%0 Journal Article
%T Epileptic Encephalopathies in Adults and Childhood
%A Zekiye Kural
%A Ali Fahir Ozer
%J Epilepsy Research and Treatment
%D 2012
%I Hindawi Publishing Corporation
%R 10.1155/2012/205131
%X Epileptic encephalopathies are motor-mental retardations or cognitive disorders secondary to epileptic seizures or epileptiform activities. Encephalopaties due to brain damage, medications, or systemic diseases are generally not in the scope of this definition, but they may rarely accompany the condition. Appropriate differential diagnosis of epileptic seizures as well as subclinical electroencephalographic discharges are crucial for management of seizures and epileptiform discharges and relative regression of cognitive deterioration in long-term followup. Proper antiepileptic drug, hormonal treatment, or i.v. immunoglobulin choice play major role in prognosis. In this paper, we evaluated the current treatment approaches by reviewing clinical electrophysiological characteristics of epileptic encephalopathies. 1. Introduction Epileptic encephalopathy is described as epilepsy with ictal and interictal epileptiform anomalies (clinical and EEG) and progressive cerebral dysfunction according to the classification and terminology criteria of International League against Epilepsy (ILAE) [1每3]. The following are syndromes meeting the criteria: Dravet syndrome, Doose syndrome, ESES (electrical status epilepticus of slow sleep) or CSWSS (continuous spike waves of slow sleep), Landau-Kleffner syndrome, Lennox-Gastaut syndrome, Ohtahara syndrome, and West syndrome [4]. Severe epilepsy with multiple independent spike foci is recently included in this group [2, 4]. Steroid-sensitive epileptic encephalopathies such as Hashimoto encephalopathy, progressive myoclonus epilepsies, and neonatal epileptic encephalopathies are not classified in this group but are worth mentioning [5每10]. 2. Dravet Syndrome or Severe Myoclonic Epilepsy of Infancy An important group of epileptic encephalopathies that are resistant to treatment, a severe myoclonic epilepsy of childhood, first described by Dravet in 1978, is characterized with recurrent febrile and/or afebrile, hemiclonic or generalized seizures, and status epilepticus. Child＊s development stops or retards after the onset of seizures [11每14]. Prevalence is unknown. The incidence is 0.5每1/40.000 and develops in 3每5% and 6.1每8.2% of all epilepsies in the first year and within the 3 years of life, respectively. Male-female ratio is 2ˋ:ˋ1. The most common cause is SCN1A mutations or deletions (35%) [11, 15每17]. Consequences in patients who reached adulthood and were observed for long term as well as neuropathology of the disease are unknown. Patients with family history of febrile convulsion or epilepsy are reported to consist
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