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 amnestic disorder and mutism. 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 Methylprednisolone hemisuccinate and Methylprednisolone have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and bone, 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 181)
# Related Disease Score Top Affiliating Genes
1 amnestic disorder 29.7 LGI1 CNTNAP2 CNR1
2 mutism 29.6 LGI1 CNTNAP2
3 stiff-person syndrome 29.6 LGI1 CNTNAP2
4 acute disseminated encephalomyelitis 29.6 LGI1 HCRT CNTNAP2
5 central nervous system disease 29.2 IFNG HCRT CNR1
6 hyperekplexia 29.1 LGI1 CNTNAP2
7 migraine with or without aura 1 29.1 TRPV1 HCRT FAAH CNR1
8 multiple sclerosis 29.0 S1PR1 IFNG FAAH CNR1
9 encephalitis 11.1
10 status epilepticus 10.5
11 limbic encephalitis 10.5
12 encephalopathy 10.4
13 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.4
14 allergic encephalomyelitis 10.4
15 autoimmune disease 10.4
16 movement disease 10.3
17 teratoma 10.3
18 ovarian germ cell teratoma 10.3
19 visual epilepsy 10.3
20 ataxia and polyneuropathy, adult-onset 10.3
21 teratoma, ovarian 10.3
22 herpes simplex 10.3
23 seizure disorder 10.3
24 epilepsy 10.2
25 glioma 10.2
26 herpes simplex encephalitis 10.2
27 glial tumor 10.2
28 polysubstance abuse 10.2 FAAH CNR1
29 hallucinogen dependence 10.2 FAAH CNR1
30 cannabis abuse 10.2 FAAH CNR1
31 creutzfeldt-jakob disease 10.2
32 withdrawal disorder 10.2 FAAH CNR1
33 aphasia 10.2
34 autonomic dysfunction 10.2
35 uvulitis 10.1 TRPV1 CNR1
36 demyelinating disease 10.1
37 aceruloplasminemia 10.1
38 dysautonomia 10.1
39 burning mouth syndrome 10.1 TRPV1 CNR1
40 sleep disorder 10.1
41 dystonia 10.1
42 relapsing-remitting multiple sclerosis 10.1
43 myoclonus 10.1
44 paraneoplastic syndromes 10.1
45 tremor 10.1
46 autosomal dominant epilepsy with auditory features 10.0 LGI1 CNTNAP2
47 dissociative amnesia 10.0 LGI1 CNTNAP2
48 la crosse encephalitis 10.0 LGI1 CNTNAP2
49 thymoma 10.0
50 purpura 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:

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 68)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
2
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
3
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
4 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
6
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
7 Hematinics Phase 4
8 Epoetin alfa Phase 4 113427-24-0
9 Gastrointestinal Agents Phase 4
10 Antiemetics Phase 4
11 Methylprednisolone Acetate Phase 4
12 Hormone Antagonists Phase 4
13 Autonomic Agents Phase 4
14 Protective Agents Phase 4
15 gamma-Globulins Phase 4
16 Neuroprotective Agents Phase 4
17 Rho(D) Immune Globulin Phase 4
18 Antineoplastic Agents, Hormonal Phase 4
19 Immunoglobulins, Intravenous Phase 4
20 glucocorticoids Phase 4
21 Hormones Phase 4
22
Naltrexone Approved, Investigational, Vet_approved Phase 3 16590-41-3 5360515
23
Ethanol Approved Phase 3 64-17-5 702
24 Narcotics Phase 3
25 Narcotic Antagonists Phase 3
26
Aldesleukin Approved Phase 1, Phase 2 110942-02-4, 85898-30-2
27
Ocrelizumab Approved, Investigational Phase 2 637334-45-3
28
rituximab Approved Phase 2 174722-31-7 10201696
29
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
30
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
31
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
32 Andrographolide Investigational Phase 1, Phase 2 5508-58-7
33 Anti-Retroviral Agents Phase 1, Phase 2
34 Anti-HIV Agents Phase 1, Phase 2
35 Anti-Inflammatory Agents Phase 1, Phase 2
36 Anti-Infective Agents Phase 1, Phase 2
37 Antiviral Agents Phase 1, Phase 2
38 Anti-Inflammatory Agents, Non-Steroidal Phase 1, Phase 2
39 Platelet Aggregation Inhibitors Phase 1, Phase 2
40 Analgesics, Non-Narcotic Phase 1, Phase 2
41 Antiparasitic Agents Phase 1, Phase 2
42 Antiprotozoal Agents Phase 1, Phase 2
43 Andrographis Phase 1, Phase 2
44 interferons Phase 1, Phase 2
45 Analgesics Phase 1, Phase 2
46 Immunoglobulin G Phase 2
47 Immunologic Factors Phase 2
48 Antibodies Phase 2
49 Immunoglobulins Phase 2
50 Antineoplastic Agents, Immunological Phase 2

