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Code System Concept

Code System Concept Code	766044005
Code System Concept Name	Acute encephalopathy with biphasic seizures and late reduced diffusion (disorder)
Code System Preferred Concept Name	Acute encephalopathy with biphasic seizures and late reduced diffusion (disorder)
Concept Status	Published
Concept Status Date	09/01/2024
Code System Name	SNOMED-CT

Concept Relationships
Concept Details

Concept Details

Code System Code	PH_SNOMED-CT
Code System OID	2.16.840.1.113883.6.96
HL7 Table 0396 Code	SCT
Superseded By Concept
SDO Concept Status	Active
SDO Concept Status Date	07/31/2018
SDO Date Created	07/31/2018
SDO Date Revised	07/31/2018
Description	Rare childhood-onset epilepsy syndrome associated with infection and a biphasic clinical course. The initial symptom is a prolonged febrile seizure on day 1 (the first phase). Afterwards, patients have variable levels of consciousness from normal to coma. Irrespective of the consciousness levels, magnetic resonance imaging (MRI) during the first 2 days shows no abnormality. During the second phase (usually days 4 - 6), patients show a cluster of seizures and deterioration of consciousness. Diffusion-weighted images (DWI) on MRI reveal the brain lesions with reduced diffusion predominantly in the subcortical white matter. After the second acute phase, consciousness levels improve with the emerging focal neurological signs. Neurological outcomes vary from normal to mild or severe sequelae including cerebral atrophy, intellectual disability, paralysis and epilepsy.
Sequence

Extended Properties

No extended properties.

Concept Alternate Designations

SDO Designation ID	Concept Designation Description	Status	Designation Type	PHIN Preferred Term Flag	Code Flag
3660590014	Acute encephalopathy with biphasic seizures and late reduced diffusion	Active	Synonym	false	false
3660591013	Acute infantile encephalopathy predominantly affecting frontal lobe	Active	Synonym	false	false

Associated Value Sets

Value Set Name	Version(s)
Clinical Finding (NNDSS)	2 , 1
Disease	10 , 9 , 8
Problem Value Set	3

Parent Concepts Parent Concepts Parent/Child (Relationship Type) Acute nervous system disorder (disorder) {127341005 , SNOMED-CT } Childhood seizure (finding) {443410001 , SNOMED-CT } Epilepsy (disorder) {84757009 , SNOMED-CT }	Other Relationships No other relationships present.
Child Concepts No child concepts present.