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Journal articles on the topic 'Epileptic Syndromes'

Author: Grafiati

Published: 7 June 2025

Last updated: 2 August 2025

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1

Khan, Sonia, and Raidah Al Baradie. "Epileptic Encephalopathies: An Overview." Epilepsy Research and Treatment 2012 (November 20, 2012): 1–8. http://dx.doi.org/10.1155/2012/403592.

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Epileptic encephalopathies are an epileptic condition characterized by epileptiform abnormalities associated with progressive cerebral dysfunction. In the classification of the International League Against Epilepsy eight age-related epileptic encephalopathy syndromes are recognized. These syndromes include early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sle

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2

Eadie, M. J. "Epileptic syndromes." Medical Journal of Australia 148, no. 8 (1988): 404. http://dx.doi.org/10.5694/j.1326-5377.1988.tb115967.x.

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3

Blinov, D. V. "Epilepsy syndromes: the 2022 ILAE definition and classification." Epilepsy and paroxysmal conditions 14, no. 2 (2022): 101–82. http://dx.doi.org/10.17749/2077-8333/epi.par.con.2022.123.

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Until recently, no classification of epileptic syndromes officially approved by the International League Against Epilepsy (ILAE) was available. In 2022, owing to many year-persistent efforts made by experts and the public community, the studies providing the definition and classification of epileptic syndromes were published. ILAE has approved the following definition of the epileptic syndrome: “a characteristic cluster of clinical and EEG features, often supported by specific etiological findings (structural, genetic, metabolic, immune, and infectious)”. The classification of epileptic syndro

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4

Stawicka, Elżbieta, Paulina Górka-Skoczylas, and Dorota Hoffman-Zacharska. "A new look at the clinical and molecular characteristics of SCN1A-related developmental and epileptic encephalopathies." Aktualności Neurologiczne 22, no. 2 (2022): 93–98. http://dx.doi.org/10.15557/an.2022.0011.

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SCN1A-related diseases are a heterogeneous group of disorders with an expanding spectrum of phenotypes. Until recently, mutations in this gene were associated with epileptic syndromes and epileptic and developmental encephalopathy – Dravet syndrome, which was contrasted with a new group of early-onset syndromes, non-Dravet developmental and epileptic encephalopathies (DEEs; OMIM: PS308350). The aim of this paper is to review published data on the phenotypic variability of SCN1A-related developmental and epileptic encephalopathies, particularly non-Dravet syndromes. These are disorders with ver

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5

Wirrell, Elaine, Kevin Farrell, and Sharon Whiting. "The Epileptic Encephalopathies of Infancy and Childhood." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 32, no. 4 (2005): 409–18. http://dx.doi.org/10.1017/s0317167100004388.

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ABSTRACT:The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usually result in intractable epilepsy. While some syndromes are relatively easily diagnosed early in their course, others take time to evolve, hampering an early, confident diagnosis. Epileptic encephalopathies are associated with slowing of cognitive function and evolution of severe behavioral disorders, which are often more distressing to families than the epilepsy. While an underlying etiology may explain some of this co-morbidity, many children have no i

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6

Benbadis, Selim R. "Neglecting Epileptic Syndromes." Journal of Clinical Neurophysiology 13, no. 5 (1996): 458. http://dx.doi.org/10.1097/00004691-199609000-00094.

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7

Wallace, S. J. "Childhood epileptic syndromes." Lancet 336, no. 8713 (1990): 486–88. http://dx.doi.org/10.1016/0140-6736(90)92024-c.

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8

Cerullo, Angelina, Carla Marini, Roberta Carcangiu, Agostino Baruzzi, and Paolo Tinuper. "Clinical and video‐polygraphic features of epileptic spasms in adults with cortical migration disorder." Epileptic Disorders 1, no. 1 (1999): 27–33. http://dx.doi.org/10.1684/j.1950-6945.1999.tb00290.x.

