Jul 2026· World Journal of Biology Pharmacy and Health Sciences· 0 citations
TL;DR
Treatment is complex and involves the use of anti-seizure medications, dietary management, neuroimaging, surgery, rehabilitation, and psychosocial support and new therapeutic approaches approved in recent years and precision medicine have changed the treatment and improved prognosis in some LGS cases.
Abstract
Lennox-Gastaut syndrome (LGS) represents a serious form of developmental and epileptic encephalopathy, which is defined by drug-resistant seizures of various types, cognitive problems and typical EEG slow spike wave discharges. LGS starts in childhood and is usually associated with a high neurologic, developmental, and psychosocial burden. The cause of LGS is different and can be represented by structural, genetic, metabolic problems, infections and idiopathic forms of LGS. Even with all progress in diagnostic and therapeutic approaches, a complete cure of seizures is difficult to attain for most of the LGS patients; thus, many individuals remain affected with seizures throughout their lifetime. The diagnosis is made based on clinical history, semiology of seizures, specific EEG pattern, neuroimaging and genetic testing. Treatment is complex and involves the use of anti-seizure medications, dietary management, neuroimaging, surgery, rehabilitation, and psychosocial support. New therapeutic approaches approved in recent years and precision medicine have changed the treatment and improved prognosis in some LGS cases.
Lennox–Gastaut Syndrome (LGS) is a severe childhood-onset epileptic encephalopathy characterised by multiple daily seizures of varying semiology, intellectual disability, and a characteristic electroencephalographic pattern. Neuropsychiatric comorbidities - including attention-deficit/hyperactivity disorder (ADHD), autistic spectrum manifestations, and learning difficulties - are well documented in this population. Management is challenging owing to poor seizure response to conventional antiepileptic medications and the complexity introduced by co-occurring psychiatric disorders.
We report the case of a patient with LGS severely resistant to antiepileptic therapy and complicated by worsening ADHD symptoms. Following years of trialling multiple antiseizure medications with limited benefit, memantine was initiated alongside other medications and was associated with meaningful improvement in seizure frequency, cognition, and behavioural manifestations. ADHD medications were subsequently reintroduced, were well tolerated, and further improved the patient’s attention and behaviour.
This case highlights a novel application of memantine - an agent most commonly used in the management of Alzheimer’s disease - and suggests its potential utility as adjunctive therapy for improving both seizure control and neuropsychiatric symptoms in patients with LGS. Further research is warranted to evaluate this therapeutic approach systematically.
Akuti Khanna, Özge Özkan, Aditi Pajiyar et al.· Journal of Global Health Neu...· 0 citations
Introduction: Rasmussen’s Syndrome (RS) is a rare, chronic, and progressive encephalitis, presumably of autoimmune origin, that predominantly affects children. It is characterized by drug-resistant focal seizures, progressive neurological deterioration, and cerebral hemispheric atrophy. Objective: To describe the clinical course, therapeutic challenges, and outcome of a pediatric patient with Rasmussen’s syndrome. Case Report: This case describes an 8-year-old female patient with a history
of delayed neuropsychomotor development who developed drugresistant focal epilepsy. The diagnosis of RS was confirmed following clinical and imaging investigations. Functional hemispherectomy was indicated due to refractoriness to medical treatment. The procedure was temporarily postponed due to SARS-CoV-2 infection and was later performed successfully. The patient developed complications postoperatively, including hemodynamic instability, electrolyte disturbances, diabetes insipidus, respiratory infections, and acute kidney injury. Complete seizure control was achieved after approximately 65 days of hospitalization. Results: This case highlights the complexity of managing RS in pediatric patients and emphasizes the importance of an individualized approach. Conclusion: Hemispherectomy proved effective in controlling seizures and stabilizing neurological progression.
André Vinícius Soares Barbosa, Arthur Andrade Resende, Clarissa Milbratz de Castro et al.· Revista Médica de Minas Gera...· 0 citations
OBJECTIVE
This study was undertaken to assess cenobamate (CNB) effectiveness, tolerability, and dosing in pediatric developmental and epileptic encephalopathies (DEEs), testing prespecified hypotheses on response by syndrome, etiology, electroencephalographic pattern, seizure type, CNB dose (mg/kg/day), and concomitant medication.
METHODS
A retrospective multicenter cohort of children (≤18 years old) with DEEs were treated with CNB at 17 Spanish hospitals. Primary outcomes were retention, response (≥50% reduction), and seizure freedom at 3, 6, and 12 months. Mixed-effects logistic and ordinal models were adjusted for age, syndrome, etiology, seizure type, and concomitant medication.
