Brodie MJ, Shorvon SD, Canger R, Halász P, Johannessen S, Thompson P, et al. Commission on European Affairs: appropriate standards of epilepsy care across Europe. ILEA Epilepsia. 1997;38:1245–50.

Article  PubMed  Google Scholar 

Chen Z, Brodie MJ, Liew D, Kwan P. Treatment outcomes in patients with newly diagnosed epilepsy treated with established and new antiepileptic drugs: a 30-year longitudinal cohort study. JAMA Neurol. 2018;75:279–86.

Article  PubMed  Google Scholar 

Victor TR, Hage Z, Tsirka SE. Prophylactic administration of cannabidiol reduces microglial inflammatory response to kainate-induced seizures and neurogenesis. Neuroscience. 2022;500:1–11.

Article  PubMed  Google Scholar 

Sills GJ, Rogawski MA. Mechanisms of action of currently used antiseizure drugs. Neuropharmacology. 2020;168:107966.

Article  PubMed  Google Scholar 

Villasana-Salazar B, Vezzani A. Neuroinflammation microenvironment sharpens seizure circuit. Neurobiol Dis. 2023;178:106027.

Article  PubMed  Google Scholar 

Prabowo AS, Anink JJ, Lammens M, Nellist M, van den Ouweland AM, Adle-Biassette H, et al. Fetal brain lesions in tuberous sclerosis complex: TORC1 activation and inflammation. Brain pathology (Zurich, Switzerland). 2013;23:45–59.

Article  PubMed  Google Scholar 

Vezzani A. Anti-inflammatory drugs in epilepsy: does it impact epileptogenesis? Expert Opin Drug Saf. 2015;14:583–92.

Article  PubMed  Google Scholar 

Falsaperla R, Collotta AD, Marino SD, Sortino V, Leonardi R, Privitera GF, et al. Drug resistant epilepsies: a multicentre case series of steroid therapy. Seizure Eur J Epilepsy. 2024;117:115–25.

Article  Google Scholar 

Grosso S, Farnetani M, Mostardini R, Cordelli D, Berardi R, Balestri P. A comparative study of hydrocortisone versus deflazacort in drug-resistant epilepsy of childhood. Epilepsy Res. 2008;81:80–5.

Article  PubMed  Google Scholar 

Marchi N, Granata T, Freri E, Ciusani E, Ragona F, Puvenna V, et al. Efficacy of anti-inflammatory therapy in a model of acute seizures and in a population of pediatric drug resistant epileptics. PLoS ONE. 2011;6:e18200.

Article  PubMed  PubMed Central  Google Scholar 

Rangarajan A, Mundlamuri RC, Kenchaiah R, Prathyusha PV, Viswanathan LG, Asranna A, et al. Efficacy of pulse intravenous methylprednisolone in epileptic encephalopathy: a randomised controlled trial. J Neurol Neurosurg Psychiatry. 2022;93:1299–305.

PubMed  Google Scholar 

Eyo UB, Peng J, Swiatkowski P, Mukherjee A, Bispo A, Wu LJ. Neuronal hyperactivity recruits microglial processes via neuronal NMDA receptors and microglial P2Y12 receptors after status epilepticus. J Neurosci Official J Soc Neurosci. 2014;34:10528–40.

Article  Google Scholar 

Vezzani A, Viviani B. Neuromodulatory properties of inflammatory cytokines and their impact on neuronal excitability. Neuropharmacology. 2015;96:70–82.

Article  PubMed  Google Scholar 

Choi J, Nordli DR Jr, Alden TD, DiPatri A Jr, Laux L, Kelley K, et al. Cellular injury and neuroinflammation in children with chronic intractable epilepsy. J Neuroinflammation. 2009;6:38.

Article  PubMed  PubMed Central  Google Scholar 

Okuneva O, Körber I, Li Z, Tian L, Joensuu T, Kopra O, et al. Abnormal microglial activation in the Cstb−/− mouse, a model for progressive myoclonus epilepsy, EPM1. Glia. 2015;63:400–11.

Article  PubMed  Google Scholar 

Pascual O, Ben Achour S, Rostaing P, Triller A, Bessis A. Microglia activation triggers astrocyte-mediated modulation of excitatory neurotransmission. Proc Natl Acad Sci U S A. 2012;109:E197-205.

Article  PubMed  Google Scholar 

Dos-Santos-Pereira M, Guimarães FS, Del-Bel E, Raisman-Vozari R, Michel PP. Cannabidiol prevents LPS-induced microglial inflammation by inhibiting ROS/NF-κB-dependent signaling and glucose consumption. Glia. 2020;68:561–73.