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ABSTRACT The International Classification of Epileptic Syndromes considers epileptic spasms to be typical seizures of West syndrome. Literature reports show that spasms are present in epileptic syndromes other than West syndrome but there are few data on their characteristics in adults. We describe ictal, clinical and video‐polygraphic findings in three patients (aged 21, 32 and 57 years) with epileptic spasms and with diffuse (case 2), focal right fronto‐parietal (case 1) and bi‐opercular (case 3) pachygyria. Spasms had been present since the ages of 1 month, 11 and 27 years respectively. Onl

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9

Bhardwaj, Ankit, Atma Ram Sharma, and Sarla Sharma. "Cefixime induce non convulsive status epileptics: a neurotoxic effect." International Journal of Basic & Clinical Pharmacology 8, no. 10 (2019): 2341. http://dx.doi.org/10.18203/2319-2003.ijbcp20194284.

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Nonconvulsive status epileptics comprises a group of syndromes that display a great diversity regarding response to anticonvulsants ranging from virtually self-limiting variants to entirely refractory forms cephalosporins are thought to provoke seizure through inhibitory effects on gamma-aminobutyric acid (GABA) transmission and GABA receptors. Interference with GABA transmission result in pre-disposition towards excitatory neurotransmission, which can leads to seizures. Antibiotics can alter the serum concentration of anti-epileptic, resulting in seizures and anti-epileptic drugs toxicity.

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10

Wong-Kisiel, Lily C., and Katherine Nickels. "Electroencephalogram of Age-Dependent Epileptic Encephalopathies in Infancy and Early Childhood." Epilepsy Research and Treatment 2013 (August 19, 2013): 1–18. http://dx.doi.org/10.1155/2013/743203.

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Epileptic encephalopathy syndromes are disorders in which the epileptiform abnormalities are thought to contribute to a progressive cerebral dysfunction. Characteristic electroencephalogram findings have an important diagnostic value in classification of epileptic encephalopathy syndromes. In this paper, we focus on electroencephalogram findings of childhood epileptic encephalopathy syndromes and provide sample illustrations.

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11

Sharma, Suvasini, and Manjari Tripathi. "Ketogenic Diet in Epileptic Encephalopathies." Epilepsy Research and Treatment 2013 (July 10, 2013): 1–5. http://dx.doi.org/10.1155/2013/652052.

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The ketogenic diet is a medically supervised high-fat, low-carbohydrate diet that has been found useful in patients with refractory epilepsy. It has been shown to be effective in treating multiple seizure types and epilepsy syndromes. In this paper, we review the use of the ketogenic diet in epileptic encephalopathies such as Ohtahara syndrome, West syndrome, Dravet syndrome, epilepsy with myoclonic atonic seizures, and Lennox-Gastaut syndrome.

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12

Ricard‐Mousnier, Brigitte, Sylvie N'Guyen, Frédéric Dubas, François Pouplard, and Agnès Guichet. "Ring chromosome 17 epilepsy may resemble that of ring chromosome 20 syndrome." Epileptic Disorders 9, no. 3 (2007): 327–31. http://dx.doi.org/10.1684/epd.2007.0121.

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ABSTRACT A four‐year‐old boy with ring chromosome 17 presenting with early‐onset, pharmacoresistant epilepsy underwent repeated 24‐hour video‐EEG monitoring and cytogenetic analyses, including fluorescent in situ hybridization with telomeric and locus‐specific probes of chromosome 17. Epilepsy was characterized by nocturnal motor seizures and by prolonged diurnal electrical status epilepticus. The 46, XY, r (17) karyotype was observed in the majority of cell lines. Fluorescent in situ hybridization revealed a deletion at the 17p telomere on the ring chromosome, whereas the 17q telomere and the

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13

Mindruta, Ioana, Alina Dimitriu, and Laura Craciun. "Insights in classification of seizures and epilepsies – review of key concepts throughout history." Romanian Journal of Neurology 10, no. 4 (2011): 161–71. http://dx.doi.org/10.37897/rjn.2011.4.1.

