Topics on Continuous Training

E. González Alguacil, J.J. García Peñas, V. Soto Insuga

Department of Pediatric Neurology. Niño Jesús University Children’s Hospital. Madrid

Abstract Epilepsy is one of the most common neurological disorders in childhood and is characterized by a persistent predisposition to recurrent seizures. The diagnosis is established when certain clinical and electroencephalographic criteria are met, and the causes may be structural, genetic, metabolic, immune, infectious, or remain unexplained. There are different types of seizures and epileptic syndromes that vary according to the age of onset. In neonates and infants, these include self-limited epilepsies and epileptic and developmental encephalopathies; while in childhood, idiopathic generalized epilepsies and syndromes defined by a specific etiology are also prominent. The current classification combines electroclinical and etiological information to better guide diagnosis and treatment. The therapeutic approach is symptomatic and focuses on seizure control. Benzodiazepines are the first-line treatment for acute seizures and status epilepticus. The choice of anti-seizure medications depends on the type of seizure or syndrome, and in certain cases, non-pharmacological options are used, such as the ketogenic diet, surgery, or neurostimulation devices. Primary care pediatricians play an essential role in the early detection of seizures, initial intervention during a first episode, timely referral to a specialist, monitoring comorbidities, and educating families, facilitating therapeutic adherence and contributing to improving patients’ quality of life.

 

Resumen La epilepsia es uno de los trastornos neurológicos más frecuentes en la infancia y se caracteriza por una predisposición persistente a presentar crisis recurrentes. El diagnóstico se establece cuando se cumplen determinados criterios clínicos y electroencefalográficos, y puede tener causas estructurales, genéticas, metabólicas, inmunes, infecciosas o permanecer sin una explicación clara. Existen distintos tipos de crisis y síndromes epilépticos que varían según la edad de inicio. En los neonatos y lactantes se incluyen epilepsias autolimitadas y encefalopatías epilépticas y del desarrollo; mientras que, en la infancia, destacan también las epilepsias generalizadas idiopáticas y los síndromes definidos por una etiología específica. La clasificación actual combina la información electroclínica con la etiológica para orientar mejor el diagnóstico y el tratamiento. El abordaje terapéutico es sintomático y se centra en el control de las crisis. Las benzodiacepinas son el tratamiento de primera línea en las crisis agudas y en el estatus epiléptico. La elección de los fármacos anticrisis depende del tipo de crisis o del síndrome y, en determinados casos, se recurre a opciones no farmacológicas, como la dieta cetogénica, la cirugía o los dispositivos de neuroestimulación. El pediatra de Atención Primaria desempeña un papel esencial en la detección precoz de las crisis, la actuación inicial ante un primer episodio, la derivación oportuna al especialista, el seguimiento de las comorbilidades y la educación a las familias, facilitando la adherencia terapéutica y contribuyendo a mejorar la calidad de vida de los pacientes.

 

Key words: Childhood epilepsy; Epileptic seizures; Status epilepticus; Epileptic syndromes; Antiseizure medications; Non-pharmacological therapies.

Palabras clave: Epilepsia infantil; Crisis epilépticas; Estatus epiléptico; Síndromes epilépticos; Fármacos anticrisis; Terapias no farmacológicas.

 

 

Pediatr Integral 2025; XXIX (7): 503 – 512

 

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Epilepsy

https://doi.org/10.63149/j.pedint.81

 

Introduction and concepts

Epileptic seizure

A transient manifestation of signs and/or symptoms caused by abnormal, excessive, or synchronous neuronal activity in the brain(1). This definition emphasizes the temporary nature of the seizure and its common pathophysiological basis, regardless of the diversity of clinical manifestations it may present.

• Acute symptomatic seizure: a clinical seizure that occurs at the time of a systemic insult or in close temporal relation to a documented brain injury, such as stroke, craniocerebral trauma, metabolic encephalopathy, central nervous system infection, among others(2).

• Unprovoked seizure: an epileptic seizure that occurs without a precipitating factor, temporary or reversible, with a possible tendency to recur.

Epilepsy

According to the practical definition proposed by the International League Against Epilepsy (ILAE) in 2014(3), epilepsy is characterized by a persistent predisposition to present recurrent epileptic seizures.

