2
19 ovarian germ cell teratoma 10.2
20 diverticulitis of colon 10.2 TRPV1 CNR1
21 creutzfeldt-jakob disease 10.2
22 multiple sclerosis 10.2
23 glioma 10.2
24 glial tumor 10.2
25 post-traumatic stress disorder 10.2 FAAH CNR1
26 epilepsy 10.2
27 herpes simplex 10.2
28 aceruloplasminemia 10.1
29 aphasia 10.1
30 demyelinating disease 10.1
31 herpes simplex encephalitis 10.1
32 autonomic dysfunction 10.1
33 diabetic neuropathy 10.1 TRPV1 CNR1
34 somatoform disorder 10.1 TRPV1 FAAH
35 choreatic disease 10.1 LGI1 CNTNAP2
36 dystonia 10.1
37 dysautonomia 10.1
38 myoclonus 10.1
39 paraneoplastic syndromes 10.1
40 bone cancer 10.0 TRPV1 CNR1
41 ocular motor apraxia 10.0
42 thymoma, familial 10.0
43 lambert-eaton myasthenic syndrome 10.0
44 psychotic disorder 10.0
45 thymoma 10.0
46 sleep disorder 10.0
47 acute disseminated encephalomyelitis 10.0
48 vasculitis 10.0
49 rem sleep behavior disorder 10.0
50 rasmussen encephalitis 10.0

Graphical network of the top 20 diseases related to Autoimmune Encephalitis:



Diseases related to Autoimmune Encephalitis

Symptoms & Phenotypes for Autoimmune Encephalitis

MGI Mouse Phenotypes related to Autoimmune Encephalitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.91 CNR1 CNTNAP2 FAAH HCRT IFNG LGI1
2 growth/size/body region MP:0005378 9.8 CNR1 CNTNAP2 HCRT IFNG LGI1 TRPV1
3 nervous system MP:0003631 9.76 CNR1 CNTNAP2 FAAH HCRT IFNG LGI1
4 integument MP:0010771 9.73 CNR1 CNTNAP2 FAAH IFNG LGI1 TRPV1
5 no phenotypic analysis MP:0003012 9.26 CNR1 IFNG S1PR1 TRPV1
6 taste/olfaction MP:0005394 8.8 CNR1 CNTNAP2 TRPV1

Drugs & Therapeutics for Autoimmune Encephalitis

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

(show top 50) (show all 72)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
2
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
3
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
4
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
5
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
6 Hematinics Phase 4
7 Epoetin alfa Phase 4 113427-24-0
8 Gastrointestinal Agents Phase 4
9 Antiemetics Phase 4
10 Prednisolone acetate Phase 4
11 Neuroprotective Agents Phase 4
12 Hormones Phase 4
13 glucocorticoids Phase 4
14 Methylprednisolone Acetate Phase 4
15 Protective Agents Phase 4
16 Antineoplastic Agents, Hormonal Phase 4
17 gamma-Globulins Phase 4
18 Hormone Antagonists Phase 4
19 Immunoglobulins, Intravenous Phase 4
20 Rho(D) Immune Globulin Phase 4
21 Autonomic Agents Phase 4
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
23
Naltrexone Approved, Investigational, Vet_approved Phase 3 16590-41-3 5360515
24
Ethanol Approved Phase 3 64-17-5 702
25 Peripheral Nervous System Agents Phase 3
26 Narcotics Phase 3
27 Narcotic Antagonists Phase 3
28
Aldesleukin Approved Phase 1, Phase 2 85898-30-2, 110942-02-4
29
Ocrelizumab Approved, Investigational Phase 2 637334-45-3
30
rituximab Approved Phase 2 174722-31-7 10201696
31
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
32
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
33
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
34 Andrographolide Investigational Phase 1, Phase 2 5508-58-7
35 Anti-HIV Agents Phase 1, Phase 2
36 Anti-Retroviral Agents Phase 1, Phase 2
37 Anti-Inflammatory Agents Phase 1, Phase 2
38 Antiviral Agents Phase 1, Phase 2
39 Anti-Infective Agents Phase 1, Phase 2
40 Analgesics Phase 1, Phase 2
41 Analgesics, Non-Narcotic Phase 1, Phase 2
42 Andrographis Phase 1, Phase 2
43 Anti-Inflammatory Agents, Non-Steroidal Phase 1, Phase 2
44 Antiparasitic Agents Phase 1, Phase 2
45 Platelet Aggregation Inhibitors Phase 1, Phase 2
46 interferons Phase 1, Phase 2
47 Antiprotozoal Agents Phase 1, Phase 2
48 Antibodies Phase 2
49 Immunologic Factors Phase 2
50 Antineoplastic Agents, Immunological Phase 2

