Rare Pulmonology News
Disease Profile
Herpes simplex encephalitis
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
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.
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.
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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.
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.
Not applicable
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;
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 | |
| 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 |
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 |
0002349 | |
| Focal impaired awareness seizure | 0002384 | |
| Functional motor deficit | 0004302 | |
| Hyperreflexia |
Increased reflexes
|
0001347 |
| Hypoglycorrhachia |
Low glucose levels in cerebral spinal fluid
|
0011972 |
|
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.
- Capsaicin (Brand name: Qutenza) Manufactured by NeurogesX, Inc.
FDA-approved indication: Management of neuropathic pain associated with postherpetic neuralgia
National Library of Medicine Drug Information Portal
Related diseases
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
|
Conditions with similar signs and symptoms from Orphanet
|
|---|
|
Differential diagnosis includes: other viral causes of encephalitis, (varicella zoster virus, enteroviruses etc); bacterial infections, including ricketsial disease, mycoplasma pneumonia, chlamydophila infections; autoimmune diseases, such as paraneoplastic limbic encephalitis, acute disseminated encephalomyelitis, Rasmussen subacute encephalitis; and other causes including space occupying lesion and non-infectious encephalopathy.
Visit the Orphanet disease page for more information.
|
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
-
Encephalitis Society
32 Castlegate
Malton, North Yorkshire YO17 7DT
United Kingdom
Telephone: +44 (0)1653 699599
E-mail: support@encephalitis.info
Website: https://www.encephalitis.info/
Social media: Twitter: @encephalitis Facebook: /encephalitissociety -
Headway the brain injury association
Bradbury House
190 Bagnall Road
Old Basford
Nottingham
NG6 8SF
United Kingdom
Telephone: 0808 800 2244
Fax: 0115 958 4446
E-mail: helpline@headway.org.uk
Website: https://www.headway.org.uk/
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
- The Mayo Clinic Web site provides further information on Herpes simplex encephalitis.
- The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
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
- Anderson WE. Herpes Simplex Encephalitis. Medscape Reference. April 2014; https://emedicine.medscape.com/article/1165183-overview.
- Klein RS. Herpes simplex virus type 1 encephalitis. UpToDate. August 2014; https://www.uptodate.com/contents/herpes-simplex-virus-type-1-encephalitis.