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Since the Babylonian era up to nowadays, the large complexity of epileptic disorders made the attempt of classifying and organizing concepts one of the most challenging. The 1981 classification was a revision of the one devised by Henri Gastaut for the ILAE and published in 1970. The distinction between simple and complex partial seizures represented a significant difference. In 1989 the report of ILAE classification and terminology task force introduced the concept of epileptic syndrome. An epileptic syndrome is defined by the sum of signs and symptoms that tend to occur together, not reflect

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14

Ohtsuka, Yoko. "West Syndrome and its Related Epileptic Syndromes." Epilepsia 39, S5 (1998): 30–37. http://dx.doi.org/10.1111/j.1528-1157.1998.tb05147.x.

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15

Chadwick, D. "Epileptic Seizures and Syndromes." Journal of Neurology, Neurosurgery & Psychiatry 60, no. 2 (1996): 248. http://dx.doi.org/10.1136/jnnp.60.2.248-a.

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16

Benbadis, Selim R. "Epileptic seizures and syndromes." Neurologic Clinics 19, no. 2 (2001): 251–70. http://dx.doi.org/10.1016/s0733-8619(05)70018-9.

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17

Niedermeyer, E. "Epileptic seizures and syndromes." Electroencephalography and Clinical Neurophysiology 95, no. 2 (1995): 145. http://dx.doi.org/10.1016/0013-4694(95)90533-2.

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18

Aicardi, Jean. "Epileptic Syndromes in Childhood." Epilepsia 29, s3 (1988): S1—S5. http://dx.doi.org/10.1111/j.1528-1157.1988.tb05803.x.

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19

Gram, L. "Epileptic seizures and syndromes." Lancet 336, no. 8708 (1990): 161–63. http://dx.doi.org/10.1016/0140-6736(90)91672-w.

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20

O'Donohoe, Niall V. "Delineation of epileptic syndromes." Current Paediatrics 2, no. 2 (1992): 68–72. http://dx.doi.org/10.1016/0957-5839(92)90125-b.

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21

Chowdhury, Nodee, Atma Ram Bansal, Rajeev Goyal, and Gowathi Nikhila. "Cerebral dominance in an unusual case of Landau-Kleffner syndrome." BMJ Case Reports 14, no. 12 (2021): e246696. http://dx.doi.org/10.1136/bcr-2021-246696.

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Landau-Kleffner syndrome (LKS) is described by the International Classification of Epileptic Syndromes since 1985 as a constellation of clinical and electrographic signs, including acquired aphasia, regression of language milestones and seizures, along with sleep-activated paroxysms on electroencephalogram which can progress to electrographic status epilepticus of sleep. In this case, a 7-year-old boy presented with an atypical history of new-onset aphasia and regression of language milestones with rare seizures. However, there was an electrographic mismatch in the form of right-sided epilepti

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22

Porter, Roger J. "Recognizing and Classifying Epileptic Seizures and Epileptic Syndromes." Neurologic Clinics 4, no. 3 (1986): 495–508. http://dx.doi.org/10.1016/s0733-8619(18)30959-9.

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23

Yoshinaga, Harumi, Tomoyuki Terasaki, Tatsuya Ogino, Hodaka Ohta, Satoshi Maniwa, and Eiji Oka. "Incidence of epileptic discharge in various epileptic syndromes." Pediatric Neurology 25, no. 1 (2001): 38–42. http://dx.doi.org/10.1016/s0887-8994(01)00275-2.

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24

Epifanio, Roberta, Roberto Giorda, Maria Carolina Merlano, et al. "SCN2A Pathogenic Variants and Epilepsy: Heterogeneous Clinical, Genetic and Diagnostic Features." Brain Sciences 12, no. 1 (2021): 18. http://dx.doi.org/10.3390/brainsci12010018.

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Pathogenic variants of the SCN2A gene (MIM 182390) are associated with several epileptic syndromes ranging from benign familial neonatal-infantile seizures (BFNIS) to early infantile epileptic encephalopathy. The aim of this work was to describe clinical features among five patients with concomitant SCN2A gene variants and cryptogenic epileptic syndromes, thus expanding the SCN2A spectrum of phenotypic heterogeneity. De novo variants were identified in four patients, while one inherited variant was identified in a patient with an unaffected carrier biological father with somatic mosaicism. Two

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25

Lima, Camila Corado Gabriel, Aline Fernandez Quevedo, Carlos Walmyr de Mattos Oliveira, et al. "Unraveling the Complexity of Seizures and Epilepsies: a Multifaceted Approach to Classification, Syndromes, Causes, and Integrated Therapies." Revista de Gestão Social e Ambiental 18, no. 4 (2024): e07064. http://dx.doi.org/10.24857/rgsa.v18n4-120.

