Lennox-Gastaut Syndrome (LGS): Development of conceptual models of health-related quality of life (HRQL) for caregivers and children

Gallop, Katy; Wild, D; Verdian, L; Kerr, Michael Patrick; Jacoby, Ann; Baker, Gus A.; Nixon, Annabel; Cramer, Joyce A.

doi:10.1016/j.seizure.2009.10.007

Cited by 47 publications

(96 citation statements)

References 16 publications

(17 reference statements)

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“…Sherman et al. (2007) focused their evaluation mainly on a cohort of severe localization‐related epilepsies, and Gallop et al. (2010) conducted semi‐structured interviews with the parents of 40 patients with Lennox‐Gastaut syndrome, finding that seizure severity and cognitive impairment had a negative impact on HRQOL.…”

Section: Discussionmentioning

confidence: 99%

“…Sabaz et al (2001) examined individuals with partial/generalized seizures who had significantly lower seizure frequency and better intellectual ability. Sherman et al (2007) focused their evaluation mainly on a cohort of severe localizationrelated epilepsies, and Gallop et al (2010) conducted semistructured interviews with the parents of 40 patients with Lennox-Gastaut syndrome, finding that seizure severity and cognitive impairment had a negative impact on HRQOL. Dravet syndrome, by contrast, is a much more clearly defined epilepsy syndrome with a distinctive pattern of seizures, motor disorder, learning, and behavioral difficulties that differ from other intractable epilepsies.…”

Section: Discussionmentioning

confidence: 99%

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Comorbidities and predictors of health‐related quality of life in Dravet syndrome

2011

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SUMMARYPurpose: Health-related quality of life (HRQOL) has emerged as a widely accepted measure to evaluate how chronic disease impacts on an individual's physical, social, and mental well-being. There is a paucity of data focusing on HRQOL in specific epilepsy syndromes and their associated needs. In this study our aim was to describe the comorbidities and disease-related predictors for HRQOL in Dravet syndrome, an epileptic encephalopathy, with defined genetic etiology. We anticipate that this will help us to better recognize and understand the needs of children and families and aid treatment planning in this severe epilepsy syndrome. Methods: One hundred sixty-three individuals with Dravet syndrome and their families participated in the study. Detailed clinical and demographic information was available for each case. HRQOL was evaluated with two epilepsy-specific instruments, the Impact of Pediatric Epilepsy Scale (IPES) and the Epilepsy & Learning Disabilities Quality of Life Questionnaire (ELDQOL); a generic HRQOL instrument; the Pediatric Quality of Life Inventory (PedsQL); and a behavioral screening tool, the Strength and Difficulties Questionnaire (SDQ). Key Findings: HRQOL was significantly lower for children with Dravet syndrome compared to normative data (p < 0.001). A cross-sectional evaluation of measures across different age groups revealed that PedsQL generic core and cognitive function scales decreased in older age categories, indicating worse HRQOL (p < 0.001). Assessment of epilepsy severity demonstrated that symptoms were rated very severe in 10 (6%) of 162 cases, somewhat severe in 78 (48%) of 162, moderate in 51 (32%) of 162, and mild in 23 (14%) of 162 cases. The epilepsy severity correlated significantly with the IPES total impact score (r = 0.466, p < 0.001, n = 162). The IPES total impact scores in the Dravet group (n = 162) were significantly higher than scores measured in the original validation sample of epileptic children with and without learning difficulties (± SD) (21.0 ± 8.7 vs. 11.6 ± 5.4, t = 8.95, p < 0.001, n = 46). On the SDQ, 35% of children scored in the abnormal range for ''conduct problems,'' 66% for ''hyperactivity/ inattention,'' and 76% for ''peer relationships.'' Regression analysis revealed that young age at seizure onset (p = 0.019), presence of myoclonic seizures (p = 0.029), motor disorder (p = 0.048), learning difficulties (p = 0.002), epilepsy severity (p < 0.001), and behavioral difficulties (p < 0.001) each independently predicted poorer HRQOL. Behavioral problems such as hyperactivity/inattention were the strongest predictors of poorer HRQOL. Significance: This is the first comprehensive study of HRQOL in an etiologically well-defined epilepsy syndrome. HRQOL in Dravet syndrome depends on a series of independent factors including seizure control, behavior, cognitive, and motor problems. Identification of specific comorbidities in Dravet syndrome will facilitate a distinct and multidisciplinary approach to management, addressing seizure control, behavior prob...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Comorbidities and predictors of health‐related quality of life in Dravet syndrome

