| 1. |
- Bjurulf, Björn, 1962, et al.
(författare)
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Caregiver reported behavior, sleep and quality of life in children with Dravet syndrome: A population-based study
- 2024
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Ingår i: Epilepsy and Behavior. - 1525-5050 .- 1525-5069. ; 150
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Tidskriftsartikel (refereegranskat)abstract
- Objective: The aim of this population-based study was to assess behavior, sleep, and quality of life, and explore factors associated with these in children with Dravet syndrome. Methods: The Developmental Behavior Checklist, the Insomnia Severity Index, and a global question regarding quality of life from the Epilepsy and Learning Disabilities Quality of Life scale were completed by primary caregivers of 42/48 Swedish children with Dravet syndrome, born 2000–2018. Factors associated with problems with insomnia, behavior and quality of life were analyzed using multivariable linear regression. Results: Scores indicating significant behavioral problems were seen in 29/40 (72 %) children, scores indicating moderate or severe clinical insomnia in 18/42 (43 %) and scores indicating poor or very poor quality of life in 7/41 (17 %). On multivariable analysis, autistic symptoms were significantly associated with behavioral problems (p = 0.013), side-effects of anti-seizure medications (ASMs) were associated with insomnia (p = 0.038), whilst insomnia was significantly associated with poor quality of life (p = 0.016). Significance: Dravet syndrome in children is associated with significant problems with behavior, sleep and quality of life. There is a need to optimize treatment via ASMs and develop and evaluate interventions to treat behavioral and sleep difficulties to optimize outcomes.
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| 2. |
- Bjurulf, Björn, 1962, et al.
(författare)
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Caregiver reported seizure precipitants and measures to prevent seizures in children with Dravet syndrome
- 2022
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Ingår i: Seizure. - : Elsevier BV. - 1059-1311. ; 103, s. 3-10
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Tidskriftsartikel (refereegranskat)abstract
- Objective: The aim of this population-based, cross-sectional study was to describe caregiver-reported seizure precipitants, measures taken to prevent seizures and rescue therapies in children with Dravet Syndrome (DS). Methods: In a population-based study, caregivers of 42/48 Swedish children with DS born between 2000 and 2018 were interviewed. Frequency of precipitants, preventive measures, and rescue therapies were compared between children born 2000-2009 and 2010-2018 and between `severe acute accent and `less severe acute accent epilepsy.Results: All children had experienced precipitants. Preventive measures were employed in all. Seizures had been provoked by a median of seven (range 2-11) out of 13 factors. A median of eight (range 1-17) preventive measures out of 19 were reported. The most common precipitants were fever (n=42, 100%), and afebrile in-fections (n=39/42, 93%). Afebrile infections (p=0.014) and reduced ambient temperature (p=0.006) were more common precipitants in younger children, and bright light in children with severe epilepsy (p=0.013).The most common factors avoided were warm weather (n=35/42, 83%) and physical activity (n=27/42, 64%). It was more common to avoid strong emotions (p=0.035) and reduced temperature (p=0.002) in younger children, and to avoid infections (p=0.024) and crowds (p=0.046) in children with 'severe' epilepsy. Many children (n=28/42, 67%) or their siblings (n=16/34, 47% of individuals with siblings) had stayed home to avoid infections in school/day-care. Use of emergency medicines was more frequent in younger children (p=0.006) and in children with 'severe' epilepsy (p=0.007). Significance: Caregiver-reported seizure precipitants are common in DS. Caregivers employ a range of measures to avoid seizures, restricting family life.
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| 3. |
- Bjurulf, Björn, 1962, et al.
(författare)
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Dravet syndrome in children - A population-based study
- 2022
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Ingår i: Epilepsy Research. - : Elsevier BV. - 0920-1211 .- 1872-6844. ; 182
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Tidskriftsartikel (refereegranskat)abstract
- Objective: The aim was to describe age at diagnosis, cumulative incidence, SCN1A variants, mortality, seizure types and treatments in children with Dravet Syndrome (DS) in Sweden. Methods: Children diagnosed with DS, born between January 1st 2000 and December 31st 2018 were included in a population-based study. Clinical data, frequency of seizure types and treatments were collected from caregivers and medical records in 42 children. Age at diagnosis, cumulative incidence and treatment were compared between children born in Sweden 2000–2009 and 2010–2018. Results: We identified 55 children with DS, 53 were born in Sweden. Three children had died of definite, probable, or possible sudden unexpected death in epilepsy, one of acute anoxic brain injury and three of pneumonia or pneumonitis. Median age at death was 4.7 (range 3.3–11) years. In 49/53 children with known SCN1A status, a pathogenic/likely pathogenic variant of SCN1A was detected. In two a SCN1A variant of unknown significance was found. For children born in Sweden 2010–2018, median age at DS diagnosis was lower (1.6 vs 4.5 years, p = 0.001) and cumulative incidence higher (1/33,000 vs 1/46,000 live-born children, p = 0.03), compared to children born in 2000–2009. The most common seizure types were focal to bilateral tonic clonic (n = 41/42) and myoclonic (n = 35/42). Tonic seizures were reported in 25/42 children. Sodium-channel inhibitors had been used in 9/24 children born in 2010–2018 and 17/18 children born in 2000–2009 (p = 0.001). Significance: A SCN1A variant that could explain the syndrome was found in over 90% of children. Tonic seizures seem to be more frequent than earlier described. Median age at diagnosis was lower, cumulative incidence higher and use of contra-indicated sodium-channel inhibitors less common for children born in 2010–2018 compared with children born in 2000–2009. This could indicate an increased awareness of DS.
