HSE
MCID: HRP009
MIFTS: 47

Herpes Simplex Encephalitis (HSE)

Categories: Blood diseases, Infectious diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Herpes Simplex Encephalitis

MalaCards integrated aliases for Herpes Simplex Encephalitis:

Name: Herpes Simplex Encephalitis 53
Herpes Simplex Meningo-Encephalitis 53 59
Herpes Simplex Virus Encephalitis 53 59
Herpes Simplex Neuroinvasion 53 59
Herpetic Encephalitis 53 59
Hsv Encephalitis 53 59
Hsve 53 59
Hse 53 59
Herpes Simplex Virus 1 Encephalitis 72
Herpes Encephalitis 72

Characteristics:

Orphanet epidemiological data:

59
herpes simplex virus encephalitis
Inheritance: Multigenic/multifactorial,Not applicable; Prevalence: 1-9/1000000 (Worldwide),1-9/1000000 (United Kingdom),1-9/1000000 (United States); Age of onset: All ages; Age of death: any age;

Classifications:



External Ids:

ICD10 via Orphanet 34 B00.4+ G05.1*
UMLS via Orphanet 73 C0019385 C0276226
Orphanet 59 ORPHA1930
UMLS 72 C0276226 C3648852

Summaries for Herpes Simplex Encephalitis

NIH Rare Diseases : 53 Herpes simplex encephalitis is a rare neurological condition that is characterized by inflammation of the brain (encephalitis). People affected by this condition may experience a headache and fever for up to 5 days, followed by personality and behavioral changes; seizures; hallucinations; and altered levels of consciousness. Without early diagnosis and treatment, severe brain damage or even death may occur. Herpes simplex encephalitis is caused by a virus called the herpes simplex virus. Most cases are associated with herpes simplex virus type I (the cause of cold sores or fever blisters), although rare cases can be caused by herpes simplex virus type II (genital herpes). It is poorly understood why some people who are infected with herpes simplex virus develop herpes simplex encephalitis while others do not. Changes (mutations) in genes such as TLR3 and TRAF3 have been observed suggesting there may be a genetic component in some cases. Treatment consists of antiviral therapy.

MalaCards based summary : Herpes Simplex Encephalitis, also known as herpes simplex meningo-encephalitis, is related to encephalitis and herpes simplex. An important gene associated with Herpes Simplex Encephalitis is UNC93B1 (Unc-93 Homolog B1, TLR Signaling Regulator), and among its related pathways/superpathways are Innate Immune System and Activated TLR4 signalling. The drugs Acyclovir and Loperamide have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and testes, and related phenotypes are hematopoietic system and homeostasis/metabolism

Related Diseases for Herpes Simplex Encephalitis

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

(show top 50) (show all 311)
# Related Disease Score Top Affiliating Genes
1 encephalitis 31.2 UNC93B1 TRAF3 TLR3 TICAM1 IRF3
2 herpes simplex 30.6 UNC93B1 TRAF3 TLR3 TICAM1 TBK1 IRF3
3 rabies 30.3 TLR3 IRF3
4 tick-borne encephalitis 30.1 TLR3 CXCL11
5 viral infectious disease 29.7 TLR3 TBK1 IRF3
6 encephalopathy, acute, infection-induced 2 12.6
7 encephalopathy, acute, infection-induced 1 12.6
8 encephalopathy, acute, infection-induced 5 12.3
9 hemorrhagic shock and encephalopathy syndrome 12.2
10 encephalopathy, acute, infection-induced 6 12.2
11 encephalopathy, acute, infection-induced 7 12.2
12 encephalopathy, acute, infection-induced 8 12.1
13 immunodeficiency 31a 11.8
14 febrile infection-related epilepsy syndrome 11.6
15 viral encephalitis 10.8
16 ocular motor apraxia 10.7
17 neonatal herpes 10.7
18 congenital herpes simplex virus infection 10.7
19 aphasia 10.6
20 kluver-bucy syndrome 10.6
21 meningoencephalitis 10.6
22 amnestic disorder 10.6
23 encephalopathy 10.6
24 status epilepticus 10.5
25 neuroretinitis 10.5
26 retinitis 10.5
27 japanese encephalitis 10.4
28 movement disease 10.4
29 glioma 10.4
30 glial tumor 10.4
31 acute necrotizing encephalitis 10.4
32 visual epilepsy 10.4
33 glioblastoma multiforme 10.4
34 brain edema 10.4
35 meningitis 10.4
36 glioblastoma 10.4
37 headache 10.4
38 seizure disorder 10.4
39 asymptomatic neurosyphilis 10.3
40 cerebritis 10.3
41 chickenpox 10.3
42 neurosyphilis 10.3
43 tuberculous meningitis 10.3
44 rare surgical neurologic disease 10.3
45 immunodeficiency, x-linked, with magnesium defect, epstein-barr virus infection, and neoplasia 10.3 UNC93B1 TLR3
46 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.3
47 chorea, childhood-onset, with psychomotor retardation 10.3
48 choreatic disease 10.3
49 papilledema 10.3
50 encephalomalacia 10.3

