Epilepsy professionals' views on sudden unexpected death in epilepsy counselling: A tale of two countries.

BACKGROUND AND PURPOSE: Sudden unexpected death in epilepsy (SUDEP) is a leading cause of epilepsy mortality. All international guidance strongly advocates for clinicians working with people with epilepsy (PWE) to discuss SUDEP. Clinician views working with PWE in the UK and Norway on SUDEP counselling are compared. METHODS: A cross-sectional online mixed methodology survey of 17 Likert and free-text response questions using validated themes was circulated via International League against Epilepsy/Epilepsy Specialist Nurses Association in the UK and International League against Epilepsy/Epilepsinet in Norway using a non-discriminatory exponential snowballing technique leading to non-probability sampling. Quantitative data were analysed using descriptive statistics and Mann-Whitney, Kruskal-Wallis, chi-squared and Fisher's exact tests. Significance was accepted at p < 0.05. Thematic analysis was conducted on free-text responses. RESULTS: Of 309 (UK 197, Norway 112) responses, UK clinicians were more likely to have experienced an SUDEP (p < 0.001), put greater importance on SUDEP communication (p < 0.001), discuss SUDEP with all PWE particularly new patients (p < 0.001), have access and refer to bereavement support (p < 0.001) and were less likely to never discuss SUDEP (p < 0.001). Significant differences existed between both countries' neurologists and nurses in SUDEP counselling with UK clinicians generally being more supportive. UK responders were more likely to be able to identify bereavement support (p < 0.001). Thematic analysis highlighted four shared themes and two specific to Norwegians. DISCUSSION: Despite all international guidelines stating the need/importance to discuss SUDEP with all PWE there remain hesitation, avoidance and subjectivity in clinicians having SUDEP-related conversations, more so in Norway than the UK. Training and education are required to improve communication, engagement and decision making.

Young people diagnosed with psychogenic nonepileptic seizures (PNES) years ago - How are they now?

BACKGROUND: Up to 30% of patients referred to epilepsy centres for drug-resistant epilepsy turn out to have psychogenic nonepileptic seizures (PNES). Patients with PNES are a very heterogeneous population with large differences in regard to underlying causes, seizures severity, and impact on quality of life. There is limited knowledge regarding the long-term seizure prognosis of youth with PNES and its influential factors. METHODS: We have performed a retrospective study on adolescents diagnosed with PNES who were receiving inpatient care at our hospital for 2-4 weeks in the period of 2012-2020. They all attended psychoeducational courses to educate them about PNES, coping with the seizures, and possible contributors to seizure susceptibility. There were 258 patients who fulfilled the inclusion criteria. We contacted them by text messages, through which they received brief information about the study and an invitation to participate. There were 62 patients (24 %) who agreed and participated in structured telephone interviews. We excluded 10 patients due to concomitant epilepsy. The mean age of the remaining 52 participants was 20.9 years (16-28 years), and 45 (87 %) were women. RESULTS: After a mean of 4.7 years (2-9 years) since discharge from our hospital, 28 patients (54 %) had been free of seizures in the last 6 months. There were 16 patients (31 %) who had better situations in regard to seizures but were not completely seizure free, while 8 patients (15 %) were either unchanged (3 patients) or worse (5 patients). There were 39 patients (75 %) who had received conversation therapy, and 37 patients (71 %) had been treated by a psychologist or psychiatrist. There were 10 patients (19 %) who had dropped out of school or work, and the percentage increased with age. There were 42 patients (80 %) who perceived their health as good or very good. CONCLUSION: Patients had a relatively favourable seizure prognosis as 54% were free of seizures and 31% had a better seizure situation, at the time of this study. However, the fact that 19% had dropped out of school or work was worrying. Young age and satisfaction with treatment were associated with being employed or receiving education. Satisfaction with perceived treatment was significantly associated with personal experience of good health. This emphasizes the importance of early diagnosis, adapted interventional measures, and long-term follow-up by healthcare for young people with PNES.

Strange episodes during sleep - epilepsy or parasomnia? / Underlige episoder under søvn ­ epilepsi eller parasomni?

