ObjectiveTuberous sclerosis complex (TSC) is a multiorgan disorder and mostly associated with intractable epilepsy. Now several individual reports suggest that epilepsy in children with TSC might benefit from a ketogenic diet (KD). We prospectively studied the curative effect of 14 children with the KD in the treatment of TSC with epilepsy. MethodsBetween 2008 and 2015, we enrolled 14 children with TSC and epilepsy who received KD treatment in Shenzhen Children's Hospital and followed up for at least three months.Outcome was measured by the change of seizure frequency before and after the KD in the use of anticonvusant drugs, adverse effects, and change in cognitive function. Results14 children aged 8 months to 7 years were included. 7/14 (50%) children had a > 50% reduction in seizure frequency at 3 months on the diet, 5/14 (36%) children had a seizure free response. 12/14 (86%) children with refractory epilepsy, 6/12 (50%) children had a > 50% reduction in seizure frequency, 2 children had reduced medications, one child did not use any antiepileptic drugs during KD. 6 of 12 children with developmental delays had cognitive function improvement. ConclusionsKD is a generally effective and safe therapy for TSC children with epilepsy, especially for refractory epilepsy. KD could reduce antiepileptic drugs, and also improve children's cognitive function.
ObjectiveTo compare the efficacy and compliance of children children with refractory epilepsy receiving ketogenic diet (KD) in outpatient department with children receiving KD treatment in inpatient department. MethodsA retrospective study of 44 children with intractable epilepsy receiving the modified classical ketogenic diets in outpatient department from June 2014 to December 2015, who were followed-up during the third, sixth and twelfth month. Records of epileptic seizures and adverse reactions were used to evaluate the efficacy and retention rate of inpatient department KD treatment in children with refractory epilepsy, and compared with 104 children receiving KD treatment in inpatient department at the same period. ResultsThirty-four of the forty-four children comleted observation after 12-month follow-up, 15 cases had been seizure freedom, 22 cases had more than 50% reduction in seizure frequency, 12 patients had less than 50% reduction in seizure frequency.The total effective rate of the KD therapy in outpatient department was 64.7%, and the retention rate was 71%. 18 of of the 104 children with KD treatment in inpatient department at the same period comleted observation after 12-month follow-up, 3 cases had been seizure freedom, 5 cases had more than 50% reduction in seizure frequency, 13 cases had less than 50% reduction in seizure frequency.The total effective rate of the KD therapy in inpatient department was 27.8%, and the retention rate was 17.3%. ConclusionThe KD therapy in outpatient department is effective to children with intractable epilepsy, and there is a highly efficacy and compliance of children receiving KD in outpatient department comparing with children receiving KD in inpatient department. Therefore, it's optional to children with refractory epilepsy who can't received KD by inpatient department because of insufficient number of beds.
Objective Compare and analyze the value of β-hydroxybutyrate from two kinds of blood ketone instruments (Optium Xceed blood ketone body instrument from American Abbott laboratories and TBS-1 blood ketone body instrument from Beijing Yicheng company), and provide instructions for clinical applications. Methods Select 7 children patients with epilepsy which have a response to ketogenic diet. The two kinds of blood ketone instruments were used to test blood ketone value of whole blood synchronously in five different periods, and compare the 70 results. Results The measuring results obtained from these two kinds of blood ketone body instruments have statistical difference significantly. The value acquired from TBS-1 blood ketone body instrument is lower than that from Optium Xceed blood ketone body instrument. Measurements using a paired t test, the value of t=12.14, P<0.01. The results from two kinds of blood ketone body instruments are statistically significant. Conclusion The values from 2 kinds of blood ketone instruments are different, which would influence the clinical judgment. At present, the key problem is that there is lack of special blood ketone measure technology for patients with ketogenic diet. How to improve the measure accuracy of high-level blood ketone value remains the future work.