The diagnosis is established when any of the following conditions occur:

• Two or more unprovoked (or reflex) seizures, separated by more than 24 hours.

• An unprovoked (or reflex) seizure, when there is a high probability of recurrence (at least 60%) in the next ten years.

• The diagnosis of a specific epileptic syndrome.

It can have a diverse origin, including genetic, structural, metabolic, immunological, infectious causes or of unknown etiology(4).

Status epilepticus (SE)

In 2015, the ILAE(5) defined status epilepticus as a condition resulting from the failure of the mechanisms responsible for terminating an epileptic seizure or from the activation of processes that prolong its duration. For better clinical application, two temporal milestones are established (Fig. 1):

 

• Time 1: clinical threshold from which a seizure should be considered as continuous epileptic activity, and it requires immediate treatment. In the case of generalized tonic-clonic seizures, this point is set at 5 minutes.

• Time 2: the point at which the seizure can cause irreversible neuronal damage and long-term consequences, estimated at 30 minutes for generalized tonic-clonic seizures.

Based on this definition, several clinical forms of SE are recognized:

• Established SE: when the seizure persists beyond time 2, usually >30 minutes.

• Refractory SE: when the seizure continues despite adequate treatment with benzodiazepines and at least one second anti-seizure drug.

• Superrefractory SE: when epileptic activity persists beyond 24 hours despite anesthetics or recurs upon attempted withdrawal.

Drug-resistant epilepsy

Drug-resistant epilepsy is defined as that in which sustained seizure control is not achieved despite the use of two well-tolerated anti-seizure drugs, at adequate doses and correctly indicated(7).

Epilepsy is defined as a persistent predisposition to unprovoked seizures, and its diagnosis requires specific criteria, while status epilepticus represents a medical emergency.

Prevalence

According to estimates from the Global Burden of Disease Study 2019(8), the global prevalence of active epilepsy was 682 cases per 100,000 inhabitants (95% CI: 586-784). Of these, 359 per 100,000 were secondary epilepsy. These figures indicate that, worldwide, approximately seven out of every thousand people live with active epilepsy.

Classification of epileptic seizures (ILAE 2025)(9)

ILAE maintains four main types of seizures:

1. Focal: these originate in networks limited to one hemisphere. They are classified as follows:

– With preserved consciousness.

– With altered consciousness.

– Focal to bilateral tonic-clonic.

2. Generalized: they start in bilateral networks from the beginning. The following are included:

– Absences (typical, atypical, myoclonic, with eyelid myoclonus).

– Tonic-clonic (classic, myoclonic-tonic-clonic).

– Other generalized ones.

3. Of unknown origin: when it cannot be determined whether the onset was focal or generalized:

– With preserved consciousness.

– With altered consciousness.

– Bilateral tonic-clonic

4. Unclassified: when there is not enough information, although the event is recognized as an epileptic seizure.

Semiology in the main types of seizures

• Tonic seizures: these are characterized by sustained muscle contraction that produces generalized or segmental rigidity. They usually affect axial and proximal muscles, often with a sudden fall if the patient is standing.

• Absence seizures: they are characterized by a sudden interruption of consciousness, of short duration, with a fixed gaze and cessation of ongoing activity. They may be accompanied by mild motor phenomena and usually have an abrupt onset and end.

• Tonic-clonic seizures: present a typical two-phase sequence:

– Tonic: generalized muscle rigidity.

– Clonic: bilateral rhythmic jerks, which decrease in frequency until resolution.

Postictal confusion is common afterward.

• Clonic seizures: they are characterized by jerking repetitive rhythmic contractions of muscle groups, generally bilateral and symmetrical, without a clear initial tonic phase.

• Myoclonic seizures: defined by brief, sudden, non-rhythmic muscle jerks, which may be single or repetitive, affect one muscle group or become generalized. Alteration of consciousness is not always present.

• Atonic seizures: sudden loss of muscle tone, leading to sudden falls, with a high risk of injury.

• Myoclonic-atonic seizures: they combine initial myoclonic jerks, immediately followed by a loss of postural tone.

• Epileptic spasms: sudden flexion, extension, or a mixture of flexion and extension, usually affecting the proximal and trunk muscles (especially of the upper limbs, producing separation and lifting of both arms relative to the torso). In bursts. More frequent upon waking.

