Evaluation and treatment in CSWS syndrome

سال انتشار: 1397
نوع سند: مقاله کنفرانسی
زبان: انگلیسی
مشاهده: 379

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EPILEPSEMED15_098

تاریخ نمایه سازی: 29 اردیبهشت 1398

چکیده مقاله:

Continuous spikes and waves during Sleep (CSWS) is an epileptic encephalopathy characterized in most patients by difficult to control seizures.it is an epileptic syndrome which occurs in childhood period and seriously affects the cognitive function of children. CSWS typically presents with seizures around 2-4 years of age. neurocognitive regression occurs around 5-6 years of age .the cause of CSWS is unknown and recognized by epilepsy with different seizure types, global or selective neuropsychological regression, motor impairment and atypical EEG pattern of continuous epileptic form activity for more than 85% of NREM sleep. treatment options for CSWS include; some antiepileptic drugs, Steroids ,immune modulating agents, Ketogenic diet and surgery. Incidence:CSWS is rare (0.2-0.5% of all childhood epilepsies).age of onset is 2 to 12 years(average 4-5 years of age).boys seem to be affected more than girls. Discussion:Cause of this epilepsy syndrome are frequently found to be brain malformations, genetic or metabolic conditions. CSWS is an age related epileptic encephalopathy that represents the most severe end of the childhood Seizure susceptibility syndrome. Family history in CSW is uncommon. However the mechanism generating CSWS is still controversially. A gene called GRIN2A is known to be involved. Materials and methods: A retrospective study was performed on all childhood’s seizures, referred to the khatam hospital in the city of Tehran (2015-2017).A total of 15 children were included (Age 2 years to 16 years). Treatment: the main aim of treatment is to control seizures. Children with CSWS are treated with AEDs and steroids, immunoglobulin, ketosis diet and surgical operation. Depakin, high dose diazepam, other drugs include Clobazam,Ethosuximide, Topiramate, Keppra, have been used. but VPA,BDZ,LEV, appear to be valuable for this syndrome. corticosteroids and immunoglobulin have a powerful effect. sometimes they have side effects such as weight gain, steroid diabetes, hypertension and Cushing syndrome.if seizures persist medication,a surgery called multiple Subpial transections may be done.in surgery makes multiple small cuts in the brain cortex where the seizures being.when brain malformation is seen resective brain surgery is recommended. Case report: The patient was a known case of CSWS. He was a 16-year-old boy with history of febrile convulsion at age 4.he had seizures during sleep once every 3-4 months. the last few years he had neurocognitive decline as impulsivity and problem in his school performance. brain MRI was normal.as soon as he was asleep, the EEG showed CSWS.Seizures were in form of partial motor, clonic, generalized tonic clonic. He waked up with right hand automatism and was unresponsive. After injecting high dose of intravenous Diazepam, no CSWS was founded during routin sleep EEG. all of CSWS disappeared immediately. Conclusion: In this report based on the semiology, EEG and MRI he was not a good candidate for epilepsy surgery. Epileptologist treated him with IVIG and several AEDs.Overall, a child with sudden decrease in school performance or language difficulties would be entirely necessary to carry out a study of sleep EEG.

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