| 1. |
- Andreasson, Ann‐Charlotte, et al.
(författare)
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Cortical excitability measured with transcranial magnetic stimulation in children with epilepsy before and after antiepileptic drugs
- 2020
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Ingår i: Developmental Medicine and Child Neurology. - : Wiley. - 0012-1622 .- 1469-8749. ; 62:7, s. 793-798
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Tidskriftsartikel (refereegranskat)abstract
- Aim To evaluate cortical excitability with transcranial magnetic stimulation (TMS) in children with new-onset epilepsy before and after antiepileptic drugs (AEDs). Method Fifty-five drug-naive patients (29 females, 26 males; 3-18y), with new-onset epilepsy were recruited from 1st May 2014 to 31st October 2017 at the Child Neurology Department, Queen Silvia's Children's Hospital, Gothenburg, Sweden. We performed TMS in 48 children (23 females, 25 males; mean [SD] age 10y [3y], range 4-15y) with epilepsy (27 generalized and 21 focal) before and after the introduction of AEDs. We used single- and paired-pulse TMS. We used single-pulse TMS to record resting motor thresholds (RMTs), stimulus-response curves, and cortical silent periods (CSPs). We used paired-pulse TMS to record intracortical inhibition and facilitation at short, long, and intermediate intervals. Results There were no differences in cortical excitability between children with generalized and focal epilepsy at baseline. After AED treatment, RMTs increased (p=0.001), especially in children receiving sodium valproate (p=0.005). CSPs decreased after sodium valproate was administered (p=0.050). As in previous studies, we noted a negative correlation between RMT and age in our study cohort. Paired-pulse TMS could not be performed in most children because high RMTs made suprathreshold stimulation impossible. Interpretation Cortical excitability as measured with RMT decreased after the introduction of AEDs. This was seen in children with both generalized and focal epilepsy who were treated with sodium valproate, although it was most prominent in children with generalized epilepsy. We suggest that TMS might be used as a prognostic tool to predict AED efficacy.
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| 2. |
- Baumgartner, T., et al.
(författare)
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A survey of the European Reference Network EpiCARE on clinical practice for selected rare epilepsies
- 2021
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Ingår i: Epilepsia Open. - : Wiley. - 2470-9239. ; 6:1, s. 160-170
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Clinical care of rare and complex epilepsies is challenging, because evidence-based treatment guidelines are scarce, the experience of many physicians is limited, and interdisciplinary treatment of comorbidities is required. The pathomechanisms of rare epilepsies are, however, increasingly understood, which potentially fosters novel targeted therapies. The objectives of our survey were to obtain an overview of the clinical practice in European tertiary epilepsy centers treating patients with 5 arbitrarily selected rare epilepsies and to get an estimate of potentially available patients for future studies. Methods: Members of the European Reference Network for rare and complex epilepsies (EpiCARE) were invited to participate in a web-based survey on clinical practice of patients with Dravet syndrome, tuberous sclerosis complex (TSC), autoimmune encephalitis, and progressive myoclonic epilepsies including Unverricht Lundborg and Unverricht-like diseases. A consensus-based questionnaire was generated for each disease. Results: Twenty-six of 30 invited epilepsy centers participated. Cohorts were present in most responding centers for TSC (87%), Dravet syndrome (85%), and autoimmune encephalitis (71%). Patients with TSC and Dravet syndrome represented the largest cohorts in these centers. The antiseizure drug treatments were rather consistent across the centers especially with regard to Dravet syndrome, infantile spasms in TSC, and Unverricht Lundborg / Unverricht-like disease. Available, widely used targeted therapies included everolimus in TSC and immunosuppressive therapies in autoimmune encephalitis. Screening for comorbidities was routinely done, but specific treatment protocols were lacking in most centers. Significance: The survey summarizes the current clinical practice for selected rare epilepsies in tertiary European epilepsy centers and demonstrates consistency as well as heterogeneity in the treatment, underscoring the need for controlled trials and recommendations. The survey also provides estimates for potential participants of clinical trials recruited via EpiCARE, emphasizing the great potential of Reference Networks for future studies to evaluate new targeted therapies and to identify novel biomarkers. © 2020 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy
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| 3. |
- 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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| 4. |
- 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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| 5. |
- 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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| 6. |
- Bjurulf, Björn, 1962, et al.