Assessment after first seizure and initial diagnosis of epilepsy(10)

In case of a first paroxysmal episode in pediatrics, a detailed medical history, neurological examination and the use of home video are key to differentiating epileptic seizures from other disorders.

1. Epileptic seizure or non-epileptic paroxysmal disorder?

In the first paroxysmal episode, it is essential to perform a standardized clinical assessment that allows us to evaluate the severity of the episode, define its nature (epileptic or non-epileptic) and guide the initial prognosis.

A thorough medical history, along with a physical and neurological examination, is usually sufficient to establish a diagnosis in most pediatric cases. The importance of home video recordings should be emphasized.

Careful follow-up is recommended to clarify the diagnosis before starting treatment with anti-seizure drugs.

2. In the face of an epileptic seizure

Distinguish between acute symptomatic vs. unprovoked seizure.

3. In the face of an unprovoked seizure, assess what type of seizure it is (Fig. 2)

 

The first-level complementary test will be the EEG (electroencephalogram), and it is recommended to perform an EEG as part of the diagnostic evaluation in children with a first apparently unprovoked seizure.

Diagnostic performance is higher if the EEG is performed within the first 24 hours; however, background findings, such as focal slowing, may be observed transiently as postictal phenomena and subsequently resolve, and should not be automatically assumed to be signs of structural injury.

Although it is uncommon to record clinical events during a routine EEG, both epileptic and non-epileptic seizures are sometimes captured. The use of simultaneous video recording during EEG can help to better understand the clinical features.

Triggering maneuvers include hyperventilation, which often triggers absence seizures in untreated childhood or juvenile absence epilepsy, and intermittent light stimulation, which can provoke seizures in photosensitive epilepsies (such as myoclonic seizures in juvenile myoclonic epilepsy). Detecting these more subtle types of seizures can point toward a specific epileptic syndrome.

EEG can also guide the need for further testing and provides information about the risk of recurrence.

Prolonged video-EEG is rarely required after a first seizure. However, it should be considered in patients without complete recovery from baseline neurological state within 60 minutes, with fluctuating disturbances of consciousness, or with unexplained focal neurological findings, to rule out frequent subtle seizures or status epilepticus.

EEG is indicated in all patients with recent-onset unprovoked seizures. Care should be taken not to misinterpret normal variations as epileptiform. EEG helps determine the seizure type and epilepsy, guide the need for further studies, and establish the prognosis regarding the risk of recurrence.

4. Epilepsy diagnosis?

See table I.

 

5. Type of epilepsy

The second level of classification focuses on the type of epilepsy, which is based on the type or types of seizures the patient experiences.

The types of epilepsy are divided into generalized, focal, combined generalized and focal, or of unknown origin.

EEG can also help determine the type of epilepsy. Focal epilepsy is suggested by focal epileptiform discharges or focal slowing, while generalized spike-and-wave discharges are associated with generalized epilepsy. Some epileptic syndromes have a characteristic electroencephalographic pattern.

EEG interpretation should always be done in the context of the patient’s medical history, since approximately 3% of people without epilepsy may exhibit epileptiform discharges. Therefore, an abnormal EEG does not necessarily equate to epilepsy.

6. Etiology (Fig. 3)

 

Epilepsy can have multiple etiologies (structural, genetic, infectious, metabolic, immune or unknown) and its identification, through MRI, genetic studies, metabolic or immunological tests, is essential, because it guides the prognosis, allows for more specific management and opens the door to precision therapies in selected cases.

Structural

Epilepsy is considered to have a structural cause if there is a structural brain abnormality that triggers the seizures. These abnormalities can be due to malformations of cortical development or acquired processes, such as infection, stroke, trauma, or tumor. In most cases, structural abnormalities are evident on magnetic resonance imaging (MRI) (especially if the images are acquired using epilepsy-specific protocols).

Additional tests: the ILAE Neuroimaging Commission has recommended that all patients with epilepsy undergo brain magnetic resonance imaging (MRI), except those with a clearly defined drug-responsive idiopathic generalized epilepsy syndrome, or with self-limiting focal epilepsy of childhood(11).

Genetics

Epilepsy is considered genetic if it is caused by a known genetic variant. In some cases, a pathogenic variant is identified in a single gene. These monogenic disorders are often, though not always, associated with early-onset, drug-resistant epileptic and developmental encephalopathies. Some genes also produce structural brain abnormalities, while others cause metabolic changes that lead to epilepsy.