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Objective: To investigate the clinical diversity, genetic bases, diagnostic complexity, and therapeutic approaches of epileptic syndromes, highlighting recent advances in understanding these disorders. Theoretical Framework: Epileptic syndromes are neurological disorders characterized by recurrent epileptic seizures, resulting from abnormal neuronal activity in the brain, whose classification has evolved towards clinical and genetic criteria. Method: A systematic approach was employed for bibliographic review on epileptic syndromes, consulting biomedical databases and applying strict selection

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26

Ali, Irfan, and Dave F. Clarke. "Febrile Seizure Plus and PCDH19-Related Epilepsy Syndromes." Journal of Pediatric Epilepsy 08, no. 03 (2019): 074–78. http://dx.doi.org/10.1055/s-0040-1701202.

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AbstractEpilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures. Practically, a patient has epilepsy if having two unprovoked seizures more than 24 hours apart, one unprovoked seizure and significant risk of another seizure, or epilepsy syndrome. Seizures induced by fever do not therefore fit this classification. An initial febrile seizure may therefore cause a false sense of security in children who evolve to febrile seizure plus syndromes. Sodium channel defects seem to predominate as the main causative factor for febrile seizure plus sy

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27

Zupanc, Mary L. "Clinical Evaluation and Diagnosis of Severe Epilepsy Syndromes of Early Childhood." Journal of Child Neurology 24, no. 8_suppl (2009): 6S—14S. http://dx.doi.org/10.1177/0883073809338151.

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The developing brain is particularly susceptible to seizures. Diffuse central nervous system pathology or injury in early infancy, when the brain is most vulnerable, may lead to catastrophic epilepsies such as Ohtahara's epileptic encephalopathy and early myoclonic epileptic encephalopathy. These epileptic encephalopathies are difficult to treat and have poor prognoses. As the brain undergoes programmed synaptogenesis, apoptosis, and myelination, the epilepsy phenotypes and electroencephalography (EEG) findings change, producing age-dependent epileptic encephalopathies. Specifically, as they g

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28

Per, Huseyin, and Mehmet Canpolat. "Malignant Epileptic Syndromes in Infancy." Erciyes Tıp Dergisi/Erciyes Medical Journal 35, no. 3 (2013): 189–97. http://dx.doi.org/10.5152/etd.2013.55.

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29

Millichap, J. Gordon. "Epileptic Syndromes and Photosensitive Seizures." Pediatric Neurology Briefs 18, no. 1 (2004): 7. http://dx.doi.org/10.15844/pedneurbriefs-18-1-8.

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30

Fukushima, Katsuyuki, Tateki Fujiwara, Kazuichi Yagi, and Masakazu Seino. "Drop Attacks and Epileptic Syndromes." Psychiatry and Clinical Neurosciences 47, no. 2 (1993): 211–16. http://dx.doi.org/10.1111/j.1440-1819.1993.tb02053.x.

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31

Vassella, F. "Seizure Types and Epileptic Syndromes." European Neurology 34, no. 1 (1994): 3–12. http://dx.doi.org/10.1159/000119501.

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32

Papazian, Oscar. "Common Epileptic Syndromes in Children." Pediatric Annals 20, no. 1 (1991): 15–24. http://dx.doi.org/10.3928/0090-4481-19910101-07.

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33

Hommet, Caroline, Hannelore C. Sauerwein, Bertrand De Toffol, and Maryse Lassonde. "Idiopathic epileptic syndromes and cognition." Neuroscience & Biobehavioral Reviews 30, no. 1 (2006): 85–96. http://dx.doi.org/10.1016/j.neubiorev.2005.06.004.