2011

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“…The potential benefits of early treatment, including decreased risk of injury from falls, decreased seizure frequency, and maintenance of quality of life [5,7,30,31] underscore the importance of identifying patients with LGS. Early diagnosis and treatment may improve clinical outcomes and help reduce the risk of psychomotor regression and intractability associated with LGS [12,32].…”

Section: Discussionmentioning

confidence: 99%

The refractory epilepsy screening tool for Lennox–Gastaut syndrome (REST-LGS)

Piña-Garza

Boyce²,

Tworek

et al. 2019

Epilepsy & Behavior

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Background: The complex clinical presentation and progression of Lennox-Gastaut syndrome (LGS) can complicate the accurate diagnosis of this severe, lifelong, childhood-onset epilepsy, often resulting in suboptimal treatment. The Refractory Epilepsy Screening Tool for LGS (REST-LGS) was developed to improve the identification of patients with LGS. Methods: Using the Modified Delphi Consensus, a group of experts developed and tested the REST-LGS Case Report Form (CRF) comprising 8 criteria (4 major, 4 minor) considered potentially indicative of LGS. Diagnosis-blinded specialist and nonspecialist raters at 2 epilepsy centers applied the CRF to deidentified patient records, including 1:1 records of patients with drug-resistant epilepsy or confirmed LGS. Interrater reliability was measured by Cohen's κ. Diagnosis was then unblinded to reveal common criteria for LGS or drug-resistant epilepsy. Cronbach's α was used to measure internal consistency between raters for all criteria combined. Results: Of 200 patients, 81% to 85% met 1 to 3 major criteria. At both sites, moderate (κ, 0.41-0.60) to good (κ, 0.61-0.80) agreement on most criteria was reached between expert and nonexpert raters. Unblinding revealed that most patients with LGS met 3 major and 2 to 3 minor criteria, while patients with drug-resistant epilepsy met ≤1 major and only 1 to 2 minor criteria. Cronbach's α of raters at both sites was 0.64. Conclusions: The combined number of major/minor criteria on the CRF may be particularly indicative of LGS. Therefore, the REST-LGS may be a valuable clinical tool in identifying patients requiring further diagnostic evaluation for LGS.

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“…12 Support from a multidisciplinary team, including epilepsy specialists, physicians, nurses, psychiatrists, psychologists, physiotherapists, education specialists, social workers, family members, and friends, is important in the treatment and management of LGS. 2,13,14 Frequent "drop attacks" caused by multiple seizure types lead to injuries, 2 as well as burdensome medical costs. 15 Although patients can use headgear to prevent some injuries, this can interfere with the quality of life (QoL), as it is highly stigmatizing and may deter participation in activities.…”

Section: Introduction: Lennox-gastaut Syndromementioning

confidence: 99%

“…2,13,14 By knowing how to classify seizures, as well as the efficacy and side effects of various AEDs, including rufinamide, nurses can assist patients and their caregivers with the treatments prescribed to patients. Table 3 lists the recommended resources for families affected by LGS.…”

mentioning

confidence: 99%

Rufinamide in patients with Lennox-Gastaut syndrome

Clark¹,

Gibson²

2016

NRR

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Objectives: Lennox-Gastaut syndrome (LGS) is an epileptic encephalopathy with an onset at the age of ~4 years.LGS is notoriously difficult to manage, as most patients experience multiple seizures per day, despite their concomitant use of several antiepileptic drugs (AEDs). Rufinamide (BANZEL ® ) is an AED approved by the US Food and Drug Administration for the adjunctive treatment of seizures associated with LGS in pediatric patients aged ≥1 year and in adults. The expert care of nurses knowledgeable in the treatment options for LGS is valuable to patients and caregivers. This review summarizes the existing knowledge on LGS and data from clinical and real-world studies on the use of rufinamide in patients with LGS. Methods: Recent review articles and information from the Epilepsy Foundation Website were reviewed for data on LGS treatment. Primary articles on rufinamide were also selected for review. Results and conclusion: The efficacy and safety of rufinamide have been evaluated in children and adults by using double-blind, open-label, and observational studies. In general, these studies indicate that rufinamide effectively reduces the frequency and severity of multiple seizure types associated with LGS and has tolerable side effects, the most common being vomiting and somnolence. Dosing modifications based on age, weight, and concomitant AED usage are recommended for patients using rufinamide.

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Lennox-Gastaut Syndrome (LGS): Development of conceptual models of health-related quality of life (HRQL) for caregivers and children

Cited by 47 publications

References 16 publications

Comorbidities and predictors of health‐related quality of life in Dravet syndrome

Comorbidities and predictors of health‐related quality of life in Dravet syndrome

The refractory epilepsy screening tool for Lennox–Gastaut syndrome (REST-LGS)

Rufinamide in patients with Lennox-Gastaut syndrome

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