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  • Li, Hua  (3)
  • Yu, Mei  (3)
  • 1
    In: Acta Epileptologica, Springer Science and Business Media LLC, Vol. 4, No. 1 ( 2022-12)
    Type of Medium: Online Resource
    ISSN: 2524-4434
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2022
    detail.hit.zdb_id: 3006206-8
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  • 2
    In: Acta Epileptologica, Springer Science and Business Media LLC, Vol. 4, No. 1 ( 2022-12)
    Abstract: Ketogenic diet (KD) therapy is one of the main treatments for drug-resistant epilepsy. However, the KD therapy has been applied in only a small number of infantile spasm cases. In this large multicenter study, we investigated the efficacy of KD therapy in the treatment of infantile spasms. Methods In this retrospective, multicenter cohort study, clinical data from main epilepsy centers were analyzed. Patients were classified into different groups according to age, type of drug and whether glucocorticoid was used before initiation of KD. Results From October 2014 to March 2020, 481 patients (308 males and 173 females) with infantile spasms were treated with the KD therapy. The age of the patients ranged from 2 months to 20 years, with a mean age of 1 year and 10 months. The number of anti-seizure medications (ASMs) used before KD initiation ranged 0–6, with a median of 3. In different time from initiation(1, 3, 6, and 12 months), the rates of seizure freedom after KD were 6.9, 11.6, 16.0 and 16.8%, respectively ( χ 2  = 27.1772, P   〈  0.0001). There was a significant difference in the rate of seizure freedom between 3 months and 1 month ( χ 2  = 6.5498, P  = 0.0105) groups, and 6 months and 3 months ( χ 2  = 3.8478, P  = 0.0498) groups, but not between 12 months and 6 months ( χ 2  = 0.1212, P  = 0.7278) groups. The rates of effectiveness were 44.7, 62.8, 49.1 and 32.0% ( χ 2  = 93.2674, P   〈  0.0001), respectively. The retention rates were 94.0, 82.5, 55.7 and 33.1% ( χ 2  = 483.7551, P   〈  0.0001), correspondingly. The rate of effectiveness and the retention rate of KD were significantly different among the 1, 3, 6 and 12 months. KD treatment was the first choice in 25 patients (5.2%), 55 patients (11.4%) started KD after the failure of the first ASM, 158 patients (32.8%) started KD after the failure of the second ASM, 157 patients (32.6%) started KD after the failure of the third drug, and 86 patients (17.9%) started KD after the failure of the fourth and more. The KD effect was not related to the number of ASMs used before KD startup ( P   〉  0.05). Two hundred and eighteen patients (45.3%) failed to respond to corticotropin or glucocorticoid before initiation. There was no significant difference in the effectiveness rate at different time points between the group of KD therapy after glucocorticoid failure and the group after non-hormone failure ( χ 2  = 0.8613, P  = 0.8348). The rate of adverse events of KD in 1, 3, 6, and 12 months after KD initiation were 22.3, 21.7, 16.8 and 6.9%, respectively. The adverse events mainly occurred during the first 3 months of KD, and the main adverse events were gastrointestinal disturbance and constipation. Conclusions The efficacy of the KD treatment for infantile spasms was not affected by age, medication, and glucocorticoid use before initiation. KD is one of the effective treatments for infantile spasms. Trial registration ChiCTR-IIR-16008342. Registered on 22 April, 2016 - Retrospectively registered, https://www.chictr.org.cn .
    Type of Medium: Online Resource
    ISSN: 2524-4434
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2022
    detail.hit.zdb_id: 3006206-8
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  • 3
    Online Resource
    Online Resource
    Frontiers Media SA ; 2022
    In:  Frontiers in Neurology Vol. 13 ( 2022-8-1)
    In: Frontiers in Neurology, Frontiers Media SA, Vol. 13 ( 2022-8-1)
    Abstract: The ketogenic diet (KD) is increasingly used to treat drug-resistant epilepsy because of its favorable effect on seizure reduction. Patients with mitochondrial diseases tend to experience seizures. Therefore, this study aimed to test the efficacy of the KD on participants with mitochondrial diseases in a controlled trial. Methods Participants from fourteen clinical centers who were diagnosed with mitochondrial disease were semi-randomized to either the intervention (KD) or control group. The KD group followed a 3-month KD intervention, while the control group received a 1-month normal diet initially and then a 3-month KD intervention. The primary outcome measure was seizure reduction. Biomarker changes, cognitive impairments, and side effects were also recorded, if available. Result A total of 33 participants were assigned to the KD ( n = 22) and control groups ( n = 11). In the KD group, 31.8% (7/22) of participants achieved ≥50% seizure reduction after 1 month of diet intervention, which increased to 40.9% (9/22) at 3 months. In the control group, only 18.2% (2/11) of the participants had ≥50% seizure reduction during the normal diet period. After the control group was transferred to the KD, 63.6% (7/11) of participants had & gt;50% seizure reduction, and this rate increased to 72.7% (8/11) at 3 months. The KD also showed high efficacy in participants with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) or pathogenic variants in mitochondrial DNA (mtDNA) (90% and 93.3% response rates, respectively). The most frequent side effects reported at the 3-month review were vomiting, cold, hyperlipidemia, and bloating. Conclusion The KD is a safe and effective therapy for seizure control in mitochondrial diseases, especially MELAS and pathogenic variants of mtDNA. KD intervention can be considered in the management of these patients.
    Type of Medium: Online Resource
    ISSN: 1664-2295
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2564214-5
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