ObjectiveTo explore the effect of Ketogenic diet (KD) in patients with Dravet syndrome (DS).MethodsThe seizures, electroencephalogram (EEG) and cognitive function of 26 DS patients receiving treatment of KD from June 2015 to October 2019 in the Epilepsy Centre of Guangdong 999 Brain Hospital were analyzed retrospectively. Modified Johns Hopkins protocol was used to initiate KD, blood glucose and urinary ketone bodies were monitored in the patients. The effect was evaluated by Engel outcome scale. And the EEG, cognition, language and motor function of the patients were assessed.Results26 DS patients (20 boys, 6 girls) received treatment of KD more than 4 weeks, the seizure of epilepsy were reduced above 50% in 15 patients (57.7%), 2 patients were seizure free. Among them, the effcet of KD can be observed in 7 patients within one week and 3 patients within two weeks, accounting for 66.7% (10/15). 26 cases (100%), 24 cases (92.3%), 20 cases (76.9%), 11 cases (42.3%) were reserved in 4, 12, 24, 28 weeks respectively. 26 patients achieved Engel Ⅰ ,Ⅱ,Ⅲ,Ⅳgrade effect, accounting for 38.5% (10/25), 54.2% (13/23), 65.0% (12/19), 81.8% (9/11) respectively according to Engel scale. The back ground rhythm of EEG showed obvious improvement in 14 patients wthin 16 patients who revieved EEG after KD. 17 patients assessed cognitive, language and motor function after KD, cognitive function was improved in 10 patients, language was enhanced in 11 patients, motor function of 8 patients was enhanced. The main adverse reactions of KD in the treatment process were mainly gastrointestinal symptoms and metabolic disorders.ConclusionKD treatment in DS has many advantages, such as fast acting, being effective in more than half of the DS patients and tolerable adverse reactions. Cognitive, language and motor function were improved in some patients. Drug resistant DS patients are suggested to receive KD treatment early.
ObjectiveTo explore the impact on the retention and effective rate of the mode of continuous guidance with wechat group in children with infantile spasms (IS) treated by ketogenic diet (KD).MethodsThe clinical data of 40 children who received KD treatment in Jiangxi Children’s Hospital from January 2017 to June 2019 were retrospectively analyzed, including 23 males and 17 females, the average age was (19.90±13.10) months and the average course was (9.95±7.61) months. They were randomly divided into control group (20 cases) and observation group (wechat group continuity Guidance Group, 20 cases), and followed up one year to compare the retention rate and efficacy.ResultsThere was no statistical difference between the general data of the observation group and the control group (P>0.05). After 9 and 12 months of KD treatment, the retention and effective rate of the observation group was significantly higher than the control group (P<0.05). And the seizure-free rate was higher than the control group (35% vs. 10%, 35% vs. 15%).ConclusionThis model of continuous guidance with wechat group can increase the KD retention rate of IS children, And increase the effective rate.
ObjectiveAnalyze and compare the differences in the efficacy and adverse reactions of various ketogenic diet (KD) in the treatment of refractory epilepsy in children.MethodsSystematic search of electronic databases, including PubMed, Embase, Ovid MEDLINE, Web of Science and the Central Register of Cochrane Controlled Trials, published in English January 2000 Relevant research from January to August 2020. Results: Finally, 11 articles were included and 781 cases were included. Meta-analysis (NMA) method was used to compare 6 classic ketogenic diets (Classic ketogenic diet, CKD), Gradual ketogenic diet initiation (GRAD-KD), and the first modified Atkins diet of 20 g carbohydrates/d (Initial 20 g of carbohydrate/day of modified Atkins diet, IMAD), modified Atkins diet (MAD), low glycemic index diet (LGID) and medium-chain fatty acid diet (Medium-chain triglyceride diet, MCT) Therapeutic effect and adverse reactions of 3, 6, and 12 months.ResultsFrom the results of the direct comparative analysis, CKD and MAD showed superior clinical efficacy in 50% seizure reduction at 3 months to CAU, and the difference was statistically significant [OR=10.58, 95%CI (3.47, 32.40), P<0.05; OR=11.31, 95%CI (5.04, 25.38), P<0.05]; the clinical efficacy of 90% seizure reduction at 3 months for MAD was superior to that of CAU with statistical significance [OR=4.95, 95%CI (1.90, 