Interventional clinical trials:

(show all 23)
# 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 Exploratory Study of Efficacy of Ocrelizumab in Autoimmune Encephalitis Recruiting NCT03835728 Phase 2 Ocrelizumab;Saline
9 Interleukin-6 Receptor Antibodies for Modulating the Systemic Inflammatory Response After Out-of-Hospital Cardiac Arrest - a Randomized Clinical Trial Recruiting NCT03863015 Phase 2 Tocilizumab 20 Mg/mL Intravenous Solution;isotonic saline
10 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
11 Prospective Assessment of Efficacy of Immunoadsorption Therapy in Managing Childhood NMDA-Receptor (NMDAR) Antibodies Encephalitis Not yet recruiting NCT03274375 Phase 2 IA session;Rituximab
12 Effect of Vigabatrin in Refractory Autoimmune Encephalitis Patients - A Pilot Study Unknown status NCT03003143 Vigabatrin 500 MG
13 Multicenter and Prospective Clinical Registry Study of Autoimmune Encephalitis in China Unknown status NCT02561260
14 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in Beijing Area Unknown status NCT02443350
15 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in China Unknown status NCT02559089
16 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
17 Effect of Sodium Intake in Multiple Sclerosis Disease Activity Completed NCT01846234
18 Prospective Randomized Controlled Trial of Plasma Exchange in Autoimmune Encephalitis Recruiting NCT03542279 IVIG, High-dose glucocorticoid
19 Mechanisms of Auto-immune Encephalitis Active, not recruiting NCT02905136
20 Study of Neuro-ophthalmology Disorders in Autoimmune Encephalitis (NODE) Not yet recruiting NCT03872284
21 Clinical, EEG and Radiological Initial and Evolutive Characterisation of LGI1-antibody Patients Not yet recruiting NCT04106765
22 Immunogenetic Characteristics in Autoimmune Encephalitis and Related Disorders: HLA Analysis Not yet recruiting NCT04106596
23 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, Bone, Thyroid, Lung, Testes, B Cells

Publications for Autoimmune Encephalitis

Articles related to Autoimmune Encephalitis:

(show top 50) (show all 1148)
# Title Authors PMID Year
1
Paraneoplastic cerebellar ataxia and antibodies to metabotropic glutamate receptor 2. 61
31826987 2020
2
Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient. 61
31953309 2020
3
Autoimmune Encephalitis is Recognised as an Important Differential Diagnosis in a Kenyan Tertiary Referral Centre. 61
32015184 2020
4
Immune mediated pediatric encephalitis - need for comprehensive evaluation and consensus guidelines. 61
32013931 2020
5
Diagnosing autoimmune encephalitis in a real-world single-centre setting. 61
31667626 2020
6
Positive oligoclonal bands and CSF pleocytosis in narcolepsy type 1: A case report supporting the immune-mediated hypothesis. 61
31756639 2020
7
Japanese encephalitis-induced anti-N-methyl-d-aspartate receptor encephalitis: A hospital-based prospective study. 61
31563418 2020
8
Recurrent panic attack and bilateral hippocampus lesions as main manifestation in an autoimmune encephalitis associated with primary biliary cirrhosis. 61
31929372 2020
9
Anti-dipeptidyl-peptidase-like protein 6 encephalitis, a rare cause of reversible rapid progressive dementia and insomnia. 61
31775073 2020
10
Autoimmune Encephalitis and Epilepsy: Evolving Definition and Clinical Spectrum. 61
32024345 2020
11
Intranasal Methylprednisolone Effectively Reduces Neuroinflammation in Mice With Experimental Autoimmune Encephalitis. 61
31886871 2020
12
Daratumumab treatment for therapy-refractory anti-CASPR2 encephalitis. 61
31630242 2020
13
Systematic review of the clinical spectrum of CASPR2 antibody syndrome. 61
31912210 2020
14
Checkpoint inhibitor-induced autoimmune encephalitis reversed by rituximab after allogeneic bone marrow transplant in a patient with Hodgkin lymphoma. 61
31502489 2020
15
Phosphatidylcholine PC ae C44:6 in cerebrospinal fluid is a sensitive biomarker for bacterial meningitis. 61
31910875 2020
16
The role of B regulatory (B10) cells in inflammatory disorders and their potential as therapeutic targets. 61
31881524 2020
17
A critical analysis of helminth immunotherapy in multiple sclerosis. 61
31971074 2020
18
Anti-leucine-rich Glioma Inactivated-1 Encephalitis Associated with Essential Thrombocythemia. 61
31941873 2020
19
Epstein Barr virus nuclear antigen 1 (EBNA-1) peptides recognized by adult multiple sclerosis patient sera induce neurologic symptoms in a murine model. 61
31515129 2020
20
Possible N-methyl-D-aspartate receptor antibody-mediated encephalitis in the setting of HIV cerebrospinal fluid escape. 61
31960135 2020
21
Hashimoto encephalopathy in the 21st century. 61
31882532 2020
22
New-onset refractory status epilepticus: A retrospective cohort study. 61
31830676 2020
23
Deciphering anti-MOG IgG antibodies: Clinical and radiological spectrum, and comparison of antibody detection assays. 61
31954354 2020
24
Maraviroc attenuates the pathogenesis of experimental autoimmune encephalitis. 61
32007705 2020
25
Acute Interstitial Nephritis in a Patient with High Aluminum Blood Levels: A Case Report. 61
32015600 2020
26
Cerebral functional activity and connectivity changes in anti-N-methyl-D-aspartate receptor encephalitis: A resting-state fMRI study. 61
32036276 2020
27
Autoimmune Encephalitis: A Diagnostic Challenge For Physician. 61
31979656 2020
28
An expanded parenchymal CD8+ T cell clone in GABAA receptor encephalitis. 61
31943946 2020
29
Different FDG-PET metabolic patterns of anti-AMPAR and anti-NMDAR encephalitis: Case report and literature review. 61
31985135 2020
30
The princess and the p-value: A case report of suspected autoimmune encephalitis and functional neurological disorder in a pediatric patient. 61
30265565 2020
31
Leucine-rich glioma-inactivated protein 1 (LGI-1) mediated limbic encephalitis associated with syndrome of inappropriate antidiuretic hormone secretion: a case report. 61
32038879 2020
32
Autoimmune psychosis: an international consensus on an approach to the diagnosis and management of psychosis of suspected autoimmune origin. 61
31669058 2020
33
Psychiatric Symptoms in Pediatric Patients With Myelin-Oligodendrocyte-Glycoprotein-Immunoglobulin G-Antibody Positive Autoimmune Encephalitis: A Case Series. 61
31980213 2019
34
Neuroimmunological antibody-mediated encephalitis and implications for diagnosis and therapy in neuropsychiatry. 61
31791436 2019
35
Subgroup comparison according to clinical phenotype and serostatus in autoimmune encephalitis: a multicenter retrospective study. 61
31814224 2019
36
Single-center study of autoimmune encephalitis-related autoantibody testing in Hungary. 61
31650706 2019
37
Serum matrix metallopeptidase-9 and tissue inhibitor of metalloproteinase-1 levels in autoimmune encephalitis. 61
31843295 2019
38
Autoimmune Encephalitis Associated with Anti-gamma-aminobutyric Acid B Receptor Antibodies Mimicking Syncope: A Case Report. 61
31813910 2019
39
Anti NMDA receptor antibody encephalitis in Pakistan: Clinicopathological features and treatment outcomes. 61
31853128 2019
40
Focus on autonomic dysfunctions in anti-NMDAR encephalitis: a case report. 61
31858566 2019
41
Psychosis as an initial symptom of autoimmune encephalitis with negative antibodies. 61
31859110 2019
42
Autoimmune encephalitis. 61
31808454 2019
43
Frequency and temporal sequence of clinical features in adults with anti-NMDA receptor encephalitis presenting with psychiatric symptoms. 61
30560745 2019
44
Multiple co-existing antibodies in autoimmune encephalitis: A case and review of the literature. 61
31655424 2019
45
Treatable causes of adult-onset rapid cognitive impairment. 61
31715517 2019
46
Refractory status epilepticus behind autoimmune encephalitis. 61
31811529 2019
47
Status epilepticus in pregnancy - Can we frame a uniform treatment protocol? 61
31303443 2019
48
Continuous EEG Findings in Autoimmune Encephalitis. 61
31800465 2019
49
Medium-chain triglyceride ketogenic diet is effective for treatment of an adult with super-refractory status epilepticus: a case report and literature review. 61
31316173 2019
50
High mobility group box 1 (HMGB1) protein in Multiple Sclerosis (MS): Mechanisms and therapeutic potential. 61
31606383 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 regulation of insulin secretion GO:0050796 9.4 IFNG CNR1
2 excitatory postsynaptic potential GO:0060079 9.37 TRPV1 HCRT
3 sensory perception of pain GO:0019233 9.32 TRPV1 CNR1
4 positive regulation of nitric oxide biosynthetic process GO:0045429 9.26 TRPV1 IFNG
5 microglial cell activation GO:0001774 9.16 TRPV1 IFNG
6 temperature homeostasis GO:0001659 8.96 TRPV1 HCRT
7 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
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