Objective:To investigate the effect and safety of ketogenic diet(KD)for the treatment of paediatric patients with super-refractory status epilepticus(SRSE)in febrile infection-related epilepsy syndrome(FIRES).Method:F...Objective:To investigate the effect and safety of ketogenic diet(KD)for the treatment of paediatric patients with super-refractory status epilepticus(SRSE)in febrile infection-related epilepsy syndrome(FIRES).Method:From January 1,2015 to October 31,2017,ten critically ill paediatric patients with SRSE in FIRES were included in this study and treated with KD.The treatment effects of KD were evaluated by using continuous encephalography(CEEG)and amplitude-integrated electro-encephalography(aEEG).Results:All 10 patients fulfilled the diagnostic criteria of SRSE in FIRES and achieved ketosis within 24–72 h following the administration of KD.CEEG and aEEG were monitored for several weeks to assess the efficacy of KD on status epilepticus(SE).SE was contained in 8 patients within 2 to 19 days after initiation of KD,and KD was discontinued in the other 2 patients.One of the 10 patients demonstrated severe adverse effects.Conclusion:KD may be an alternative and safe treatment option in critical paediatric patients with SRSE in FIRES.展开更多
Super-refractory status epilepticus(SRSE)is an important neurological emergency associated with high mortality and morbidity and poses a heavy economic burden on patients.Ketogenic diet parenteral nutrition(KD-PN)is k...Super-refractory status epilepticus(SRSE)is an important neurological emergency associated with high mortality and morbidity and poses a heavy economic burden on patients.Ketogenic diet parenteral nutrition(KD-PN)is ketogenic diet therapy provided through parenteral administration and may be an adjuvant treatment for these who cannot accept enteral diet.However,the calculation and management of KD-PN presents a challenge for clinicians.This review focuses on the practical aspects of KD-PN therapy for treatment of SRSE,including the dietary composition,potential drug-diet interactions,and monitoring during KD-PN treatment.As with all SRSE treatments,KD-PN has many adverse effects,like hyperlipemia,hepatotoxicity,metabolic acidosis,insufficient ketosis or hyper-ketosis,and propofol infusion syndrome.We summarize monitoring and treatment methods in our review.This review provides some practical aspects for treatment of SRSE.展开更多
Super-refractory status epilepticus(SRSE)is a serious and life-threatening neurological condition.Ketogenic diet(KD)is a diet characterized by high fat,low carbohydrate,and moderate protein.As KD shows effectiveness i...Super-refractory status epilepticus(SRSE)is a serious and life-threatening neurological condition.Ketogenic diet(KD)is a diet characterized by high fat,low carbohydrate,and moderate protein.As KD shows effectiveness in controlling seizures in more than half of SRSE patients,it can be a treatment option for SRSE.Currently,KD treatment for SRSE is based on personal experience and observational evidence has been published.In the context of a lack of a validated guideline,we convened a multicenter expert panel within the China Association Against Epilepsy(CAAE)Ketogenic Diet Commission to work out the Chinese expert recommendations on KD for SRSE.We summarize and discuss the latest clinical practice of KD for SRSE in critical care settings.Recommendations are given on patient selection,the timing of KD,diet implementation,and follow-up.More research data are needed in this area to support better clinical practice.展开更多
癫发作依据持续的时程和对治疗的反应分为早期癫持续状态( early status epilepticus)、确认的癫持续状态( established status epilepticus)、难治性癫持续状态( refractory status epilepticus)和超级难治性癫持续状态( super-...癫发作依据持续的时程和对治疗的反应分为早期癫持续状态( early status epilepticus)、确认的癫持续状态( established status epilepticus)、难治性癫持续状态( refractory status epilepticus)和超级难治性癫持续状态( super-refractory status epilepticus)。本研究主要针对难治性及超级难治性癫的治疗策略进行系统回顾,包括致病相关因素、脑损伤的分析,指出超级难治性癫是一个重要的临床问题,有着较高的病死率和发病率。现就近期世界范围发表的文献提出的治疗方法进行评价,包括麻醉药物,抗癫药物,镁剂,吡哆醇,类固醇和免疫治疗,生酮饮食,低温疗法,神经外科干预,经颅磁刺激,迷走神经刺激,脑深部刺激,电休克疗法,脑脊液引流及其他传统药物等疗法。强调原因的识别与对因治疗的重要性,推荐难治性癫分段治疗方案及超级难治性癫的管理流程。展开更多
基金“Omics-based precision medicine of epilepsy”entrusted by Key ResearchProject of the Ministry of Science and Technology of China(GrantNo.2016YFC0904400).
