Epileptic Syndromes

EPILEPTIC SEIZURES that are of similar type and age of onset and have other similar features (e.g., clinical course, EEG findings, genetic association and neuropathology).

Also Known As:

Epilepsy Syndromes; Familial Epilepsies; Syndromic Epilepsies; Epilepsies, Familial; Epilepsies, Syndromic; Epilepsy Syndrome; Epilepsy, Familial; Epilepsy, Syndromic; Epileptic Syndrome; Familial Epilepsy; Syndromic Epilepsy

Networked: 883 relevant articles (83 outcomes, 79 trials/studies)

Relationship Network

Disease Context: Research Results

Nervous System Diseases: 14178
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Epilepsy: 37791
      - Epileptic Syndromes: 883
        Temporal Lobe Epilepsy: 3740
        Infantile Spasms: 1454
        Myoclonic Epilepsies: 1199
        Absence Epilepsy: 850
        Lennox Gastaut Syndrome: 668
        Frontal Lobe Epilepsy: 158
        Rolandic Epilepsy: 156
        CDKL5 deficiency disorder: 87
        Landau-Kleffner Syndrome: 60

Related Diseases

1.	Epilepsy (Aura)
2.	Seizures (Absence Seizure)
3.	Drug Resistant Epilepsy
4.	Infections
5.	Febrile Seizures (Febrile Seizure)

Experts

1.	Striano, Pasquale: 13 articles (01/2022 - 03/2006)
2.	Lerche, Holger: 13 articles (12/2019 - 01/2005)
3.	Guerrini, Renzo: 12 articles (09/2022 - 10/2002)
4.	Verrotti, Alberto: 10 articles (01/2022 - 11/2005)
5.	Macdonald, Robert L: 10 articles (12/2020 - 07/2002)
6.	Scheffer, Ingrid E: 10 articles (12/2019 - 09/2002)
7.	Kang, Jing-Qiong: 9 articles (01/2021 - 09/2009)
8.	Nabbout, Rima: 9 articles (01/2021 - 03/2013)
9.	Shen, Wangzhen: 9 articles (01/2021 - 10/2010)
10.	Arzimanoglou, Alexis: 9 articles (11/2020 - 09/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Epileptic Syndromes:

1.	Anticonvulsants (Antiepileptic Drugs)IBA 01/01/1999 - "This antiepileptic drug can be a major improvement in treatment of some of the epileptic syndromes and needs further investigation." 01/01/2022 - "To determine the long-term probability of remission without antiepileptic treatment of common epileptic syndromes and of children without a specific syndromic diagnosis. " 10/01/2021 - "However, the detailed phenotype-genotype correlations are unclear, along with the efficacy of various antiepileptic drugs in the treatment of this epilepsy syndrome. " 10/01/2017 - "Efficacy of a third or later antiepileptic drug regimen according to epilepsy syndrome among adult patients." 04/01/2000 - "The selection of antiepileptic drugs is based on efficacy for specific seizure types and epileptic syndromes. "
2.	Valproic Acid (Depakote)FDA LinkGeneric 01/01/2014 - "Valproic acid has demonstrated efficacy in such epileptic syndromes with other medications providing an adjunctive role. " 08/01/2021 - "The high prevalence of epilepsy syndromes amenable to valproate treatment in women of childbearing age and the little information available on the teratogenic potential of alternative treatments have created uncertainty on how to manage these patients. " 01/01/2020 - "Patients were grouped into those currently using valproate (n = 54), those taking drugs other than valproate (n = 47), and drug-naïve patients (n = 51) at the time of MRI, irrespectively of their epilepsy syndrome. " 01/01/2017 - "Sodium valproate is a commonly used antiseizure drug with broad indications for different seizuretypes and epilepsy syndromes. " 12/01/2008 - "Valproate teratogenicity and epilepsy syndrome."
3.	Levetiracetam (Keppra)FDA LinkGeneric 03/01/2017 - "Levetiracetam has a high tolerability and is effective against various seizure types and epilepsy syndromes. " 01/01/2018 - "In our sample, brand-to-generic levetiracetam switching was effective and safe in both monotherapy and polytherapy regardless of the epilepsy syndrome." 01/01/2016 - "However there is still a need for well-designed trials to justify the widespread use of levetiracetam monotherapy in children with specific epilepsy syndromes." 05/01/2011 - "A prospective open-labeled trial with levetiracetam in pediatric epilepsy syndromes: continuous spikes and waves during sleep is definitely a target." 05/01/2010 - "The aim of this study was to evaluate efficacy and tolerability of levetiracetam (LEV) in patients with different epilepsy syndromes. "
4.	Lamotrigine (Lamictal)FDA LinkGeneric 05/01/2001 - "The results of this study contribute to the overall understanding of the spectrum of lamotrigine effectiveness across seizure types and epileptic syndromes. " 05/01/2001 - "Because of the available evidence from controlled studies, indicating the large spectrum of action of lamotrigine, we designed this prospective study to investigate the efficacy and safety of lamotrigine in everyday clinical practice, to collect useful information on its action in specific epilepsy syndromes and on the clinical results of specific co-medications. " 12/01/2016 - "Lamotrigine (LTG) is a second generation anti-epileptic drug (AED) with broad spectrum of activity, a favourable side-effect profile, simpler dosing than earlier drugs and efficacious in diverse epilepsy syndromes. " 11/01/2006 - "Lamotrigine is well-tolerated and secondary aggravation of epileptic syndromes has rarely been reported. " 09/01/2005 - "In people with both epilepsy syndromes who have depression, lamotrigine was treatment of choice. "
5.	CannabidiolIBA 01/01/2020 - "Furthermore, cannabidiol is more effective than placebo, regardless of the etiology of epileptic syndromes and dosage. " 05/01/2019 - "The approval comes at a time of other treatment options also receiving approval (cannabidiol) and several products in ongoing studies (fenfluramine, TAK-935) providing additional treatment options and hope on the horizon for those impacted by this severe epilepsy syndrome." 01/01/2018 - "We begin by exploring the early and somewhat anecdotal evidence that was recently replaced by high-quality data from randomized controlled studies, which subsequently led to the US Food and Drug Administration approval of a purified cannabidiol extract for the treatment of 2 highly refractory pediatric epilepsy syndromes (Dravet and Lennox-Gastaut)." 01/01/2017 - "As studies continue to reveal favorable findings for the use of cannabidiol in the management of childhood epilepsy syndromes and other disorders, best practices for the large-scale production of Cannabis are needed for timely product development and research purposes. " 01/01/2023 - "Cannabidiol may have similar effects in various epilepsy syndromes. "
6.	Topiramate (Topamax)FDA LinkGeneric 04/01/2006 - "As it is not yet wholly clear which specific epilepsy syndromes may benefit most from topiramate with respect to other drugs, more accurate indications for initial monotherapy would require syndrome-oriented trials and more clinical experience." 06/01/2022 - "Topiramate (TPM) is an ASM used in the treatment of various seizure types and in different epileptic syndromes, as well as in other groups of morbidities, and it is available in many generic and similar forms, besides the innovator. " 04/01/2005 - "Efficacy and safety of topiramate in infants according to epilepsy syndromes." 03/01/2003 - "Topiramate: efficacy and tolerability in children according to epilepsy syndromes." 11/01/2005 - "In conclusion, the present long-term study confirms that topiramate represents a useful drug effective in a wide range of seizures and epilepsy syndromes. "
7.	Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link 06/11/2021 - "This review first discusses the effectiveness of anakinra for intractable epileptic syndromes. " 10/01/2019 - "Febrile Infection-Related Epilepsy Syndrome Treated Successfully With Anakinra in a 21-Year-Old Woman." 01/01/2023 - "Long-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra." 12/01/2020 - "Anakinra usage in febrile infection related epilepsy syndrome: an international cohort." 12/01/2016 - "Febrile infection-related epilepsy syndrome treated with anakinra."
8.	CannabinoidsIBA 01/13/2020 - "Whilst the efficacy of cannabinoids in the treatment of some childhood epilepsy syndromes is increasingly recognized, medical cannabinoids may also have potential clinical roles in the treatment of older adults. " 01/01/2022 - "Moreover, sex differences have been proposed in epilepsy syndromes and in cannabinoid action. " 01/01/2021 - "Editorial: Challenges and Conundrums in Cannabinoid-Based Treatments for Epilepsy Syndromes and Associated Neurobehavioral Comorbidities." 01/01/2018 - "Existing RCT evidence is mostly in paediatric samples with rare and severe epilepsy syndromes; RCTs examining other syndromes and cannabinoids are needed. " 01/01/2018 - "Over the past decade there has been an increasing interest in using cannabinoids to treat a range of epilepsy syndromes following reports of some remarkable responses in individual patients. "
9.	Adrenal Cortex Hormones (Corticosteroids)IBA 09/01/2014 - "The efficacy of corticosteroid on ESES suppression, seizure control of three epilepsy syndrome were analyzed. " 08/01/2009 - "Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep." 09/01/2015 - "Many different corticosteroid treatment schedules have been used in order to treat refractory epileptic seizures with encouraging effects on seizure reduction in many epileptic syndromes. " 03/01/2008 - "Corticosteroids have been used only in the treatment of special epileptic syndromes or epileptic encephalopathies, such as infantile spasms. " 08/01/2009 - "To assess the efficacy and tolerability of steroids in epileptic syndromes with continuous spike-waves during slow-wave sleep (CSWS), charts of 44 children (25 boys) who received corticosteroids for cognitive and/or behavioral deterioration associated with CSWS were retrospectively reviewed. "
10.	perampanelIBA 01/01/2019 - "The efficacy, safety, and tolerability of perampanel have been reproduced in real-world clinical practice, and the agent has been shown to be effective in other epilepsy syndromes. " 01/01/2016 - "These findings suggest that perampanel is safe, well-tolerated, and effective in treatment of various types of adult and pediatric epilepsy syndromes. " 01/01/2016 - "Safety and efficacy of perampanel in children and adults with various epilepsy syndromes: A single-center postmarketing study." 08/08/2016 - "Partial onset epilepsy was the predominant epilepsy syndrome treated with perampanel. " 01/01/2021 - "We describe perampanel as an adjunctive treatment for FIRES (febrile infection-related epilepsy syndrome) and other drug-resistant epilepsies. "

