MCID: ATM075
MIFTS: 38

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 52
Hashimoto's Encephalitis 71

Classifications:



External Ids:

UMLS 71 C0393639

Summaries for Autoimmune Encephalitis

NIH Rare Diseases : 52 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 stiff-person syndrome and amnestic disorder. 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 Hematinics and Epoetin alfa have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and testes, and related phenotypes are behavior/neurological and integument

Wikipedia : 74 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 186)
# Related Disease Score Top Affiliating Genes
1 stiff-person syndrome 29.9 LGI1 CNTNAP2
2 amnestic disorder 29.7 LGI1 CNTNAP2 CNR1
3 acute disseminated encephalomyelitis 29.7 LGI1 HCRT CNTNAP2
4 lingual-facial-buccal dyskinesia 29.6 LGI1 CNTNAP2
5 mutism 29.6 LGI1 CNTNAP2
6 migraine with or without aura 1 29.1 TRPV1 HCRT FAAH CNR1
7 multiple sclerosis 29.0 S1PR1 IFNG FAAH CNR1
8 encephalitis 11.1
9 status epilepticus 10.5
10 limbic encephalitis 10.5
11 encephalopathy 10.4
12 autoimmune disease 10.4
13 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.4
14 movement disease 10.4
15 allergic encephalomyelitis 10.4
16 ataxia and polyneuropathy, adult-onset 10.3
17 visual epilepsy 10.3
18 teratoma 10.3
19 seizure disorder 10.3
20 teratoma, ovarian 10.3
21 ovarian germ cell teratoma 10.3
22 herpes simplex 10.3
23 glioma 10.3
24 glial tumor 10.3
25 herpes simplex encephalitis 10.2
26 polysubstance abuse 10.2 FAAH CNR1
27 hallucinogen dependence 10.2 FAAH CNR1
28 creutzfeldt-jakob disease 10.2
29 epilepsy 10.2
30 autonomic dysfunction 10.2
31 cannabis abuse 10.2 FAAH CNR1
32 aphasia 10.2
33 withdrawal disorder 10.1 FAAH CNR1
34 demyelinating disease 10.1
35 sleep disorder 10.1
36 dystonia 10.1
37 dysautonomia 10.1
38 myoclonus 10.1
39 trigeminal nerve disease 10.1 TRPV1 CNR1
40 burning mouth syndrome 10.1 TRPV1 CNR1
41 thymoma, familial 10.1
42 relapsing-remitting multiple sclerosis 10.1
43 thymoma 10.1
44 thyroiditis 10.1
45 vasculitis 10.1
46 hashimoto encephalopathy 10.1
47 paraneoplastic syndromes 10.1
48 tremor 10.1
49 infectious encephalitis 10.1
50 autosomal dominant epilepsy with auditory features 10.0 LGI1 CNTNAP2

