MCID: ATM075
MIFTS: 42

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 54
Hashimoto's Encephalitis 74

Classifications:



External Ids:

UMLS 74 C0393639

Summaries for Autoimmune Encephalitis

NIH Rare Diseases : 54 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 encephalitis and multiple sclerosis. An important gene associated with Autoimmune Encephalitis is CNTNAP2 (Contactin Associated Protein Like 2), and among its related pathways/superpathways are Neuroscience and Eicosanoid ligand-binding receptors. The drugs Epoetin alfa and Hematinics have been mentioned in the context of this disorder. Affiliated tissues include brain, lung and testes, and related phenotypes are behavior/neurological and growth/size/body region

Wikipedia : 77 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

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

(show top 50) (show all 87)
# Related Disease Score Top Affiliating Genes
1 encephalitis 11.0
2 multiple sclerosis 10.3
3 choreatic disease 10.2 CNTNAP2 LGI1
4 creutzfeldt-jakob disease 10.1
5 status epilepticus 10.1
6 epilepsy 10.1
7 amnestic disorder 10.1 CNR1 LGI1
8 internuclear ophthalmoplegia 10.1 HCRT LGI1
9 diverticulitis of colon 10.1 CNR1 TRPV1
10 seizure disorder 10.1
11 diabetic neuropathy 10.1 CNR1 TRPV1
12 glioma 10.1
13 bone cancer 10.0 CNR1 TRPV1
14 herpes zoster 10.0 IFNG TRPV1
15 herpes simplex 10.0
16 encephalopathy 10.0
17 upper respiratory tract disease 10.0 IFNG TRPV1
18 overnutrition 10.0 CNR1 TRPV1
19 bone inflammation disease 10.0 IFNG TRPV1
20 hallucinogen dependence 10.0 CNR1 FAAH
21 polysubstance abuse 9.9 CNR1 FAAH
22 specific developmental disorder 9.9 CNR1 TRPV1
23 small cell cancer of the lung 9.9
24 lung cancer 9.9
25 thymoma, familial 9.9
26 dementia 9.9
27 thymoma 9.9
28 movement disease 9.9
29 herpes simplex encephalitis 9.9
30 disease of mental health 9.9 CNR1 HCRT TRPV1
31 migraine with or without aura 1 9.9 CNR1 HCRT TRPV1
32 speech and communication disorders 9.9 CNTNAP2 LGI1 TRPV1
33 focal epilepsy 9.9 CNTNAP2 LGI1 TRPV1
34 post-traumatic stress disorder 9.9 CNR1 FAAH
35 somatoform disorder 9.8 FAAH TRPV1
36 alcohol abuse 9.8 CNR1 FAAH
37 mouth disease 9.8 IFNG TRPV1
38 rheumatoid arthritis 9.8
39 myasthenia gravis 9.8
40 panencephalitis, subacute sclerosing 9.8
41 nance-horan syndrome 9.8
42 ataxia and polyneuropathy, adult-onset 9.8
43 aceruloplasminemia 9.8
44 vitiligo-associated multiple autoimmune disease susceptibility 1 9.8
45 malaria 9.8
46 lung cancer susceptibility 3 9.8
47 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 9.8
48 arthritis 9.8
49 brain injury 9.8
50 neuromyelitis optica 9.8

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:

47
# 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 51)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Epoetin alfa Phase 4 113427-24-0
2 Hematinics Phase 4
3
Aldesleukin Approved Phase 1, Phase 2 110942-02-4, 85898-30-2
4
Methylprednisolone Approved, Vet_approved Phase 2,Not Applicable 83-43-2 6741
5
Methylprednisolone hemisuccinate Approved Phase 2,Not Applicable 2921-57-5
6
rituximab Approved Phase 2 174722-31-7 10201696
7
Prednisolone Approved, Vet_approved Phase 2,Not Applicable 50-24-8 5755
8
Prednisolone phosphate Approved, Vet_approved Phase 2,Not Applicable 302-25-0
9
Ocrelizumab Approved, Investigational Phase 2 637334-45-3
10
Prednisolone hemisuccinate Experimental Phase 2,Not Applicable 2920-86-7
11 Anti-Infective Agents Phase 1, Phase 2
12 Anti-Retroviral Agents Phase 1, Phase 2
13 Antiviral Agents Phase 1, Phase 2
14 Anti-HIV Agents Phase 1, Phase 2
15 Immunologic Factors Phase 2
16 Antibodies Phase 2
17 Rho(D) Immune Globulin Phase 2
18 Methylprednisolone Acetate Phase 2,Not Applicable
19 Prednisolone acetate Phase 2,Not Applicable
20 Immunoglobulins Phase 2
21 Antineoplastic Agents, Immunological Phase 2
22 Antirheumatic Agents Phase 2
23 Immunoglobulins, Intravenous Phase 2
24 gamma-Globulins Phase 2
25 Autoantibodies Phase 2
26 Pharmaceutical Solutions Phase 2
27
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
28
Heparin Approved, Investigational Not Applicable 9005-49-6 46507594 772
29
Vigabatrin Approved Not Applicable 68506-86-5, 60643-86-9 5665
30 N-Methylaspartate
31 Aspartic Acid
32 Antineoplastic Agents, Hormonal
33 Gastrointestinal Agents ,Not Applicable
34 Antiemetics ,Not Applicable
35 Alkylating Agents
36 Peripheral Nervous System Agents ,Not Applicable
37 Autonomic Agents ,Not Applicable
38 Hormones ,Not Applicable
39 glucocorticoids ,Not Applicable
40 Immunosuppressive Agents
41 Hormones, Hormone Substitutes, and Hormone Antagonists ,Not Applicable
42 Anti-Inflammatory Agents ,Not Applicable
43 Protective Agents ,Not Applicable
44 Neuroprotective Agents ,Not Applicable
45 Hormone Antagonists ,Not Applicable
46 Antineoplastic Agents, Alkylating
47 calcium heparin Not Applicable
48 Anticoagulants Not Applicable
49 Neurotransmitter Agents Not Applicable
50 GABA Agents Not Applicable

Interventional clinical trials:

(show all 16)
# Name Status NCT ID Phase Drugs
1 Effect of Erythropoietin in Refractory Autoimmune Encephalitis Patients Unknown status NCT03004209 Phase 4 Erythropoietin
2 IL-2 in Refractory Autoimmune Encephalitis Unknown status NCT02714959 Phase 1, Phase 2 Proleukin
3 Efficacy of Ocrelizumab in Autoimmune Encephalitis Recruiting NCT03835728 Phase 2 Ocrelizumab;Saline
4 IVIG and Rituximab in Antibody-associated Psychosis - SINAPPS2 Recruiting NCT03194815 Phase 2 Intravenous immunoglobulin;Placebo;Rituximab
5 Multicenter and Prospective Clinical Registry Study of Autoimmune Encephalitis in China Unknown status NCT02561260
6 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in Beijing Area Unknown status NCT02443350
7 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in China Unknown status NCT02559089
8 To Explore Cognitive Neural Mechanism of Autoimmune Encephalitis by Using Neuropsychological Tests and Multi-modal MRI Completed NCT03530462 rituximab;cyclophosphamide;Steroids;Intravenous immunoglobulin
9 Sodium Intake in Multiple Sclerosis Completed NCT01846234
10 Immunotherapy in Autoimmune Encephalitis Recruiting NCT03542279 Not Applicable IVIG, High-dose glucocorticoid
11 Effect of Vigabatrin in Refractory Autoimmune Encephalitis Patients Recruiting NCT03003143 Not Applicable Vigabatrin 500 MG
12 Antibodies Causing Epilepsy Syndromes: The ACES Study. Recruiting NCT02802475
13 Mechanisms of Auto-immune Encephalitis Active, not recruiting NCT02905136
14 Incidence of Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis Not yet recruiting NCT03957616
15 Neuro-ophthalmology and Autoimmune Encephalitis (NODE) Not yet recruiting NCT03872284
16 Phenotypic and Functional Study of 4BL B Cells in Multiple Sclerosis (MS) Not yet recruiting NCT03796611