Another large group comprises idiopathic generalized epilepsies, in which family studies show a strong genetic predisposition, although no single causative gene has been identified. The etiology is considered polygenic.

Additional tests: large-scale molecular genetic studies have made it possible to identify a growing number of novel genes associated with epilepsy.

The diagnostic yield of genetic testing is highest in infants and young children with epileptic and developmental encephalopathies of unknown cause. It should also be considered in patients whose clinical examination or other studies suggest a likely genetic cause.

This has diverse repercussions, but the probability of initiating precision medicine based on the affected gene is becoming increasingly common.

Several genetic testing strategies exist: karyotyping, CGH arrays, single-gene sequencing, epilepsy gene panel, whole-exome sequencing, or whole-genome sequencing. A recent meta-analysis evaluating the cost-effectiveness of various genetic tests commonly used in epilepsy suggests that epilepsy gene panel or whole-exome sequencing should be the first diagnostic option in these cases(12).

Infectious

Infectious etiologies are the most frequent cause of epilepsy worldwide, especially in developing countries. Chronic epilepsy is considered structural, likely due to structural lesions secondary to infection during the acute phase.

This category should not be used to describe acute symptomatic seizures that occur during brain infections, such as encephalitis or meningitis.

Metabolic

A metabolic etiology implies that epilepsy and seizures are due to biochemical abnormalities resulting from a known metabolic disorder. Some treatments are specific and can stop seizures and prevent developmental impairment.

Additional tests: a standardized metabolic study will be performed on patients with epilepsy and suspected inborn errors of metabolism. In many cases, the metabolic disease has a genetic basis, and the diagnosis will be made through genetic testing.

Immune

Immune epilepsy is considered to be that which results directly from an underlying immune disorder.

Additional tests: in the presence of suggestive clinical features, the performance of antineuronal antibodies in serum and CSF should be strongly considered.

Unknown

The more thorough the investigation, the greater the likelihood of identifying a cause. However, even after comprehensive studies, a clear etiology cannot be identified in one-third to one-half of patients with recent-onset unprovoked seizures.

Epileptic encephalopathy or epileptic and developmental encephalopathy?

Epileptic encephalopathy

Epilepsies in which the epileptic activity itself contributes to a more severe cognitive and behavioral impairment than expected from the underlying pathology, with potential reversibility if early and effective treatment is achieved(13).

Epileptic and developmental encephalopathy

Cases in which cognitive-behavioral impairment is due to both epilepsy and the underlying neurobiological etiology. In these patients, seizure control may partially improve the clinical picture, but the most promising intervention comes from precision medicine strategies targeting the causal mechanism(4) (Table II).

 

Epileptic syndromes

ILAE 2022 proposes a hybrid classification of epilepsy, which combines the electroclinical syndrome with etiology, recognizing the genotype-phenotype complexity and allowing for a more accurate diagnosis, which better guides treatment and prognosis.

Traditionally, epilepsies were classified into epileptic syndromes defined by common electroclinical characteristics, comorbidities, prognosis, treatment response, and EEG or neuroimaging findings. With advances in genetics, causative genes were identified, although the relationship between mutation and phenotype proved complex: the same gene can cause different syndromes, and the same syndrome can be caused by different genes.

Therefore, in 2022, ILAE proposed a hybrid classification, which integrates both the electroclinical syndrome and the etiology (structural, genetic, metabolic, immune, or infectious) to guide the therapeutic plan. It also redefines the epileptic syndrome as a set of clinical and electroencephalographic characteristics, frequently supported by specific etiological findings, and describes genetic syndromes with homogeneous electroclinical profiles(14,15).

Electro-clinically defined epileptic syndromes with onset in neonates and infants

In the 2022 ILAE classification, epileptic syndromes with onset in newborns and infants (Table III) are divided into two main groups: self-limiting epileptic syndromes, in which spontaneous remission is likely to occur; and epileptic and developmental encephalopathies (EDE).

 

Childhood-onset epileptic syndromes

They are divided into three main groups (Table IV):

 

• Self-limiting focal epilepsies: focal epilepsies that begin in childhood are usually self-limiting and typically of unknown cause. They were previously called “benign” or “idiopathic,” but ILAE recommends the term self-limiting because they can be associated with comorbidities and because the natural course is age-dependent in onset and remission.