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| 4. |
- Dellenmark-Blom, Michaela, 1983, et al.
(författare)
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Schooling experiences in children with long-gap esophageal atresia compared with children with esophageal atresia and primary anastomosis: a Swedish study
- 2023
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Ingår i: Orphanet Journal of Rare Diseases. - : BioMed Central (BMC). - 1750-1172. ; 18:1
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundChildren with long-gap esophageal atresia (LGEA) risk living with aerodigestive morbidity and mental health difficulties. No previous study has investigated their experiences of schooling, despite the importance of schools in children's development, learning and social relationships. We aimed to describe experiences of schooling in children with LGEA in Sweden in comparison with children with EA who had primary anastomosis.MethodChildren with LGEA aged 3-17 were recruited nationwide in Sweden. One parent completed a survey on their child's school-based supports (according to definitions from the Swedish National Agency for Education), school absence, school satisfaction, school functioning (PedsQL 4.0), mental health (Strength and Difficulties Questionnaire) and current symptomatology. School data were compared between 26 children with LGEA to that from 95 children with EA who had PA, a hypothesized milder affected group. Mental health level was determined using validated norms; abnormal & GE; 90 percentile. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05.ResultsFormal school-based support was reported in 17 (65.4%) children with LGEA and concerned support with nutritional intake (60%), education (50%) and medical/special health needs (35%). The prevalence of school-based support was significantly higher compared to children with PA overall (36.8%, p = 0.013) and regarding nutritional intake support (20%, p < 0.001). In children with LGEA, school-based support was related to low birth weight (p = 0.036), young child age (p = 0.014), height & LE; -2SD for age/sex (p = 0.024) and an increased number of aerodigestive symptoms (p < 0.05). All children with LGEA who had abnormal mental health scores had school-based support, except for one child. Nine children with LGEA (36%) had school absence & GE; 1times/month the past year, more frequently because of colds/airway infections (p = 0.045) and GI-specific problems compared to PA (p = 0.003). School functioning scores were not significantly different from children with PA (p = 0.34) but correlated negatively with school-based support (< 0.001) and school absence (p = 0.002). One parent out of 26 reported their child's school satisfaction as "not good".ConclusionsChildren with LGEA commonly receive school-based support, reflecting multifaceted daily needs and disease severity. School absence is frequent and related to poorer school functioning. Future research focusing on academic achievement in children with EA is needed.
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| 5. |
- Goselink, Rianne J. M., et al.
(författare)
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Transition to adult care in epilepsy: A systematic review
- 2022
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Ingår i: Seizure. - : Elsevier BV. - 1059-1311 .- 1532-2688. ; 101, s. 52-59
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Tidskriftsartikel (refereegranskat)abstract
- The transfer from paediatric to adult care can be a complex process in children with epilepsy. Inadequate care during this phase can affect long-term medical and psychosocial outcomes. The aim of this study was to review studies on transitional care from paediatric to adult healthcare for young persons with epilepsy in order to synthesize evidence for best practice. We undertook a systematic review following PRISMA guidelines and employed narrative synthesis. A total of 36 articles were included, of which 11 were interventional studies and 25 observational studies. Study quality was rated as 'good' for only four studies. Interventions included joint or multidisciplinary clinics, education (patient and health professional education) and extended service provision (Saturday clinics, peer-groups). All studies observed a positive effect experienced by the participants, regardless of intervention type. Observational studies showed that transition plans/programmes are asked for but frequently not existing or not adapted to subgroups with intellectual disability or other neurodevelopmental conditions. The results of this systematic review on transitional care in epilepsy suggest that a planned transition process likely enhances medical and psychosocial outcomes for young people with epilepsy, but the body of evidence is limited and there are significant gaps in knowledge of what efficacious transition constitutes. More studies are needed employing qualitative and quantitative methods to further explore the needs of young people with epilepsy and their families but also robust study designs to investigate the impact of interventions on medical and psychosocial outcomes.
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| 6. |
- Hunter, R. M., et al.
