Diagnosis of Lennox-Gastaut syndrome and strategies for early recognition

INTRODUCTION: Lennox Gastaut syndrome (LGS) as an electroclinical diagnosis has been utilized as a clinical entity for more than 70 years. However, with the recognition of other distinct electroclinical epilepsy syndromes, no consistent single etiology, and the variability of criteria used in clinical trials, the clinical utility of such a diagnosis has been questioned. Recently, the International League Against Epilepsy for the first time defined diagnostic criteria for epilepsy syndromes, thereby allowing consistent language and inclusion criteria to be utilized. AREAS COVERED: Recent diagnostic criteria for syndrome diagnosis are explored as defined by the International League Against Epilepsy, with further literature reviewed to highlight relevant features, and differential diagnosis explored. EXPERT OPINION: Developmental and Epileptic Encephalopathy (DEE) is an overall term that may be descriptive of many different epilepsies, most of early onset, whether electroclinically or etiologically defined, of which LGS is one. Although we have moved forward in defining an increasing number of etiologically specific syndromes, this to date remains a minority of the DEEs. Although there is progress with precision medicine targeted at specific causes, the term LGS still remains useful as a diagnosis in defining treatment options, as well as overall prognosis

Physical activity in people with epilepsy: A systematic review

This study aimed to systematically review studies focusing on levels of physical activity (PA) in people with epilepsy (PWE) compared with non‐epilepsy controls, and identify factors associated with PA in PWE. Intervention studies were also reviewed to consider the effects of psychological interventions on levels of PA, and the effects of PA‐based interventions on seizure activity, psychiatric comorbidity, and health‐related quality of life (HRQoL). PRISMA guidelines were followed. Searches were conducted using PubMed, Cochrane Controlled Register of Trials, PsycINFO, and Embase. Forty‐six studies met inclusion criteria, including case‐control, cross‐sectional, and intervention studies. Assessment measures included questionnaires, activity trackers, and measures of physiological fitness. Twelve of 22 (54.5%) case‐control studies utilizing self‐report questionnaire measures reported that PWE were performing lower levels of PA, less likely to be engaging in PA, or less likely to meet PA guidelines than controls. The remaining studies did not find a difference between PWE and controls. Eight of 12 (67%) case‐control studies utilizing exercise/fitness tests reported that PWE performed significantly poorer than controls, whereas in two studies PWE performed better than controls. One of three studies investigating the relationship between PA and seizure frequency found that increased self‐reported PA was associated with having fewer seizures, whereas two did not find a significant relationship. All seven cross‐sectional studies that included measures of HRQoL and depression/anxiety found a positive relationship between levels of PA and HRQoL/reduced levels of depression and anxiety. All four studies that used PA‐based interventions demonstrated improvements in levels of PA and increased HRQoL. Study quality was almost universally low. In conclusion, there is some evidence that PWE engage in less PA than peers, and that interventions can improve PA levels and HRQoL. However, there is a need for more robust study designs to better understand PA in individuals with epilepsy

Incidence of Epilepsy and Seizures Over the First 6 Months After a COVID-19 Diagnosis: A Retrospective Cohort Study

BACKGROUND: The relationship between COVID-19 and epilepsy is uncertain. We studied the potential association between COVID-19 and seizures or epilepsy in the six months after infection. METHODS: We applied validated methods to an electronic health records network (TriNetX Analytics) of 81 million people. We closely matched people with COVID-19 infections to those with influenza. In each cohort, we measured the incidence and hazard ratios (HRs) of seizures and of epilepsy. We stratified data by age and by whether the person was hospitalized during the acute infection. We then explored time-varying HRs to assess temporal patterns of seizure or epilepsy diagnoses. RESULTS: We analyzed 860,934 electronic health records. After matching, this yielded two cohorts each of 152,754 patients. COVID-19 was associated with an increased risk of seizures and epilepsy compared to influenza. The incidence of seizures within 6 months of COVID-19 was 0.81% (95% CI, 0.75-0.88; HR compared to influenza 1.55 (1.39-1.74)). The incidence of epilepsy was 0.30% (0.26-0.34; HR compared to influenza 1.87 (1.54-2.28)). The HR of epilepsy after COVID-19 compared to influenza was greater in people who had not been hospitalized and in individuals aged under 16 years. The time of peak HR after infection differed by age and hospitalization status. CONCLUSIONS: The incidence of new seizures or epilepsy diagnoses in the six months following COVID-19 was low overall, but higher than in matched patients with influenza. This difference was more marked in people who were not hospitalized, highlighting the risk of epilepsy and seizures even in those with less severe infection. Children appear at particular risk of seizures and epilepsy after COVID-19 providing another motivation to prevent COVID-19 infection in pediatric populations. That the varying time of peak risk related to hospitalization and age may provide clues as to the underlying mechanisms of COVID-associated seizures and epilepsy