Graphical network of the top 20 diseases related to Herpes Simplex Encephalitis:



Diseases related to Herpes Simplex Encephalitis

Symptoms & Phenotypes for Herpes Simplex Encephalitis

MGI Mouse Phenotypes related to Herpes Simplex Encephalitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.92 APOE ELANE IL2RB TBK1 TICAM1 TLR3
2 homeostasis/metabolism MP:0005376 9.86 APOE ELANE IRF3 TBK1 TICAM1 TLR3
3 immune system MP:0005387 9.81 APOE ELANE IL2RB IRF3 TBK1 TICAM1
4 liver/biliary system MP:0005370 9.43 APOE IL2RB TBK1 TICAM1 TRAF3 UNC93B1
5 mortality/aging MP:0010768 9.23 APOE ELANE IL2RB TBK1 TICAM1 TLR3

Drugs & Therapeutics for Herpes Simplex Encephalitis

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

(show all 24)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Acyclovir Approved Phase 3 59277-89-3 2022
2
Loperamide Approved Phase 3 53179-11-6 3955
3
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
4
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
5 Anti-Infective Agents Phase 3
6 valacyclovir Phase 3
7 Antiviral Agents Phase 3
8 Antisecretory factor Phase 3
9 Antidiarrheals Phase 3
10 Gastrointestinal Agents Phase 3
11 Anti-Inflammatory Agents Phase 3
12 Antiemetics Phase 3
13 HIV Protease Inhibitors Phase 3
14 Hormones Phase 3
15 glucocorticoids Phase 3
16
protease inhibitors Phase 3
17 Antineoplastic Agents, Hormonal Phase 3
18 Hormone Antagonists Phase 3
19 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
20 Peripheral Nervous System Agents Phase 3
21 Autonomic Agents Phase 3
22 BB 1101 Phase 3
23 Antibodies
24 Immunoglobulins

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase III Double-Blind, Placebo-Controlled Trial of Long Term Therapy of Herpes Simplex Encephalitis (HSE): An Evaluation of Valacyclovir (CASG-204) Completed NCT00031486 Phase 3 Valacyclovir;Placebo
2 Salovum (Antisecretory Factor) in Patients With Severe Traumatic Brain Injury Recruiting NCT03339505 Phase 3
3 Dexamethasone in Herpes Simplex Virus Encephalitis Open Label Randomized Controlled Trial With an Observer-blinded Evaluation at 6 Months Not yet recruiting NCT03084783 Phase 3 Dexamethasone
4 Evaluation of Specific PET Radioligand Binding To Translocator Protein Completed NCT02181582 Phase 1 Emapunil
5 The Association Between Herpes Simplex Viruses (Type One and Two) and Autism Spectrum Disorders Unknown status NCT03180112
6 The Natural History of Severe Viral Infections and Characterization of Immune Defects Recruiting NCT01011712
7 Swiss Trial of Decompressive Craniectomy Versus Best Medical Treatment of Spontaneous Supratentorial Intracerebral Hemorrhage (SWITCH): a Randomized Controlled Trial Recruiting NCT02258919
8 Management of Acute Disseminated Encephalomyelitis in Neurology Unit of Assiut University Children Hospital Not yet recruiting NCT03284801

