MCID: HRP009
MIFTS: 50

Herpes Simplex Encephalitis

Categories: Rare diseases, Neuronal diseases, Blood diseases, Infectious 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 73
Herpes Encephalitis 73

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:

Orphanet 59 ORPHA1930
ICD10 via Orphanet 34 B00.4+ G05.1*
UMLS via Orphanet 74 C0276226 C0019385

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 103)
# Related Disease Score Top Affiliating Genes
1 encephalitis 30.4 IRF3 TICAM1 TLR3 TRAF3 UNC93B1
2 herpes simplex 30.4 IRF3 TICAM1 TLR3 TRAF3 UNC93B1
3 rabies 29.7 IRF3 TLR3
4 tick-borne encephalitis 29.3 CXCL11 TLR3
5 encephalopathy, acute, infection-induced 1 12.3
6 encephalopathy, acute, infection-induced 2 12.2
7 encephalopathy, acute, infection-induced 5 12.1
8 encephalopathy, acute, infection-induced 6 12.1
9 encephalopathy, acute, infection-induced 7 12.0
10 hemorrhagic shock and encephalopathy syndrome 11.9
11 encephalopathy, acute, infection-induced 8 11.4
12 febrile infection-related epilepsy syndrome 11.2
13 immunodeficiency 31a 11.2
14 neonatal herpes 10.4
15 retinitis 10.3
16 cerebritis 10.2
17 kluver-bucy syndrome 10.1
18 meningitis 10.1
19 glioblastoma 10.1
20 alzheimer disease 10.1
21 status epilepticus 10.1
22 epilepsy 10.1
23 neurosyphilis 10.1
24 encephalopathy 10.1
25 glioma susceptibility 1 10.0
26 aging 10.0
27 mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes 10.0
28 cerebral hemorrhage 10.0
29 lymphoma 10.0
30 japanese encephalitis 10.0
31 glioblastoma multiforme 10.0
32 movement disease 10.0
33 neuronitis 10.0
34 glioma 10.0
35 retinitis pigmentosa 33 9.9 IRF3 TBK1
36 breast cancer 9.8
37 creutzfeldt-jakob disease 9.8
38 multiple sclerosis 9.8
39 medulloblastoma 9.8
40 trigeminal neuralgia 9.8
41 autism 9.8
42 myeloma, multiple 9.8
43 stroke, ischemic 9.8
44 acute hemorrhagic leukoencephalitis 9.8
45 human immunodeficiency virus type 1 9.8
46 burns 9.8
47 hepatitis 9.8
48 hepatitis b 9.8
49 malignant glioma 9.8
50 neutropenia 9.8

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 TLR3 APOE TRAF3 ELANE UNC93B1 IL2RB
2 homeostasis/metabolism MP:0005376 9.86 TLR3 APOE TRAF3 ELANE UNC93B1 IRF3
3 immune system MP:0005387 9.81 TLR3 APOE TRAF3 ELANE UNC93B1 IL2RB
4 liver/biliary system MP:0005370 9.43 APOE TRAF3 UNC93B1 IL2RB TBK1 TICAM1
5 mortality/aging MP:0010768 9.23 TICAM1 TLR3 APOE TRAF3 ELANE UNC93B1

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 Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
4 valacyclovir Phase 3
5 Anti-Infective Agents Phase 3
6 Antiviral Agents Phase 3
7 Gastrointestinal Agents Phase 3
8 Antidiarrheals Phase 3
9 Antisecretory factor Phase 3
10 Dexamethasone acetate Phase 3 1177-87-3
11 glucocorticoids Phase 3
12
protease inhibitors Phase 3
13 HIV Protease Inhibitors Phase 3
14 Hormone Antagonists Phase 3
15 Hormones Phase 3
16 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
17 Peripheral Nervous System Agents Phase 3
18 Antiemetics Phase 3
19 Anti-Inflammatory Agents Phase 3
20 BB 1101 Phase 3
21 Autonomic Agents Phase 3
22 Antineoplastic Agents, Hormonal 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 73 / NDF-RT 51 :


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, Cortex, T Cells, Eye, Parietal Lobe

Publications for Herpes Simplex Encephalitis

Articles related to Herpes Simplex Encephalitis:

(show top 50) (show all 611)
# Title Authors Year
1
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
2
Clinical significance of IgM and IgA class anti-NMDAR antibodies in herpes simplex encephalitis. ( 29698873 )
2018
3
Efficacy of pritelivir and acyclovir in the treatment of herpes simplex virus infections in a mouse model of herpes simplex encephalitis. ( 29113740 )
2018
4
A Rare Case of Concurrent Herpes Simplex Encephalitis and Glioblastoma Multiforme. ( 29492128 )
2018
5
Semantic language deficit developing following herpes simplex encephalitis: reorganization "cannibalising" language centers? ( 29350572 )
2018
6
Herpes simplex encephalitis relapse associated with positive N-methyl-D-aspartate receptor antibodies. ( 29450730 )
2018
7
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
8
Rapid acquisition of acyclovir resistance in an immunodeficient patient with herpes simplex encephalitis. ( 29249385 )
2018
9
Functional failure of TLR3 and its signaling components contribute to herpes simplex encephalitis. ( 29305044 )
2018
10
Decompressive craniectomy as salvage treatment in herpes simplex encephalitis: Two case reports. ( 29870796 )
2018
11
Herpes Simplex Encephalitis. ( 28076016 )
2017
12
Herpes Simplex Encephalitis Complicated by Cerebral Hemorrhage during Acyclovir Therapy. ( 28090058 )
2017
13
MRI diagnosis of herpes simplex encephalitis in an elderly man with nonspecific symptoms. ( 28228902 )
2017
14
Potential risk of developing herpes simplex encephalitis in patients treated with sildenafil following primary exposure to genital herpes. ( 28956417 )
2017
15
Epilepsy surgery for epileptic encephalopathy as a sequela of herpes simplex encephalitis: case report. ( 28452654 )
2017
16
Concomitant neurosyphilis and herpes simplex encephalitis in an immunocompetent patient: a case report. ( 28914386 )
2017
17
Herpes simplex encephalitis with thalamic, brainstem and cerebellar involvement. ( 28627955 )
2017
18
Psychosis in a 15-Year-Old Female with Herpes Simplex Encephalitis in a Background of Mannose-Binding Lecithin Deficiency. ( 28261514 )
2017
19
Herpes simplex encephalitis and Alzheimer's disease: Is there a link? ( 28870568 )
2017
20
Incidence and mortality of herpes simplex encephalitis in Denmark: A nationwide registry-based cohort study. ( 27717782 )
2017
21
Early rehabilitation results in a child who developed herpes simplex encephalitis. ( 29270579 )
2017
22
Nosocomial herpes simplex encephalitis: Does it really exist? ( 28209321 )
2017
23
Predominant area of brain lesions in neonates with herpes simplex encephalitis. ( 28726789 )
2017
24
Screening Cerebrospinal Fluid Prior to Herpes Simplex Virus PCR Testing Might Miss Cases of Herpes Simplex Encephalitis. ( 28947509 )
2017
25
Anti-N-methyl-D-aspartate receptor encephalitis during relapse of herpes simplex encephalitis in a young boy: A brief review of literature. ( 28290410 )
2017
26
CXCL11 production in cerebrospinal fluid distinguishes herpes simplex meningitis from herpes simplex encephalitis. ( 28693588 )
2017
27
Intracranial Hemorrhage Complicating Herpes Simplex Encephalitis on Antiviral Therapy: A Case Report and Review of the Literature. ( 29057131 )
2017
28
Herpes Simplex Encephalitis: an Update. ( 28251511 )
2017
29
Pure word deafness due to herpes simplex encephalitis. ( 28017194 )
2017
30
Acyclovir resistant acute herpes simplex encephalitis associated with acute retinal necrosis: A case report and review of the literature. ( 28450685 )
2017
31
T-cell Responses to HSV-1 in Persons Who Have Survived Childhood Herpes Simplex Encephalitis. ( 28471862 )
2017
32
Painful loss of vision after an episode of herpes simplex encephalitis. ( 28994251 )
2017
33
50 Years Ago in The Journal of Pediatrics: Brain Scan in a Patient with Herpes Simplex Encephalitis. ( 28843301 )
2017
34
Herpes simplex encephalitis presenting as stroke-like symptoms with atypical MRI findings and lacking cerebrospinal fluid pleocytosis. ( 28637937 )
2017
35
Bilateral Cerebral Hemorrhage in Herpes Simplex Encephalitis: Rare Occurrence. ( 28163530 )
2016
36
Herpes simplex encephalitis initially presented with parietal cortex lesions mimicking acute ischemic stroke: A case report. ( 26797480 )
2016
37
Can we differentiate between herpes simplex encephalitis and Japanese encephalitis? ( 27288787 )
2016
38
Prolonged acyclovir treatment in a child with opercular syndrome related to herpes simplex encephalitis. ( 27452287 )
2016
39
Acute and prolonged complement activation in the central nervous system during herpes simplex encephalitis. ( 27235358 )
2016
40
The effect of captopril on the expression of MMP-9 and the prognosis of neurological function in herpes simplex encephalitis mice. ( 27354147 )
2016
41
Prevalence of Intrathecal Acyclovir Resistant Virus in Herpes Simplex Encephalitis Patients. ( 27171421 )
2016
42
Nosocomial herpes simplex encephalitis: A challenging diagnosis. ( 27686257 )
2016
43
Anti-N-Methyl-D-Aspartate Receptor Antibody Mediated Neurologic Relapse Post Herpes Simplex Encephalitis: A Case Series. ( 27171680 )
2016
44
N-Methyl-D-Aspartate receptor Autoimmunity Affects Cognitive Performance in herpes simplex encephalitis. ( 27497810 )
2016
45
Autoimmune Post-Herpes Simplex Encephalitis. ( 27396274 )
2016
46
Prognostic Value of Initial Standard EEG and MRI in Patients with Herpes Simplex Encephalitis. ( 26833985 )
2016
47
Normocellular CSF in herpes simplex encephalitis. ( 26879928 )
2016
48
Subtle presentation of herpes simplex encephalitis. ( 27510472 )
2016
49
Gnann JW et al (Clin Infect Dis 2015; 61:683-91). Herpes Simplex Encephalitis: Lack of Clinical Benefit of Long-term Valacyclovir Therapy. ( 26703861 )
2016
50
Temporal Lobe Encephalitis Need not Always be Herpes Simplex Encephalitis: Think of Tuberculosis. ( 27437274 )
2016

