| Background:The ketogenic diet (KD) is a kind of special diet, composed by high proportion of fat, low carbohydrate and appropriate amount of protein. Via body metabolism, it is fat that is burned to produce energy and fat can be transferred into ketone bodies. Ketone bodies include acetone, acetoacetate and beta hydroxybutyrate, which can be used by brain as energy, thereby reducing the use of sugar and protein. Refractory childhood epilepsy refers to that, with the formal application of 2 kinds of antiepileptic drugs (AEDs) and the application of sufficient dose and long enough, the attack still cannot be completely controlled. Till now, it is thought that its pathogenesis may be related to neural network reorganization, the expression increase of multidrug resistance (MDR) gene related protein, mitochondrial dysfunction, abnormal signal transduction, autoantibodies producing and so on. At present, KD is mostly used in the treatment of children with refractory epilepsy. Since 1921, KD has been used for the refractory childhood epilepsy in the USA. However, KD couldn’t be widely applied because of differences between Chinese and Western diets. In recent years, there are an increasing number of Chinese epilepsy experts exploring in the area of KD treatment for refractory epilepsy and so, more and more children with refractory epilepsy are treated by KD in China.Objective:Refractory epilepsy children with KD treatment are mainly discussed in this study. We analyzed the seizure frequency change, the improvement of EEG, the adverse reactions and the factors that may cause KD withdrawal and evaluate the efficacy and safety of KD in order to provide a reliable basis for the clinical application, and make it safer and more effective.Methods:This study retrospectively analyzed 126 cases that were treated with Atkins therapy improved by John Hopki hospital (MAD) for refractory childhood epilepsy in our hospital from June 2012 to June 2014. All the children were followed up for more than 1 year and those who stop KD wouldn’t receive the follow up. We observed and described the change of seizure frequency and their EEG, the adverse reactions and the factors that may cause KD withdrawal. Factors that may affect the efficacy of KD were children’s gender, age of onset, course of disease, etiology, change of seizure frequency and EEG, adverse reactions and so on.Results:This study contained a total of 126 cases, and to the end of follow-up, a total of 20 cases exit, and the reasons include that parents not cooperating in 15 cases, recurrent pulmonary infection in 2 cases, antifeedant in 1 case, and attack completely controlled in 2 cases.126 cases included 68 cases of West syndrome,33 cases of Lennox-Gastaut Syndrome,4 of Doose syndrome,4 of Dravet syndrome,5 of tuberous sclerosis,4 of viral encephalitis and 8 of partial epilepsy, with boys 69 cases (54.8%) and girls 57 cases (45.2%). Their ages ranged from 7 to 11 years old, with the average age 4.75 ± 3.02 years old, and they suffered from the disease for 3 months to 120 months. We found that, the possible various factors including gender, age, course of disease, etiology, and the age, initial ratio of KD couldn’t make statistical difference.After 3 months,6 months and 12 months of treatment, the numbers of children with spike wave reduction≥ 50% were respectively 56 (48.7%),74 (68.3%), and 78 (73.6%). With KD treatment for 3 months,6 months,12 months, the difference of the spike wave grade and that of the efficiency were both statistically significant. KD treatment for 3 months and 6 months and 12 months, the difference was statistically significant.After 3 months,6 months and 12 months treatment, in 68 cases of infantile spasms (West syndrome), the data of spike wave reduction ≥50% was 30 (49.2%),40(70.2%),48(76.8%). With KD treatment for 3 months,6 months,12 months, the difference of the spike wave grade and that of the efficiency were both statistically significant. KD treatment for 3 months and 6 months,6months and 12 months, the difference was statistically significant. As to 33 cases of Lennox-Gastaut syndrome (LGS) patients, after 3 months,6 months and 12 months treatment, the data of spike wave reduction≥50% was 16 (51.6%),19(65.5%),20(69.0%). With KD treatment for 3 months,6 months,12 months, the difference of the spike wave grade and that of the efficiency were not statistically significant. KD treatment for 3 months and 12 months, the difference was statistically significant. For 25 cases of Doose syndrome, Dravet syndrome, viral encephalitis and partial epilepsy, after 3 months,6 months and 12 months treatment, the data of spike wave reduction≥ 50% was 10 (43.5%),15(68.2%),15(71.4%). With KD treatment for 3 months,6 months,12 months, the difference of the spike wave grade and that of the efficiency were not statistically significant.The frequency of seizure and the associated factors of spike wave were 0.710 for West syndrome,0.495 for LGS,0.572 for the other 25 cases, and the total 0.606.After 3 months,6 months and 12 months of treatment, the numbers of children with seizure frequency reduction≥ 50% were respectively 44 (38.3%),64 (59.3%), and 58 (54.7%). With KD treatment for 3 months,6 months,12 months, the difference of the seizure grade and that of the efficiency were both statistically significant. KD treatment for 3 months and 6 months and 12 months, the difference was statistically significant.After 3 months,6 months and 12 months treatment, in 68 cases of infantile spasms (West syndrome), the data of seizure reduction≥ 50% was 25 (41.0%),37(64.9%),32(57.1%). The treatment efficiency would reach to the top at the 6th month. With KD treatment for 3 months,6 months,12 months, the difference of the seizure grade wasn’t statistically significant. With KD treatment for 3 months,6 months,12 months, the difference of the efficiency was statistically significant. KD treatment for 3 months and 6 months, the difference was statistically significant.As to 33 cases of Lennox-Gastaut syndrome (LGS) patients, after 3 months,6 months and 12 months treatment, the data of seizure reduction≥50% was 10 (32.3%),17 (58.6%),14 (48.3%). The treatment efficiency would reach to the top at the 6th month. With KD treatment for 3 months,6 months,12 months, the difference of the seizure grade and that of the efficiency were not statistically significant. KD treatment for 3 months and 6 months, the difference was statistically significant.For 25 cases of Doose syndrome, Dravet syndrome, viral encephalitis and partial epilepsy, after 3 months,6 months and 12 months treatment, the difference of the seizure grade and that of the efficiency were not statistically significant. And seizure of 4 patients with Doose syndrome was totally controlled.Conclusion:KD is an effective method in the treatment of children with kinds of refractory epilepsy, and the effect is stable. In control of seizures, KD can also significantly improve the EEG, and this effect may be greater than that of seizure control. There was a certain correlation between the frequency of attack and that of spike wave in the children with refractory epilepsy. The frequency of spike wave could be decreased with the decrease of the frequency of attacks. The early adverse reaction may be a common occurrence, but generally mild and most children were tolerable. |
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