They represent up to 25% of all pediatric epilepsies. Most children fall into a specific syndrome; however, in some cases, there may be overlap or progression from one syndrome to another.

• Genetic generalized epilepsies: virtually all generalized epileptic syndromes with onset in childhood have a genetic etiology. They are considered to have complex inheritance, with a polygenic basis, with or without contribution from environmental factors.

• Epileptic and developmental encephalopathies: epileptic and developmental encephalopathies constitute a group of severe syndromes in which frequent and treatment-resistant epileptic seizures are associated with a significant impairment of neurodevelopment, as well as the underlying etiology.

Early recognition of these syndromes is fundamental, as it allows for targeted treatment, including the use of specific therapies in certain cases.

Specific syndromes by etiology

Etiologically specific syndromes are those in which there is a defined cause of epilepsy, associated with a relatively uniform and characteristic clinical phenotype in most patients (form of presentation, types of seizures, comorbidities, evolutionary course and/or response to specific treatments), in addition to consistent correlates in EEG, neuroimaging and/or genetics.

The ILAE working group did not seek to describe all specific syndromes by etiology, but to offer definitions of some relevant ones (Table V).

 

Idiopathic generalized epilepsies

Idiopathic generalized epilepsy (IGE) refers to epilepsies that begin in childhood or adolescence, with the following characteristics(16).

• Generalized seizures (absences, myoclonus and/or tonic-clonic seizures).

• Neurological development is generally normal, although with frequent comorbidities (learning disorders, ADHD, anxiety or depression).

• Characteristic EEG: spike-wave discharges or generalized polyspike-wave (2.5-5.5 Hz).

• Polygenic genetic basis, although in some cases monogenic causes may exist.

• Good response to broad-spectrum drugs, although complete remission is not always possible.

There is overlap between the syndromes, since the same patient can evolve from one to another, which reflects that they are part of a continuum within IGE, rather than completely separate entities (Fig. 4).

 

Epilepsy treatment

The management of childhood epilepsy includes a growing list of anti-seizure drugs and non-pharmacological therapies.

The initial choice of treatment should be based on the type of seizure or epileptic syndrome.

Action to take in the event of an episode consistent with an epileptic seizure

• Postural management (place the patient on his/her side, avoiding holding the patient and do not insert anything in the mouth).

• If duration is longer than 2-3 minutes, administer rescue medication (buccal midazolam vs. rectal diazepam).

Pharmacological treatment in epilepsy

Benzodiazepines in epileptic seizures (ES) and status epilepticus (SE)(17):
are the initial treatment of choice (recommendation IA): safe, effective and with better control if administered within the first 3 minutes.

• Intravenous (IV) route:

– Diazepam (DZP): similar efficacy; DZP is the most used in our setting due to better evidence.

– Midazolam (MDZ): alternative option, as a first line.

• If venous access is unavailable: quick options include: Intranasal, oral, or intramuscular administration of MDZ, and rectal DZP. MDZ demonstrates greater efficacy than rectal DZP.

• Precautions:

– Always monitor respiratory function.

– Avoid in myasthenia gravis, neuromuscular diseases with orofacial involvement or respiratory failure.

In infants between 1 and 3 months, the administration of benzodiazepines should be individualized (e.g., they are not recommended in spasm seizures) and, preferably, the intravenous route should be used rather than the oral route.

In newborns, it is important to detect and treat the most common metabolic disorders (hypoglycemia, hypocalcemia, hypomagnesemia, etc.). Inborn errors of metabolism can present with epileptic seizures that respond to appropriate vitamin supplements, so it is important to consider them in routine treatment.

Anti-seizure drugs for the treatment of epilepsy

Currently available pharmacological treatment is symptomatic, aimed at controlling seizures, but it does not modify the natural course of epilepsy or affect epileptogenesis. For this reason, the term antiepileptic drugs (AEDs) has been replaced in recent literature by that of antiseizure drugs (ASDs).

The ideal ASD would be one capable of eliminating seizures without producing significant adverse effects, thus respecting the patient’s quality of life. Its optimal characteristics would include:

• Flexible administration via oral and parenteral routes, with formulations adapted to pediatric age (oral suspension).