RESULTS
Among 152 children (median age = 12 years), 27.6% had Lennox-Gastaut syndrome (LGS) and 58.6% unspecified DEE, with a median of 9 prior antiseizure medications. Retention was 88%, 90%, and 93% and responder rates 64%, 73%, and 79% at 3, 6, and 12 months; seizure freedom was 8%, 12%, and 18% (evaluable n = 152, 105, and 57 at 3, 6, and 12 months, respectively). LGS and other DEEs reached identical 12-month responder rates (both 79%), whereas Dravet syndrome showed limited sustained benefit. By etiology, structural cases had the highest 12-month responder rate (93% vs. 68% in nonstructural, p = .04), but no etiology was independently associated with response. By seizure type, responder rates were highest for tonic (81%) and bilateral tonic-clonic (76%) and lowest for absences (54%, adjusted odds ratio [OR] = .43, p = .032). Treatment-emergent seizure worsening occurred in 10.5%. Sodium channel blockers were the main risk factor for adverse events (OR = 2.10); 10.5% discontinued CNB due to adverse events.
SIGNIFICANCE
CNB was associated with sustained effectiveness and acceptable tolerability across pediatric DEEs. Slow weight-based titration and proactive simplification of sodium channel blockers and clobazam may optimize benefit-risk.
Ángel Aledo-Serrano, Adrián Valls-Carbó, E. González-Alguacil et al.· Epilepsia· 0 citations
This critical narrative review synthesises evidence on the clinical features, aetiologies, and therapeutic options for these syndromes, and evaluates the strength, consistency, and limitations of that evidence rather than cataloguing individual studies.
S. Bittmann, E. Luchter, Elena Moschüring-Alieva· Asian Journal of Pediatric R...· 0 citations
Comprehensive care extends beyond seizure management and includes addressing developmental, educational, and psychosocial needs, and a multidisciplinary, family-centred approach involving neurologists, dietitians, psychologists, and educators is essential.
Jayesh S. Patil, Hitendra S. Chaudhari, S. Pawar et al.· Research and reviews : a jou...· 0 citations
0.5–1% of children worldwide suffer from epilepsy, a widespread neurological condition that affects people of all genders and demographics. It is a collection of disorders rather than a single illness that is typified by frequent, erratic seizures brought on by aberrant brain activity. These seizures can take many different forms, such as convulsions, sensory or behavioral abnormalities, or loss of consciousness. Recurrent unprovoked seizures, a high likelihood of recurrence, or an epileptic syndrome are the criteria used by the International League Against epileptic (ILAE) to describe epilepsy. Patients' neurological, emotional, and social well-being are greatly impacted by epilepsy, which is caused by neuronal hyperexcitability. Seizure type, EEG results, and related neurological characteristics determine classification. All things considered, epilepsy is a complicated condition with a wide range of causes and symptoms. In children, epilepsy and Attention Deficincy Hyperactivity Disorder (ADHD) often co-occur; approximately 30–40% of children with epilepsy also have ADHD. There are two types of epileptic seizures: focal and generalized. Automatisms, atonic or tonic episodes, clonic jerks, spasms, hyperkinetic movements, or myoclonus are examples of motor symptoms that can accompany focal seizures. Non-motor symptoms include autonomic changes, behavior arrest, cognitive disturbances, emotional changes, or sensory abnormalities. Absence seizures, myoclonic seizures, atonic seizures, tonic seizures, and tonic-clonic seizures are examples of generalized seizures that affect both hemispheres of the brain. This categorization aids in the diagnosis, management, and comprehension of epileptic seizure patterns. A common neurological condition in children, epilepsy is most common in the first year of life and is more common in low- and middle-income nations, where the majority of cases go untreated. The six main categories of its causes are structural, genetic, infectious, metabolic, immunological, and unknown. An imbalance between neuronal excitation and inhibition causes aberrant electrical activity during seizures, which can extend to different parts of the brain. Ion channel malfunction, neurotransmitter imbalance, and altered neuronal circuitry are some of the factors that lead to epileptogenesis and can have long-term repercussions on cognition, particularly following protracted or frequent seizures. When assessing epilepsy, a physical examination is crucial. This includes measuring blood pressure, looking for signs of neurocutaneous syndromes on the skin, and looking for anomalies in the skull that can point to underlying neurological conditions. Electroencephalography (EEG), neuroimaging, and genetic testing are used to diagnose epilepsy in children. Sleep EEG is crucial for focal epilepsies and epileptic encephalopathies. EEG is an easy-to-use method for identifying aberrant cortical excitability. While genetic testing, including next-generation sequencing, has identified over 265 genes associated with epilepsy, increasing the identification of genetic epilepsies, neuroimaging detects structural abnormalities in the brain. Antiepileptic medications including carbamazepine, ethosuximide, and levetiracetam are used in treatment; each is customized for a particular type of seizure and age group while taking side effects and effectiveness into account. An alternate strategy is offered by dietary therapy, especially the ketogenic diet, which lowers seizure frequency by altering neurotransmitter activity and brain metabolism. Together, these therapeutic and diagnostic approaches allow children with epilepsy to be Effecetiveiy managed.
N. P. Patil, Divakar. R. Patil, Akash S. Jain et al.· Research Journal of Pharmaco...· 0 citations
