Epileptic encephalopathy: Doose syndrome

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.