Search NIH Clinical Center for Autoimmune Encephalitis

Genetic Tests for Autoimmune Encephalitis

Anatomical Context for Autoimmune Encephalitis

MalaCards organs/tissues related to Autoimmune Encephalitis:

42
Brain, Lung, Testes, Bone, T Cells, B Cells, Thyroid

Publications for Autoimmune Encephalitis

Articles related to Autoimmune Encephalitis:

(show top 50) (show all 284)
# Title Authors Year
1
Autoimmune Encephalitis as an Extra-articular Manifestation of Rheumatoid Arthritis. ( 30449780 )
2019
2
Psychosis of dual origin in HIV infection: Viral escape syndrome and autoimmune encephalitis. ( 31041138 )
2019
3
Grading the severity of autoimmune encephalitis: advances and pitfalls. ( 31049995 )
2019
4
Reply to "Grading the severity of autoimmune encephalitis: advances and pitfalls". ( 31050012 )
2019
5
Heidenhain variant sporadic Creutzfeldt-Jakob disease diagnosed as an autoimmune encephalitis due to a false-positive GAD autoantibody. ( 31061195 )
2019
6
Autoimmune encephalitis with anti-IgLON5 and anti-GABAB-receptor antibodies: A case report. ( 31096519 )
2019
7
Efficacy of subcutaneous immunoglobulin therapy in a patient with autoimmune encephalitis: a case report. ( 30232671 )
2019
8
Admission diagnoses of patients later diagnosed with autoimmune encephalitis. ( 30421340 )
2019
9
Proportion and spectrum of movement disorders in adolescent and adult patients of autoimmune encephalitis of non-neoplastic aetiology. ( 30455136 )
2019
10
Ictal piloerection is associated with high-grade glioma and autoimmune encephalitis-Results from a systematic review. ( 30497014 )
2019
11
Management and prognostic markers in patients with autoimmune encephalitis requiring ICU treatment. ( 30568992 )
2019
12
Prognosticating autoimmune encephalitis: A systematic review. ( 30595145 )
2019
13
Autoimmune encephalitis: A costly condition. ( 30674590 )
2019
14
Development of the clinical assessment scale in autoimmune encephalitis. ( 30675918 )
2019
15
Diagnosing autoimmune encephalitis based on clinical features and autoantibody findings. ( 30676128 )
2019
16
Pembrolizumab-induced autoimmune encephalitis in a patient with advanced non-small cell lung cancer: A case report. ( 30680206 )
2019
17
Antibody-LGI 1 autoimmune encephalitis manifesting as rapidly progressive dementia and hyponatremia: a case report and literature review. ( 30732585 )
2019
18
Sleep disturbances are common in patients with autoimmune encephalitis. ( 30741377 )
2019
19
Autoimmune Encephalitis Secondary to Melanoma. ( 30743260 )
2019
20
Fulminant Subacute Sclerosing Panencephalitis Mimicking Autoimmune Encephalitis. ( 30762710 )
2019
21
A transdiagnostic pattern of psychiatric symptoms in autoimmune encephalitis. ( 30765327 )
2019
22
A Rasmussen encephalitis, autoimmune encephalitis, and mitochondrial disease mimicker: expanding the DNM1L-associated intractable epilepsy and encephalopathy phenotype. ( 30767894 )
2019
23
A clinical TRIAD for early suspicion of autoimmune encephalitis as a possibility in patients presenting with progressive cognitive decline. ( 30836326 )
2019
24
Editorial: Emerging Challenges in the Diagnosis and Treatment of Autoimmune Encephalitis. ( 30858821 )
2019
25
Autoimmune encephalitis: frequency and prognosis. ( 30877379 )
2019
26
Immune-Checkpoint-Inhibitor-Induced Severe Autoimmune Encephalitis Treated by Steroid and Intravenous Immunoglobulin. ( 30877699 )
2019
27
An Assay to Determine Mechanisms of Rapid Autoantibody-Induced Neurotransmitter Receptor Endocytosis and Vesicular Trafficking in Autoimmune Encephalitis. ( 30881339 )
