HSE
MCID: HRP009
MIFTS: 50

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 54
Herpes Simplex Meningo-Encephalitis 54 60
Herpes Simplex Virus Encephalitis 54 60
Herpes Simplex Neuroinvasion 54 60
Herpetic Encephalitis 54 60
Hsv Encephalitis 54 60
Hsve 54 60
Hse 54 60
Herpes Simplex Virus 1 Encephalitis 74
Herpes Encephalitis 74

Characteristics:

Orphanet epidemiological data:

60
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 35 B00.4+ G05.1*
UMLS via Orphanet 75 C0019385 C0276226
Orphanet 60 ORPHA1930

Summaries for Herpes Simplex Encephalitis

NIH Rare Diseases : 54 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 145)
# Related Disease Score Top Affiliating Genes
1 encephalitis 31.5 IRF3 TICAM1 TLR3 TRAF3 UNC93B1
2 herpes simplex 31.1 IRF3 TBK1 TICAM1 TLR3 TRAF3 UNC93B1
3 rabies 30.2 IRF3 TLR3
4 tick-borne encephalitis 30.1 CXCL11 TLR3
5 encephalopathy, acute, infection-induced 1 12.4
6 encephalopathy, acute, infection-induced 2 12.3
7 hemorrhagic shock and encephalopathy syndrome 12.1
8 encephalopathy, acute, infection-induced 5 11.8
9 encephalopathy, acute, infection-induced 6 11.8
10 encephalopathy, acute, infection-induced 7 11.8
11 encephalopathy, acute, infection-induced 8 11.6
12 febrile infection-related epilepsy syndrome 11.4
13 immunodeficiency 31a 11.4
14 neonatal herpes 10.5
15 kluver-bucy syndrome 10.3
16 multiple sclerosis 10.2
17 japanese encephalitis 10.2
18 retinitis 10.2
19 meningitis 10.2
20 glioblastoma 10.2
21 neurosyphilis 10.2
22 alzheimer disease 10.2
23 status epilepticus 10.2
24 epilepsy 10.2
25 encephalopathy 10.2
26 amnestic disorder 10.2
27 immunodeficiency, x-linked, with magnesium defect, epstein-barr virus infection, and neoplasia 10.2 TLR3 UNC93B1
28 glioma susceptibility 1 10.1
29 mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes 10.1
30 cerebral hemorrhage 10.1
31 aphasia 10.1
32 lymphoma 10.1
33 mumps 10.1
34 glioblastoma multiforme 10.1
35 astrocytoma 10.1
36 acute disseminated encephalomyelitis 10.1
37 autoimmune encephalitis 10.1
38 glioma 10.1
39 retinitis pigmentosa 33 10.0 IRF3 TBK1
40 thrombophilia due to thrombin defect 10.0
41 myxedema 10.0
42 stroke, ischemic 10.0
43 common variable immunodeficiency 10.0
44 temporal lobe epilepsy 10.0
45 thrombosis 10.0
46 bell's palsy 10.0
47 acoustic neuroma 10.0
48 cerebral degeneration 10.0
49 neuroma 10.0
50 agnosia 10.0

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:

47
# 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 22)
# 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 valacyclovir Phase 3
6 Antiviral Agents Phase 3
7 Anti-Infective Agents Phase 3
8 Gastrointestinal Agents Phase 3
9 Antidiarrheals Phase 3
10 Antisecretory factor Phase 3
11 Peripheral Nervous System Agents Phase 3
12 HIV Protease Inhibitors Phase 3
13 Anti-Inflammatory Agents Phase 3
14 Autonomic Agents Phase 3
15 BB 1101 Phase 3
16 Hormones Phase 3
17
protease inhibitors Phase 3
18 Antiemetics Phase 3
19 Antineoplastic Agents, Hormonal Phase 3
20 Hormone Antagonists Phase 3
21 glucocorticoids Phase 3
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Long Term Treatment of Herpes Simplex Encephalitis (HSE) With Valacyclovir Completed NCT00031486 Phase 3 Valacyclovir;Placebo
2 Effect of Antisecretory Factor, Given as a Food Supplement to Adult Patients With Severe Traumatic Brain Injury Recruiting NCT03339505 Phase 3
3 Dexamethasone in Herpes Simplex Virus Encephalitis Not yet recruiting NCT03084783 Phase 3 Dexamethasone

Search NIH Clinical Center for Herpes Simplex Encephalitis

Inferred drug relations via UMLS 74 / NDF-RT 52 :