Search NIH Clinical Center for Herpes Simplex Encephalitis

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Acyclovir
Acyclovir Sodium
Cidofovir

Genetic Tests for Herpes Simplex Encephalitis

Anatomical Context for Herpes Simplex Encephalitis

MalaCards organs/tissues related to Herpes Simplex Encephalitis:

41
Brain, Temporal Lobe, Testes, T Cells, Cortex, Liver, Eye

Publications for Herpes Simplex Encephalitis

Articles related to Herpes Simplex Encephalitis:

(show top 50) (show all 1602)
# Title Authors PMID Year
1
Mutations in the TLR3 signaling pathway and beyond in adult patients with herpes simplex encephalitis. 38 71
26513235 2015
2
Functional IRF3 deficiency in a patient with herpes simplex encephalitis. 38 71
26216125 2015
3
TLR3 deficiency in herpes simplex encephalitis: high allelic heterogeneity and recurrence risk. 38 71
25339207 2014
4
Herpes simplex encephalitis in children with autosomal recessive and dominant TRIF deficiency. 38 71
22105173 2011
5
Human TRAF3 adaptor molecule deficiency leads to impaired Toll-like receptor 3 response and susceptibility to herpes simplex encephalitis. 38 71
20832341 2010
6
TLR3 deficiency in patients with herpes simplex encephalitis. 38 71
17872438 2007
7
Herpes simplex virus encephalitis in a patient with complete TLR3 deficiency: TLR3 is otherwise redundant in protective immunity. 71
21911422 2011
8
Herpes simplex virus encephalitis in human UNC-93B deficiency. 71
16973841 2006
9
Herpes Simplex Encephalitis Initially Erroneously Diagnosed as Glioma of the Cerebellum: Case Report and Literature Review. 38
31254700 2019
10
Herpes Simplex Encephalitis Shortly After Surgery for a Secondary Glioblastoma: A Case Report and Review of the Literature. 38
31150854 2019
11
Functional outcomes in adult patients with herpes simplex encephalitis admitted to the ICU: a multicenter cohort study. 38
31292686 2019
12
Hyperammonemia in a case of herpes simplex and anti-N-methyl-d-aspartate receptor encephalitis. 38
30961960 2019
13
Herpes simplex reactivation following neurosurgery: case report and review of the literature. 38
31370030 2019
14
An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. 38
31326280 2019
15
Cerebral venous thrombosis: a rare complication of herpes simplex encephalitis. 38
31278537 2019
16
Bilateral independent periodic lateralised epileptiform discharges at presentation followed by rapid recovery: novel observations from a case of Epstein-Barr virus encephalitis. 38
31326904 2019
17
Acute retinal necrosis following herpes simplex encephalitis: a nationwide survey in Japan. 38
31054049 2019
18
Outcomes with acyclovir treatment in herpes simplex encephalitis after surgery for solid CNS tumors: a case report and systematic review of the literature. 38
31386089 2019
19
Severe presentation of antibody-negative, postinfectious steroid-responsive encephalitis and atonic bladder after herpes simplex encephalitis. 38
31337628 2019
20
[Recent progress in autoimmune encephalitis and its related disorders]. 38
31341132 2019
21
Correction to: Functional outcomes in adult patients with herpes simplex encephalitis admitted to the ICU: a multicenter cohort study. 38
31346675 2019
22
