Abstract:
Introduction. The term epileptic encephalopathies are severe brain disorders of early age with a
different manifestation, depending on the age of onset, developmental outcome, etiologies,
neuropsychological deficits, electroencephalographic (EEG) patterns, seizure types, and
prognosis, but all may have a significant impact on neurological development. Doose syndrome,
otherwise traditionally known as myoclonic-astatic epilepsy is an epileptic encephalopathy with
multiple seizure types. About a third of children may have episodes of convulsive status
epilepticus. The disease is characterized by the following criteria: genetic predisposition (high
incidence of seizures and/or genetic EEG patterns in relatives); mostly normal development and
no neurological deficits before onset; primarily generalized myoclonic, astatic or myoclonicastatic
seizures, short absences and mostly generalized tonic-clonic seizures; no tonic seizures or tonic drop attacks during daytime, generalized EEG patterns, and often normal neuroimaging .
The prognosis is variable and difficult to predict, and the seizures may remit in 54-89% of
patients.