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34

Stephenson, John B. P. "Epileptic Syndromes and their Treatment." European Journal of Paediatric Neurology 14, no. 2 (2010): 195–96. http://dx.doi.org/10.1016/j.ejpn.2009.12.004.

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35

Luders, Hans. "Epileptic syndromes may be misleading." Epilepsia 55, no. 10 (2014): 1677–78. http://dx.doi.org/10.1111/epi.12737.

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36

Benbadis, Selim R., and Hans O. Luders. "Epileptic Syndromes: An Underutilized Concept." Epilepsia 37, no. 11 (1996): 1029–34. http://dx.doi.org/10.1111/j.1528-1157.1996.tb01021.x.

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37

Eslava-Cobos, Jorge. "Treatment strategies for epileptic syndromes." Pediatric Neurology 8, no. 5 (1992): 394. http://dx.doi.org/10.1016/0887-8994(92)90283-5.

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38

Anastasescu, Catalina Mihaela, Veronica Gheorman, Simona Viorica Godeanu, et al. "KIAA2022/NEXMIF c.1882C>T (p.Arg628*) Variant in a Romanian Patient with Neurodevelopmental Disorders and Epilepsy: A Case Report and Systematic Review." Life 15, no. 3 (2025): 497. https://doi.org/10.3390/life15030497.

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Pathogenic variants in the NEXMIF gene are associated with a broad neurodevelopmental phenotype, including autism spectrum disorder (ASD), intellectual disability (ID), and epilepsy. However, the role of NEXMIF in specific epileptic syndromes remains insufficiently explored. We present the case of an 11.9-year-old Romanian girl diagnosed with ASD, attention-deficit/hyperactivity disorder (ADHD), mild ID, and Jeavons syndrome (generalized epilepsy characterized by eyelid myoclonia, absence seizures, and photosensitivity). Genetic testing identified a pathogenic NEXMIF variant: c.1882C>T (p.A

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39

Arkhipov, V. V., N. V. Chebanenko, D. M. Mednaya, and K. M. Mantserov. "Current Approaches to Assessing the Safety and Efficacy of Lamotrigine in Children and Adolescents with Epileptic Syndromes Associated with Focal Seizures." Safety and Risk of Pharmacotherapy 11, no. 3 (2023): 348–60. http://dx.doi.org/10.30895/2312-7821-2023-11-3-348-360.

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Scientific relevance. Epileptic syndromes associated with focal seizures often develop in children and adolescents and may be accompanied by cognitive impairment, mental disorders, and endocrine disorders, which require additional medication apart from anti-epileptic medicinal products. Currently, the selection of safe and effective therapies for epileptic syndromes, especially in paediatric populations, is a pressing challenge for epileptology.Aim. This study aimed at a comprehensive assessment of the safety and efficacy of lamotrigine in therapeutic doses for children and adolescents with ep

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40

Arya, Kapil. "Reflex Photosensitive Epilepsy." Journal of Pediatric Epilepsy 08, no. 04 (2019): 103–8. http://dx.doi.org/10.1055/s-0039-1700946.

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AbstractReflex seizure provoked by light or pattern stimulation are an interesting and, at times, difficult to treat entity. Such seizures occur in patients with a variety of well-described epileptic syndromes, although in most spontaneous seizures are also described. This paper attempts to describe epileptic syndromes with primarily light or pattern provoked seizures.

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41

Mukhin, K. Yu, O. A. Pylaeva, V. S. Kakaulina, and M. Yu Bobylova. "Classification and definition of epilepsy. Position paper by the International League Against Epilepsy on Nosology and Definitions of Epilepsy Syndromes dated 2021." Russian Journal of Child Neurology 17, no. 1 (2022): 6–95. http://dx.doi.org/10.17650/2073-8803-2022-17-1-6-95.

