Rare Neurology News

Disease Profile

Herpes simplex encephalitis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

All ages

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ICD-10

B00.4+ G05.1*

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Herpetic encephalitis; Herpes simplex meningo-encephalitis; Herpes simplex neuroinvasion;

Categories

Congenital and Genetic Diseases; Immune System Diseases; Nervous System Diseases;

Summary

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.[1][2]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
CSF lymphocytic pleiocytosis
0200149
EEG abnormality
0002353
30%-79% of people have these symptoms
Fatigue
Tired
Tiredness

[ more ]

0012378
Fever
0001945
Headache
Headaches
0002315
Hyponatremia
Low blood sodium levels
0002902
Increased CSF protein
0002922
Leukocytosis
Elevated white blood count
High white blood count
Increased blood leukocyte number

[ more ]

0001974
Loss of consciousness
Passing out
0007185
Nausea and vomiting
0002017
Neurological speech impairment
Speech disorder
Speech impairment
Speech impediment

[ more ]

0002167
Neutrophilia
Increased blood neutrophil counts
0011897
Nuchal rigidity
0031179
Respiratory failure requiring assisted ventilation
0004887
5%-29% of people have these symptoms
Alcoholism
0030955
Cerebral edema
Swelling of brain
0002181
Chills
0025143
Coma
0001259
Elevated C-reactive protein level
0011227
Excessive daytime somnolence
Excessive daytime sleepiness
More than typical sleepiness during day

[ more ]

0001262
Focal aware seizure
0002349
Focal impaired awareness seizure
0002384
Functional motor deficit
0004302
Hyperreflexia
Increased reflexes
0001347
Hypoglycorrhachia
Low glucose levels in cerebral spinal fluid
0011972
Immunodeficiency
Decreased immune function
0002721
Status epilepticus
Repeated seizures without recovery between them
0002133

Treatment

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Herpes simplex encephalitis. Click on the link to view a sample search on this topic.

        References

        1. Anderson WE. Herpes Simplex Encephalitis. Medscape Reference. April 2014; https://emedicine.medscape.com/article/1165183-overview.
        2. Klein RS. Herpes simplex virus type 1 encephalitis. UpToDate. August 2014; https://www.uptodate.com/contents/herpes-simplex-virus-type-1-encephalitis.