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.8 CNR1 CNTNAP2 FAAH HCRT IFNG LGI1
2 integument MP:0010771 9.63 CNR1 CNTNAP2 FAAH IFNG LGI1 TRPV1
3 nervous system MP:0003631 9.56 CNR1 CNTNAP2 FAAH HCRT IFNG LGI1
4 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 63)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Hematinics Phase 4
2 Epoetin alfa Phase 4 113427-24-0
3 Hormones Phase 4
4 Hormone Antagonists Phase 4
5 Rho(D) Immune Globulin Phase 4
6 gamma-Globulins Phase 4
7 glucocorticoids Phase 4
8 Antineoplastic Agents, Hormonal Phase 4
9
Naltrexone Approved, Investigational, Vet_approved Phase 3 16590-41-3 5360515
10
Ethanol Approved Phase 3 64-17-5 702
11 Narcotics Phase 3
12 Narcotic Antagonists Phase 3
13
Aldesleukin Approved Phase 1, Phase 2 85898-30-2, 110942-02-4
14
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
15
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
16
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
17
Ocrelizumab Approved, Investigational Phase 2 637334-45-3
18
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
19
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
20
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
21
rituximab Approved Phase 2 174722-31-7 10201696
22
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
23 Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
24
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
25
Andrographolide Investigational Phase 1, Phase 2 5508-58-7
26
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
27 Anti-HIV Agents Phase 1, Phase 2
28 Anti-Retroviral Agents Phase 1, Phase 2
29 Anti-Inflammatory Agents Phase 1, Phase 2
30 Antiviral Agents Phase 1, Phase 2
31 Anti-Infective Agents Phase 1, Phase 2
32 Analgesics, Non-Narcotic Phase 1, Phase 2
33 interferons Phase 1, Phase 2
34 Platelet Aggregation Inhibitors Phase 1, Phase 2
35 Analgesics Phase 1, Phase 2
36 Antiprotozoal Agents Phase 1, Phase 2
37 Antiparasitic Agents Phase 1, Phase 2
38 Andrographis Phase 1, Phase 2
39 Anti-Inflammatory Agents, Non-Steroidal Phase 1, Phase 2
40 Immunoglobulin G Phase 2
41 Pharmaceutical Solutions Phase 2
42 Antibodies Phase 2
43 Immunoglobulins, Intravenous Phase 2
44 Immunologic Factors Phase 2
45 Immunoglobulins Phase 2
46 Methylprednisolone Acetate Phase 2
47 Antirheumatic Agents Phase 2
48 Antineoplastic Agents, Immunological Phase 2
49
Vigabatrin Approved 68506-86-5, 60643-86-9 5665
50
Heparin Approved, Investigational 9005-49-6 46507594 772

Interventional clinical trials:

(show all 25)
# 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 A Phase 2a, Prospective, Open-label, Single-arm, Single Center, Proof of Concept Study to Evaluate the Safety and Efficacy of IGIV 10% in Patients With Autoimmune Encephalitis Recruiting NCT04175522 Phase 2 Immunoglobulin G
8 A Multicenter Randomized, Controlled, Double-blinded Trial to Evaluate Efficacy and Safety of Bortezomib in Patients With Severe Autoimmune Encephalitis Recruiting NCT03993262 Phase 2 Bortezomib;Placebo
9 Exploratory Study of Efficacy of Ocrelizumab in Autoimmune Encephalitis Recruiting NCT03835728 Phase 2 Ocrelizumab;Saline
10 Interleukin-6 Receptor Antibodies for Modulating the Systemic Inflammatory Response After Out-of-Hospital Cardiac Arrest - a Randomized Clinical Trial Active, not recruiting NCT03863015 Phase 2 Tocilizumab 20 Mg/mL Intravenous Solution;isotonic saline
11 A Phase-2b, Double-Blind, Randomized Controlled Trial to Evaluate the Activity and Safety of Inebilizumab in Anti-Nmda Receptor Encephalitis and Assess Markers of Disease Not yet recruiting NCT04372615 Phase 2 Inebilizumab;Placebo
12 Prospective Assessment of Efficacy of Immunoadsorption Therapy in Managing Childhood NMDA-Receptor (NMDAR) Antibodies Encephalitis Not yet recruiting NCT03274375 Phase 2 IA session;Rituximab
13 Effect of Vigabatrin in Refractory Autoimmune Encephalitis Patients - A Pilot Study Unknown status NCT03003143 Vigabatrin 500 MG
14 Multicenter and Prospective Clinical Registry Study of Autoimmune Encephalitis in China Unknown status NCT02561260
15 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in Beijing Area Unknown status NCT02443350
16 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in China Unknown status NCT02559089
17 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
18 Effect of Sodium Intake in Multiple Sclerosis Disease Activity Completed NCT01846234
19 Study of Neuro-ophthalmology Disorders in Autoimmune Encephalitis (NODE) Recruiting NCT03872284
20 Study of 12 Cases of Autoimmune Encephalitis With Anti-NMDA Receptor Antibodies Following Herpetic Encephalitis and Review of the Literature Recruiting NCT04339127
21 Prospective Randomized Controlled Trial of Plasma Exchange in Autoimmune Encephalitis Recruiting NCT03542279 IVIG, High-dose glucocorticoid
22 Mechanisms of Auto-immune Encephalitis Active, not recruiting NCT02905136
23 Clinical, EEG and Radiological Initial and Evolutive Characterisation of LGI1-antibody Patients Not yet recruiting NCT04106765
24 Immunogenetic Characteristics in Autoimmune Encephalitis and Related Disorders: HLA Analysis Not yet recruiting NCT04106596
25 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:

