/early-infantile DEE

early-infantile DEE

Rare Disease

MONDO:0800491

Also Known As

epileptic seizures - myoclonicepileptic seizures, myoclonicmyoclonia epilepticamyoclonic epilepsymyoclonic seizuremyoclonic seizure disorderEIDEEEIEEEMEOhtahara syndromeearly infantile epileptic encephalopathyearly infantile epileptic encephalopathy with burst-suppressionearly infantile epileptic encephalopathy with suppression-burstsearly myoclonic encephalopathyearly myoclonic encephalopathy with suppression-burstsearly-infantile developmental and epileptic encephalopathy syndromeepileptic encephalopathy, early infantileepileptic encephalopathy, infantileinfantile epileptic encephalopathymyoclonus epilepsy

Definition

A neonatal/infantile epilepsy syndrome characterized by frequent drug-resistant seizures that begin ≤3 months of age, with abnormal interictal EEG and neurological examination. In up to 80% of patients, EIDEE is caused by an underlying structural, genetic, or metabolic reason.