The Role of Physical Examinations in Psychiatry as Illustrated in a Case of Neuroleptic Malignant Syndrome Versus Viral Encephalitis: A Case Report and Literature Review. 38
31410323 2019
23
A case of fulminant Epstein-Barr virus encephalitis in an immune-competent adult. 38
30671776 2019
24
Mechanisms of Blood-Brain Barrier Disruption in Herpes Simplex Encephalitis. 38
30456443 2019
25
C1Q/TNF-related protein 4 expression correlates with herpes simplex encephalitis progression. 38
31317005 2019
26
Immune-mediated encephalitis for the infectious disease specialist. 38
31021956 2019
27
An Adult Case of Herpes Simplex Virus-associated Granulomatous Encephalitis. 38
30626834 2019
28
Bacteroides fragilis polysaccharide A induces IL-10 secreting B and T cells that prevent viral encephalitis. 38
31089128 2019
29
Amentoflavone Inhibits HSV-1 and ACV-Resistant Strain Infection by Suppressing Viral Early Infection. 38
31121928 2019
30
Acute retinal necrosis in a patient with remote severe herpes simplex encephalitis. 38
31138593 2019
31
UNC93B1 promotes tumoral growth by controlling the secretion level of granulocyte macrophage colony-stimulating factor in human oral cancer. 38
30935694 2019
32
Proteomics analysis of HSV-1-induced alterations in mouse brain microvascular endothelial cells. 38
31144288 2019
33
Efficacy of acyclovir for herpes simplex encephalitis: A protocol for a systematic review of randomized controlled trial. 38
30985731 2019
34
Suspected Herpes Simplex Encephalitis with Low Copy Number DNA. 38
30607771 2019
35
Mouse model of anti-NMDA receptor post-herpes simplex encephalitis. 38
30697582 2019
36
Rel-Dependent Immune and Central Nervous System Mechanisms Control Viral Replication and Inflammation during Mouse Herpes Simplex Encephalitis. 38
30683700 2019
37
Evaluation of cholinergic functions in patients with Japanese encephalitis and Herpes simplex encephalitis. 38
30468725 2019
38
[Selected viral neuroinfections in children]. 38
30830896 2019
39
Acyclovir Unresponsive Herpes Simplex Encephalitis in a child successfully treated with the addition of Foscarnet: Case report. 38
30652455 2019
40
Autoimmune encephalitis: A costly condition. 38
30674590 2019
41
HSV-Encephalitis Reactivation after Cervical Spine Surgery. 38
31093411 2019
42
Cerebral Venous Thrombosis as Rare Presentation of Herpes Simplex Virus Encephalitis. 38
30800483 2019
43
Anterior Thalamic Stimulation Induced Relapsing Encephalitis. 38
31055582 2019
44
Corilagin Interferes With Toll-Like Receptor 3-Mediated Immune Response in Herpes Simplex Encephalitis. 38
31080403 2019
45
[Dramatic improvement after decompressive craniectomy in a fulminant stroke-like case of herpes simplex encephalitis]. 38
30528146 2019
46
Frequently used bioinformatics tools overestimate the damaging effect of allelic variants. 38
29217828 2019
47
Herpes simplex encephalitis relapse associated with positive N-methyl-D-aspartate receptor antibodies. 38
29450730 2018
48
Herpes simplex encephalitis and pseudotumour cerebri due to ısotretinoin. 38
30504951 2018
49
Increased level of compleasomes in cerebrospinal fluid of patients with herpes simplex encephalitis. 38
30094629 2018
50
Acute disseminated encephalomyelitis: A rare autoimmune complication of herpes simplex encephalitis in the adult. 38
30336448 2018