40
Brain, T Cells, Testes, B Cells, Bone, Lung, Thyroid

Publications for Autoimmune Encephalitis

Articles related to Autoimmune Encephalitis:

(show top 50) (show all 1226)
# Title Authors PMID Year
1
Loss of Phosphatidylinositol 3-Kinase Activity in Regulatory T Cells Leads to Neuronal Inflammation. 61
32414808 2020
2
Safety and feasibility of various fasting-mimicking diets among people with multiple sclerosis. 61
32408153 2020
3
Glial fibrillary acidic protein (GFAP) associated autoimmune meningoencephalitis in a patient receiving nivolumab. 61
32416558 2020
4
Clinical analysis of anti-NMDAR encephalitis combined with MOG antibody in children. 61
32234601 2020
5
Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient. 61
32295822 2020
6
Clinical and imaging features of children with autoimmune encephalitis and MOG antibodies. 61
32358225 2020
7
Novel findings of HLA association with anti-LGI1 encephalitis: HLA-DRB1*03:01 and HLA-DQB1*02:01. 61
32417596 2020
8
Reply to "Grading the Severity of Autoimmune Encephalitis: When to Evaluate?" 61
32356329 2020
9
[Parainfectious CNS small vessel vasculitis and autoimmune encephalitis after herpes simplex type 1 encephalitis]. 61
32347328 2020
10
Neuronal Surface Antibody Syndrome: A Review of the Characteristics of the Disease and Its Association with Autoantibodies. 61
32554968 2020
11
Sleep Disturbances in Patients with Autoimmune Encephalitis. 61
32524504 2020
12
Searching for autoimmune encephalitis: Beware of normal CSF. 61
32563126 2020
13
Grading the Severity of Autoimmune Encephalitis: When to Evaluate? 61
32356319 2020
14
Autoimmune encephalitis and immune therapy: lessons from Argentina. 61
30182259 2020
15
Astrocytes and their potential role in anti-NMDA receptor encephalitis. 61
32085980 2020
16
Neural autoantibodies and autoimmune encephalitis - the conjunction of both counts. 61
32502286 2020
17
Efficacy and safety of rituximab in autoimmune encephalitis: A meta-analysis. 61
32484900 2020
18
Acute amnestic syndromes. 61
32203745 2020
19
Infectious and Autoimmune Causes of Encephalitis in Children. 61
32358069 2020
20
Anti-gamma-aminobutyric acid receptor type A encephalitis: a review. 61
32374573 2020
21
Psychiatric Manifestation of Anti-LGI1 Encephalitis. 61
32560097 2020
22
Acute symptomatic seizures secondary to autoimmune encephalitis and autoimmune-associated epilepsy: Conceptual definitions. 61
32544279 2020
23
Autoimmune encephalitis as an increasingly recognised cause of non-convulsive status epilepticus: A retrospective, multicentre evaluation of patient characteristics and electroencephalography (EEG) results. 61
32574837 2020
24
Seizures in autoimmune encephalitis: Kindling the fire. 61
32400012 2020
25
Olanzapine and Lorazepam Used in the Symptomatic Management of Excited Catatonia Secondary to Anti-N-Methyl-D-Aspartate Receptor Encephalitis. 61
32577332 2020
26
Encephalitis in a Patient With Melanoma Treated With Immune Checkpoint Inhibitors: Case Presentation and Review of the Literature. 61
32554976 2020
27
The neuropsychological spectrum of anti-LGI1 antibody mediated autoimmune encephalitis. 61
32480239 2020
28
Dietary factors in experimental autoimmune encephalomyelitis and multiple sclerosis: A comprehensive review. 61