文摘Objective:To investigate the effect and safety of ketogenic diet(KD)for the treatment of paediatric patients with super-refractory status epilepticus(SRSE)in febrile infection-related epilepsy syndrome(FIRES).Method:From January 1,2015 to October 31,2017,ten critically ill paediatric patients with SRSE in FIRES were included in this study and treated with KD.The treatment effects of KD were evaluated by using continuous encephalography(CEEG)and amplitude-integrated electro-encephalography(aEEG).Results:All 10 patients fulfilled the diagnostic criteria of SRSE in FIRES and achieved ketosis within 24–72 h following the administration of KD.CEEG and aEEG were monitored for several weeks to assess the efficacy of KD on status epilepticus(SE).SE was contained in 8 patients within 2 to 19 days after initiation of KD,and KD was discontinued in the other 2 patients.One of the 10 patients demonstrated severe adverse effects.Conclusion:KD may be an alternative and safe treatment option in critical paediatric patients with SRSE in FIRES.
文摘Super-refractory status epilepticus(SRSE)is an important neurological emergency associated with high mortality and morbidity and poses a heavy economic burden on patients.Ketogenic diet parenteral nutrition(KD-PN)is ketogenic diet therapy provided through parenteral administration and may be an adjuvant treatment for these who cannot accept enteral diet.However,the calculation and management of KD-PN presents a challenge for clinicians.This review focuses on the practical aspects of KD-PN therapy for treatment of SRSE,including the dietary composition,potential drug-diet interactions,and monitoring during KD-PN treatment.As with all SRSE treatments,KD-PN has many adverse effects,like hyperlipemia,hepatotoxicity,metabolic acidosis,insufficient ketosis or hyper-ketosis,and propofol infusion syndrome.We summarize monitoring and treatment methods in our review.This review provides some practical aspects for treatment of SRSE.
基金This work was supported by the Horizontal Scientific Research Project of Sichuan University(No.20H0072).
文摘Super-refractory status epilepticus(SRSE)is a serious and life-threatening neurological condition.Ketogenic diet(KD)is a diet characterized by high fat,low carbohydrate,and moderate protein.As KD shows effectiveness in controlling seizures in more than half of SRSE patients,it can be a treatment option for SRSE.Currently,KD treatment for SRSE is based on personal experience and observational evidence has been published.In the context of a lack of a validated guideline,we convened a multicenter expert panel within the China Association Against Epilepsy(CAAE)Ketogenic Diet Commission to work out the Chinese expert recommendations on KD for SRSE.We summarize and discuss the latest clinical practice of KD for SRSE in critical care settings.Recommendations are given on patient selection,the timing of KD,diet implementation,and follow-up.More research data are needed in this area to support better clinical practice.
文摘癫发作依据持续的时程和对治疗的反应分为早期癫持续状态( early status epilepticus)、确认的癫持续状态( established status epilepticus)、难治性癫持续状态( refractory status epilepticus)和超级难治性癫持续状态( super-refractory status epilepticus)。本研究主要针对难治性及超级难治性癫的治疗策略进行系统回顾,包括致病相关因素、脑损伤的分析,指出超级难治性癫是一个重要的临床问题,有着较高的病死率和发病率。现就近期世界范围发表的文献提出的治疗方法进行评价,包括麻醉药物,抗癫药物,镁剂,吡哆醇,类固醇和免疫治疗,生酮饮食,低温疗法,神经外科干预,经颅磁刺激,迷走神经刺激,脑深部刺激,电休克疗法,脑脊液引流及其他传统药物等疗法。强调原因的识别与对因治疗的重要性,推荐难治性癫分段治疗方案及超级难治性癫的管理流程。