(författare)
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The health, education, and social care costs of school-aged children with active epilepsy: A population-based study
- 2015
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Ingår i: Epilepsia. - : Wiley. - 0013-9580. ; 56:7, s. 1056-1064
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Tidskriftsartikel (refereegranskat)abstract
- ObjectiveTo provide data on the health, social care, and education costs of active childhood epilepsy and factors associated with these costs over an 18-month period in a population-based sample. MethodsThe Children with Epilepsy in Sussex Schools (CHESS) study is a population-based study involving school-aged children (5-15years) with active epilepsy (taking one or more antiepileptic drug and/or had a seizure in the last year) in a defined geographical area in England. Clinical data were collected on 85 children (74% of eligible population) who underwent comprehensive psychological assessment. Health, education, and social care resource use was collected retrospectively over an 18-month period. Regression analysis was used to identify variables associated these with costs. ResultsThe mean (standard deviation) 18-month cost of health care for a child with active epilepsy was 3,635 (5,339) pound, with mean education and social care cost of 11,552 pound (8,937) pound and 1,742 pound (8,158) pound, respectively, resulting in total mean costs per participant of 16,931 pound (14,764) pound. Health care costs were significantly associated with seizure frequency and etiology (all p-values<0.05). Combined health care, social care, and education costs were significantly related to cognitive impairment (intelligence quotient [IQ] <85) and seizure frequency (p<0.05). The mean cost of health care, social care, and education over 18months for participants with cognitive impairment was 23,579 pound (95% confidence interval [CI] 16,489- pound 30,670) pound compared to 7,785 pound (95% CI 4,943- pound 10,627) pound for those without impairment. SignificanceActive childhood epilepsy has significant health, social care, and education costs. This is the first study to comprehensively document the economic impact on these sectors as well as factors associated with these costs. When caring for children with epilepsy in England, costs incurred by education and social care sectors are approximately four times the costs incurred by the health care sector. Increased costs were associated with cognitive impairment (IQ <85) and weekly or greater seizure frequency.
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| 7. |
- Johnson, Emma C, et al.
(författare)
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Physical activity in people with epilepsy: A systematic review.
- 2020
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Ingår i: Epilepsia. - : Wiley. - 1528-1167 .- 0013-9580.
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Forskningsöversikt (refereegranskat)abstract
- 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.
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| 8. |
- Jones, C., et al.
(författare)
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Parental anxiety in childhood epilepsy: A systematic review
- 2016
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Ingår i: Epilepsia. - : Wiley. - 0013-9580. ; 57:4, s. 529-537
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Tidskriftsartikel (refereegranskat)abstract
- The aim was to systematically review studies that have focused on symptoms of anxiety reported by parents of children (0-18 years) with epilepsy. PubMed was used to identify relevant studies. Selected studies were reviewed with respect to prevalence of above threshold scores and comparisons with controls on standardized measures of anxiety. Studies are also reported with respect to factors associated with parental anxiety, impact on child outcomes, and comparisons with studies that have included equivalent measures of symptoms of depression. Fifteen studies that met inclusion criteria were identified. None of the studies were population based. The percentage of parents scoring above cutoffs on standardized measures of anxiety was 9-58%. In comparison with parents of healthy controls, parents of children with epilepsy had higher mean scores in two of three studies where this was measured. Possible correlates of parental anxiety in childhood epilepsy that were considered varied widely across studies. Factors such as seizure frequency and use of antiepileptic drugs (AEDs) have been associated with parental anxiety in some but not all studies. With respect to child outcome, increased parental anxiety has been associated with lower quality of life and lower scores on adaptive behavior domains. Symptoms of anxiety are common among parents of children with epilepsy. There is a need for more systematic, representative studies to identify the prevalence of clinically significant anxiety and track the course of symptoms. Such studies will help to identify more clearly factors associated with parental anxiety and impact of symptoms on child and parent outcomes. Intervention studies are needed to evaluate approaches that target a reduction in symptoms and the potential impact on parental and child functioning. Furthermore, there is a need to evaluate the impact of antiepileptic therapies and interventions that focus on child neurobehavioral comorbidities on parental anxiety
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| 9. |
- Krantz, Mattias, et al.
(författare)
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Parental experiences of having a child with CLN3 disease (juvenile Batten disease) and how these experiences relate to family resilience
- 2022
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Ingår i: Child: Care, Health and Development. - : Wiley. - 0305-1862 .- 1365-2214. ; 48:5, s. 842-851
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Tidskriftsartikel (refereegranskat)abstract
- Background: CLN3 disease is a neurodegenerative condition presenting in the first decade of life typically leading to death in the third decade. The earliest symptom is rapidly progressive visual impairment followed by intellectual and motor impairments, epilepsy and behavioural disturbances. There are limited data on how the condition affects the family system or the role of family resilience in paediatric neurodegenerative diseases. Methods: Semi-structured interviews were conducted with eight parents (five mothers and three fathers) of five children with CLN3. Interview questions focused on the experience of having a child with CLN3, its impact on the family system as well as the concept of family resilience. Data were analysed via thematic analysis. Results: The thematic analysis resulted in four main themes. The theme ‘recurring losses’ included the feeling of losing a healthy child, the child's loss of abilities and loss of relationships. The theme ‘disruption to the family system’ included that siblings could be ‘side-lined’, the potential negative impact on romantic relationships and difficulties finding time to oneself. The theme ‘Society is not developed for a progressive disease’ highlighted the difficulties parents faced with respect to contacts with the health and/or social insurance system. The paediatric health care system was seen as supportive, but the adult health care system was not seen as fit for the purpose. Regarding family resilience, parents felt that the disease forced them to reconsider what was important in life. Several parents described that they learned to value small moments of joy and create deep connections through involvement in family routines and rituals. Conclusions: CLN3 places a very significant burden on the family system including parental feelings of loss, impact on family relationships and lack of understanding within the health/social insurance systems. The concept of family resilience may be useful in understanding the experiences of families affected by paediatric neurodegenerative conditions.
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| 10. |
- McWilliams, A., et al.