(författare)
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Potassium citrate and metabolic acidosis in children with epilepsy on the ketogenic diet: a prospective controlled study
- 2020
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Ingår i: Developmental Medicine & Child Neurology. - : Wiley. - 0012-1622 .- 1469-8749. ; 61:1, s. 57-61
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Tidskriftsartikel (refereegranskat)abstract
- Aim To investigate if potassium citrate, a mild alkaline compound, can prevent metabolic acidosis in children with epilepsy treated with the ketogenic diet without reducing antiepileptic efficacy. Method In this prospective controlled study, we investigated the frequency of initial uncompensated metabolic acidosis in 51 participants. There were 22 participants with and 29 without potassium citrate supplementation. The ketogenic diet was used as add-on treatment to children with drug resistant epilepsy. We also estimated the proportion of participants with a greater than 50% seizure reduction after 7 months. Results None of the 22 participants (15 males, seven females; median age 1y 7mo, interquartile range [IQR] 3y 3mo) with, and 10 of 29 (12 males, 17 females; median age 6y 1mo, IQR 4y 8mo) without potassium citrate developed metabolic acidosis (odds ratio=0.04, 95% CI 0.00–0.75 [p<0.01]); median pH 7.32 vs 7.24; [p<0.001]), and median bicarbonate 19.7mmol/L vs 14.0mmol/L (p<0.001). The number of seizures was reduced by more than 50% in 9 of 22 with potassium citrate and 8 of 29 participants without potassium citrate, 7 months after introducing a ketogenic diet (p=0.4). Interpretation In the ketogenic diet, potassium citrate supplementation can prevent metabolic acidosis, without reducing antiepileptic efficacy.
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| 7. |
- Bomfim, I L, et al.
(författare)
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The immunogenetics of narcolepsy associated with A(H1N1)pdm09 vaccination (Pandemrix) supports a potent gene-environment interaction.
- 2017
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Ingår i: Genes and immunity. - : Springer Science and Business Media LLC. - 1476-5470 .- 1466-4879. ; 18, s. 75-81
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Tidskriftsartikel (refereegranskat)abstract
- The influenza A(H1N1)pdm09 vaccination campaign from 2009 to 2010 was associated with a sudden increase in the incidence of narcolepsy in several countries. Narcolepsy with cataplexy is strongly associated with the human leukocyte antigen (HLA) class II DQB1*06:02 allele, and protective associations with the DQB1*06:03 allele have been reported. Several non-HLA gene loci are also associated, such as common variants of the T-cell receptor-α (TRA), the purinergic receptor P2RY11, cathepsin H (CTSH) and TNFSF4/OX40L/CD252. In this retrospective multicenter study, we investigated if these predisposing gene loci were also involved in vaccination-associated narcolepsy. We compared HLA- along with single-nucleotide polymorphism genotypes for non-HLA regions between 42 Pandemrix-vaccinated narcolepsy cases and 1990 population-based controls. The class II gene loci associations supported previous findings. Nominal association (P-value<0.05) with TRA as well as suggestive (P-value<0.1) associations with P2RY11 and CTSH were found. These associations suggest a very strong gene-environment interaction, in which the influenza A(H1N1)pdm09 strain or Pandemrix vaccine can act as potent environmental triggers.Genes and Immunity advance online publication, 23 March 2017; doi:10.1038/gene.2017.1.
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| 8. |
- Chaplin, John, 1955, et al.