Genetic Tests for Herpes Simplex Encephalitis

Anatomical Context for Herpes Simplex Encephalitis

MalaCards organs/tissues related to Herpes Simplex Encephalitis:

42
Brain, Temporal Lobe, Testes, T Cells, Cortex, Eye, Heart

Publications for Herpes Simplex Encephalitis

Articles related to Herpes Simplex Encephalitis:

(show top 50) (show all 810)
# Title Authors Year
1
Acyclovir Unresponsive Herpes Simplex Encephalitis in a child successfully treated with the addition of Foscarnet: Case report. ( 30652455 )
2019
2
Rel-Dependent Immune and Central Nervous System Mechanisms Control Viral Replication and Inflammation during Mouse Herpes Simplex Encephalitis. ( 30683700 )
2019
3
Mouse model of anti-NMDA receptor post-herpes simplex encephalitis. ( 30697582 )
2019
4
Suspected Herpes Simplex Encephalitis with Low Copy Number DNA. ( 30607771 )
2019
5
Why do herpes simplex encephalitis and semantic dementia show a different pattern of semantic impairment in spite of their main common involvement within the anterior temporal lobes? ( 29573378 )
2018
6
Clinical significance of IgM and IgA class anti-NMDAR antibodies in herpes simplex encephalitis. ( 29698873 )
2018
7
Efficacy of pritelivir and acyclovir in the treatment of herpes simplex virus infections in a mouse model of herpes simplex encephalitis. ( 29113740 )
2018
8
A Rare Case of Concurrent Herpes Simplex Encephalitis and Glioblastoma Multiforme. ( 29492128 )
2018
9
Semantic language deficit developing following herpes simplex encephalitis: reorganization "cannibalising" language centers? ( 29350572 )
2018
10
Herpes simplex encephalitis relapse associated with positive N-methyl-D-aspartate receptor antibodies. ( 29450730 )
2018
11
Acyclovir Sensitivity and Neurovirulence of Herpes Simplex Virus Type 1 with Amino Acid Substitutions in the Viral Thymidine Kinase Gene, Which Were Detected in the Patients with Intractable Herpes Simplex Encephalitis Previously Reported. ( 29848849 )
2018
12
Rapid acquisition of acyclovir resistance in an immunodeficient patient with herpes simplex encephalitis. ( 29249385 )
2018
13
Functional failure of TLR3 and its signaling components contribute to herpes simplex encephalitis. ( 29305044 )
2018
14
Decompressive craniectomy as salvage treatment in herpes simplex encephalitis: Two case reports. ( 29870796 )
2018
15
Acute disseminated encephalomyelitis: A rare autoimmune complication of herpes simplex encephalitis in the adult. ( 30336448 )
2018
16
Autoimmune encephalitis after herpes simplex encephalitis: insights into pathogenesis. ( 30049613 )
2018
17
Frequency, symptoms, risk factors, and outcomes of autoimmune encephalitis after herpes simplex encephalitis: a prospective observational study and retrospective analysis. ( 30049614 )
2018
18
Approach to recurrent Herpes Simplex Encephalitis in children. ( 30805531 )
2018
19
Mechanisms of Blood-Brain Barrier Disruption in Herpes Simplex Encephalitis. ( 30456443 )
2018
20
Evaluation of cholinergic functions in patients with Japanese encephalitis and Herpes simplex encephalitis. ( 30468725 )
2018
21
Herpes simplex encephalitis and pseudotumour cerebri due to ısotretinoin. ( 30504951 )
2018
22
A Fatal Case of Herpes Simplex Encephalitis with Two False-Negative Polymerase Chain Reactions. ( 30283319 )
2018
23
Decompressive craniectomy for herpes simplex encephalitis complicated by frank intracerebral hemorrhage: a case report and review of the literature. ( 30352560 )
2018
24
Increased level of compleasomes in cerebrospinal fluid of patients with herpes simplex encephalitis. ( 30094629 )
2018
25
Insights into the pathogenesis of herpes simplex encephalitis from mouse models. ( 30167845 )
2018
26
MRI Findings Suggestive of Herpes Simplex Encephalitis in Patients with Anti-NMDA Receptor Encephalitis. ( 30213819 )
2018
27
Herpes Simplex Encephalitis. ( 28076016 )
2017
28
Herpes Simplex Encephalitis Complicated by Cerebral Hemorrhage during Acyclovir Therapy. ( 28090058 )
2017
29
MRI diagnosis of herpes simplex encephalitis in an elderly man with nonspecific symptoms. ( 28228902 )
2017
30
Potential risk of developing herpes simplex encephalitis in patients treated with sildenafil following primary exposure to genital herpes. ( 28956417 )
2017
31
Epilepsy surgery for epileptic encephalopathy as a sequela of herpes simplex encephalitis: case report. ( 28452654 )
2017
32
Concomitant neurosyphilis and herpes simplex encephalitis in an immunocompetent patient: a case report. ( 28914386 )
2017
33
Herpes simplex encephalitis with thalamic, brainstem and cerebellar involvement. ( 28627955 )
2017
34
Reply to Galen, "Screening Cerebrospinal Fluid Prior to Herpes Simplex Virus PCR Testing Might Miss Cases of Herpes Simplex Encephalitis". ( 28947510 )
2017
35
Psychosis in a 15-Year-Old Female with Herpes Simplex Encephalitis in a Background of Mannose-Binding Lecithin Deficiency. ( 28261514 )
2017
36
Herpes simplex encephalitis and Alzheimer's disease: Is there a link? ( 28870568 )
2017
37
Incidence and mortality of herpes simplex encephalitis in Denmark: A nationwide registry-based cohort study. ( 27717782 )
2017
38
Early rehabilitation results in a child who developed herpes simplex encephalitis. ( 29270579 )
2017
39
Nosocomial herpes simplex encephalitis: Does it really exist? ( 28209321 )
2017
40
Predominant area of brain lesions in neonates with herpes simplex encephalitis. ( 28726789 )
2017
41
Screening Cerebrospinal Fluid Prior to Herpes Simplex Virus PCR Testing Might Miss Cases of Herpes Simplex Encephalitis. ( 28947509 )
2017
42
Anti-N-methyl-D-aspartate receptor encephalitis during relapse of herpes simplex encephalitis in a young boy: A brief review of literature. ( 28290410 )
2017
43
CXCL11 production in cerebrospinal fluid distinguishes herpes simplex meningitis from herpes simplex encephalitis. ( 28693588 )
2017
44
Intracranial Hemorrhage Complicating Herpes Simplex Encephalitis on Antiviral Therapy: A Case Report and Review of the Literature. ( 29057131 )
2017
45
Herpes Simplex Encephalitis: an Update. ( 28251511 )
2017
46
Pure word deafness due to herpes simplex encephalitis. ( 28017194 )
2017
47
Acyclovir resistant acute herpes simplex encephalitis associated with acute retinal necrosis: A case report and review of the literature. ( 28450685 )
2017
48
T-cell Responses to HSV-1 in Persons Who Have Survived Childhood Herpes Simplex Encephalitis. ( 28471862 )
2017
49
Painful loss of vision after an episode of herpes simplex encephalitis. ( 28994251 )
2017
50
50 Years Ago in The Journal of Pediatrics: Brain Scan in a Patient with Herpes Simplex Encephalitis. ( 28843301 )
2017