In some forms of epilepsy, the seizures occur almost exclusively during sleep. This is particularly the case with hypermotor frontal lobe seizures. Clinically it can be difficult to distinguish such seizures from parasomnias and psychogenic non-epileptic seizures. This clinical review article aims to highlight the importance of making the correct diagnosis, as these conditions require completely different treatment.

Reduced Interhemispheric Coherence and Cognition in Children with Fetal Alcohol Spectrum Disorder (FASD)-A Quantitative EEG Study.

BACKGROUND: Magnetic resonance imaging in fetal alcohol spectrum disorder (FASD) children showed altered connectivity, suggesting underlying deficits in networks, which may be related to cognitive outcome. Functional connectivity has been of interest in neurophysiological research with quantitative electroencephalography (QEEG) as useful tool for measuring pathology, not detectable by normal EEG. The aim of this study was to investigate differences in the EEG interhemispheric coherence (ICoh) in children diagnosed with FASD compared with healthy controls and to relate the results to cognitive scores. METHOD: Analysis of ICoh in 81 FASD children (4-Digit Code) compared with 31 controls. The children underwent cognitive assessment, and EEG was performed and used for analysis. Group comparisons and analysis of covariance interaction models were used to test for differences between FASD and controls but also to look for differences between FASD subgroups. Significant findings were correlated to cognitive scores. RESULTS: Lower ICoh was found in the frontal and temporal derivations in the FASD group. When comparing FASD subgroups, children with fetal alcohol syndrome had lower ICoh occipital. Reduced ICoh in the temporal alpha band was correlated with lower performance IQ in the FASD group. CONCLUSION: Our findings could imply hypoconnectivity between the hemispheres with impact on cognition. We suggest that EEG coherence analysis could be a sensitive parameter in the detection of electrophysiological abnormalities in FASD with possible clinical relevance. These results may indicate that QEEG could be used as biomarker for FASD. However, further research is needed to determine the role of QEEG analysis in the diagnosis of FASD.

Norwegian population-based study of effectiveness of vagus nerve stimulation in patients with developmental and epileptic encephalopathies.

OBJECTIVE: Evaluate the long-term efficacy of vagus nerve stimulation (VNS) in patients with developmental and epileptic encephalopathies (DEE) compared with epilepsy patients without intellectual disability (ID). METHODS: Long-term outcomes from a Norwegian VNS quality registry are reported in 105 patients with DEEs (Lennox-Gastaut syndrome [LGS] n = 62; Dravet n = 16; Rett n = 9; other syndromes n = 18) were compared with 212 epilepsy patients without ID, with median follow-up of 88 and 72 months, respectively. Total seizure reduction was evaluated at 6, 12, 24, 36, and 60 months. Effect on different seizure types was evaluated at baseline and last observation carried forward (LOCF). RESULTS: Median monthly seizure frequency at LOCF was reduced by 42.2% (p < 0.001) in patients with DEE and by 55.8% (p < 0.001) in patients without ID. In DEE patients, ≥50% seizure reduction at 6 and 24 months were 17.1% and 37.1%, respectively, and 33.5% and 48.6% for patients without ID. Seizure reduction ≥75% at 60 months occurred in 14.3% of DEE patients and 23.1% of patients without ID. Highest median reduction was for atonic seizures, most notably 64.6% for LGS patients. A better effect was seen at 2 years among DEE patients with unchanged medication compared with those with changed medication (54.5% vs. 35.6% responders, p = 0.078). More DEE patients were reported to have greater improvement in ictal or postictal severity (43.8% vs. 28.3%, p = 0.006) and alertness (62.9% vs. 31.6%, p < 0.001) than patients without ID. For both groups, use of the magnet reduced seizure severity. Hoarseness was the most common adverse effect in both groups. In addition, DEE patients were frequently reported to have sleep disturbance, general discomfort, or abdominal problems. SIGNIFICANCE: Our data indicate that VNS is very effective for atonic seizures. Patients without ID had best overall seizure reduction, however, patients with DEE had higher retention rates probably due to other positive effects. PLAIN LANGUAGE SUMMARY: DEE refers to a group of patients with severe epilepsy and intellectual disability. Many of these patients have restricted lifestyles with frequent seizures. VNS is a treatment option for patients who do not respond well to medicines, either because of insufficient effect or serious adverse effects. Our study shows that VNS is well tolerated in this patient group and leads to a reduction in all seizure types, most notably for seizures leading to fall. Many patients experience other positive effects like shorter and milder seizures, as well as improvement in alertness.

