Background:Infantile spasms(IS)are the most common childhood epileptic encephalopathy.Focal cortical dysplasia(FCD)and gray matter heterotopias(GH)are common structural causes of IS.The recommended first-line treatmen...Background:Infantile spasms(IS)are the most common childhood epileptic encephalopathy.Focal cortical dysplasia(FCD)and gray matter heterotopias(GH)are common structural causes of IS.The recommended first-line treatment for IS patients with structural causes is surgical intervention,according to the International League Against Epilepsy(ILAE)commission guidelines.However,there is currently no consensus on appropriate timings of surgery.Case presentations:Two structural IS cases are presented here:one was caused by FCD,and the other by GH.Both patients exhibited recurrent seizures at the age of 2 months,had poor responses to various antiepileptic drugs(AEDs)and displayed severe mental and motor developmental retardation.Seizure types included focal seizures and spasms.Brain magnetic resonance imaging showed abnormal gray signal or suspicious FCD lesions that coincided with the origin of the focal seizures.The patients underwent lesion resection before the age of 6 months.Follow-up observation showed that seizures of both patients were completely controlled several days after the surgery.All AEDs were gradually reduced in dosage within 1 year,and the mental and motor development almost returned to normal.Conclusion:Early resection of lesions in structural IS patients has benefits of effectively controlling convulsions and improving developmental retardation.Infants at several months of age can well tolerate craniotomy,and their cognitive development is more likely to return to normal after early surgery.展开更多
基金the National Natural ScienceFoundation of China(No.81671297).
文摘Background:Infantile spasms(IS)are the most common childhood epileptic encephalopathy.Focal cortical dysplasia(FCD)and gray matter heterotopias(GH)are common structural causes of IS.The recommended first-line treatment for IS patients with structural causes is surgical intervention,according to the International League Against Epilepsy(ILAE)commission guidelines.However,there is currently no consensus on appropriate timings of surgery.Case presentations:Two structural IS cases are presented here:one was caused by FCD,and the other by GH.Both patients exhibited recurrent seizures at the age of 2 months,had poor responses to various antiepileptic drugs(AEDs)and displayed severe mental and motor developmental retardation.Seizure types included focal seizures and spasms.Brain magnetic resonance imaging showed abnormal gray signal or suspicious FCD lesions that coincided with the origin of the focal seizures.The patients underwent lesion resection before the age of 6 months.Follow-up observation showed that seizures of both patients were completely controlled several days after the surgery.All AEDs were gradually reduced in dosage within 1 year,and the mental and motor development almost returned to normal.Conclusion:Early resection of lesions in structural IS patients has benefits of effectively controlling convulsions and improving developmental retardation.Infants at several months of age can well tolerate craniotomy,and their cognitive development is more likely to return to normal after early surgery.