(författare)
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Nonepileptic seizures in the pediatric population: A qualitative study of patient and family experiences
- 2016
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Ingår i: Epilepsy & Behavior. - : Elsevier BV. - 1525-5050. ; 59, s. 128-136
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Tidskriftsartikel (refereegranskat)abstract
- Objective: The objective of this study was to characterize the experience of nonepileptic seizures (NES) in young people (0-19 years) and their families, referred to a UK specialist (tertiary) pediatric hospital. The topics investigated include: accessing healthcare, how the diagnosis was first explained, impact on home life and school, coping strategies, and ideas about naming and causes. Methods: Ten young people with NES and 29 family members took part in focus groups and telephone interviews. The data generated were analyzed qualitatively with thematic analysis. Results: Six themes were identified from participant experiences: upset and afraid, missing out, feeling misunderstood, confusion and uncertainty, less than epilepsy, and making sense and moving on. Participants described severe disruption to multiple domains of functioning at home, educationally, and in social activities. Young people felt guilty but also overprotected, while family members felt that they were failing as parents. The journey to diagnosis and treatment was seen as unnecessarily tortuous, with access to care and treatment pathways poorly defined. Participants described feeling that a wide variety of professionals did not believe their experiences, showed pejorative attitudes, and left them feeling isolated and marginalized. The young people and family members found NES a difficult disorder to understand and sometimes could not differentiate it from epilepsy. Epilepsy was used as a benchmark for several comparisons, including highlighting the lack of support for and information about NES. Families disliked being told that it was "good news" that their child did not have epilepsy and questioned if their child should be present during initial diagnostic discussions. Participants described stressful situations as a common trigger for NES. Young people showed ambivalence towards the need to understand the condition or the choice of name used for it, whereas family members considered this crucial for achieving recovery. Conclusions: Young people and families who live with NES experience considerable distress and impairment. Pathways to diagnosis need to be streamlined, and better integration of pediatric, mental health, and educational services is required. The use of the "good news" story to discuss the diagnosis with families should be reconsidered, as families seem to interpret this as indicating that there is no effective treatment. Educational resources and support groups for young people and families are needed. Greater understanding of experiences may allow investigation of the pathogenic mechanism and inform possible management approaches. Training of health professionals in communicating with young people and families with NES must be improved. (C) 2016 Elsevier Inc. All rights reserved.
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| 11. |
- Reilly, Colin, 1977, et al.
(författare)
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Academic achievement in school-aged children with active epilepsy: A population-based study.
- 2014
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Ingår i: Epilepsia. - : Wiley. - 1528-1167 .- 0013-9580. ; 55:12, s. 1910-1917
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: To provide population-based data on the performance of school-aged children with epilepsy on measures of academic achievement and factors associated with this performance after controlling for IQ. METHODS: Eighty-five (74%) of 115 children with "active" epilepsy (experienced a seizure in the past year and/or on antiepileptic drugs [AEDs]) underwent psychological assessment including measures of IQ, aspects of working memory and processing speed. Sixty-five of the 85 were able to complete subtests on the Wide Range Achievement Test-Fourth Edition (WRAT-4). Paired sample t-tests were conducted to compare subtest scores. Factors associated with academic performance after controlling for IQ were examined using linear regression. RESULTS: Seventy-two percent of the children, who could complete subtests on the WRAT-4, displayed "low achievement" (1 standard deviation [SD] below test mean) and 42% displayed "underachievement" (1 SD below assessed IQ) on at least one of the four WRAT-4 subtests. The mean scores on the Math Computation subtest and Sentence Comprehension subtest were significantly lower than scores on the Word Reading (p < 0.05) and Spelling (p < 0.001) subtests. Younger age at seizure onset was associated (p < 0.05) with decreased scores on three of the four WRAT-4 subtests after controlling for IQ. Difficulties with auditory working memory were associated with difficulties on reading comprehension (p < 0.05), and parent-reported difficulties with school attendance were associated with decreased scores on the Spelling and Word Reading subtests after controlling for IQ (p < 0.05). SIGNIFICANCE: Difficulties with academic achievement are common in school-aged children with "active" epilepsy. Much of the difficulties can be attributed to lowered global cognition. However, specific cognitive deficits, younger onset of first seizure, and school attendance difficulties may contribute to difficulties independent of global cognition. There is a need to screen all children with "active" epilepsy for difficulties in school achievement, to identify contributory factors and to identify efficacious interventions for ameliorating such difficulties.
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| 12. |
- Reilly, Colin, 1977, et al.
(författare)
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Autism and attention-deficit/hyperactivity disorder in children with Dravet syndrome: A population-based study
- 2024
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Ingår i: DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY. - 0012-1622 .- 1469-8749.
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Tidskriftsartikel (refereegranskat)abstract
- AimTo identify on a population basis the prevalence of autism and attention-deficit/hyperactivity disorder (ADHD) in children with Dravet syndrome and factors associated with symptoms of autism and ADHD. MethodForty-one of 48 children with Dravet syndrome living in Sweden, born between 1st January 2000 and 31st December 2018 underwent assessment including measures of autism and ADHD. Diagnoses of autism and ADHD were made with respect to DSM-5 criteria. Factors associated with features of autism and ADHD were analysed via regression. ResultsTwenty-five of the 41 children fulfilled DSM-5 criteria for autism spectrum disorder and 12 of 37 children considered for an ADHD diagnosis fulfilled DSM-5 criteria for ADHD. Severe intellectual disability was significantly associated with a greater degree of autistic features (p < 0.001) and a DSM-5 diagnosis of autism spectrum disorder (p = 0.029). Younger children had significantly more features of ADHD (p = 0.004) and features of inattention were significantly more common than features of hyperactivity/impulsivity (p < 0.001). InterpretationChildren with Dravet syndrome often have significant features of autism and ADHD, primarily inattentive type. Screening for autism and ADHD should be routine in children with Dravet syndrome.