• Good bioavailability, with complete absorption unmodifiable by food or other drugs.

• Long half-life allowing for a single daily dose.

• Linear and predictable kinetics.

• Low plasma protein binding and no interactions with other medications.

• Broad therapeutic range, without tolerance or addiction.

• Minimal adverse effects, with no significant analytical alterations.

• That it does not worsen previous seizures or induce new types of seizures.

• Broad therapeutic spectrum and adequate efficacy-safety balance.

In practice, no available drug fully meets all these characteristics, but these criteria guide research and the rational choice of treatment in the clinic.

Table VI details the drugs used according to the type of epileptic seizure.

 

Non-pharmacological therapies

• Ketogenic diet: the ketogenic diet has a multimodal mechanism of action distinct from that of anti-seizure drugs, highlighting its anti-inflammatory effect and its regulatory effect on mitochondrial redox metabolism, which give it a potential neuroprotective and anti-epileptogenic effect.

It consists of a diet with a high percentage of fats, a low amount of carbohydrates and an adequate supply of protein.

In general, up to 50-60% of patients with epilepsy treated with a ketogenic diet may be considered responders. Response rates and seizure freedom rates vary depending on the different epileptic syndromes and etiologies considered(18,19).

• Epilepsy surgery: surgery is considered in drug-resistant epilepsy, after assessing risks and benefits at a reference center, distinguishing between:

– Resective surgery: it involves removing the epileptic focus, which can achieve seizure freedom in a significant percentage of patients with drug-resistant focal epilepsy. It requires pre-surgical evaluation at specialized centers and precise localization of the focus.

– Palliative surgery: it aims to reduce the frequency and intensity of seizures. It is indicated in certain drug-resistant epilepsies when complete resection of the epileptic focus is not possible.

• Neurostimulation(20) (Table VII): neurostimulation is a therapeutic option for patients with drug-resistant epilepsy. It consists of devices that apply electrical impulses to modulate brain activity and reduce the frequency and intensity of seizures.

 

Table VIII presents the drugs and non-pharmacological therapies indicated for each epileptic syndrome.

 

Role of the Primary Care Pediatrician

• Early detection of epileptic seizures and referral to a neuropediatrician for diagnostic confirmation, syndrome classification, and etiological study.

• Initial approach to a seizure: safety measures, administration of rescue medication if indicated and coordination with the Emergency Services.

• Shared monitoring of the already diagnosed patient, supervising adherence and tolerance to treatment, as well as clinical progress.

• Management of common comorbidities: sleep disorders, learning difficulties, behavioral disorders and associated medical problems.

• Family support: detection of caregiver burden, guidance on available resources and promotion of adherence to the therapeutic plan.

Conflict of interest

There is no conflict of interest in the preparation of this manuscript nor any source of funding.

Bibliography

The asterisks indicate the article’s level of interest, in the authors’ opinion.

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2. Beghi E, Carpio A, Forsgren L, Hesdorffer DC, Malmgren K, Sander JW, et al. Recommendation for a definition of acute symptomatic seizure. Epilepsia. 2010; 51: 671-5.

3. Fisher RS, Acevedo C, Arzimanoglou A, Bogacz A, Cross JH, Elger CE, et al. ILAE Official Report: A practical clinical definition of epilepsy. Epilepsia. 2014; 55: 475-82.

4. Scheffer IE, Berkovic S, Capovilla G, Connolly MB, French J, Guilhoto L, et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017; 58: 512-21.

5. Trinka E, Cock H, Hesdorffer D, Rossetti AO, Scheffer IE, Shinnar S, et al. A definition and classification of status epilepticus – Report of the ILAE Task Force on Classification of Status Epilepticus. Epilepsia. 2015; 56: 1515-23.

6. Soto Insuga V, González Alguacil E, García Peñas JJ. Pediatric status epilepticus. Estado epiléptico pediátrico. Rev Neurol. 2022; 75: 225.

7. Kwan P, Arzimanoglou A, Berg AT, Brodie MJ, Allen Hauser W, Mathern G, et al. Definition of drug resistant epilepsy: Consensus proposal by the ad hoc Task Force of the ILAE Commission on Therapeutic Strategies. Epilepsia. 2010; 51: 1069-77.

8. Institute for Health Metrics and Evaluation. Years lived with disability attributable to epilepsy. 2019. Available at: https://vizhub.healthdata.org/gbd-compare.