Article  PubMed  Google Scholar 

Wechsler RT, Burdette DE, Gidal BE, Hyslop A, McGoldrick PE, Thiele EA, et al. Consensus panel recommendations for the optimization of EPIDIOLEX® treatment for seizures associated with Lennox-Gastaut syndrome, Dravet syndrome, and tuberous sclerosis complex. Epilepsia Open. 2024;9:1632–42.

Article  PubMed  PubMed Central  Google Scholar 

Wirrell EC, Lagae L, Scheffer IE, Cross JH, Specchio N, Strzelczyk A. Practical considerations for the use of fenfluramine to manage patients with Dravet syndrome or Lennox-Gastaut syndrome in clinical practice. Epilepsia Open. 2024;9:1643–57.

Article  PubMed  PubMed Central  Google Scholar 

Cha J, Filatov G, Smith SJ, Gammaitoni AR, Lothe A, Reeder T. Fenfluramine increases survival and reduces markers of neurodegeneration in a mouse model of Dravet syndrome. Epilepsia Open. 2024;9:300–13.

Article  PubMed  Google Scholar 

Wirrell EC, Hood V, Knupp KG, Meskis MA, Nabbout R, Scheffer IE, et al. International consensus on diagnosis and management of Dravet syndrome. Epilepsia. 2022;63:1761–77.

Article  PubMed  PubMed Central  Google Scholar 

Craner MJ, Damarjian TG, Liu S, Hains BC, Lo AC, Black JA, et al. Sodium channels contribute to microglia/macrophage activation and function in EAE and MS. Glia. 2005;49:220–9.

Article  PubMed  Google Scholar 

Boerma RS, Braun KP, van de Broek MPH, van Berkestijn FMC, Swinkels ME, Hagebeuk EO, et al. Remarkable phenytoin sensitivity in 4 children with SCN8A-related epilepsy: a molecular neuropharmacological approach. Neurotherapeutics. 2016;13:192–7.

Article  PubMed  Google Scholar 

Makridis KL, Friedo AL, Kellinghaus C, Losch FP, Schmitz B, Boßelmann C, Kaindl AM. Successful treatment of adult Dravet syndrome patients with cenobamate. Epilepsia. 2022;63(12):e164–71.

Article  PubMed  Google Scholar 

Anderson LL, Hawkins NA, Thompson CH, Kearney JA, George AL Jr. Unexpected efficacy of a novel sodium channel modulator in Dravet syndrome. Sci Rep. 2017;7:1682.

Article  PubMed  PubMed Central  Google Scholar 

Zelenka L, Pägelow D, Krüger C, Seele J, Ebner F, Rausch S, et al. Novel protocol for the isolation of highly purified neonatal murine microglia and astrocytes. J Neurosci Methods. 2022;366:109420.

Article  PubMed  Google Scholar 

Boccazzi M, Van Steenwinckel J, Schang AL, Faivre V, Le Charpentier T, Bokobza C, et al. The immune-inflammatory response of oligodendrocytes in a murine model of preterm white matter injury: the role of TLR3 activation. Cell Death Dis. 2021;12:166.

Article  PubMed  PubMed Central  Google Scholar 

Bokobza C, Joshi P, Schang AL, Csaba Z, Faivre V, Montané A, et al. MiR-146b protects the perinatal brain against microglia-induced hypomyelination. Ann Neurol. 2022;91:48–65.

Article  PubMed  Google Scholar 

Livak KJ, Schmittgen TD. Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) method. Methods. 2001;25:402–8.

Article  PubMed  Google Scholar 

Vezzani A, Balosso S, Ravizza T. Neuroinflammatory pathways as treatment targets and biomarkers in epilepsy. Nat Rev Neurol. 2019;15:459–72.

Article  PubMed  Google Scholar 

Zographos GA, Russ-Hall SJ, Scheffer IE. Does long-term phenytoin have a place in Dravet syndrome? Ann Clin Transl Neurol. 2022;9:2036–40.

Article  PubMed  PubMed Central  Google Scholar 

Paolicelli RC, Sierra A, Stevens B, Tremblay M-E, Aguzzi A, Ajami B, et al. Microglia states and nomenclature: a field at its crossroads. Neuron. 2022;110:3458–83.

Article  PubMed  PubMed Central  Google Scholar 

Pinto MV, Fernandes A. Microglial phagocytosis-rational but challenging therapeutic target in multiple sclerosis. Int J Mol Sci. 2020. https://doi.org/10.3390/ijms21175960.

Article  PubMed  PubMed Central  Google Scholar 

Guerrini R, Conti V, Mantegazza M, Balestrini S, Galanopoulou AS, Benfenati F. Developmental and epileptic encephalopathies: from genetic heterogeneity to phenotypic continuum. Physiol Rev. 2023;103:433–513.

Article  PubMed