(författare)
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The development of a health-related quality-of-life instrument for young people with narcolepsy: NARQoL-21
- 2017
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Ingår i: Health and Quality of Life Outcomes. - : Springer Science and Business Media LLC. - 1477-7525. ; 15:1
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Narcolepsy is a lifelong sleep disorder with a prevalence of between 0.03% and 0.06% and onset at around puberty. It is associated with psychiatric comorbidities and cognitive difficulties. No valid and reliable condition-specific health-related quality-of-life (HrQoL) instrument has been developed for this population. METHODS: A questionnaire based on four mixed-gender age-defined focus group discussions and a patient panel analysis was administered to young people with narcolepsy and a control group. External reliability was measured by a test-retest procedure and internal reliability was measured using Cronbach's alpha. Convergent validity with the KIDSCREEN-10 index was assessed using with intraclass correlation coefficients (ICC) and receiver operating characteristic (ROC) curves. Factor analysis techniques were used to identify suitable items and confirm the factor structure. Baseline values were assessed for convergent validity, ceiling effects, agreement and sensitivity. Comparison with KIDSCREEN-10 was made on the basis of area under the curve (AUC). RESULTS: One hundred young people with narcolepsy and 95 control subjects returned questionnaires. The factor structure revealed two main factors with five domains and 21 questions, which was confirmed with confirmatory factor analysis. The domains of the NARQoL-21 showed good independence while the floor and ceiling effects were acceptable. The external reliability (0.928), convergent validity (rs = 0.769) and internal consistency (Cronbach's alpha = 0.886) were excellent. A Bland-Altman plot revealed some proportional bias. Good discriminant validity was detected for control/patient (Cohen's d = 2.114). ROC analysis showed significantly better AUC for NARQoL-21 (0.939) than KIDSCREEN (0.877). A cut-off score equivalent to KIDSCREEN-10 for suboptimal HrQoL which maximized sensitivity (84%) and specificity (92%) was found at NARQoL-21 score below 42. CONCLUSIONS: Establishing the validity of a disease-specific HrQoL instrument in a population of people with a rare condition poses significant challenges. The mixed-methods approach adopted here has resulted in a questionnaire of 21 items with good discrimination and convergent validity, and excellent internal and external reliability, allowing precise and stable measurements. The cut-off score can be useful to identify patients with very poor HrQoL and thus improve the design of treatment options. Further testing in a longitudinal cohort is recommended in order to establish responsiveness.
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| 9. |
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| 10. |
- Edelvik, Anna, 1971, et al.
(författare)
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Hälften är långsiktigt fria från anfall efter epilepsikirurgi
- 2018
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Ingår i: Läkartidningen. - 0023-7205. ; 115:21
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Forskningsöversikt (refereegranskat)abstract
- Personer med läkemedelsresistent epilepsi bör remitteras till regionala epilepsiteam för utredning och bedömning av behandlingsalternativ, bland annat epilepsikirurgi. Om magnetresonanstomografi visar en epileptogen lesion som är åtkomlig för kirurgi är operation ett effektivt behandlingsalternativ, och en epilepsikirurgisk utredning kan vara relativt begränsad. Även om magnetresonanstomografi bedöms normal kan en epilepsikirurgisk utredning vara aktuell, men är då mera omfattande. Små barn med läkemedelsresistent epilepsi bör tidigt remitteras till regionala epilepsiteam. Ungefär hälften av dem som opereras blir anfallsfria på lång sikt, med bättre resultat för dem som opererats för välavgränsade lesioner.
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| 11. |
- Engström, Maria, 1958-, et al.
(författare)
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Functional magnetic resonance imaging in narcolepsy and the Kleine-Levin syndrome
- 2014
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Ingår i: Frontiers in Neurology. - Switzerland : Frontiers Media SA. - 1664-2295. ; 5
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Tidskriftsartikel (refereegranskat)abstract
- This work aims at reviewing the present state of the art when it comes to understanding the pathophysiology of narcolepsy and the Kleine-Levin syndrome (KLS) from a neuroimaging point of view. This work also aims at discussing future perspectives of functional neuroimaging in these sleep disorders. We focus on functional magnetic resonance imaging (fMRI), which is a technique for in vivo measurements of brain activation in neuronal circuitries under healthy and pathological conditions. fMRI has significantly increased the knowledge on the affected neuronal circuitries in narcolepsy and the Kleine-Levin syndrome. It has been shown that narcolepsy is accompanied with disturbances of the emotional and the closely related reward systems. In the Kleine Levin syndrome, fMRI has identified hyperactivation of the thalamus as a potential biomarker that could be used in the diagnostic procedure. The fMRI findings in both narcolepsy and the Kleine-Levin syndrome are in line with previous structural and functional imaging studies. We conclude that fMRI in combination with multi-modal imaging can reveal important details about the pathophysiology in narcolepsy and the Kleine-Levin syndrome. In the future, fMRI possibly gives opportunities for diagnostic support and prediction of treatment response in individual patients.
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| 12. |
- Hahne, Oscar, et al.
