生酮饮食添加治疗儿童癫痫性痉挛临床和随访研究

Clinical obsevr ation and follow-ups tudy of ketogenic diet adding treatment for epileptic spasms in chil-dren

目的：初步了解生酮饮食（ ketogenic diet，KD）添加治疗对儿童癫痫性痉挛的短期疗效、不良反应及依从性。方法对2012年7月至2014年12月在我院接受KD添加治疗的20例癫痫性痉挛患儿，总结其临床特点，并临床随访。结果20例癫痫性痉挛患儿，男14例，女6例，年龄10个月至8岁2个月，＜1岁2例，-3岁10例，-6岁5例，＞6岁3例。隐源性癫痫12例，围生期脑损伤6例，先天性脑发育畸形及重症病毒性脑炎后遗症各1例。20例患儿均以痉挛发作为主要发作形式，其中7例患儿同时伴随其他发作形式，包括部分性发作泛化全身、阵挛发作、强直发作、不典型失神、不典型失神持续状态。19例患儿诊断为婴儿痉挛症或晚发性癫痫性痉挛，1例诊断为Lennox-Gaustaut综合征。20例患儿病程最短11 d，最长7年9个月。1例患儿因口服抗癫痫药物后短期（11 d）出现较严重肝功损害，予KD治疗，余患儿均为难治性癫痫。20例患儿中6例完全缓解，占30％，1例患儿癫痫发作明显改善，达Engel分级Ⅱ级。12例患儿大运动能力进步，6例患儿同时出现语言能力进步。主要不良反应为消化道症状和代谢紊乱，均较轻微，无严重不良反应。治疗疗程＞6个月者8例，其中4例仍继续治疗，其余12例因短期治疗无效、饮食不耐受、腹泻、治疗过程中饮食控制欠佳及复发等原因终止治疗。结论 KD添加治疗对癫痫性痉挛治疗有效，短期内尚未观察到严重不良反应。还需要加强长期管理，改善饮食配方，提高优质脂肪比例，对更大样本进行更长时间临床观察，为癫痫性痉挛患儿提供更好的治疗方案及更多治疗选择。

Objective To preliminarily study the efficacy and sfa ety of ketogenic diet（ KD） adding treatment for epileptic spasm s in children.Methods The clinical features,efficacies,side effects and compli-ances of patients adding KD treatment in our hospital from July 2012 to December 2014 were analyzed. Results Totally 20 patients（10 months old to 8 years and 2 months old） of epileptic spasms with 14 boys and 6 girls were enrolled in the study.Among them 2 cases were less than 1 year old,10 cases were 1-3 years old,5 cases were 3-6 years old,and 3 casesw ere older thna 6 years.Twelve cases were cryptogenic epilepsy, 6 cases were symptomatic epilepsy（perian tal brain injury）,1 case was brain malformation,and 1 case was severe viral encehp alitis sequela.Sp asm was the main episode of rm of all the cases.Amogn 20 caes s,7 cases were accompanied by some oteh r forms of episode, inlc uding secondarily generalized seizures, clonic seizures,tonic seizures,atypical absence seizures,and atypical absence status.The epileptic syndromes includ-ed infantile spasms or epileptic spasms（19 cases） and Lennox-Gastaut syndrome（1 case）.The courses of disease were 11 days to 7 years and 9 months.One of them had severe liver function injury after oral antiepi-leptic medicine （11 days）,and others were diagnosed intractable epilepsy.Overall,the KD dei t achieved the seizure-free state in 30%（6/20） of patients and a greater than 90% reduction of seizure frequency in one patient.In addition,12 patients got improvement in development of gross motor,while 6 of them had lingual developments.Severe side effects were not observed in follow-up studies.Eight cases com pleted the KD treat-ment for at least 6 months,12 cases stopped because of no efficacy,poor tolerance,diar hea and recrudesces. Conclusion KD adding treatment is probably an effective therapy for epileptic spasms with few side effects. While large samples and long-term observations are still desired to better recipes, and to provide possibly effective alternative to other therapies for epileptic spasms.