12.88), P<0.05]. The results of further network meta-analysis suggested that for the comparison of 50% seizure reduction at 3 months, IMAD, GRAD-KD, CKD, MAD, and MCT were superior to CAU, and the difference was statistically significant [OR=0.03; 95%CI (0.00, 0.30), P<0.05; OR=0.07; 95%CI (0.01, 0.76), P<0.05; OR=0.11; 95%CI (0.03, 0.35), P<0.05; OR=0.11; 95%CI (0.04, 0.35), P<0.05; OR=0.13; 95%CI (0.03, 0.67), P<0.05; OR=0.11; 95%CI (0.03, 0.35), P<0.05; OR=0.11; 95%CI (0.04, 0.35), P<0.05]. For the comparison of 90% seizure reduction at 3 months, CKD, GRAD-CK, IMAD, MAD, and MCT were superior to CAU, and the differences were statistically significant [OR=0.05; 95%CI (0.00, 0.31), P<0.05; OR=0.22; 95%CI (0.00, 0.39), P<0.05; OR=0.03; 95%CI (0.00, 0.62), P<0.05; OR=0.12; 95%CI (0.01, 0.60), P<0.05; OR=0.09; 95%CI (0.00, 0.91), P<0.05]. It is suggested in the cumulative probability plot that: the optimal clinical regimen for 50% seizure reduction at 3 months was IMAD (Rank1=0.91), the optimal clinical regimen for 50% seizure reduction at 6 months was CKD (Rank1=0.40), the optimal clinical regimen for 50% seizure reduction at 12 months was MCT (Rank1=0.64); the optimal clinical regimen for 90% seizure reduction at 3 months was IMAD (Rank1=0.94), the optimal clinical regimen for 90% seizure reduction at 6 months was LGIT (Rank1=0.44), and the optimal clinical regimen for 90% seizure reduction at 12 months was MCT (Rank1=0.41); the optimal clinical regimen for seizure reduction at 3 months was GRAD-CK (Rank1=0.46), the optimal clinical regimen for seizure reduction at 6 months was LGIT (Rank1=0.58), and the optimal clinical regimen for seizure reduction at 12 months was CKD (Rank1=0.56). It is suggested in the benefit-risk assessment that among the three KDs (CKD, MAD, MCT) with better 50% and 90% seizure reduction at 3 months and 6 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.47, CF=0.86); among the two KDs (CKD, MAD) with better seizure reduction at 3 months and 6 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.45); among the two KDs (CKD, MCT) with better 50% and 90% seizure reduction at 12 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.65).ConclusionsIn this study, IMAD showed the optimal clinical efficacy at 3 months and MCT at 12 months. With stable efficacy and low incidence of adverse reactions in 12 months, CKD was the optimal treatment regimen for children with refractory epilepsy after the comprehensive evaluation.
ObjiectiveTo explore the efficacy and safety of ketogenic diet therapy (KDT) in the rapidly progressive stage of childhood developmental epileptic encephalopathy Dravet syndrome (DS). Methods The clinical data of all patients who added KDT in the Children’s Hospital of Fudan University from 2011 to 2022 were retrospectively collected, and the age of <6 years was used as the criterion for the rapid progression of the disease. The clinica data, genotype and the efficacy of KDT were analyzed in DS patients who met the criteria. Results A total of 32 patients met the criteria for rapid disease progress, including 22 males and 10 females. The age at onset was (5.69±2.10) months. All patients had multiple seizure phenotypes and monthly seizures despite reasonable Antiseizure medications treatment. After 3, 6, 12, and ≥24 months, 93.8% (30/32), 87.5% (28/32), 53.1% (17/32), 34.4% (11/32) remained on the KDT, while 76.7% (23/30), 75.0% (21/28), 70.6% (12/17), 54.5% (6/11) showed >50% reduction in seizure. Status epileptius (SE) was reduced by 100% at 3 months, 71.0% at 6 months, 86.0% at 12 months. After 12 months, 14 patients experienced efficacy degradation. After 3 months, the EEG background rhythm showed improvement in 75.0% patients, interictal epileptic discharges was decreased in 54.5% patients and cognitive function was improved in 78.6% patients. At the initial stage of KDT, 62.5% (20/32) patients had transisent adverse reactions, including diarrhea, vomiting, fatigue, lethargy, hypoglycemia, and metabolic acidosis, but no mid- and long-term adverse reactions were found. ConclusionKDT is an efficective and safe treatment for DS. KDT can effectively control seizures, reduce the incidence of Status SE and shorten the duration of SE. With the prolongation of the KDT course, some patients experienced a degraded effect. KDT can improve abnormal EEG and cognitive function in DS patients. Pharmoco-resistant DS patients are suggested to receive KDT in the early stage of disease progression.