Interventional clinical trials:

(show all 19)
# Name Status NCT ID Phase Drugs
1 Effect of Erythropoietin in Refractory Autoimmune Encephalitis Patients Unknown status NCT03004209 Phase 4 Erythropoietin
2 Immunotherapy in Intractable Cryptogenic Epilepsy Patients With Autoimmune Antibody Unknown status NCT02695797 Phase 4 Prednisolone
3 A Randomized Placebo-Controlled, Crossover-Design Study of the Effects of Low Dose Naltrexone on Quality of Life as Measured by the Multiple Sclerosis Quality of Life Inventory (MSQLI54) Completed NCT00501696 Phase 3 4.5 mg Naltrexone;Naltrexone
4 Effect of IL-2 in Refractory Autoimmune Encephalitis Patients: A Pilot Study Unknown status NCT02714959 Phase 1, Phase 2 Proleukin
5 Efficacy and Safety of Tamibarotene (OAM80) for Alzheimer's Disease Unknown status NCT01120002 Phase 2 Tamibarotene;Placebo
6 Randomized, Comparative, Double Blind Controlled Phase II Clinical Trial, to Evaluate the Efficacy of ApE in Patients With Multiple Sclerosis (MS). Completed NCT02280876 Phase 1, Phase 2 1 - Andrographis paniculata p/st extract;2 - Excipients
7 Exploratory Study of Efficacy of Ocrelizumab in Autoimmune Encephalitis Recruiting NCT03835728 Phase 2 Ocrelizumab;Saline
8 A Multicenter Randomized, Controlled, Double-blinded Trial to Evaluate Efficacy and Safety of Bortezomib in Patients With Severe Autoimmune Encephalitis Not yet recruiting NCT03993262 Phase 2 Bortezomib;Placebo
9 Prospective Assessment of Efficacy of Immunoadsorption Therapy in Managing Childhood NMDA-Receptor (NMDAR) Antibodies Encephalitis Not yet recruiting NCT03274375 Phase 2 IA session;Rituximab
10 Multicenter and Prospective Clinical Registry Study of Autoimmune Encephalitis in China Unknown status NCT02561260
11 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in Beijing Area Unknown status NCT02443350
12 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in China Unknown status NCT02559089
13 The Usage of Neuropsychological Tests and Multi-mode Magnetic Resonance Imaging in Patients With Autoimmune Encephalitis for Cognitive Neural Mechanism Completed NCT03530462 rituximab;cyclophosphamide;Steroids;Intravenous immunoglobulin
14 Effect of Sodium Intake in Multiple Sclerosis Disease Activity Completed NCT01846234
15 Effect of Vigabatrin in Refractory Autoimmune Encephalitis Patients - A Pilot Study Recruiting NCT03003143 Vigabatrin 500 MG
16 Prospective Randomized Controlled Trial of Plasma Exchange in Autoimmune Encephalitis Recruiting NCT03542279 IVIG, High-dose glucocorticoid
17 Mechanisms of Auto-immune Encephalitis Active, not recruiting NCT02905136
18 Study of Neuro-ophthalmology Disorders in Autoimmune Encephalitis (NODE) Not yet recruiting NCT03872284
19 Incidence of Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis in France Between 2016 and 2018 Not yet recruiting NCT03957616

