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230407006: Hemiconvulsion-hemiplegia-epilepsy syndrome (disorder)


Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jan 2002. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module
345260011 Hemiconvulsion-hemiplegia-epilepsy syndrome en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
345261010 H-H-E syndrome en Synonym (core metadata concept) Inactive Entire term case sensitive (core metadata concept) SNOMED CT core
345262015 Hemiplegia-hemiconvulsion-epilepsy syndrome en Synonym (core metadata concept) Inactive Entire term case insensitive (core metadata concept) SNOMED CT core
618209011 Hemiplegia-hemiconvulsion-epilepsy syndrome (disorder) en Fully specified name Inactive Entire term case insensitive (core metadata concept) SNOMED CT core
5373695018 Hemiconvulsion-hemiplegia-epilepsy syndrome (disorder) en Fully specified name Active Entire term case insensitive (core metadata concept) SNOMED CT core
5373696017 HHE - hemiplegia-hemiconvulsion-epilepsy syndrome en Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT core
5464933019 A rare type of epilepsy that presents with seizures prior to 4 years of age with an episode of febrile, hemiclonic status epilepticus, which is immediately followed by permanent hemiparesis (acute phase). Within 3 years of status epilepticus, drug-resistant unilateral focal seizures develop (chronic phase). Previous development and neurological examination are normal. Immediately after status, patients develop hemiparesis, that usually leaves a permanent motor deficit. Aphasia may present acutely and typically resolves. Many individuals are left with various degrees of intellectual disability. EEG shows slowing of background activity over the affected hemisphere. Focal or multifocal epileptiform abnormalities over the affected hemisphere are evident after seizure onset. MRI shows unilateral edematous swelling of the affected hemisphere in the acute phase, followed by hemispheric atrophy at follow-up. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core
5465533016 A rare type of epilepsy that presents with seizures prior to 4 years of age with an episode of febrile, hemiclonic status epilepticus, which is immediately followed by permanent hemiparesis (acute phase). Within 3 years of status epilepticus, drug-resistant unilateral focal seizures develop (chronic phase). Previous development and neurological examination are normal. Immediately after status, patients develop hemiparesis, that usually leaves a permanent motor deficit. Aphasia may present acutely and typically resolves. Many individuals are left with various degrees of intellectual disability. EEG shows slowing of background activity over the affected hemisphere. Focal or multifocal epileptiform abnormalities over the affected hemisphere are evident after seizure onset. MRI shows unilateral oedematous swelling of the affected hemisphere in the acute phase, followed by hemispheric atrophy at follow-up. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core


0 descendants.

Expanded Value Set


Outbound Relationships Type Target Active Characteristic Refinability Group Values
Hemiconvulsion-hemiplegia-epilepsy syndrome Is a Focal epilepsy true Inferred relationship Some
Hemiconvulsion-hemiplegia-epilepsy syndrome Finding site Cerebrum false Inferred relationship Some 1
Hemiconvulsion-hemiplegia-epilepsy syndrome Has definitional manifestation Seizure false Inferred relationship Some
Hemiconvulsion-hemiplegia-epilepsy syndrome Is a Epilepsy syndrome with progressive neurological deterioration (disorder) true Inferred relationship Some
Hemiconvulsion-hemiplegia-epilepsy syndrome Finding site Brain structure true Inferred relationship Some 1

Inbound Relationships Type Active Source Characteristic Refinability Group

Reference Sets

Component annotation with string value reference set (foundation metadata concept)

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