Objective To evaluate the efficacy of Wechat combined with continuing nursing on the quality of life of epilepsy children with ketogenic diet, parents' mood and the time commitment of medical staff. MethodsData were collected from 140 children with intractable epilepsy with ketogenic diet admitted to the Department of Neurology, Children's Hospital Affiliated to Chongqing Medical University from November 2014 to June 2022, including 116 males and 24 females, with an average age of (8.42±2.44) years. The random sampling method was divided into control group (continuing nursing) 71 cases, intervention group (Wechat combined with continuing nursing) 69 cases. The quality of life of the children (QOLCE-16) in the two groups was compared before discharge and 3 months after discharge, as well as parental anxiety (SAS), depression (SDS), and the amount of time medical staff spent with both groups. ResultsThere was no difference in the quality of life and parental emotion between the two groups before intervention. After 3 months, the quality of life of the two groups was significantly improved [(43.59±10.00) vs. (40.14±10.44), P<0.05], and the QOLCE-16 score of the intervention group was higher than that of the control group (P<0.05). The parental mood SAS and SDS in both groups were significantly improved [(37.19±2.90) vs. (50.85±3.76), (40.14±3.52) vs. (49.29±3.37), P<0.01], and the SAS and SDS scores of anxiety and depression of parents in the intervention group were lower than that of the control group (P<0.01). Medical staff spent more time on intervention group [(136.17±7.43) vs. (65.55±7.48), P<0.01]. ConclusionContinuing nursing can improve the quality of life of children with ketogenic diet and the negative emotions of their parents. The combination of Wechat and continuing nursing can further strengthen this positive effect, and requires more time of medical staff.
Objective To investigate the compliance of ketogenic diet in children with refractory epilepsy and its correlation with the curative effect, and to provide support and basis for the implementation and improvement of the long-term management of ketogenic diet in this patient population. MethodsA total of 106 children and their families who were followed up on ketogenic diet in the Department of Pediatrics of Fudan University from March 2019 to January 2022 in the Department of Ketogenic Multidisciplinary Treatment (MDT) were selected by convenience sampling method. General data questionnaire and ketogenic compliance questionnaire were used for investigation and follow-up. ResultsThe mean compliance of ketogenic diet in refractory epilepsy children was (13.27±3.68). The compliance scores of ketogenic children with different therapeutic effects and cognitive functions were significantly different. The compliance score was significantly correlated with the therapeutic effect and cognitive level, that is, the higher the therapeutic effect of ketogenic diet in children with higher compliance score, the better the cognitive improvement. ConclusionThe compliance of ketogenic diet in children with refractory epilepsy needs to be further improved. Improving the compliance of ketogenic diet is of great significance for the efficacy of ketogenic diet. Medical staff should actively develop the corresponding intervention program and follow-up management mode to further improve the treatment compliance of children's families, improve the treatment effect and improve the quality of life of children's families.
ObjectiveAccording to the diagnosis and treatment analysis of 5 children with Febrile infection-related epilepsy syndrome (FIRES) and literature review, to explore the therapeutic effect of Ketogenic diet (KD).MethodsA retrospective analysis of the clinical status of 5 children with KD treatment of FIRES admitted to the Department of Pediatric Neurology, West China Second Hospital of Sichuan University from August 2016 to September 2019, combined with literature data, summarized their disease characteristics, prognosis and KD treatment effects.ResultsThe 5 cases of FIRES children were (5.8±2.0) years old and had a male to female ratio of 2∶3. They were all induced by fever followed by a status epilepticus that was difficult to control with drugs. The interval between fever and first seizure was 4 to 7 days, and the prodromal symptoms were higher respiratory tract infections, dizziness, vomiting, fatigue, listlessness, loss of appetite, etc., convulsions manifested as focal or focal secondary systemic or general seizures, EEG showed slowing background rhythm, changes in multifocal epileptic discharge, early course of disease The cranial imaging examination was basically normal. As the course of the disease progressed, changes in brain atrophy gradually appeared, and abnormal signal shadows were seen in the forehead, parietal, occipital lobe, and periventricular. A variety of anti-epileptic drugs, hormones, gamma globulin, plasma exchange and other treatments have poor therapeutic effects and severe cognitive impairment. The KD treatment started to take effect within 2 weeks, and the convulsions were reduced. One case was completely controlled, and the cognitive function basically returned to normal with only mild learning disabilities; the convulsions were reduced by more than 50% in 2 cases, leaving mild to moderate cognitive impairment, The other 2 cases had poor long-term treatment effects, left intractable seizures and severe mental retardation.ConclusionFIRES is a serious epileptic encephalopathy, most of which leave severe cognitive impairment and refractory seizures. Drug therapy and prognosis are poor. KD treatment is beneficial to control seizures in children with FIRES in the acute stage.