ArticleTherapeutic advances in neurological disorders2020
Effective and safe diet therapies for Lennox-Gastaut syndrome with mitochondrial dysfunction.
Article in Therapeutic advances in neurological disorders, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 1 of them a synthesis that pooled it.
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Who cites it
18 citing papers in PubMed, 1 synthesis or guideline pooled it, 29 citations in OpenAlex.
- Efficacy and safety of corpus callosotomy and ketogenic diet in children with Lennox Gastaut syndrome: a systematic review and meta-analysis.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2021Pooled it
- Mitochondrial quality control in health and disease: Updates 2026.Chinese medical journal · 2026Review
- Role of Diabetes and its metabolic pathways in Epilepsy: An insight to various target approaches.Naunyn-Schmiedeberg's archives of pharmacology · 2025Review
- Non-Pharmacological Treatment Methods of Lennox-Gastaut Syndrome-Review of the Literature.Biomedicines · 2025Review
- Clinical Efficacy and Safety of the Ketogenic Diet in Patients with Genetic Confirmation of Drug-Resistant Epilepsy.Nutrients · 2025Review
- Therapeutic Approach to Epilepsy in Patients with Mitochondrial Diseases.Yonsei medical journal · 2025Review
- Progress of ketogenic diet in the treatment of developmental epileptic encephalopathy.Frontiers in pediatrics · 2025Review
- Diagnosis and Management of Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes Syndrome.Biomolecules · 2024Review
- Efficacy and Safety of Ketogenic Diet Treatment in Pediatric Patients with Mitochondrial Disease.Nutrients · 2024Article
- Expanded clinical phenotype and untargeted metabolomics analysis in RARS2-related mitochondrial disorder: a case report.BMC neurology · 2024Article
- Therapeutic outcome of patients with Lennox-Gastaut syndrome with mitochondrial respiratory chain complex I deficiency.Frontiers in neurology · 2024Article
- Long-term ketogenic diet therapy improves mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS): A case report.CNS neuroscience & therapeutics · 2023Article
- Review
- Treatment strategies for Lennox-Gastaut syndrome: outcomes of multimodal treatment approaches.Therapeutic advances in neurological disorders · 2022Article
- Effective application of corpus callosotomy in pediatric intractable epilepsy patients with mitochondrial dysfunction.Therapeutic advances in neurological disorders · 2022Article
- Dietary Regulation of Oxidative Stress in Chronic Metabolic Diseases.Foods (Basel, Switzerland) · 2021Review
- Epilepsy in Mitochondrial Diseases-Current State of Knowledge on Aetiology and Treatment.Children (Basel, Switzerland) · 2021Review
- Association Between Epilepsy and Leigh Syndrome WithFrontiers in neurology · 2021Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundLennox-Gastaut syndrome (LGS) is a typical intractable form of epilepsy that most often occurs between the second and sixth year of life. This study aimed to evaluate the clinical efficacy and safety of ketogenic diet therapies (DTs) for LGS with mitochondrial dysfunction.
methodsThis was a retrospective study involving 20 LGS patients with mitochondrial dysfunction who received several DTs from 2004 to 2014 at a single tertiary care center. Seizure reduction rate, cognitive function, retention rate, electroencephalography (EEG) changes, and adverse effects were examined before and after DTs.
resultsThe retention rates at 1 and 2 years after initiation of DTs were 45% and 40%, respectively. After 1-year follow up, we observed seizure freedom in two patients, 75% seizure reduction in two patients, 50% reduction in three patients, and 25% reduction in one patient. After 2-year follow up, the outcomes were seizure freedom in two patients, 90% seizure reduction in one patient, 75% reduction in two patients, and 50% reduction in two patients. EEG findings improved in nine patients. Nine patients were treated with DTs for 1 year; all patients demonstrated improved cognitive status. Eight patients were treated with DTs for 2 years, of whom seven had improved cognitive status. Poor tolerability of DTs was due to poor oral intake and gastrointestinal problems.
conclusionsWe demonstrate that, in LGS with mitochondrial dysfunction, improvement of seizures and cognitive function are not inferior to those in other patients treated with DTs. This study showed that DTs are efficacious and feasible for LGS patients with mitochondrial dysfunction and can significantly improve their prognosis.
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