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| 13. |
- Reilly, Colin, 1977, et al.
(författare)
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Cognition in school-aged children with "active" epilepsy: A population-based study.
- 2015
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Ingår i: Journal of clinical and experimental neuropsychology. - : Informa UK Limited. - 1744-411X .- 1380-3395. ; 37:4, s. 429-438
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Tidskriftsartikel (refereegranskat)abstract
- INTRODUCTION: There is a lack of population-based data on specific cognitive profiles in childhood epilepsy. This study sought to determine the frequency of impairments in global cognition and aspects of working memory and processing speed in a population-based sample of children with "active" epilepsy (on antiepileptic Drugs (AEDs), and/or had a seizure in the last year). Factors significantly associated with global and specific difficulties in cognition were also identified. METHOD: A total of 85 (74% of eligible population) school-aged children (5-15 years) with "active" epilepsy underwent comprehensive psychological assessment including assessment of global cognition, working memory, and processing speed. Scores on cognitive subtests were compared via paired-samples t tests. The factors associated with cognitive difficulties were analyzed via linear regression. RESULTS: A total of 24% of children were functioning below IQ 50, and 40% had IQ scores below 70. Scores on the Processing Speed Index were significantly lower than scores on the Verbal or Performance indexes on Wechsler instruments. The Coding subtest was a significant weakness compared with the other Wechsler subtests. A total of 58% of children displayed "memory underachievement" (memory score 1 SD below assessed IQ) on at least one of the four administered working memory subtests. Factors significantly associated with globally impaired cognition included being on polytherapy (β = -13.0; 95% CI [-19.3, -6.6], p = .000) and having attention-deficit/hyperactivity disorder (ADHD; β = -11.1, 95% CI [-3.0, -19.3], p = .008). Being on polytherapy was also associated with lower scores on the working memory and processing speed composite scores. Having developmental coordination disorder (DCD) was associated with a lower score on the processing speed composite. CONCLUSIONS: There is a high rate of global and specific cognitive difficulties in childhood epilepsy. Difficulties are most pronounced in aspects of working memory and processing speed. Predictors of cognitive impairment in childhood epilepsy include epilepsy-related and behavioral factors, which may differ depending on the domain of cognition assessed.
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| 14. |
- Reilly, Colin, 1977, et al.
(författare)
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Epilepsy
- 2015
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Ingår i: Comorbid Conditions Among Children with Autism Spectrum Disorders. - Cham : Springer. - 9783319191829 ; , s. 235-256
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Various theories have emerged regarding common neural pathways between autism spectrum disorder (ASD) and other conditions. Assumptions such as this are bolstered by the high rate of overlap with other genetic conditions such as epilepsy. This chapter describes the disorder, its prevalence and relationship to ASD.
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| 15. |
- Reilly, Colin, 1977, et al.
(författare)
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Factors associated with quality of life in active childhood epilepsy: A population-based study.
- 2015
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Ingår i: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. - : Elsevier BV. - 1532-2130. ; 19:3, s. 308-313
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Improving health-related quality of life (HRQOL), rather than just reducing seizures, should be the principal goal in comprehensive management of childhood epilepsy. There is a lack of population-based data on predictors of HRQOL in childhood epilepsy. METHODS: The Children with Epilepsy in Sussex Schools (CHESS) study is a prospective, population-based study involving school-aged children (5-15 years) with active epilepsy (on one or more AED and/or had a seizure in the last year) in a defined geographical area in the UK. Eighty-five of 115 (74% of eligible population) children underwent comprehensive psychological assessment including measures of cognition, behaviour, and motor functioning. Parents of the children completed the Quality of Life in Childhood Epilepsy (QOLCE).Clinical data on eligible children was extracted using a standardised pro forma. Linear regression analysis was undertaken to identify factors significantly associated with total Quality of Life in this population. RESULTS: Factors independently significantly associated (p < .05) with total QOLCE scores were seizures before 24 months, cognitive impairment (IQ < 85), anxiety, and parent reported school attendance difficulty. These factors were also significantly associated with total QOLCE when children with IQ < 50 were excluded from analysis. CONCLUSIONS: The majority of factors associated with parent reported HRQOL in active childhood epilepsy are related to neurobehavioural and/or psychosocial aspects of the condition.
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| 16. |
- Reilly, Colin, 1977, et al.
(författare)
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Features of autism spectrum disorder (ASD) in childhood epilepsy: A population-based study.
