| A type of epilepsy that presents with epileptic spasms and an epileptic encephalopathy between 1 and 24 months of age (peak 3 and 12 months). Epileptic spasms usually occur in clusters often over a period of minutes and are often seen on awakening. They may be symmetric or asymmetric and may be subtle, with minor head nods, or eye or chin movements. Focal seizures may also be seen particularly if there is a structural etiology. Prior to seizure onset the development can be normal, but there is often a history of preceding clear or suspected abnormal development. Developmental slowing, arrest, or regression is seen with the onset of epileptic spasms, although it may not be apparent very early in the course. Neurological exam may be normal or reveal abnormalities associated with the underlying etiology. Interictal EEG shows hypsarrhythmia, multifocal or focal epileptiform discharges. |
