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.
Objective To investigate the efficacy and safety of the ketogenic diet (KD) treatment in refractory epilepsy (RE) with myoclonic seizures as the predominant seizure type. Methods Clinical data were collected from 34 pediatric patients with RE characterized by myoclonic seizures who underwent KD treatment at the Department of Neurology, Jinan Children's Hospital from January 2021 to October 2023. The classic KD protocol was applied, and seizure frequency and adverse reactions were monitored at 1, 3, and 6 months post-treatment. Results Among the 34 patients, intention-to-treat (ITT) analysis revealed seizure-free rates of 11.8%, 17.6%, and 14.7% at 1, 3, and 6 months KDT, respectively. Seizure control response rates were 41.2%, 52.9%, and 38.2%. Proximal protocol (PP) analysis showed the KDT treatment achieved seizure-free rates of 11.8%, 25%, and 5% at 1, 3, and 6 months, respectively, with seizure control efficacy rates of 41.2%, 75%, and 86.7%. Subgroup analysis: Among 34 patients, 16 had epilepsy syndrome (ES) and 18 had myoclonic epilepsies (MEs). Comparing efficacy rates, the ES group showed significantly higher efficacy than the MEs group at 1 month (P=0.045), with no significant differences observed at 3 or 6 months. Adverse reactions occurred in some patients: 6 developed gastrointestinal adverse events, 2 experienced somnolence, and 2 had urinary system adverse reactions during KD treatment. Conclusion KD treatment is an effective treatment for RE with predominant myoclonic seizures. Short-term application demonstrates good safety, but adherence issues require attention.
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.
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.
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.
Febrile infection-related epilepsy syndrome (FIRES) is a rare and severe epileptic encephalopathy characterized by critical illness, complex nursing requirements, the need for multidisciplinary collaboration, and high-intensity care during its acute phase. Based on a review of relevant literature and specific nursing practices, this article summarizes the latest advancements in the acute-phase care of children with FIRES. It focuses on aspects such as the management of status epilepticus, fever care, airway management, nutritional support and ketogenic diet, family support, and multidisciplinary collaboration. The aim is to provide a reference for clinical nursing practices and related research.
ObjectiveTo investigate the effect of medical counseling games on ketogenic diet therapy for drug-resistant epilepsy children. MethodsA total of 98 children with drug-resistant epilepsy admitted to the neurology ward of Shenzhen Children's Hospital from January 2023 to June 2024 who were treated with ketogenic diet for the first time were selected as the study objects by random number table method, and were divided into observation group (n=49) and control group (n=49). The control group received the traditional multidisciplinary team health education mode, while the observation group received the ketogenic diet treatment based on the multidisciplinary team health education mode and participated in the customized medical counseling games intervention. The time of children reaching ketosis, the knowledge level of ketogenic diet caregivers and the retention rate of children on ketogenic diet were compared between the two groups. ResultsThe time of ketosis in observation group was earlier than that in control group (P<0.05). The knowledge level of the main caregivers of ketogenic diet and the retention rate of children with ketogenic diet at 3 months and 6 months in observation group were higher than those in control group (P<0.05). ConclusionThe use of medical counseling games in the ketogenic diet for medically refractory epilepsy is an effective therapeutic strategy that facilitates the early attainment of ketosis in children with medically refractory epilepsy, improves the knowledge of caregivers on the ketogenic diet, improves retention of children on the ketogenic diet, and serves to optimize the effectiveness of clinical outcomes, which may contribute to the quality of life of children with medically refractory epilepsy.
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.
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.
ObjectiveKetogenic diet (KD) has shown promising efficacy in the treatment of super-refractory status epilepticus (SRSE); however, its adverse effects have not been systematically evaluated. This study aimed to analyze the safety profile of adjunctive KD therapy for SRSE and explore potential risk factors. MethodsProspective data from 13 SRSE patients (3 adolescents, 10 adults; mean age 34.6±18.4 years) at Xuanwu Hospital, Capital Medical University (July 2020–December 2024) who received KD adjunctive therapy after failing conventional treatments were collected. Adverse reactions were observed, and a systematic literature review (up to March 2025) was conducted for meta-analysis. ResultsIn the single-center cohort of 13 patients, common adverse events included gastrointestinal intolerance (53.8%), hematologic and metabolic abnormalities such as thrombocytosis (84.6%), hyperammonemia (76.9%), dyslipidemia (69.2%), and hypocalcemia (69.2%), as well as nutritional deficits including hypoalbuminemia (61.5%), anemia (53.8%), and transient weight loss (61.5%). Most adverse events were transient and reversible with timely adjustments to the KD regimen. The meta-analysis (25 studies, 251 cases; mean age 16.1±19.0 years) revealed a spectrum of major adverse events, including gastrointestinal intolerance (26.7%), hypoglycemia (19.1%), acidosis (17.5%), and hyperlipidemia (12.0%). ConclusionsThe ketogenic diet as adjunctive therapy for super-refractory status epilepticus demonstrates a manageable safety profile. Reported adverse events are primarily confined to gastrointestinal intolerance, metabolic derangements, and nutritional deficits, with notable occurrences of thrombocytosis and hyperammonemia requiring timely clinical management. This study provides critical evidence-based support for KD implementation in SRSE treatment protocols.