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ObjectiveEpileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep‐induced epileptic discharges and acquired impairment of cognition or behavior. Treatment of ESES is assumed to improve cognitive outcome. The aim of this study is to create an overview of the current evidence for different treatment regimens in children with ESES syndrome...
PurposeDravet syndrome is an autosomal dominant epileptic encephalopathy of childhood, which is caused mainly by SCN1A and PCHD19 mutations. Although Dravet syndrome is well recognized, the causes of acute encephalopathy are still elusive, and reported data on ictal electroencephalography (EEG) and structural brain abnormalities are scarce.
MethodsWe studied 30 children who fulfilled the clinical...
Recent studies reported mutations in the gene encoding the proline-rich transmembrane protein 2 (PRRT2) to be causative for paroxysmal kinesigenic dyskinesia (PKD), PKD combined with infantile seizures (ICCA), and benign familial infantile seizures (BFIS). PRRT2 is a presynaptic protein which seems to play an important role in exocytosis and neurotransmitter release. PKD is the most common form of...
Purpose: We analyzed clinical and electroencephalography (EEG) outcomes of 13 patients with pharmacoresistant encephalopathy with electrical status epilepticus during sleep (ESES) following epilepsy surgery.
Methods: All patients had symptomatic etiology of ESES and preoperative neuropsychological deterioration. Ten patients had daily atypical absences. Clinical outcome was assessed at 6 months...
Purpose: To prospectively evaluate the efficacy of drug treatment and long‐term cognitive outcome in children with encephalopathy with status epilepticus during sleep (ESESS).
Methods: Thirty‐two children were diagnosed and prospectively followed up for at least 3 years at our unit between 1991 and 2007. Twenty‐seven children were included in the prospective treatment study with valproate (VPA)...
Research findings concerning cognitive effects of pediatric epilepsy surgery form an important basis for decisions about surgery. However, most follow-up studies have been of limited duration. In this study, a 2-year follow-up of intelligence was undertaken. Risk factors were analyzed. Included were 38 patients aged 3 to 17 years. Surgery was left in 19 patients and right in 19 patients. Types of...
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