- 2015
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Ingår i: Epilepsy & behavior : E&B. - : Elsevier BV. - 1525-5069. ; 42C, s. 86-92
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Tidskriftsartikel (refereegranskat)abstract
- In a defined geographical area in the south of the UK, 115 children with active epilepsy (i.e., children who had seizures in the last year and/or children who are currently taking antiepileptic drugs (AEDs)) were identified via a computerized database and liaison with local pediatricians. Eighty-five (74%) of the children (5-15years of age) underwent a comprehensive psychological assessment. Twenty-one percent of the children met the DSM-IV-TR criteria for ASD, and 61% of them had another DSM-IV-TR behavioral or motor disorder. The Autism Spectrum Screening Questionnaire (ASSQ) was completed by parents (n=69) and by teachers (n=67) of children with an IQ>34. Only 9% of children on parent ratings and 15% of children on teacher ratings had no features of ASD. Parents reported significantly (p<.05) more features of ASD on the ASSQ compared with teachers. Factors significantly associated with responses on the ASSQ included respondent (parents reported more features), school placement (more features in specialized settings), and respondent by school placement interaction. Effective screening for ASD in children with epilepsy will need a consideration of the impact of informant and school placement on ratings. In conclusion, features of ASD were common in children with epilepsy regardless of cognitive ability. The ASSQ was a useful screening instrument in this population, and combining parent and teacher forms was optimal in terms of screening properties.
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| 17. |
- Reilly, Colin, 1977, et al.
(författare)
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Features of developmental coordination disorder in active childhood epilepsy: a population-based study.
- 2015
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Ingår i: Developmental medicine and child neurology. - : Wiley. - 1469-8749 .- 0012-1622. ; 57:9, s. 829-834
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Tidskriftsartikel (refereegranskat)abstract
- AIMS: To provide data on parent-reported features of developmental coordination disorder (DCD) and describe neurobehavioural comorbidity in children with epilepsy and DCD. METHOD: Eighty-five (74% of those eligible) children (44 males, 41 females; age range 5-15y) with active childhood epilepsy (an epileptic seizure in the last year and/or currently taking antiepileptic drugs) in a population-based cohort underwent comprehensive multidisciplinary assessment. The DCD Questionnaire (DCD-Q) was completed by parents (n=69) of children with an IQ>34, of whom 56 did not have cerebral palsy (CP), and were considered for a diagnosis of DCD. RESULTS: Of those considered for a DCD diagnosis, 16 (29%) met DSM-IV-TR criteria whereas 34 (61%) scored in the at-risk range on the DCD-Q. The sensitivity of the DCD-Q was 100% (95% CI 76-100) and specificity was 55% (95% CI 39-70). Significant predictors of higher scores on the DCD-Q included the presence of autism spectrum disorder, CP, and early seizure onset. Increasing age and IQ were independently associated with higher DCD-Q scores. Intellectual disability, attention-deficit-hyperactivity disorder, academic underachievement, and specific memory problems were the most common neurobehavioural difficulties in those with both DCD and epilepsy. INTERPRETATION: Parent-reported symptoms of DCD are very common in childhood epilepsy. The DCD-Q has good sensitivity but lower specificity in this population.
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| 18. |
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| 19. |
- Reilly, Colin, 1977, et al.
(författare)
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Health-related quality of life and emotional wellbeing improve in parents after their children have undergone epilepsy surgery A prospective population-based study
- 2017
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Ingår i: Epilepsy & Behavior. - : Elsevier BV. - 1525-5050. ; 75, s. 196-202
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Tidskriftsartikel (refereegranskat)abstract
- The objective was to compare parental health-related quality of life (HRQoL), anxiety, and depression at baseline and 2 years after epilepsy surgery in a population-based series of children and young people who underwent surgery between 1995 and 1999 and to compare with population norms. Fifty mothers and 44 fathers of 50 children and young people (age: 1-20 years) completed the Medical Outcome Study 36-item Short Form Health Survey (SF-36) and Hospital Anxiety and Depression (HAD) scale at baseline and at follow-up. Changes in SF-36 and HAD scores between baseline and follow-up were compared using Wilcoxon signed rank test. Scores on the SF-36 were compared with a reference sample from the Swedish population using the Mann Whitney U test. Factors associated with changes in SF-36 and HAD scores were analyzed using regression analysis. On the SF-36, the Physical Component Summary (PCS) scores were not significantly different between baseline and follow-up for mothers (p = 0.177) or fathers (p = 0.054). Mental Component Summary (MCS) scores improved significantly for mothers (p = 0.008) and fathers (p < 0.001). Mothers' baseline scores on seven of eight SF-36 domains were significantly lower than reference values. Scores at follow-up improved on these seven domains, but on three domains (primarily mental health domains), scores remained significantly lower than reference values. Fathers' baseline scores on four of eight SF-36 domains were significantly lower than reference values, and scores at follow-up remained significantly lower on the four primarily mental health domains. The proportions of mothers and fathers classified as HAD-A and HAD-D cases decreased at follow-up but did not reach statistical significance. Child epilepsy variables were in the main not associated with parental outcomes, but a greater reduction in AEDs was associated with a greater reduction in PCS scores. Parents of young people/children with seizure-free outcome were significantly more likely to have a reduction in depression scores than parents of young people/children with continued seizures. Many aspects of HRQoL and emotional wellbeing improved at 2-year follow-up for parents after epilepsy surgery on their children. There is a need to comprehensively identify factors associated with changes in parental HRQoL and emotional wellbeing to provide adequate support. (C) 2017 Elsevier Inc All rights reserved.
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| 20. |
- Reilly, Colin, 1977, et al.