Search NIH Clinical Center for Autoimmune Encephalitis

Genetic Tests for Autoimmune Encephalitis

Anatomical Context for Autoimmune Encephalitis

MalaCards organs/tissues related to Autoimmune Encephalitis:

41
Brain, T Cells, B Cells, Testes, Thyroid, Bone, Bone Marrow

Publications for Autoimmune Encephalitis

Articles related to Autoimmune Encephalitis:

(show top 50) (show all 1036)
# Title Authors PMID Year
1
"Limbic encephalitis with acute onset and Hu antibodies treated with rituximab: Paraneoplastic or non-paraneoplastic disorder?" 38
31330415 2019
2
Status epilepticus in Hashimoto's encephalopathy. 38
31228700 2019
3
Autoimmune Encephalitis and Epilepsy: Evolving Definition and Clinical Spectrum. 38
31431603 2019
4
Putting a Band-Aid on a Broken Leg: Antiseizure Medications Are Inferior to Immune Therapies in Autoimmune Epilepsy. 38
31436112 2019
5
Neurosyphilis in the modern era: Literature review and case series. 38
31427238 2019
6
N-Methyl-D-Aspartate Receptor Encephalitis with Psychiatric Symptoms and an Ovarian Teratoma Detected by MRI in a 17-Year-Old Girl. 38
31163454 2019
7
Clinical characteristics and factors associated with short-term prognosis in adult patients with autoimmune encephalitis of non-neoplastic etiology. 38
30955116 2019
8
Seizure specificities in patients with antibody-mediated autoimmune encephalitis. 38
31283859 2019
9
PDE10A antibodies in autoimmune encephalitis: A possible marker of cancer immunotherapy? 38
31315974 2019
10
Clinical Presentation and Outcome in Autoimmune Encephalitis Associated With N-Type Voltage-Gated Calcium Channels in Children. 38
31014180 2019
11
Update on HSV and VZV infections of the brain. 38
31402052 2019
12
B cell depletion therapy resulting in sustained remission of severe autoimmune complications following Alemtuzumab treatment of Multiple Sclerosis. 38
31357122 2019
13
[Autoantibody-associated schizophreniform psychoses: pathophysiology, diagnostics, and treatment]. 38
31197396 2019
14
Factors Affecting the Response to First-Line Treatments in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis. 38
31286710 2019
15
A case study: Therapeutic plasma exchange in voltage-gated potassium channel autoimmune encephalitis. 38
31378682 2019
16
Pediatric autoimmune encephalitis in Denmark during 2011-17: A nationwide multicenter population-based cohort study. 38
31128894 2019
17
Serum and CSF neurofilament light chain levels in antibody-mediated encephalitis. 38
30944980 2019
18
Reply to "Grading the severity of autoimmune encephalitis: Advances and pitfalls". 38
31050012 2019
19
Grading the severity of autoimmune encephalitis: Advances and pitfalls. 38
31049995 2019
20
Medium-chain triglyceride ketogenic diet is effective for treatment of an adult with super-refractory status epilepticus: a case report and literature review. 38
31316173 2019
21
Functional Immunoregulation by Heme Oxygenase 1 in Juvenile Autoimmune Diseases. 38
31288720 2019
22
Three cases of antibody-LGI1 limbic encephalitis and review of literature. 38
30112956 2019
23
Neuronal damage and neuroinflammation markers in patients with autoimmune encephalitis and multiple sclerosis. 38
31267347 2019
24
Severe Epididymo-Orchitis and Encephalitis Complicating Anti-PD-1 Therapy. 38
30936376 2019
25
Clinical features and long-term outcomes of seizures associated with autoimmune encephalitis: A follow-up study in East China. 38
31331752 2019
26
The role of infections in autoimmune encephalitides. 38
31371185 2019
27
The Price of Immune Responses and the Role of Vitamin D in the Inner Ear. 38
31194714 2019
28
Autoimmunity in psychotic disorders. Where we stand, challenges and opportunities. 38
