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Start free with EleplanHemiconvulsion-hemiplegia-epilepsy syndrome
ORPHA:86908Disease
Also called IHHS · HHE · HHE syndrome
What it is
A rare childhood-onset epilepsy syndrome characterized by hemiplegia occurring in children (typically under the age of 4) following prolonged focal seizures, often febrile in nature. The condition progresses through three stages: initial prolonged seizures, subsequent hemiplegia (weakness or paralysis on one side of the body), and eventual epilepsy, following a latent period. The condition may be idiopathic or associated with structural, infectious, traumatic, or degenerative brain abnormalities. Patients present with acute unilateral brain swelling, followed by cerebral hemiatrophy. Inflammation, genetic predisposition, and prolonged seizure activity may play a role in the disease development/progression.
Key facts
- Classified as
- Disease
ICD-10 codes
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.
Benefit programs to look at
Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.
Cross-references
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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