(författare)
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Intellectual functioning and adaptive behaviour in children with Dravet syndrome: A population-based study
- 2023
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Ingår i: Developmental Medicine & Child Neurology. - : Wiley. - 0012-1622 .- 1469-8749. ; 65:6, s. 831-837
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Tidskriftsartikel (refereegranskat)abstract
- Aim: To identify, on a population basis, the prevalence of intellectual disability in children with Dravet syndrome, profiles on a measure of adaptive behaviour, and factors associated with intellectual functioning and adaptive behaviour. Method: Forty-two out of 48 children with Dravet syndrome living in Sweden, born between 1st January 2000 and 31st December 2018, underwent assessment of intellectual functioning and adaptive behaviour. Factors associated with level of intellectual functioning and adaptive behaviour were analysed. Results: Eight-six per cent (n = 36) of the children fulfilled DSM-5 criteria for intellectual disability (29% [n = 12] mild intellectual disability, 24% [n = 10] moderate intellectual disability, 33% [n = 14] severe intellectual disability, 0% profound intellectual disability) and 93% (n = 39) had an adaptive behaviour composite more than two standard deviations below the mean. Communication was a significant weakness compared with daily living skills (p < 0.001; mean difference 95% confidence interval [CI] -8.193 to -4.092) and socialization (p = 0.001; mean difference 95% CI 6.511 to -1.775) on the Vineland Adaptive Behavior Scales, Second Edition. The only factors significantly associated with both decreased adaptive behaviour and presence of severe intellectual disability was the presence of increased autistic symptoms and younger age. Interpretation: Children with Dravet syndrome have a very high level of intellectual disability and almost all have significant deficits in adaptive behaviour. Greater deficits in adaptive behaviour and greater severity of intellectual disability are associated with the presence of increased autistic symptoms, highlighting the need for comprehensive neurodevelopmental assessment for all affected children.
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| 21. |
- Reilly, Colin, 1977, et al.
(författare)
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Neurobehavioral Comorbidities in Children With Active Epilepsy: A Population-Based Study.
- 2014
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Ingår i: Pediatrics. - : American Academy of Pediatrics (AAP). - 1098-4275 .- 0031-4005. ; 133:6
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Tidskriftsartikel (refereegranskat)abstract
- In addition to recurrent epileptic seizures, children with epilepsy can have coexisting cognitive and behavioral difficulties but the spectrum and prevalence of such difficulties are uncertain.METHODS: The Children with Epilepsy in Sussex Schools study is a prospective, community-based study involving school-aged children (5-15 years) with active epilepsy in a defined geographical area in the United Kingdom. Participants underwent comprehensive psychological assessment, including measures of cognition, behavior, and motor functioning. Consensus neurobehavioral diagnoses were made with respect to Diagnostic and Statistical Manual, Fourth Edition-Text Revision (DSM-IV-TR) criteria.RESULTS: A total of 85 children (74% of eligible population) were enrolled; 80% of children with active epilepsy had a DSM-IV-TR behavioral disorder and/or cognitive impairment (IQ <85). Intellectual disability (ID) (IQ <70) (40%), attention-deficit/hyperactivity disorder (ADHD) (33%), and autism spectrum disorder (ASD) (21%) were the most common neurobehavioral diagnoses. Of those who met criteria for a DSM-IV-TR behavioral disorder, only one-third had previously been diagnosed. Logistic regression revealed that seizures in the first 24 months compared with first seizures at 24 to 60 or 61+ months (odds ratio [OR] 13, 95% confidence interval 2.2-76.9; OR 21.3, 3.2-148.9) and polytherapy (OR 7.7, 1.6-36.3) were independently associated with ID and the presence of ID was associated with a diagnosis of ASD (OR 14.1, 2.3-87.1) after Bonferroni adjustment. Epilepsy-related factors did not independently predict the presence of behavioral disorders.CONCLUSIONS: Screening for neurobehavioral comorbidities should be an integral part of management in children with "active" epilepsy. There is a need for research to identify neurobiological mechanisms underpinning neurobehavioral impairments and studies to evaluate possible treatments.
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| 22. |
- Reilly, Colin, 1977, et al.
(författare)
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Parent- and Teacher-Reported Symptoms of ADHD in School-Aged Children With Active Epilepsy: A Population-Based Study.
- 2017
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Ingår i: Journal of attention disorders. - : SAGE Publications. - 1557-1246 .- 1087-0547. ; 21:11, s. 887-897
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: Provide data on the distribution of parent- and teacher-reported symptoms of ADHD in childhood epilepsy and describe coexisting cognitive and behavioral disorders in children with both epilepsy and ADHD. METHOD: Eighty-five (74% of those eligible) children (5-15 years) in a population-based sample with active epilepsy underwent psychological assessment. The ADHD Rating Scale-IV (ADHD-RS-IV) scale was completed by parents (n = 69) and teachers (n = 67) of participating children with an IQ > 34. ADHD was diagnosed with respect to Diagnostic and Statistical Manual of Mental Disorders (4th ed., text rev.). RESULTS: Parents reported significantly more symptoms of ADHD than teachers (p < .001). Symptoms of inattention were more commonly reported than symptoms of hyperactivity-impulsivity (p < .001). Neurobehavioral comorbidity was similar in those with ADHD and non-ADHD with the exception of oppositional defiant disorder (ODD) and developmental coordination disorder (DCD), which were more common in those with both epilepsy and ADHD. CONCLUSION: Symptoms of ADHD are very common in childhood epilepsy but prevalence is influenced by informant.