2019
28
Atypical Autoimmune Encephalitis With Neuropil Antibodies Against a Yet Unknown Epitope. ( 30891000 )
2019
29
Commentary: Detection Methods for Autoantibodies in Suspected Autoimmune Encephalitis. ( 30915021 )
2019
30
Epilepsy Emergencies: Status Epilepticus, Acute Repetitive Seizures, and Autoimmune Encephalitis. ( 30921018 )
2019
31
In vitro neuronal network activity as a new functional diagnostic system to detect effects of Cerebrospinal fluid from autoimmune encephalitis patients. ( 30944364 )
2019
32
Clinical characteristics and factors associated with short-term prognosis in adult patients with autoimmune encephalitis of non-neoplastic etiology. ( 30955116 )
2019
33
Recognizing autoimmune encephalitis as a cause of seizures: Treating cause and not effect. ( 30979858 )
2019
34
Editorial: Recent Advances in Psychiatry From Psycho-Neuro-Immunology Research: Autoimmune Encephalitis, Autoimmune Encephalopathy, and Mild Encephalitis. ( 31001151 )
2019
35
Clinical Presentation and Outcome in Autoimmune Encephalitis Associated With N-Type Voltage-Gated Calcium Channels in Children. ( 31014180 )
2019
36
Rare case of Hashimoto's encephalitis mimicking grief reaction. ( 30642847 )
2019
37
Nivolumab-Induced Autoimmune Encephalitis in Two Patients with Lung Adenocarcinoma. ( 30073101 )
2018
38
Evaluation and Management of Autoimmune Encephalitis: A Clinical Overview for the Practicing Child Psychiatrist. ( 29157501 )
2018
39
Febrile infection-related epilepsy syndrome (FIRES) with super-refractory status epilepticus revealing autoimmune encephalitis due to GABAAR antibodies. ( 29203057 )
2018
40
Mechanisms of Caspr2 antibodies in autoimmune encephalitis and neuromyotonia. ( 29244234 )
2018
41
Autoimmune encephalitis with GABAA receptor antibodies in a 10-year-old girl. ( 29245105 )
2018
42
Post-Herpetic Autoimmune Encephalitis. ( 29260374 )
2018
43
Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. ( 29293273 )
2018
44
High albumin level is a predictor of favorable response to immunotherapy in autoimmune encephalitis. ( 29343812 )
2018
45
Autoimmune encephalitis associated with glutamic acid decarboxylase antibodies: a case series. ( 29349679 )
2018
46
Autoimmune encephalitis: a review of diagnosis and treatment. ( 29364393 )
2018
47
Glioblastoma as differential diagnosis of autoimmune encephalitis. ( 29383516 )
2018
48
Treatment strategies for autoimmune encephalitis. ( 29399043 )
2018
49
Autoimmune Encephalitis With Multiple Autoantibodies: A Diagnostic and Therapeutic Challenge. ( 29494437 )
2018
50
Autoimmune encephalitis positive for both anti-γ-aminobutyric acid B receptor and anticollapsin response-mediator protein 5 antibodies: A case report. ( 29504979 )
2018

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 CNR1 CNTNAP2 HCRT
2
Show member pathways
10.64 CNR1 S1PR1
3 10.07 CNR1 FAAH

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 CNTNAP2 HCRT LGI1 TRPV1
2 intrinsic component of plasma membrane GO:0031226 8.62 S1PR1 TRPV1

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 HCRT TRPV1
2 regulation of insulin secretion GO:0050796 9.32 CNR1 IFNG
3 sensory perception of pain GO:0019233 9.26 CNR1 TRPV1
4 positive regulation of nitric oxide biosynthetic process GO:0045429 9.16 IFNG TRPV1
5 temperature homeostasis GO:0001659 8.96 HCRT TRPV1
6 transmission of nerve impulse GO:0019226 8.62 CNTNAP2 S1PR1

Sources for Autoimmune Encephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....