Frequency of epilepsy and pathological EEG findings in a Norwegian sample of children with fetal alcohol spectrum disorder: Impact on cognition and adaptive functioning.

BACKGROUND: Fetal alcohol spectrum disorder (FASD) comprises a combination of developmental, cognitive, and behavioral disabilities that occur in children exposed to alcohol prenatally. A higher prevalence of epilepsy and pathological electroencephalographic (EEG) features have also been reported in individuals with FASD. We examined the frequency of epilepsy, pathological EEG findings, and their implications for cognitive and adaptive functioning in children with FASD. METHODS: We conducted a cross-sectional study of 148 children with FASD who underwent a multidisciplinary assessment and a 120-min EEG recording. Group comparisons and regression analyses were performed to test the associations between epilepsy and pathological EEG findings, FASD subgroups and neurocognitive test results and adaptive functioning. RESULTS: The frequency of epilepsy was 6%, which compares with 0.7% in Norway overall. Seventeen percent of children without epilepsy had pathological EEG findings. Attention-deficit hyperactivity disorder (ADHD) was diagnosed in 64% of the children. Children with epilepsy and/or pathological EEG findings had comparable cognitive and adaptive scores to those with normal EEG. However, children with frontal EEG pathology (without epilepsy) had significantly lower scores on the IQ indices Processing speed and Working memory than FASD children without such findings, irrespective of ADHD comorbidity. CONCLUSIONS: There was a greater prevalence of epilepsy among children with FASD than in the general Norwegian population. A greater frequency of EEG pathology was also evident in children without epilepsy, across all FASD subgroups. Irrespective of epilepsy, ADHD comorbidity, and FASD subgroup, children with frontal EEG pathology, despite having a normal total IQ, showed significantly slower processing speed and poorer working memory, which may indicate specific executive function deficits that could affect learning and adaptive functioning.

Temporal lobe epilepsy. / Temporallappsepilepsi.

The temporal lobes are the part of the brain most likely to give rise to epileptic seizures. Seizures originating in the temporal lobes vary greatly in character; some may be so unusual that they are not even recognised as epileptic. For patients who have been diagnosed with hippocampal sclerosis and whose seizures cannot be controlled with drugs, epilepsy surgery may be a good treatment option. In this brief clinical review, we summarise the key features of epilepsy and highlight the importance of accurate and early diagnosis for achieving good clinical outcomes.

Use of screening tools to assess comorbidities and adverse events in patients with epilepsy. A European Reference Network for Rare and Complex Epilepsies (EpiCARE) survey.

PURPOSE: As comorbidities can affect treatment decisions, quality of life, and prognosis in epilepsy, it is important that they are detected and addressed as soon as possible. Screening tools can help by rapidly assessing various additional challenges in epilepsy. METHODS: To map the use and perceived benefit of different screening instruments for quality of life, psychiatric comorbidity, and cognition, along with side effects from anti-seizure medication in Europe, we sent an online questionnaire to dedicated epilepsy centres departments within the European Reference Network for Rare and Complex Epilepsies (EpiCARE). RESULTS: Among the 40 hospitals in the EpiCARE network, we received responses from 25 (63%), with 28 individual respondents. Most respondents reported using screening for quality of life (86%) and psychiatric comorbidity (82%), but relatively few (14%) screen for sexual problems. Many (47) different tools were used for evaluation of cognitive dysfunction, but just a few (5) different tools were used to screen for adverse events. The optimization of individual patient care was one main reason given for using screening tools (58%-100% - depending on purpose of tool), another was research (50% - 88% - depending on purpose of tool). A major benefit of using screening tools perceived by the respondents is the detection of "hidden" comorbidity (67% - 90% - depending on purpose of tool). CONCLUSION: In the absence of a broad consensus regarding use of screening tools, practices vary considerably among epilepsy centres. Greater emphasis should be directed towards harmonizing use of screening tools. Future research should address how screening results influence treatment choices, and how these might affect clinical care.