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 14)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.69 ELANE IL2RB IRF3 TBK1 TICAM1 TLR3
2
Show member pathways
13.27 IRF3 TBK1 TICAM1 TLR3 TRAF3 UNC93B1
3
Show member pathways
12.83 CXCL11 IRF3 TBK1 TICAM1 TLR3 TRAF3
4
Show member pathways
12.67 IRF3 TBK1 TICAM1 TLR3 TRAF3
5
Show member pathways
12.57 IL2RB IRF3 TBK1 TICAM1 TLR3 TRAF3
6
Show member pathways
12.47 IRF3 TBK1 TICAM1 TLR3 TRAF3
7
Show member pathways
12.3 IRF3 TBK1 TICAM1 TLR3 TRAF3
8
Show member pathways
12.15 IRF3 TBK1 TICAM1 TLR3
9
Show member pathways
12.13 IRF3 TBK1 TICAM1 TLR3
10
Show member pathways
12.11 IL2RB IRF3 TBK1 TICAM1 TLR3 TRAF3
11
Show member pathways
12.01 IRF3 TICAM1 TLR3
12
Show member pathways
11.63 IRF3 TBK1 TICAM1 TLR3 TRAF3
13 11.61 IL2RB IRF3 TBK1 TICAM1 TLR3 TRAF3
14 10.9 TICAM1 TLR3

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 TBK1 TICAM1 TLR3
2 endosome GO:0005768 8.92 IL2RB TLR3 TRAF3 UNC93B1

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

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 APOE CXCL11 ELANE

Sources for Herpes Simplex 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
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