9. Beniczky S, Trinka E, Wirrell E, Abdulla F, Al Baradie R, Alonso Vanegas M, et al. Updated classification of epileptic seizures: Position paper of the International League against Epilepsy. Epilepsia. 2025; 66: 1804-23.

10.** Wirrell E. Evaluation of First Seizure and Newly Diagnosed Epilepsy. Continuum. 2022; 28: 230-60.

11. Gaillard WD, Cross JH, Duncan JS, Stefan H, Theodore WH; Task Force on Practice Parameter Imaging Guidelines for the International League against Epilepsy, Commission for Diagnostics. Epilepsy imaging study guideline criteria: Commentary on diagnostic testing study guidelines and practice parameters: Epilepsy Diagnostic Testing Guidelines. Epilepsia. 2011; 52: 1750-6.

12. Sánchez Fernández I, Loddenkemper T, Gaínza-Lein M, Sheidley BR, Poduri A. Diagnostic yield of genetic tests in epilepsy: A meta-analysis and cost-effectiveness study. Neurology. 2019; 92: e418-e428. Available at: https://doi.org/10.1212/wnl.0000000000006850.

13. Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia. 2010; 51: 676-85.

14.** Specchio N, Wirrell EC, Scheffer IE, Nabbout R, Riney K, Samia P, et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022; 63: 1398-442.

15.** Zuberi SM, Wirrell E, Yozawitz E, Wilmshurst JM, Specchio N, Riney K, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022; 63: 1349-97.

16. Hirsch E, French J, Scheffer IE, Bogacz A, Alsaadi T, Sperling MR, et al. ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022; 63: 1475-99.

17. Martín Martínez A, García Morales I, Serratosa Fernández JM, Gil-Nagel Rein A. BVCM051050; Proceso asistencial crisis epiléptica urgente de la Comunidad de Madrid. Urgent epileptic seizure care process in the Community of Madrid. 2023. Available at: https://repositorio.uam.es/bitstream/handle/10486/719178/10388472.pdf.

18. Pedrón Giner C. Manual para la práctica de la dieta cetogénica. Manual for the practice of the ketogenic diet. 1st ed. Madrid: Nutricia. 2021.

19. Sourbron J, Klinkenberg S, Van Kuijk SMJ, Lagae L, Lambrechts D, Braakman HMH, et al. Ketogenic diet for the treatment of pediatric epilepsy: review and meta-analysis. Childs Nerv Syst. 2020; 36: 1099-109.

20. Gouveia FV, Warsi NM, Suresh H, Matin R, Ibrahim GM. Neurostimulation treatments for epilepsy: Deep brain stimulation, responsive neurostimulation and vagus nerve stimulation. Neurotherapeutics. 2024; 21: e00308.

21. Cancho Candela R, Andrés de Álvaro M. Síndromes epilépticos según la edad. Epileptic syndromes according to age. Pediatr Integral. 2020; 7: 375-82. Available at: https://www.pediatriaintegral.es/publicacion-2020-10/sindromes-epilepticos-segun-la-edad/.

 

Recommended bibliography

- Wirrell E. Evaluation of First Seizure and Newly Diagnosed Epilepsy. Continuum. 2022; 28: 230-60.

This article is key because it explains how to approach a first seizure and differentiate it from a diagnosis of epilepsy. It indicates which complementary tests to perform, provides guidance on initiating treatment based on the risk of recurrence and the patient’s characteristics, and offers tools for recognizing epileptic syndromes from the outset and understanding their impact on prognosis.

- Specchio N, Wirrell EC, Scheffer IE, Nabbout R, Riney K, Samia P, et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsy. 2022; 63: 1398-442.

This article is essential because it establishes the official classification and definition of childhood-onset epileptic syndromes developed by the ILAE working group. It allows for a more precise and standardized diagnosis, which directly impacts patient treatment and prognosis.

- Zuberi SM, Wirrell E, Yozawitz E, Wilmshurst JM, Specchio N, Riney K, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsy. 2022; 63: 1349-97.

This article is important because it provides the official classification and definition of epileptic syndromes with onset in newborns and infants, according to the ILAE. It contributes to earlier and more accurate diagnosis, leading to better therapeutic management and prognosis.