acute encephalopathy with biphasic seizures and late reduced diffusion
Findings
No curated finding names acute encephalopathy with biphasic seizures and late reduced diffusion yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a rare childhood-onset epilepsy syndrome associated with infection and characterized by 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 of AESD vary from normal to mild or severe sequelae including cerebral atrophy, mental retardation, paralysis and epilepsy.
Definition from the Mondo Disease Ontology (MONDO:0018198), read 2026-09-29. CC BY 4.0.
- Onset and course
- Seizure cluster
HPO, annotations 2026-09-02
Features
11 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Bilateral tonic-clonic seizureHPOHP:0002069
- Very frequent (80% to 99% of cases)
- Abnormal metabolic brain imaging by MRSHPOHP:0012705
- Frequent (30% to 79% of cases)
- Complex febrile seizureHPOHP:0011172
- Frequent (30% to 79% of cases)
- Hypointensity of cerebral white matter on MRIHPOHP:0007103
- Frequent (30% to 79% of cases)
- Increased circulating procalcitonin concentrationHPOHP:0032308
- Frequent (30% to 79% of cases)
Where it sits
Other names
3 names
Resolves to: acute encephalopathy with biphasic seizures and late reduced diffusion
- Also called
- acute infantile encephalopathy predominantly affecting the frontal lobesAESDAIEF