(författare)
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Epilepsy surgery in patients with hypothalamic hamartomas-Population-based two-year and long-term outcomes
- 2023
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Ingår i: European Journal of Paediatric Neurology. - 1090-3798. ; 46, s. 24-29
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Hypothalamic hamartomas are benign lesions associated with drug resistant epilepsy. Surgical treatment has become an increasingly utilised approach with promising results. This study aims to evaluate seizure outcome and complications after surgery in a population-based series of patients with intractable epilepsy and hypothalamic hamartoma.Methods: All patients with hypothalamic hamartoma treated with epilepsy surgery in Sweden since 1995 with at least two years of follow-up were included. Preoperative, two-, five- and ten-year prospective longitudinal data were collected from The Swedish National Epilepsy Surgery Register. Data included seizure types and frequency, duration of epilepsy, clinical characteristics, neurological deficits, cognitive level and complications. In a subgroup from Gothenburg, we also analysed data not included in the register such as classification of hamartomas, surgical procedures and gelastic seizures.Results: Eighteen patients were operated on during the period 1995-2020. The median age at epilepsy onset was 6 months and age at surgery 13 years. Four were seizure free and another four had >75% reduction in seizure frequency at the two-year follow-up. Two of the 13 patients with a long-term follow-up (five or ten years) were seizure-free and four had >75% reduction in seizure frequency. Three had an increased seizure frequency. No major complications were seen. Five had minor complications. In the Gothenburg subgroup all had open pterional disconnection or intraventricular endoscopic disconnection. Six of 12 were free from gelastic seizures at the two-year follow-up and six of eight at the long-term follow-up. Conclusion: This study supports surgical treatment of hypothalamic hamartomas as a safe method with a low risk of permanent complications. The seizure reduction seems to be persistent over time.
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| 13. |
- Hallböök, Tove, et al.
(författare)
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Beneficial effects on sleep of vagus nerve stimulation in children with therapy resistant epilepsy.
- 2005
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Ingår i: European Journal of Paediatric Neurology. - : Elsevier BV. - 1090-3798. ; 9:6, s. 399-407
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Tidskriftsartikel (refereegranskat)abstract
- The study purpose was to evaluate sleep structure following Vagus Nerve Stimulation (VNS) in 15 children with therapy resistant epilepsy and to correlate possible alterations with changes in epileptiform activity and clinical effects. Fifteen children were examined with ambulatory polysomnographic recordings initially, and after 3 and 9 months of VNS-treatment. Sleep parameters, all-night delta power activity and movement times (MTs), used to account for arousals were estimated. Epileptiform activity was evaluated by spike detection. Seizure frequency was recorded in a diary. The severity of the seizures was scored with the National Hospital Seizure Severity Scale (NHS3). Quality of life (QOL) was assessed by a visual analogue scale. Behaviour problems were quantified by using the total score of the Child Behaviour Checklist (CBCL). VNS induces a significant increase in slow wave sleep (SWS) and a decrease in sleep latency and in stage 1 sleep. The number and density of MTs during total night sleep were significantly increased. There was also a significant increase in the number of MTs immediately related to the VNS stimulation periods. Of the 14 children with increased MTs, 10 had a reduction in epileptiform activity, and in clinical seizures, all had an improvement in NHS3, and 11 in QOL. Of the 10 children with increased SWS, eight also improved in QOL and eight in behaviour. Our findings indicate that VNS counteracts known adverse effects of epilepsy on sleep and increases slow wave sleep. This possibly contributes to the reported improvement in well-being. We also see an increase in MTs. This arousal effect seems to be of minor importance for QOL and could possibly be related to the antiepileptic mechanisms in VNS.
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| 14. |
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| 15. |
- Hallböök, Tove, et al.
(författare)
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Effectiveness of the ketogenic diet used to treat resistant childhood epilepsy in Scandinavia.
- 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. ; 19:1, s. 29-36
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Tidskriftsartikel (refereegranskat)abstract
- This Scandinavian collaborative retrospective study of children treated with ketogenic diet (KD) highlights indications and effectiveness over two years follow-up.
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| 16. |
- Hallböök, Tove, et al.
