{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T10:14:29Z","timestamp":1790158469101,"version":"4.1.0"},"reference-count":168,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T00:00:00Z","timestamp":1790121600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Developmental and epileptic encephalopathies (DEEs) are severe, early-onset, frequently drug-resistant epilepsies in which recurrent seizures and epileptiform activity contribute to cognitive impairment beyond the underlying etiology. Ketogenic dietary therapies (KDTs) exert broad, multimodal antiseizure and neuroprotective effects and occupy a distinct position among nonpharmacologic treatments for DEEs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To synthesize the evidence for KDT across the principal electroclinical DEE syndromes, an expanding spectrum of gene-specific DEEs, and acute presentations such as febrile infection-related epilepsy syndrome (FIRES) and new-onset refractory status epilepticus (NORSE), with attention to outcome measures, mechanisms, adverse effects, and monitoring.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A narrative review of the literature on the classic ketogenic diet (KD), modified Atkins diet, medium-chain triglyceride diet, and low-glycemic-index treatment in DEEs, drawing on randomized controlled trials, cohort studies, systematic reviews, and meta-analyses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>KDT has the strongest and most methodologically rigorous evidence base in infantile epileptic spasms syndrome, including comparative-effectiveness data vs. hormonal therapy, followed by strong consensus supporting early use in epilepsy with myoclonic-atonic seizures. Meaningful benefit has also been reported in Dravet syndrome and, to a somewhat lesser extent, Lennox\u2013Gastaut syndrome. Responses are more variable in early-infantile developmental and epileptic encephalopathy, epilepsy of infancy with migrating focal seizures, developmental and epileptic encephalopathy with spike-wave activation in sleep, and hemiconvulsion\u2013hemiplegia\u2013epilepsy syndrome, with tolerability and long-term adherence often declining over time. In FIRES\/NORSE, KDT may serve as an acute anti-inflammatory treatment for super-refractory status epilepticus. Gene-specific responses vary substantially, with the most favorable outcomes observed in transporter-related disorders, followed by conditions involving other membrane-associated proteins, including ion channels and neurotransmitter receptors, whereas disorders affecting cellular structural integrity or homeostatic regulation appear less responsive.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>KDT has an increasingly well-supported, syndrome- and endpoint-specific role across DEEs. Future research should prioritize comparative-effectiveness trials, standardized outcome measures, predictors of response, better-tolerated formulations, and equitable access to dietary therapy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2026.1958733","type":"journal-article","created":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T09:57:06Z","timestamp":1790157426000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Ketogenic diet therapy in developmental and epileptic encephalopathies: toward syndrome- and gene-informed precision therapy"],"prefix":"10.3389","volume":"14","author":[{"given":"Debopam","family":"Samanta","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Child Neurology, University of Arkansas for Medical Sciences","place":["Little Rock, AR, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher Paul","family":"Manbeck","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Child Neurology, University of Arkansas for Medical Sciences","place":["Little Rock, AR, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,9,23]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1014","DOI":"10.1111\/epi.18265","article-title":"Operational definition of developmental and epileptic encephalopathies to underpin the design of therapeutic trials","volume":"66","author":"Scheffer","year":"2025","journal-title":"Epilepsia"},{"key":"B2","doi-asserted-by":"publisher","first-page":"1398","DOI":"10.1111\/epi.17241","article-title":"International league against epilepsy classification and definition of epilepsy syndromes with onset in childhood: position paper by the Ilae task force on nosology and 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