Norwegian population-based study of long-term effects, safety, and predictors of response of vagus nerve stimulation treatment in drug-resistant epilepsy: The NORPulse study.

OBJECTIVE: This study was undertaken to evaluate the efficacy of vagus nerve stimulation (VNS) over time, and to determine which patient groups derive the most benefit. METHODS: Long-term outcomes are reported in 436 epilepsy patients from a VNS quality registry (52.8% adults, 47.2% children), with a median follow-up of 75 months. Patients were stratified according to evolution of response into constant responders, fluctuating responders, and nonresponders. The effect was evaluated at 6, 12, 24, 36, and 60 months. Multivariate regression analysis was used to identify predictors of response. RESULTS: The cumulative probability of ≥50% seizure reduction was 60%; however, 15% of patients showed a fluctuating course. Of those becoming responders, 89.5% (230/257) did so within 2 years. A steady increase in effect was observed among constant responders, with 48.7% (19/39) of those becoming seizure-free and 29.3% (39/133) with ≥75% seizure reduction achieving these effects within 2-5 years. Some effect (25%-<50%) at 6 months was a positive predictor of becoming a responder (odds ratio [OR] = 10.18, p < .0001) and having ≥75% reduction at 2 years (OR = 3.34, p = .03). Patients without intellectual disability had ORs of 3.34 and 3.11 of having ≥75% reduction at 2 and 5 years, respectively, and an OR of 6.22 of being seizure-free at last observation. Patients with unchanged antiseizure medication over the observation period showed better responder rates at 2 (63.0% vs. 43.1%, p = .002) and 5 years (63.4% vs. 46.3%, p = .031) than patients whose antiseizure medication was modified. Responder rates were higher for posttraumatic (70.6%, p = .048) and poststroke epilepsies (75.0%, p = .05) than other etiologies (46.5%). SIGNIFICANCE: Our data indicate that the effect of VNS increases over time and that there are important clinical decision points at 6 and 24 months for evaluating and adjusting the treatment. There should be better selection of candidates, as certain patient groups and epilepsy etiologies respond more favorably.

Feasibility of transcutaneous auricular vagus nerve stimulation in treatment of drug resistant epilepsy: A multicenter prospective study.

BACKGROUND: Transcutaneous auricular vagus nerve stimulation (ta-VNS) is a new non-invasive technique developed as treatment option for drug resistant epilepsy. A few studies have been carried out showing that the efficacy and tolerability of ta-VNS is comparable with traditional implanted VNS but the feasibility of the therapy has been poorly described. This study aimed to explore potential clinical benefits of ta-VNS and to evaluate adaptation, compliance, as well as the usability of the device from a service design perspective. METHODS: A prospective, multicenter, clinical, investigator-initiated trial was conducted using the NEMOS® ta-VNS device. After eight weeks baseline, all subjects started ta-VNS with individually adjusted currents for four hours per day for six-months (first endpoint) followed by optional 12 months follow-up (second endpoint). The primary outcome was six months retention rate of ta-VNS therapy. Secondary outcomes included the user retention rate at 12 months follow-up, compliance, changes in scores of psychometric measures. For the study of feasibility, a service design questionnaire on medical devices used in the home was developed. RESULTS: In total 37 subjects had been included in the study after 45 months where the study was prematurely terminated due to recruitment problems and due to a high drop-out rate. Twenty-two subjects (59 %) completed the first six months of the study and in total six subjects (16 %) completed the following 12 months follow-up. The reasons for discontinuation were a mixture of medical and practical issues of which the majority were related to a combination of both. Those, who managed to continue to use ta-VNS throughout the study, gave generally higher scores for the device usability and compatibility with lifestyle. The study turned out to be inadequately powered to reach any conclusion in terms of the clinical benefits of ta-VNS but present an example of difficulties that are encountered in conducting high-quality studies with digital devices. CONCLUSION: The feasibility of ta-VNS therapy showed to be relatively modest which is most likely due to practical usability issues and lifestyle fits. The results of this study stress the importance of generating data based on patients experiences at an early stage during the development phase and when designing clinical trials on medical devices that depend on patient's active participation and motivation.

