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.
The correlation between gut microbes and epilepsy is a hot research topic. This review aims to summarize the effects of Ketogenic diet (KD) on gut microbes and the preclinical and clinical progress of the use of Fecal microbiota transplants (FMT) and Probiotics in the intervention of epilepsy to provide clinical reference. Gut microbes mediates the antiepileptic effect of KD. Many studies have found that bactericides decreased in epileptic patients, and KD can increase bactericides abundance, which may be one of its effective mechanisms. Both FMT and probiotics showed antiepileptic effects on epileptic model mice with different pathogenesis, suggesting that gut microbes is an important target for epilepsy treatment. Preliminary clinical studies of small samples suggest that the use of probiotics can effectively treat refractory epilepsy and autoimmune-associated epilepsy, and can improve comorbidities. No serious and long-term side effects of probiotics have been found in epileptic patients. In the future, more high-quality studies are needed to further clarify its efficacy and mechanisms, which could lead to new strategies for epilepsy treatment and refresh our understanding of the causes of epilepsy.
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 analyze the efficacy of ketogenic diet (KD) in the treatment of epilepsy syndrome associated with Electrical status epilepticus during sleep (ESES) and the changes of electroencephalogram (EEG). MethodsThe clinical data of 58 children with ESES-related epilepsy syndrome and cognitive impairment admitted to the outpatient and inpatient department of Xuzhou Children’s Hospital from March 2020 to June 2022 were collected. They were divided into observation group (group A) and control group (group B) by random number table method, with 29 cases in each group. On the basis of not changing the original anti-seizure drugs, group A was treated with ketogenic diet, and group B was treated with hormone. The clinical efficacy, cognitive function and Spike-wave index (SWI) of children with ESES-related epilepsy syndrome were observed before and 3 and 6 months after ketogenic diet and hormone treatment. Results The total clinical effective rate and control rate of group A after 3 months of treatment; The total effective rate and significant efficiency of EEG and the verbal quotient, operation quotient and total intelligence quotient of Wechsler Intelligence Scale for children were significantly lower than those of group B, and the differences were statistically significant (P<0.05). However, the total clinical effective rate and control rate of group A after 6 months of treatment; The total effective rate and significant efficiency of electroencephalogram and the verbal quotient, operation quotient and total intelligence quotient measured by Wechsler Intelligence Scale for children were similar to those of group B, and the difference was not statistically significant (P>0.05). There were no cases of EEG SWI and recurrent clinical symptoms in group A during the follow-up period; In group B, the recurrence rate of EEG SWI was 55.2%, and the recurrence rate of clinical symptoms was 37.9% after 6 months of treatment. There were no serious adverse reactions in the two groups during the treatment period. In group A, about 27.6% (8 cases) of the children had gastrointestinal symptoms, but they were mild and quickly relieved, and no serious adverse reactions occurred in the maintenance period. However, 89.7% (26 cases) of the children in group B showed different degrees of weight gain, and 69% (20 cases) of them showed kexing features such as full moon face and buffalo back. ConclusionsKetogenic diet can improve the curative effect and EEG changes of children with ESES related epilepsy syndrome, and improve their cognition. Compared with hormone pulse therapy, the recurrence rate of ESES related epilepsy syndrome in the treatment of ketogenic diet is lower, and the incidence of adverse reactions is relatively mild and low.
Ketogenic diet (KD) is one of the effective treatments for refractory epilepsy (RE) and is recommended when anti-seizure medications (ASMs) are ineffective or less effective, inoperable or ineffective. The efficacy of the medium-chain triglyceride (MCT) ketogenic diet is as good as the classical KD (CKD), which has been demonstrated in several retrospective, prospective, and randomized studies, and MCT is more ketogenic than long-chain triglycerides, so MCTD allows more carbohydrate and protein foods, which makes MCTD more palatable than CKD more palatable. Research advances in the mechanisms and clinical efficacy associated with MCTD in the treatment of refractory epilepsy are reviewed.
Objective To explore the efficacy and safety of the ketogenic diet (KD) in the treatment of genetic developmental and epileptic encephalopathy (DEE). Methods Clinical data from 42 children with genetically confirmed refractory epileptic encephalopathy treated in the Department of Neurology, Jinan Children’s Hospital, between January 2021 and October 2023 were retrospectively analyzed. A classic KD protocol was implemented, and outcomes including seizure frequency, electroencephalogram (EEG) improvement, and adverse reactions were observed at 3, 6, and 12 months post-treatment. Results Among the 42 children, the seizure-free rates at 3, 6, and 12 months of KD treatment were 16.7%, 16.7%, and 14.3%, respectively, while the effective seizure control rates were 69.0%, 52.4%, and 35.7%. At 3 months, comparison of baseline characteristics between the effective and ineffective groups showed no statistically significant differences in gender (P=0.095), age at onset (P=0.648), age at KD initiation(P=0.768), disease duration before KD (P=0.519), presence of abnormal brain MRI findings (P=0.226), epilepsy syndrome classification(P=0.344), or ion channel gene involvement (P=0.066). EEG improvement rates at 6 and 12 months were 54.2% (24 cases) and 42.8% (14 cases), respectively. Retention rates for KD at 3, 6, and 12 months were 100.0%, 71.4%, and 42.8%. Adverse reactions occurred in 7 patients (16.7%), primarily gastrointestinal symptoms (vomiting, constipation, diarrhea; 6 cases) and elevated uric acid (1 case), with no severe adverse events reported. Conclusion KD is an effective treatment for genetic DEE with favorable short-term safety, though long-term adherence requires attention.
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.
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.
Lennox-Gastaut syndrome (LGS) is a refractory epileptic encephalopathy that mainly affects children, but can also involve adults, and is characterized by multiple seizure types, electroencephalographic (EEG) abnormalities, and mental retardation. This review focuses on the etiology, pathogenesis, diagnostic criteria, and treatment of LGS. In terms of etiology, LGS may be caused by a variety of factors such as abnormal brain development, perinatal brain injury, inherited metabolic diseases, and gene mutations. The pathogenesis involves multiple gene mutations that affect the balance of neuronal excitability and inhibition.LGS is diagnosed on the basis of multiple seizure types with an age of onset of less than 18 years, an EEG that shows widespread slow (1.5~2.5 Hz) spiking slow complex waves, and a triad of intellectual and psychosocial dysfunction. Therapeutically, LGS is treated with antiepileptic seizure medications (ASMs) , including valproate, lamotrigine, and rufinamide, but patients often develop resistance to ASMs. Non-pharmacological treatments include ketogenic diet, vagus nerve stimulation (VNS) , and corpus callosotomy (CC) , which provide palliative treatment options for patients who have difficulty controlling seizures. Despite the variety of therapeutic options, the prognosis for LGS is usually poor, with patients often experiencing intellectual disability and seizures persisting into adulthood. This review emphasizes the importance of further research into the etiology and pathogenesis of LGS and the need to develop new therapeutic approaches to improve patients' quality of life and reduce the burden of disease.
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.