Therapies and Procedures

1.	Therapeutics 09/01/2005 - "However, compared to the new AEDs, VNS Therapy has similar efficacy results in clinical trials and in many epilepsy syndromes and the long-term efficacy results are even more positive, with continued improvement in seizure reduction for up to two years. " 08/01/2010 - "First, specific EEG abnormalities help characterize the seizure type and epilepsy syndrome, which allows more informed decisions regarding therapy and more accurate prediction of seizure control and ultimate remission. " 01/01/2015 - "Immune-modulating therapies along with the administration of TPM effectively treated this severe epileptic syndrome in our patient." 09/01/1998 - "To study the medical therapy according to epileptic syndromes in the everyday practice of a hospital based outpatient neuropediatric clinic. " 06/01/1989 - "Development of effective therapy for these intractable epileptic syndromes will be an important subject of future studies."
2.	Ketogenic Diet 07/01/2012 - "Is the ketogenic diet (KD) more effective in certain epilepsy syndromes? " 07/01/2012 - "Is the ketogenic diet effective in specific epilepsy syndromes?" 10/01/2011 - "In this Argentinean retrospective, collaborative, multicenter study, we examine the efficacy and tolerability of the ketogenic diet (KD) for different epilepsy syndromes. " 06/01/2010 - "Although there are few data regarding the use of the ketogenic diet in the treatment of adult epilepsy syndromes, it may be an option for the treatment of adults with refractory, prolonged SE." 11/01/2008 - "The ketogenic diet (KD) has been used successfully in a variety of epilepsy syndromes. "
3.	Vagus Nerve Stimulation 01/01/2009 - "The efficacy of surgery ranges from curative (resection) to palliative (vagus nerve stimulation, callosotomy, multiple subpial transections) depending on the epilepsy syndrome and etiology. " 12/06/2020 - "In addition, implanted vagus nerve stimulation had different effects on epilepsy syndromes. " 01/01/2009 - "Vagus nerve stimulation in pediatric epileptic syndromes." 01/01/2020 - "Background: Vagus nerve stimulation (VNS) is a palliative treatment for medical intractable epileptic syndromes not eligible for resective surgery. " 12/06/2020 - "To identify the safety and efficacy of vagus nerve stimulation in children with intractable epilepsy and analyze the effects on different epilepsy syndromes. "
4.	Hemispherectomy 06/01/2020 - "Although hemispherectomy is an effective treatment for children with intractable hemispheric epilepsy syndromes, as many as 40% of patients eventually develop seizure recurrence. " 01/01/2018 - "Hemispherectomy remains the only treatment that leads to seizure freedom for this refractory epileptic syndrome. " 10/01/2014 - "While seizure outcomes in pediatric epilepsy surgery may be improved by the use of high-resolution neuroimaging and invasive electrographic studies, a more aggressive resection should be considered in certain patients, including hemispherectomy if a hemispheric epilepsy syndrome is suspected. " 12/01/2011 - "In this study we report the clinical outcomes of hemispherectomy for epilepsy in pediatric patients with special emphasis on the epileptic syndromes and their etiologies. " 12/01/2011 - "Hemispherectomy in pediatric patients with epilepsy: a study of 45 cases with special emphasis on epileptic syndromes."
5.	Precision Medicine 01/01/2019 - "In this study, we highlight the importance of earlier identification of the genetic etiology of non-familial epilepsies, which leads us to the best treatment options in terms of precision medicine and to future neurobiological research for novel drug development. " 10/01/2020 - "It could also provide insights into precision medicine approaches to the treatment of epileptic syndromes. " 10/01/2018 - "Some of the compounds in development target the etiology of specific epilepsy syndromes (including orphan conditions) through precision medicine, and some offer hope of producing disease-modifying effects rather than symptomatic seizure suppression. " 01/01/2023 - "It will also determine the long-term efficacy of the treatment of seizures and the development of precision medicine for epilepsy syndromes due to gain-of-function variants. " 01/01/2022 - "Such a molecule provides a precision-medicine approach to target SCN8A-related epilepsy syndromes (SCN8A-RES), where gain-of-function (GoF) mutations lead to excess NaV1.6 sodium current, or other indications where NaV1.6 mediated hyper-excitability contributes to disease (Gardella and Møller, 2019; Johannesen et al., 2019; Veeramah et al., 2012). "