Herpes simplex virus encephalitis

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Herpes simplex virus encephalitis

ORPHA:1930Disease

Also called HSE · HSV encephalitis · HSVE · Herpes simplex meningo-encephalitis · Herpes simplex neuroinvasion · Herpetic encephalitis

What it is

A rare disorder caused by infection of the central nervous system by Herpes simplex virus (HSV) that could have a devastating clinical course and a potentially fatal outcome particularly with delay or lack of treatment. This disorder often involves the frontal and temporal lobes, usually asymmetrically, resulting in personality changes, cognitive impairment, aphasia, seizures, and focal weakness.

Key facts

Prevalence
1-9 / 1 000 000 (annual incidence)
Age of onset
All ages
Inheritance
Multigenic/multifactorial, Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

TBK1Major susceptibility factor
TICAM1Major susceptibility factor
TLR3Major susceptibility factor
TRAF3Major susceptibility factor
UNC93B1Major susceptibility factor

ICD-10 codes

B00.4+ICD-10 names this disease exactly
G05.1*filed under a broader ICD-10 category — shared with 1 other rare disease

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 6649MEDDRA 10019953MESH D020803MONDO 0012521MONDO 12521OMIM 610551OMIM 613002OMIM 614849OMIM 614850OMIM 616532OMIM 617900UMLS C0276226

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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