(författare)
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Effects of ketogenic diet on epileptiform activity in children with therapy resistant epilepsy
- 2007
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Ingår i: Epilepsy Research. - : Elsevier BV. - 1872-6844 .- 0920-1211. ; 77:2-3, s. 134-140
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: The purpose was to quantify changes of epileptiform activity during ketogenic diet (KD) treatment in children with therapy resistant epilepsy, and evaluate how these changes are related to activity stage and to clinical effects on seizure frequency, seizure severity, attentional behaviour, quality of life (QOL), and beta-hydroxybutyrate (betaOHb). METHODS: Eighteen children were investigated with 24h ambulatory EEG monitoring 1 week prior to KD initiation and, after 3 months of KD treatment. Epileptiform activity was evaluated by automated spike detection. This data was compared with data presented in a previous study published in Epilepsia 2006, on sleep structure and different activity stages, clinical data on seizure frequency, seizure severity, QOL and attentional behaviour on the same children [Hallbook, T., Lundgren, J., Rosen, I., 2007. Ketogenic diet improves sleep quality in children with therapy resistant epilepsy. Epilepsia 48, 59-65]. RESULTS: After 3 months of KD treatment the number of interictal epileptiform discharges (IEDs) was significantly reduced (p<0.001). When considering the four activity stages separately, the reduction was significant during non-rapid eye movement sleep stage 2, slow wave sleep (SWS) and rapid eye movement (REM) sleep (p=0.001, 0.001, 0.002), and not significantly so during awake (p=0.07). Beta-hydroxybutyrate was significantly increased (p<0.001). There was a significant correlation between the reduction in IEDs and clinical seizures (Spearman r=0.6, p=0.005) and between improvement in attentional behaviour and the increase in betaOHb (Spearman r=0.5, p=0.03). There was no significant correlation between changes in attentional behaviour and IEDs or clinical seizures. CONCLUSION: This study shows that KD reduces the number of IEDs, especially during sleep. It shows a correlation between reduction in epileptiform activity and clinical seizures. There were no correlations between reduction in epileptiform activity and clinical seizures and improvement in QOL or attention. The increase in betaOHb correlated with improvement in attention.
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| 17. |
- Hallböök, Tove
(författare)
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Effects of Vagus Nerve Stimulation and Ketogenic Diet on Quality of Life and Changes in EEG and Sleep
- 2006
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- When anti-epileptic drugs fail, and epilepsy surgery is found unfeasible or ineffective, there remains a group of at least 25% of children with epilepsy in whom seizure control cannot be achieved. Vagus nerve stimulation (VNS) is an adjunctive treatment for medically refractory epilepsy. It is an implantable, multi-programmable pulse generator that delivers current electrical stimulation to the vagus nerve for the purpose of suppressing and reducing the frequency and/or severity of epileptic seizures. Ketogenic diet (KD) is a high fat, low carbohydrate and low protein diet that has been used for childhood therapy resistant epilepsy since the1920s. It was developed to mimic the ketotic state of starvation. The general aim of this thesis was to evaluate effects of VNS on epileptiform activity, deltapower and sleep characteristics and to correlate these to clinical aspects on behaviour, mood and QOL in children with therapy resistant epilepsy. Initially, and after three and nine months of VNS-treatment, 15 children were investigated. Sleep characteristics and clinical correlations were also evaluated after 3 and 12 months in 18 children with KD. A diary of seizure frequency and the National Hospital Seizure Severity Scale (NHS3) were collected. In the first study (paper I) cognitive functioning was recorded, a Visual Analogue Scale for validating QOL, Child Behaviour Checklist (CBCL) for quantifying behaviour problems, Dodrill Mood Analogue Scale and Birleson Depression Self-Rating Scale were used. Six of fifteen children showed a 50% or more reduction in seizure frequency; one of these became seizure-free. Two children had a 25-50% seizure reduction. Two children showed increased seizure frequency. In 13 of 15 children there was an improvement in NHS3. The parents reported shorter duration of seizure and recovery phase. There were no changes in cognitive functioning. Twelve children showed an improvement in QOL. Eleven of these also improved in seizure severity and mood and 5 also in depressive parameters. In the second study (paper II) 24 hours ambulatory EEG monitoring for spike detection were used. This study shows that VNS reduces interictal epileptiform discharges (IEDs), especially in rapid eye movements (REM) and slow wave sleep, and the number of electrographic seizures. It also shows a concordance between reduction in IEDs and electrographic seizures. There was no correlation between the extent of improvement in clinical data and the degree of spike reduction. In the third and forth study (paper III and IV) children with VNS and KD were examined with ambulatory polysomnographic recordings. Sleep parameters, and in VNS, movement times (MTs), used to account for arousals were estimated. Our findings indicate that VNS counteracts known adverse effects of epilepsy on sleep and increases slow wave sleep. This possibly contributes to the reported improvement in wellbeing. We also see an increase in MTs. This arousal effect seems to be of minor importance for QOL and could possibly be related to the antiepileptic mechanisms in VNS. KD decreases night sleep and improves sleep quality. The improvement in sleep quality, with increased REM sleep, seems to contribute to the improvement in QOL. In the fifth study (paper V) the 24 hours ambulatory EEG monitoring were used to study immediate effects of VNS stimulation on epileptiform activity, arousals and background frequency in EEG. The findings lend no support to earlier studies suggesting immediate VNS stimulation related changes in IEDs och IEIs. There were no VNS stimulation related changes in background EEG frequency despite the significant increase in the number of MTs immediately related to the VNS stimulation periods.