31323365 2019
29
[Recent progress in autoimmune encephalitis and its related disorders]. 38
31341132 2019
30
Status epilepticus in pregnancy - Can we frame a uniform treatment protocol? 38
31303443 2019
31
Long-term seizure outcome in patients with status epilepticus due to acute encephalitis. 38
30981052 2019
32
[Anti-N-methyl-D-aspartate receptor encephalitis, an essential differential diagnosis in psychiatry: a case report.] 38
30656512 2019
33
Nivolumab-Associated Acute Demyelinating Encephalitis: A Case Report and Literature Review. 38
31160476 2019
34
Immunoneuropsychiatry - novel perspectives on brain disorders. 38
30988501 2019
35
[AUTOIMMUNE ENCEPHALITIS: A BRIDGE BETWEEN NEUROLOGY AND PSYCHIATRY]. 38
31215187 2019
36
Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report. 38
31195997 2019
37
Autoimmune encephalitis presenting with cognitive decline and hyponatraemia. 38
31180781 2019
38
Immune-mediated encephalitis for the infectious disease specialist. 38
31021956 2019
39
Herpes simplex virus encephalitis update. 38
30921087 2019
40
Imaging in Encephalitis. 38
31378867 2019
41
Nivolumab-induced autoimmune encephalitis in an anti-neuronal autoantibody-positive patient. 38
31187869 2019
42
HLA class II allele DRB1*16:02 is associated with anti-NMDAR encephalitis. 38
30636700 2019
43
Little Children, Bigger Problems: Anti-N-Methyl D-Aspartate Receptor Encephalitis! 38
31197643 2019
44
Impact of anti-CASPR2 autoantibodies from patients with autoimmune encephalitis on CASPR2/TAG-1 interaction and Kv1 expression. 38
31176559 2019
45
Elevated Adenosine Deaminase Levels in the Cerebrospinal Fluid in Immune Checkpoint Inhibitor-induced Autoimmune Encephalitis: A Report of Two Cases. 38
31243206 2019
46
Management of antibody-mediated autoimmune encephalitis in adults and children: literature review and consensus-based practical recommendations. 38
31161339 2019
47
18F-Flurodeoxyglucose positron emission tomography with computed tomography (FDG PET/CT) findings in children with encephalitis and comparison to conventional imaging. 38
30863933 2019
48
Bidirectional regulatory potentials of short-chain fatty acids and their G-protein-coupled receptors in autoimmune neuroinflammation. 38
31222050 2019
49
The role of gut microbiota in shaping the relapse-remitting and chronic-progressive forms of multiple sclerosis in mouse models. 38
31061496 2019
50
Encephalopathy Associated With Neurochondrin Autoantibodies. 38
31138003 2019

Variations for Autoimmune Encephalitis

Expression for Autoimmune Encephalitis

Search GEO for disease gene expression data for Autoimmune Encephalitis.

Pathways for Autoimmune Encephalitis

Pathways related to Autoimmune Encephalitis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.87 HCRT CNTNAP2 CNR1
2
Show member pathways
10.64 S1PR1 CNR1
3 10.07 FAAH CNR1

GO Terms for Autoimmune Encephalitis

Cellular components related to Autoimmune Encephalitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.26 TRPV1 LGI1 HCRT CNTNAP2
2 intrinsic component of plasma membrane GO:0031226 8.62 TRPV1 S1PR1

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

# Name GO ID Score Top Affiliating Genes
1 excitatory postsynaptic potential GO:0060079 9.37 TRPV1 HCRT
2 regulation of insulin secretion GO:0050796 9.32 IFNG CNR1
3 sensory perception of pain GO:0019233 9.26 TRPV1 CNR1
4 positive regulation of nitric oxide biosynthetic process GO:0045429 9.16 TRPV1 IFNG
5 temperature homeostasis GO:0001659 8.96 TRPV1 HCRT
6 transmission of nerve impulse GO:0019226 8.62 S1PR1 CNTNAP2

Sources for Autoimmune 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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