32406797 2020
29
Autoimmune encephalitis associated with Ma2 antibodies and immune checkpoint inhibitor therapy. 61
32303632 2020
30
Psychiatric Presentation of Probable Seronegative Autoimmune Encephalitis in a Late Middle-Aged Woman With Ovarian Teratoma. 61
31607502 2020
31
Clinical characteristics of GAD 65-associated autoimmune encephalitis. 61
32416610 2020
32
Autoimmune glial fibillary acidic protein astrocytopathy associated meningoencephalomyelitis and bilateral sensorineuronal deafness. 61
31981881 2020
33
A Teenager With Acute Anterograde Amnesia. 61
29346236 2020
34
Refractory status epilepticus behind autoimmune encephalitis. 61
31811529 2020
35
Possible N-methyl-D-aspartate receptor antibody-mediated encephalitis in the setting of HIV cerebrospinal fluid escape. 61
31960135 2020
36
Clinical characteristics and outcome of patients with autoimmune encephalitis: clues for paraneoplastic aetiology. 61
32412135 2020
37
Seropositive Anti-NMDAR Mediated Autoimmune Encephalitis. 61
32405773 2020
38
Anti-Glutamic Acid Decarboxylase-65 Autoimmune Encephalitis Presenting With Mania and Delirium. 61
31648777 2020
39
Pediatric autoimmune encephalitis: Recognition and diagnosis. 61
32047077 2020
40
Autoimmune Encephalitis in Children: A Case Series at a Tertiary Care Center. 61
32458722 2020
41
Autoimmune Encephalitis With Elevated N-Type Calcium Channel Antibodies Presenting as Psychotic Depression. 61
32408401 2020
42
18F-FDG-PET Imaging Patterns in Autoimmune Encephalitis: Impact of Image Analysis on the Results. 61
32486044 2020
43
Utility of brain fluorodeoxyglucose PET in children with possible autoimmune encephalitis. 61
32459714 2020
44
CSF studies which ultimately led to the possible diagnosis of anti-NMDAR encephalitis. 61
32385119 2020
45
Autoimmune encephalitis with psychosis: Warning signs, step-by-step diagnostics and treatment. 61
30513253 2020
46
Anti-NMDAR Encephalitis Following Herpes Simplex Virus Encephalitis: 2 Cases From Portugal. 61
32373278 2020
47
Catatonia with GABAA receptor antibodies. 61
31771952 2020
48
Feasibility & safety of plasma exchange in paediatric neuro-immunology: A single center experience. 61
32307218 2020
49
Ictal Catatonia in Autoimmune Encephalitis. 61
32236165 2020
50
Worldwide survey of neurologists on approach to autoimmune encephalitis. 61
32309032 2020

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 synapse GO:0045202 9.26 TRPV1 LGI1 HCRT CNR1
2 intrinsic component of plasma membrane GO:0031226 9.16 TRPV1 S1PR1
3 cell junction GO:0030054 9.02 TRPV1 LGI1 HCRT CNTNAP2 CNR1

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

# Name GO ID Score Top Affiliating Genes
1 regulation of insulin secretion GO:0050796 9.43 IFNG CNR1
2 excitatory postsynaptic potential GO:0060079 9.4 TRPV1 HCRT
3 sensory perception of pain GO:0019233 9.37 TRPV1 CNR1
4 positive regulation of nitric oxide biosynthetic process GO:0045429 9.32 TRPV1 IFNG
5 microglial cell activation GO:0001774 9.26 TRPV1 IFNG
6 regulation of metabolic process GO:0019222 9.16 S1PR1 CNR1
7 temperature homeostasis GO:0001659 8.96 TRPV1 HCRT
8 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 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
Content
Loading form....