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This article presents updated and revised diagnostic criteria for epilepsy syndromes proposed in 2021 by the Nosology and Definitions Taskforce of the International League Against Epilepsy (ILAE). The current classification of epilepsy syndromes was developed in 1989. The new ILAE Classification and Definition of Epilepsy Syndromes is a result of huge work of many experts from all over the world. The draft of the new classification is presented on the ILAE website for wide discussion. It includes 4 main sections: 1) classification and definition of epilepsy syndrome with onset in neonates and

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42

Zeka, Naim, Abdurrahim Gerguri, Ramush Bejiqi, Ragip Retkoceri, and Armend Vuciterna. "Compare Of the West Syndrome with Other Syndromes in the Epileptic Encephalopathy - Kosovo Experience." Open Access Macedonian Journal of Medical Sciences 5, no. 7 (2017): 925–28. http://dx.doi.org/10.3889/oamjms.2017.192.

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BACKGROUND: West Syndrome (WS) represents as a specific epileptic encephalopathy characterised with a unique type of attacks, called infantile spasms, severe forms of abnormalities in electroencephalographic (EEG) records as a hypsarythmias and delays in the psychomotoric development. The characteristics of the disease, mostly affecting male gender, are infantile spasms and typical findings in EEG as a hypsarythmia. Infantile spasms are a consequence of many factors in the undeveloped brain.AIM: We aimed: (1) to see the incidence of the illness and the spreading out because of gender in rappor

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43

Hasan, H., Caner F. Demir, and Hasan S. Cura. "Absence status seen in an adult patient." Journal of Neurosciences in Rural Practice 04, no. 03 (2013): 342–44. http://dx.doi.org/10.4103/0976-3147.118809.

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ABSTRACTAbsence status epilepticus (ASE) is a type of nonconvulsive status epilepticus in which continuous or recurrent generalized epileptiform discharges are associated with a varying grade of consciousness impairment. Absence status epilepticus may be obtained during progress of many epileptic syndromes, in several metabolic disturbances and related to use of several drugs. Absence status epilepticus is generally seen in childhood; rarely it can be seen in adulthood. In this paper, the case which has never diagnosed until now in spite of many absence seizures for years, applied for absence

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44

Rešić, B., J. Rešić, and M. Antičić. "New approach to etiology factors in treatment of neonatal seizures." Paediatria Croatica 52, no. 3 (2008): 183–88. http://dx.doi.org/10.13112/pc.918.

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Neonatal convulsions are a very alarming and in the most cases the only manifestation of an underlying neural disorder. Seizures occur in 3.5-4.4/1000 live births. Convulsions in the immature brain can cause irreversible changes to the synapsis and can cause progress of convulsions. According to clinical manifestations, EEG changes and outcome, disorders are divided into benign and severe epileptic syndromes. Seizures may also be caused by genetic disorders, several of which are benign, familial, and caused by channelopathies involving potassium channels. Convulsions caused by these disorders

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45

Saltik, Sema. "Febrile seizures and related epileptic syndromes." Türk Pediatri Arşivi 53, no. 4 (2019): 203–4. http://dx.doi.org/10.5152/turkpediatriars.2018.86458.

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46

Millichap, J. Gordon. "Prevalence of Epileptic Syndromes in Finland." Pediatric Neurology Briefs 12, no. 1 (1998): 7. http://dx.doi.org/10.15844/pedneurbriefs-12-1-12.

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47

Wolf, P., and R. Goosses. "Relation of photosensitivity to epileptic syndromes." Journal of Neurology, Neurosurgery & Psychiatry 49, no. 12 (1986): 1386–91. http://dx.doi.org/10.1136/jnnp.49.12.1386.

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48

Guerrini, Renzo, and Pierre Genton. "Epileptic Syndromes and Visually Induced Seizures." Epilepsia 45, s1 (2004): 14–18. http://dx.doi.org/10.1111/j.0013-9580.2004.451011.x.

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49

Thadani, V., and P. Williamson. "Classification of Epileptic Seizures and Syndromes." Seminars in Neurology 10, no. 04 (1990): 328–38. http://dx.doi.org/10.1055/s-2008-1063977.

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50

Novotny, Edward. "Epileptic Syndromes and Seizures in Infants." Seminars in Neurology 10, no. 04 (1990): 366–79. http://dx.doi.org/10.1055/s-2008-1063981.

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