Epilepsy in Onchocerca volvulus Sero-Positive Patients From Northern Uganda-Clinical, EEG and Brain Imaging Features.

Globally, epilepsy is the most common chronic neurological disorder. The incidence in sub-Saharan Africa is 2-3 times higher than that in high income countries. Infection by Onchocerca volvulus may be an underlying risk factor for the high burden and based upon epidemiological associations, has been proposed to cause a group of disorders-Onchocerca associated epilepsies (OAE) like nodding syndrome (NS). To improve our understanding of the disease spectrum, we described the clinical, electroencephalographic (EEG) and magnetic resonance imaging (MRI) features of children with epilepsy and sero-positive for Onchocerca volvulus (possible OAEs other than nodding syndrome). Twenty-nine children and adolescents with non-nodding syndrome OAE in northern Uganda were enrolled. A diagnosis of OAE was made in patients with epilepsy and seizure onset after age 3 years, no reported exposure to perinatal severe febrile illness or traumatic brain injury, no syndromic epilepsy diagnosis and a positive Ov-16 ELISA test. Detailed clinical evaluation including psychiatric, diagnostic EEG, a diagnostic brain MRI (in 10 patients) and laboratory testing were performed. Twenty participants (69%) were male. The mean age was 15.9 (standard deviation [SD] 1.9) years while the mean age at seizure onset was 9.8 (SD 2.9) years. All reported normal early childhood development. The most common clinical presentation was a tonic-clonic seizure. The median number of seizures was 2 (IQR 1-4) in the previous month. No specific musculoskeletal changes, or cranial nerve palsies were reported, neither were any vision, hearing and speech difficulties observed. The interictal EEG was abnormal in the majority with slow wave background activity in 52% (15/29) while 41% (12/29) had focal epileptiform activity. The brain MRI showed mild to moderate cerebellar atrophy and varying degrees of atrophy of the frontal, parietal and occipital lobes. The clinical spectrum of epilepsies associated with Onchocerca may be broader than previously described. In addition, focal onset tonic-clonic seizures, cortical and cerebellar atrophy may be important brain imaging and clinical features.

COVID-19 and epilepsy. / Covid-19 og epilepsi.

Can COVID-19 cause epilepsy, or increase the tendency to seizures in those with epilepsy? Is it safe for persons with epilepsy to be vaccinated against COVID-19?

People with epilepsy still feel stigmatized.

OBJECTIVES: Those affected with epilepsy have long been subject to stigmatization. This may have manifold negative effects, for example social isolation, low self-esteem, reduced quality of life and worsening of seizures. In Norway educational programs have been arranged at the National Centre for Epilepsy, aiming at reducing stigma and shame associated with epilepsy, and thereby increase the quality of life for those affected and their families. Thus, we wanted to explore the extent of self-reported perceived stigma and experienced discrimination in a Norwegian cohort with epilepsy. MATERIALS AND METHODS: We conducted a web-based questionnaire survey in Norway. Participants were asked to provide background and epilepsy-related information. In addition, they were encouraged to answer questions regarding felt stigmatization in different situations and to rate stigma according to the Jacoby stigma scale. RESULTS: Of 1182 respondents, 56% reported to have felt being stigmatized, and 35% reported to have experienced discrimination solely on the ground of the disease. 70% of respondents reported at least one type of perceived or experienced stigma. After controlling for gender, age, perceived depression and seizure freedom, reports of experienced stigmatization was a statistically significant independent predictor for reduced quality of life. CONCLUSIONS: A considerable proportion of people with epilepsy in Norway feel stigmatized and/or subject to discrimination, which negatively affects their quality of life.

