Objective To study the clinical features and SCN1A genes detection results in children with Dravet syndrome in order to provide reference for clinical treatment. Methods The clinical data，SCN1A genes reports and antiepileptic drug effects of 60 DS children who were diagnosed from December 2013 to December 2015 were collected from the Children’s Hospital of Fudan University. Results The onset of seizures occured during 1-9 months with a median of 6 months and 83.3% of patients were febrile seizures at frist onset；they were heat sensitive，and hot water bath induced seizures in 63.3%（38/60）. There were multiple phenotypes，including generalized tonic-clonic seizures（95.0%，57/60），partial seizures（alternating unilateral seizure）（78.3%，47/60），status epilepticus（65.0%,39/60），myoclonic seizures（65.0%，39/60），and atypical absence （63.3%，38/60）. Seizure ouccurred most frequently（2-3 times per month） in 1-3 years of age. The median age of mental retardation was 18 months. The number of mental retardation and the positive rate of EEG increased with age. Dravet syndrome were intractable. In patients who used sodium ion blocking drugs 40.0%（24/60） children had aggravated seizures. 80.0%（48/60） patients had SCN1A mutation with missense and nonsense mutation accounting for over a half. There was no correlation between SCN1A mutations and onset age，sex，seizure type or seizure frequency. Conclusion Dravet syndrome is a childhood-onset epileptic encephalopathy，which is not rare in the national seizure center. The positive rate of SCNIA mutation is high，which can help the diagnosis of DS. Anti-epiletic drug treatment for DS is difficult and the misuse of drugs is in a high proportion，so the diagnosis and treatment level still needs to be improved.