Variations for Herpes Simplex Encephalitis

Expression for Herpes Simplex Encephalitis

Search GEO for disease gene expression data for Herpes Simplex Encephalitis.

Pathways for Herpes Simplex Encephalitis

Pathways related to Herpes Simplex Encephalitis according to GeneCards Suite gene sharing:

(show all 13)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.67 UNC93B1 TRAF3 TLR3 TICAM1 TBK1 IRF3
2
Show member pathways
13.26 UNC93B1 TRAF3 TLR3 TICAM1 TBK1 IRF3
3
Show member pathways
12.67 TRAF3 TLR3 TICAM1 TBK1 IRF3 CXCL11
4
Show member pathways
12.65 TRAF3 TLR3 TICAM1 TBK1 IRF3
5
Show member pathways
12.47 TRAF3 TLR3 TICAM1 TBK1 IRF3 IL2RB
6
Show member pathways
12.43 TRAF3 TLR3 TICAM1 TBK1 IRF3
7
Show member pathways
12.25 TRAF3 TLR3 TICAM1 TBK1 IRF3
8
Show member pathways
12.11 TLR3 TICAM1 TBK1 IRF3
9
Show member pathways
12.11 TLR3 TICAM1 TBK1 IRF3
10
Show member pathways
12 TLR3 TICAM1 IRF3
11
Show member pathways
11.63 TRAF3 TLR3 TICAM1 TBK1 IRF3
12 11.61 TRAF3 TLR3 TICAM1 TBK1 IRF3 IL2RB
13 10.89 TLR3 TICAM1

GO Terms for Herpes Simplex Encephalitis

Cellular components related to Herpes Simplex Encephalitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endosome membrane GO:0010008 9.13 TLR3 TICAM1 TBK1
2 endosome GO:0005768 8.92 UNC93B1 TRAF3 TLR3 IL2RB

Biological processes related to Herpes Simplex Encephalitis according to GeneCards Suite gene sharing:

(show all 26)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.88 UNC93B1 TRAF3 TLR3 TICAM1 TBK1 IRF3
2 inflammatory response GO:0006954 9.86 TLR3 TICAM1 TBK1 CXCL11
3 innate immune response GO:0045087 9.85 UNC93B1 TRAF3 TLR3 TICAM1 TBK1 IRF3
4 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.8 TLR3 TICAM1 TBK1 IRF3
5 response to lipopolysaccharide GO:0032496 9.77 TICAM1 IRF3 ELANE
6 cellular response to lipopolysaccharide GO:0071222 9.77 TICAM1 IRF3 CXCL11
7 I-kappaB kinase/NF-kappaB signaling GO:0007249 9.69 TLR3 TICAM1 TBK1
8 toll-like receptor signaling pathway GO:0002224 9.65 UNC93B1 TRAF3 TLR3
9 defense response to virus GO:0051607 9.65 UNC93B1 TLR3 TICAM1 TBK1 IRF3
10 apoptotic signaling pathway GO:0097190 9.63 TLR3 TICAM1
11 response to exogenous dsRNA GO:0043330 9.63 TLR3 TICAM1 IRF3
12 positive regulation of interleukin-6 production GO:0032755 9.62 TLR3 TICAM1
13 positive regulation of tumor necrosis factor production GO:0032760 9.62 TLR3 TICAM1
14 lipopolysaccharide-mediated signaling pathway GO:0031663 9.61 TICAM1 IRF3
15 negative regulation of type I interferon production GO:0032480 9.61 TBK1 IRF3
16 positive regulation of interferon-beta production GO:0032728 9.61 TLR3 TBK1 IRF3
17 necroptotic process GO:0070266 9.58 TLR3 TICAM1
18 positive regulation of interferon-alpha production GO:0032727 9.58 TBK1 IRF3
19 regulation of type I interferon production GO:0032479 9.57 TBK1 IRF3
20 MyD88-independent toll-like receptor signaling pathway GO:0002756 9.55 TLR3 TICAM1
21 positive regulation of chemokine biosynthetic process GO:0045080 9.54 TLR3 TICAM1
22 negative regulation of MyD88-independent toll-like receptor signaling pathway GO:0034128 9.52 TLR3 TICAM1
23 toll-like receptor 3 signaling pathway GO:0034138 9.5 UNC93B1 TLR3 TICAM1
24 positive regulation of interferon-beta biosynthetic process GO:0045359 9.33 TLR3 TICAM1 TBK1
25 positive regulation of type I interferon production GO:0032481 9.26 TLR3 TICAM1 TBK1 IRF3
26 TRIF-dependent toll-like receptor signaling pathway GO:0035666 9.02 TRAF3 TLR3 TICAM1 TBK1 IRF3

Molecular functions related to Herpes Simplex Encephalitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 heparin binding GO:0008201 8.8 ELANE CXCL11 APOE

Sources for Herpes Simplex 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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