Inclusion and participation of children with epilepsy in schools: Views of young people, school staff and parents

OBJECTIVE: To gain an understanding of the views of children with epilepsy, their parents and staff regarding inclusion and participation of children with epilepsy in school. METHODS: During the study period, 136 children with 'active' epilepsy (taking anti-seizure Medications (ASMs) for epilepsy), were identified in the study area and of these 68 (50% of those eligible) families agreed to participate. Children (n = 20) with 'active epilepsy' their parents (n = 68) and staff (n = 56) were interviewed or completed surveys. The quantitative data were analysed using descriptive statistics and chi-square analyses. The answers to open questions were analysed using thematic analyses. RESULTS: Staff in mainstream schools were more concerned about the child's attendance than staff in special schools (p = 0.008).Parents and school staff cited a number of negative aspects of the child's attendance difficulties including social-emotional and academic aspects. The majority of parents and staff felt that young people with epilepsy were included in school to the same extent as peers. Parents were however, significantly less likely than staff to agree that children were included in all playground activities (p = 0.045). Parents of children in special schools were more likely to agree that their child was included in school than parents of children attending mainstream schools (p = 0.041). Thematic analysis revealed that parents felt that their child could be excluded in school due to staff decisions, child's own choice and peer led exclusion. The majority of children (64%) and parents (56%) agreed that the child with epilepsy was restricted from doing things their peers could do because of their epilepsy. While more than half of children with epilepsy (63%) reported that they had been bullied, most did not attribute this bullying to having epilepsy. Parents were more likely to agree that their child was bullied because of their epilepsy (p = 0.035) and non-epilepsy reasons than staff (p<0.001). Parents of children with epilepsy attending mainstream schools were more likely to agree that their child was bullied because of their epilepsy (p = 0.017) and non-epilepsy reasons (p = 0.026), compared to parents of children with epilepsy attending special schools. CONCLUSIONS: School attendance difficulties for children with epilepsy can contribute to academic and social-emotional difficulties. Most parents and teachers feel that children with epilepsy are included in classroom activities to the same extent as peers. Children with epilepsy and their parents believe that they are more restricted in non-classroom activities compared with their peers. Difficulties with participation, friendships and bullying for children with epilepsy may be due to presence of other conditions as opposed to epilepsy per se. There is a need to increase understanding of the wide ranging impact of epilepsy on school life in order to enhance attendance and inclusion and to reduce bullying

3D figure of epilepsy syndromes

We propose an instructive figure that summarized the classification of epilepsy syndromes according to the 2022 report of the ILAE Task Force on Nosology and Definitions. Our aim is to present, in one figure, different concepts such as the names of epilepsy syndromes, their extreme and classical ages of onset, their epilepsy types (generalized, focal or generalized and focal) but also their membership in groups of epilepsy syndromes as for self-limited or developmental and epileptic encephalopathy. With this figure, we provided an interactive tool, as supplementary data, helping to present this classification and link it to electro-clinical mandatory, alerts and exclusionary criteria of each syndrome, in accordance with the ILAE position papers on syndromes classification and nosology. This report may be used as an illustrative tool for teaching epilepsy syndromes and as a practical and comprehensive aid for the classification of epilepsy individuals' syndromes

