Clinical Research Of Tuberous Sclerosis Complex Complicated With Epilepsy In Children

Background and ObjectiveTuberous sclerosis (Tuberous sclerosis complex, TSC) is a neurocutaneous syndrome. This disease is an autosomal dominant genetic disease by Gunther in1935, there is a significant pathological features of multiple organ hamartoma like hyperplasia, it can damage the skin, brain, kidneys, heart and other organs and so on. The incidence of the disease is about1/6000, sporadic patients accounts for about2/3, the study found that seizures among the children with the first (91.7%) and newly diagnosed (94.6%) is the most common neurological manifestations, the peak of seizures age among the TSC patients is less than1year.the bigger the TSC patients,the onset of a downward trend in the proportion of relatively smaller,but for different starting age of tuberous sclerosis with epilepsy clinical episodes proportion. The starting age children with seizure type distribution, medication species, growth and development status, initial starting age with newly diagnosed seizure type distribution characteristic EEG abnormalities, skin lesions characteristics and sporadic and familial epilepsy in children, children with epilepsy age, skin damage and growth retardation relationship is still insufficient.ObjectiveExplore the clinical features of tuberous sclerosis in children with epilepsy.MethodsCollect clinical data of39patients with tuberous sclerosis with epilepsy in children with seizures in children, such as personal history, family history, course of disease and drug therapy were retrospectively analyzed.Results1、39cases of children with seizures starting age were from lmonth to11years old, with the average (2.01±2.48) years, a median age of9month. Epilepsy starting age0to1year of age,1to3years old,3to5years old, five over the age of onset of the respective proportions were53.8%,20.5%,15.4%,10.3%.2、In the Tuberous sclerosis children,35.9%children had a genetic family history,64.1%children had not family history of hereditary;59.0%children had skin damage,performance of64.1%growth retardation or smart waning.3、 Single drug, two drugs and three or more drugs in the treatment efficiency were41.0%,28.2%,17.9%.4> Different starting age children of seizure types (X2=5.012, P=0.082), medication species (X2=1.437, P=0.488), growth retardation (P=0.337), the initial EEG abnormalities (P=0.667) and skin damage were no statistically significant difference (P=0.469).5、Dissemination in children with familial seizure types was statistically significant (X2=6.520, P=0.038), medication (P=0.607) difference was not statistically significant.6、 Skin lesions in children with growth retardation was no correlation (X2=1.400, P=0.237). 7、 Daily attack group and choose an auspicious day in finding out the abnormal EEG (P=0.029) and the application of drug treatment programs(P=0.008) was different.Conclusion1、 Epileptic children starting age is gradually increasing, the epileptogenesis proportion may decreasing.2、 With the gradual increase of the number of species of antiepileptic drugs, medication efficient downward trend may relate to the severity of the disease in children, the use of three or more medications may be refractory epilepsy in children.3、 Generalized seizures in children with familial disseminated in children, is higher than in children with familial distribution of children with partial seizures.