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| 18. |
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| 19. |
- Hallböök, Tove, et al.
(författare)
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Epilepsy surgery in children with drug-resistant epilepsy, a long-term follow-up
- 2013
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Ingår i: Acta Neurologica Scandinavica. - : Hindawi Limited. - 1600-0404 .- 0001-6314. ; 128:6, s. 414-421
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Tidskriftsartikel (refereegranskat)abstract
- ObjectivesIn this follow-up study, we wanted to present the long-term outcome (5-21years) in terms of seizure freedom, seizure reduction, and the cognitive development in the first 47 children who underwent epilepsy surgery at the University Hospital in Lund from 1991 to 2007. Materials and methodsAll children who underwent epilepsy surgery in the southern region of Sweden were assessed for cognitive function before surgery and at follow-up. A review of medical documents for demographic data and seizure-related characteristics was made by retrospectively examining the clinical records. ResultsForty-seven children with a median age at surgery of 8years (range 0.5-18.7years) were included. Twenty-three children achieved seizure freedom, six demonstrated >75% improvement in seizure frequency, and none of the children experienced an increase in seizure frequency. Twenty-one children required a reoperation to achieve satisfactory seizure outcomes. Cognitive functional level was preserved, and the majority of patients, 34 (76%), followed their expected cognitive trajectory. The patients who became seizure free significantly improved their cognitive processing speed, even after long-term follow-up. ConclusionsEpilepsy surgery in children offers suitable candidates a good chance of significantly improved outcome and low rates of complications. Several children, however, required a reoperation to achieve satisfactory seizure outcomes. Cognitive level was preserved, and the majority of patients followed their expected cognitive trajectory. Cognitive improvements in processing speed appear to occur in parallel with seizure control and were even more pronounced in subjects with no anti-epilepsy drugs. These improvements persisted even after long-term follow-up.
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| 20. |
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| 21. |
- Hallböök, Tove, et al.
(författare)
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Ketogenic Diet Improves Sleep Quality in Children with Therapy-resistant Epilepsy.
- 2007
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Ingår i: Epilepsia. - : Wiley. - 0013-9580 .- 1528-1167. ; 48:1, s. 59-65
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Tidskriftsartikel (refereegranskat)abstract
- Summary: Purpose: The study purpose was to evaluate sleep structure during ketogenic diet (KD) treatment in children with therapy-resistant epilepsy and to correlate possible alterations with changes in clinical effects on seizure reduction, seizure severity, quality of life (QOL), and behavior. Methods: Eighteen children were examined with ambulatory polysomnographic recordings initially and after 3 months of KD treatment. Eleven children continued with the KD and were also evaluated after 12 months. Sleep parameters were estimated. Seizure frequency was recorded in a diary and seizure severity in the National Health Seizure Severity Scale (NHS3). QOL was assessed with a visual analogue scale. Child behavior checklist and Ponsford and Kinsella's rating scale of attentional behavior were used. Results: KD induced a significant decrease in total sleep (p = 0.05) and total night sleep (p = 0.006). Slow wave sleep was preserved, rapid eye movement (REM) sleep increased (p = 0.01), sleep stage 2 decreased (p = 0.004), and sleep stage 1 was unchanged. Eleven children continued with the KD and were also evaluated after 12 months. They showed a significant decrease in daytime sleep (p = 0.01) and a further increase in REM sleep (p = 0.06). Seizure frequency (p = 0.001, p = 0.003), seizure severity (p < 0.001, p = 0.005) and QOL (p < 0.001, p = 0.005) were significantly improved at 3 and 12 months. Attentional behavior was also improved, significantly so at 3 months (p = 0.003). There was a significant correlation between increased REM sleep and improvement in QOL (Spearman r = 0.6, p = 0.01) at 3 months. Conclusion: KD decreases sleep and improves sleep quality in children with therapy-resistant epilepsy. The improvement in sleep quality, with increased REM sleep, seems to contribute to the improvement in QOL.