The start of epilepsy care in Norway. / Epilepsiomsorgens start i Norge.

In 1912, an epilepsy colony was established in Bærum, inspired by similar colonies in other countries. Its first years were marked by financial constraints and limited treatment options. In the basement of the National Centre for Epilepsy in Bærum we recently came across a number of historical documents. These give an insight into the first years of what can be referred to as organised epilepsy care in Norway, and include patient protocols for the period 1913­20, five copybooks for the period 1905­23 and a ledger for the period 1905­20.

Patient satisfaction with information provided by epilepsy specialist nurses: Results of an online survey.

The aim of this study was to investigate the perspective of Norwegian patients with epilepsy regarding the information that they have received about epilepsy-related issues and to determine whether there was a difference in information received between those who had been followed up by an epilepsy specialist nurse (ESN) and those who had not. Further, were there differences regarding satisfaction with the information between the two groups? We conducted an online survey in close collaboration with the Norwegian Epilepsy Association. A total of 1859 respondents (1182 patients with epilepsy and 677 carers for patients with epilepsy) completed a web-based questionnaire. They were asked about epilepsy-related issues on which they had received information, the extent to which they were satisfied with this information, and whether they were being followed up by an ESN or not. Significantly more patients followed up by an ESN had received information about the epilepsy diagnosis, antiseizure drugs (ASDs), routine use of ASD, and risk of seizure-related injuries as compared to those not followed up by an ESN. In addition, patients followed by an ESN were more likely to be satisfied with the information they received. Just above or under half of the respondents had received or were satisfied with information about depression, anxiety, premature death, and sexual wellbeing. Our results indicate that follow-up by ESNs results in improvements in the information provided to patients with epilepsy; ESNs should be an integral part of comprehensive epilepsy service.

EEG in fitness to drive evaluations in people with epilepsy - Considerable variations across Europe.

PURPOSE: Epilepsy patients consider driving issues to be one of their most serious concerns. Ideally, decisions regarding fitness to drive should be based upon thorough evaluations by specialists in epilepsy care. In 2009, an EU directive was published aiming to harmonize evaluation practices within European countries, but, despite these recommendations, whether all epileptologists use the same criteria is unclear. We therefore conducted this study to investigate routine practices on how epileptologists at European epilepsy centers evaluate fitness to drive. METHODS: A questionnaire was sent to 63 contact persons identified through the European Epi-Care and the E-pilepsy network. The questionnaire addressed how fitness-to-drive evaluations were conducted, the involvement of different professionals, the use and interpretation of EEG, and opinions on existing regulations and guidelines. RESULTS: The questionnaire was completed by 35 participants (56 % response rate). Results showed considerable variation regarding test routines and the emphasis placed on the occurrence and extent of epileptiform discharges revealed by EEG. 82 % of the responders agreed that there was a need for more research on how to better evaluate fitness-to-drive in people with epilepsy, and 89 % agreed that regulations on fitness to drive evaluations should be internationally coordinated. CONCLUSION: Our survey showed considerable variations among European epileptologists regarding use of EEG and how findings of EEG pathology should be assessed in fitness-to-drive evaluations. There is a clear need for more research on this issue and international guidelines on how such evaluations should be carried out would be of value.

How often do doctors discuss drug withdrawal with their seizure-free patients with epilepsy?

Among patients with epilepsy, almost 70% become seizure-free with the current antiseizure drugs (ASDs) within 20 years following seizure onset. Of those who have been seizure-free for many years, around 70% remain seizure-free after withdrawal of ASDs. The purpose of this study was to determine the extent to which seizure-free patients with epilepsy in Norway discuss drug discontinuation with their physician. An online questionnaire was used; among the respondents were 186 adult patients who had been seizure-free for at least five years and were still using ASDs. Of these, 60 patients (32%) reported that they had discussed the question of drug withdrawal with their treating physician. Those patients who reported being involved in treatment decisions were more likely to have discussed ASD withdrawal. In conclusion, it is our opinion that discontinuation of drug treatment in patients with long-term seizure freedom is discussed far too seldom and that many patients may be living with an unnecessary drug burden.