Epilepsy management during difficult times

Major disruption in the delivery of healthcare services can occur in exceptional situations such as natural disasters, conflicts, periods of severe economic hardship, and epidemics. These disruptions typically affect to the greatest extent the most vulnerable segments of the population, including people with epilepsy. Inability to access healthcare services can lead to failure to undergo necessary diagnostic investigations, or to receive needed therapeutic interventions, including epilepsy surgery. Stress and other factors associated with the nature or the cause of the disruption can adversely affect seizure control status, or precipitate the occurrence of psychiatric disorders and other comorbid conditions. Failure to access antiseizure medications is a common occurrence in these situations and can result in loss of seizure control, withdrawal seizures, and status epilepticus. In this article, we provide examples of recent disruptions in healthcare and their implications for people with epilepsy. We discuss the consequences of natural disasters, conflicts, economic sanctions, and focus in greater detail on lessons learnt during the COVID-19 pandemic. We also discuss possible mitigation procedures, focusing in particular on the application of telemedicine to epilepsy care. Finally, we underline the need for governments, healthcare authorities, and international organizations to improve their preparedness to deal with exceptional situations that may arise in the future

Expert Opinion On The Management Of Lennox-gastaut Syndrome: Treatment Algorithms And Practical Consideration

Lennox-Gastaut syndrome (LGS) is a severe epileptic and developmental encephalopathy that is associated with a high rate of morbidity and mortality. It is characterized by multiple seizure types, abnormal electroencephalographic features, and intellectual disability. Although intellectual disability and associated behavioral problems are characteristic of LGS, they are not necessarily present at its outset and are therefore not part of its diagnostic criteria. LGS is typically treated with a variety of pharmacological and non-pharmacological therapies, often in combination. Management and treatment decisions can be challenging, due to the multiple seizure types and comorbidities associated with the condition. A panel of five epileptologists met to discuss consensus recommendations for LGS management, based on the latest available evidence from literature review and clinical experience. Treatment algorithms were formulated. Current evidence favors the continued use of sodium valproate (VPA) as the first-line treatment for patients with newly diagnosed de novo LGS. If VPA is ineffective alone, evidence supports lamotrigine, or subsequently rufinamide, as adjunctive therapy. If seizure control remains inadequate, the choice of next adjunctive antiepileptic drug (AED) should be discussed with the patient/parent/caregiver/clinical team, as current evidence is limited. Non-pharmacological therapies, including resective surgery, the ketogenic diet, vagus nerve stimulation, and callosotomy, should be considered for use alongside AED therapy from the outset of treatment. For patients with LGS that has evolved from another type of epilepsy who are already being treated with an AED other than VPA, VPA therapy should be considered if not trialed previously. Thereafter, the approach for a de novo patient should be followed. Where possible, no more than two AEDs should be used concomitantly. Patients with established LGS should undergo review by a neurologist specialized in epilepsy on at least an annual basis, including a thorough reassessment of their diagnosis and treatment plan. Clinicians should always be vigilant to the possibility of treatable etiologies and alert to the possibility that a patient's diagnosis may change, since the seizure types and electroencephalographic features that characterize LGS evolve over time. To date, available treatments are unlikely to lead to seizure remission in the majority of patients and therefore the primary focus of treatment should always be optimization of learning, behavioral management, and overall quality of life

A six-year longitudinal study of neurocognitive problems in children with epilepsy

INTRODUCTION: This study describes the specific neuropsychological abnormalities among children with epilepsy (CH-E) living in Georgia. METHODS: A cohort of CH-E and children without epilepsy (CH-NoE), aged 6-13 years, admitted to the epilepsy center of the Institute of Neurology and Neuropsychology from 1st January 2010 to 31st December 2015, was selected and investigated with a structured protocol. Neurological/epileptological assessments were made and neuropsychological testing was done on all study subjects. RESULTS: Abnormalities in praxis, verbal functions, verbal learning, visual-spatial matching, visual-motor ability, and fine motor skills, working memory, and phonological memory span were often revealed in CH-E as compared to CH-NoE. Early age of seizure onset, epilepsy duration, and anti-seizure medication (ASM) use, in combination with brain structural abnormalities on neuroimaging, and structural etiology were independent predictors of impaired functioning in various neuropsychological domains. DISCUSSION: More than half of children with epilepsy have a variety of cognitive impairments, which may increase with ASM therapy, especially when the cause of seizures is structural damage to the brain. Therefore, in the process of diagnosing epilepsy, evaluation of cognitive functions should become an integral part to ensure effective management of the disorder