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| 22. |
- Hallböök, Tove, et al.
(författare)
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Long term effects on epileptiform activity with vagus nerve stimulation in children.
- 2005
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Ingår i: Seizure. - : Elsevier BV. - 1532-2688 .- 1059-1311. ; 14:8, s. 527-533
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: We report tong-term effects of vagus nerve stimulation (VNS) on epileptiform activity in 15 children, and how these changes are related to activity stage and to clinical effects on seizure reduction, seizure severity (NHS3) and quality of life (QOL). Methods: Initially, and after 3 and 9 months of VNS-treatment, 15 children were investigated with 24 h ambulatory EEG monitoring for spike detection. The number of interictal epiteptiform discharges (IEDs) and the inter spike intervals (ISIs) were analysed during 2 h in the awake state, and 1 h of rapid eye movement (REM)-, spindle- and delta-steep, respectively. Total number and duration of electrographic seizure episodes were also analysed. Results: At 9 months the total number of IEDs was significantly reduced (p = 0.04). There was a tendency of reduction in all activity stages, and significantly so in delta-steep (p = 0.008). Total etectrographic seizure number was significantly reduced in the 24 h EEG at 3 and 9 months (p = 0.03, 0.05). There was a significant concordance in direction of changes in epileptiform activity and etectrographic seizures at 9 months (p = 0.04). Concordance in direction of changes was seen in 9 of 15 children between clinical seizures and IED (p > 0.3), in 10 of 15 children between QOL and IED (p = 0.3) and in 8 of 15 children between NHS3 and IED (p > 0.3). There was no direct correlation between the extent of improvement in these clinical data and the degree of spike reduction. Conclusion: This study shows that VNS reduces IEDs especially in REM and delta steep, as well as the number of electrographic seizures. It also shows a concordance between reduction in IEDs and etectrographic seizures.
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| 23. |
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| 24. |
- Hallböök, Tove, et al.
(författare)
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Narkolepsi – ovanlig sjukdomsom fått ökad uppmärksamhet : Pandemrixvaccination ledde till fler insjuknanden bland barn och ungdomar
- 2014
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Ingår i: Läkartidningen. - 0023-7205 .- 1652-7518. ; 111:41, s. 1770-1773
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Tidskriftsartikel (refereegranskat)abstract
- Narkolepsi är en ovanlig sjukdom där symtomen beror på störd reglering av sömn och vakenhet. Kardinalsymtom är uttalad dagsömnighet, kataplexiattacker och nattlig dyssomni. Etiologin anses betingad av en autoimmun process hos predisponerade individer, där omgivningsfaktorer är av betydelse för att utlösa sjukdomen.Pandemrixvaccinationen ledde till ökat insjuknande hos barn och ungdomar och en viss ökning hos unga vuxna.Behandlingen utgör en kombination av läkemedel med effekt på kardinalsymtomen och livsstilsråd, där teamomhändertagande är viktigt.Svenska narkolepsiregistret ger möjlighet att följa sjukdomsförlopp och behandling, vilket är viktigt inte minst eftersom dokumentationen av de läkemedel som används är begränsad.
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| 25. |
- Hallböök, Tove, et al.
(författare)
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Pediatric neurovascular disease.
- 2006
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Ingår i: Acta Pædiatrica. - : Wiley. - 1651-2227 .- 0803-5253. ; 95:6, s. 766-766
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Tidskriftsartikel (refereegranskat)
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