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.
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.
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.
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.
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.
ObjectiveTo investigate the current situation of ketogenic diet treatment centers in China, including the target, quantity, indication diseases, staffing and regional distribution of ketogenic diet therapy centers.MethodsMembers from the China Association Against Epilepsy (CAAE) received an investigation on the development situation of ketogenic diet therapy in their own epilepsy centers through an online survey. ResultsAt present, there are 91 centers for ketogenic diet therapy in China, and among them, there are 74 centers (81.32%) only treat pediatric patients. 59 centers of them (64.83%) were established between 2010 and 2020; patients of 63 centers (69.23%) have been treated with ketogenic therapy for status epilepticus (SE), among which only 9 centers (14.29%) have 5 or more epileptic patients on averge per month. 35 centers (38.46%) are offering ketogenic diet treatment for indications other than epilepsy. The main diseases are autism spectrum disorder, encephalitis, febrile infection related epilepsy syndrome (FIRES), rare diseases and glycolipid metabolic diseases. All ketogenic diet treatment centers are equipped with high-quality professionals, mainly neurologists and pediatric physicians; There are significant differences in regional distribution, with centers in the eastern region accounting for more than half (53.85%). ConclusionsThe development of ketogenic diet therapy in China has achieved initial results, but there are still some problems such as small number of patients treated and uneven geographical distribution.
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 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.
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.
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.