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| 23. |
- Reilly, Colin, 1977, et al.
(författare)
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Parent reported health related quality of life (HRQoL) and behaviour in young people with epilepsy before and two years after epilepsy surgery.
- 2020
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Ingår i: Seizure. - : Elsevier BV. - 1059-1311. ; 74, s. 1-7
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Tidskriftsartikel (refereegranskat)abstract
- The aim of this study was to compare parent-reported Health Related Quality of Life (HRQoL) and behaviour of young people before (baseline) and two years after paediatric epilepsy surgery (follow-up).The parents of 107 children who underwent epilepsy surgery completed surveys focussing on different aspects of child HRQoL and behaviour at baseline and follow-up. Parents of children with multiple disabilities (n=27) completed five additional questions focussing on child HRQoL. Changes in scores between baseline and follow-up were compared using Wilcoxon signed-rank tests. Factors associated with changes in scores were analyzed using linear regression.HRQoL and behaviour were significantly improved at follow-up (p<0.001). HRQoL was also significantly improved for children with multiple disabilities (p=0.003). Factors independently associated with improvement in HRQoL on multivariable analysis were lower baseline scores (p<0.001), seizure-free status (p<0.001) and improvement in behaviour (p=0.022). Factors independently associated with improvement in behaviour were higher baseline difficulties (p<0.001), reduction in antiepileptic drug (AED) usage, (p<0.001), seizure-free status (p=0.04), younger age (p=0.03), and improvements in HRQoL (p=0.028).Parent rated HRQoL and behaviour had improved two years after epilepsy surgery. Seizure freedom was associated with both improvements in HRQoL and behaviour. Additionally, a reduction in AED usage contributed to reduced behavioural difficulties. All children undergoing epilepsy surgery should undergo assessment of HRQoL and behaviour at baseline and follow-up.
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| 24. |
- Reilly, Colin, 1977, et al.
(författare)
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Parent-reported symptoms of ADHD in young people with epilepsy before and two years after epilepsy surgery
- 2019
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Ingår i: Epilepsy and Behavior. - : Elsevier BV. - 1525-5050 .- 1525-5069. ; 94, s. 29-34
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Tidskriftsartikel (refereegranskat)abstract
- The aim was to compare parent-reported symptoms of attention-deficit/hyperactivity disorder (ADHD) before (baseline) and two years after pediatric epilepsy surgery (follow-up). The parents of 107 children who underwent epilepsy surgery completed surveys including the Conners 10-item scale at baseline and follow-up. Changes in scores between baseline and follow-up were compared using paired sample t-test. Factors associated with changes in scores were analyzed using linear regression. Features of ADHD were significantly reduced at follow-up (p < 0.001). Items with the greatest reduction were items focusing on core aspects of the diagnostic criteria for ADHD. Fewer children were in the at-risk range for ADHD on the Conners 10-item scale at follow-up but this did not reach statistical significance (49% vs. 43%; p = 0.481). Factors independently significantly associated with improvement in ADHD symptoms on multivariable analysis were higher baseline scores (p < 0.001), seizure-free status (p = 0.029), and right-sided surgery (p = 0.031). Children who undergo epilepsy surgery have a high rate of ADHD symptoms. Parent-rated symptoms of ADHD improved at 2-year follow-up after epilepsy surgery. All children undergoing epilepsy surgery should undergo assessment for ADHD at baseline and follow-up.
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| 25. |
- Reilly, Colin, 1977, et al.
(författare)
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Pathological Demand Avoidance in a population-based cohort of children with epilepsy: Four case studies.
- 2014
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Ingår i: Research in developmental disabilities. - : Elsevier BV. - 1873-3379 .- 0891-4222. ; 35:12, s. 3236-3244
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Tidskriftsartikel (refereegranskat)abstract
- Childhood epilepsy is associated with a range of neurobehavioural comorbidities including Attention-Deficit/Hyperactivity Disorder (ADHD), Autism Spectrum Disorder (ASD), motor impairments and emotional problems. These difficulties frequently have a greater impact on quality of life than seizures. Pathological Demand Avoidance (PDA) is a term increasingly in use in the UK and Europe to describe behaviours associated with an extreme resistance to demands and requests and the need to be in control in social interactions. In a population-based group of 85 children with epilepsy, four (5%) were identified as displaying significant symptoms of PDA, were assessed using the Extreme Demand Avoidance Questionnaire (EDA-Q) and are described in detail. As well as significant symptoms of PDA, the four children met criteria for a range of neurobehavioural disorders; all four had cognitive impairment (IQ<85) and met DSM-IV-TR criteria for ADHD. Three, in addition, met criteria for ASD and Developmental Coordination Disorder (DCD) and two for Oppositional Defiant Disorder (ODD). All four experienced their first seizure before 5 years of age. School and parent reports indicated very significant functional impairment and management concerns, particularly with respect to complying with everyday demands. Symptoms of PDA should be considered when evaluating neurobehavioural comorbidity in childhood epilepsy.
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