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 15)
# 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
12.73 CXCL11 IRF3 TBK1 TICAM1 TLR3 TRAF3
4
Show member pathways
12.63 IL2RB IRF3 TBK1 TICAM1 TLR3 TRAF3
5
Show member pathways
12.5 IRF3 TBK1 TICAM1 TLR3 TRAF3
6
Show member pathways
12.42 IL2RB IRF3 TBK1 TICAM1 TLR3 TRAF3
7
Show member pathways
12.28 IRF3 TBK1 TICAM1 TLR3 TRAF3
8
Show member pathways
12.17 IRF3 TBK1 TICAM1 TLR3
9
Show member pathways
12.15 IRF3 TBK1 TICAM1 TLR3
10 12.03 IRF3 TBK1 TRAF3
11
Show member pathways
12 IRF3 TICAM1 TLR3
12 11.95 IL2RB IRF3 TBK1 TICAM1 TLR3 TRAF3
13
Show member pathways
11.83 IRF3 TBK1 TICAM1 TLR3 TRAF3
14 11.14 IRF3 TBK1 TICAM1 TLR3 TRAF3
15 10.92 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 25)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.88 CXCL11 TBK1 TICAM1 TLR3
2 immune system process GO:0002376 9.88 IRF3 TBK1 TICAM1 TLR3 TRAF3 UNC93B1
3 innate immune response GO:0045087 9.85 IRF3 TBK1 TICAM1 TLR3 TRAF3 UNC93B1
4 response to lipopolysaccharide GO:0032496 9.83 CXCL11 ELANE IRF3 TICAM1
5 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.8 IRF3 TBK1 TICAM1 TLR3
6 I-kappaB kinase/NF-kappaB signaling GO:0007249 9.71 TBK1 TICAM1 TLR3
7 toll-like receptor signaling pathway GO:0002224 9.69 TLR3 TRAF3 UNC93B1
8 negative regulation of type I interferon production GO:0032480 9.65 IRF3 TBK1 TRAF3
9 defense response to virus GO:0051607 9.65 IRF3 TBK1 TICAM1 TLR3 UNC93B1
10 response to exogenous dsRNA GO:0043330 9.63 IRF3 TICAM1 TLR3
11 positive regulation of interleukin-6 production GO:0032755 9.62 TICAM1 TLR3
12 positive regulation of tumor necrosis factor production GO:0032760 9.62 TICAM1 TLR3
13 lipopolysaccharide-mediated signaling pathway GO:0031663 9.61 IRF3 TICAM1
14 MyD88-independent toll-like receptor signaling pathway GO:0002756 9.61 TICAM1 TLR3
15 positive regulation of interferon-beta production GO:0032728 9.61 IRF3 TBK1 TLR3
16 regulation of type I interferon production GO:0032479 9.6 IRF3 TBK1
17 necroptotic process GO:0070266 9.59 TICAM1 TLR3
18 positive regulation of interferon-alpha production GO:0032727 9.58 IRF3 TBK1
19 positive regulation of chemokine biosynthetic process GO:0045080 9.57 TICAM1 TLR3
20 negative regulation of MyD88-independent toll-like receptor signaling pathway GO:0034128 9.56 TICAM1 TLR3
21 cellular response to dsRNA GO:0071359 9.55 IRF3 TLR3
22 toll-like receptor 3 signaling pathway GO:0034138 9.5 TICAM1 TLR3 UNC93B1
23 positive regulation of type I interferon production GO:0032481 9.46 IRF3 TBK1 TICAM1 TLR3
24 positive regulation of interferon-beta biosynthetic process GO:0045359 9.13 TBK1 TICAM1 TLR3
25 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 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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