Effectiveness of cognitive behavioral therapy for insomnia: influence of slight-to-moderate depressive symptom severity and worrying.

BACKGROUND: Cognitive behavioral therapy for insomnia (CBT-I) is a well-known, effective treatment for primary insomnia. However, the majority of sleeping problems occur in the presence of another medical or psychiatric disorder. Depression and general anxiety disorder (with a main feature of excessive generalized worrying) are disorders that frequently co-occur with insomnia. The purpose of this study is to evaluate whether depressive symptom severity or worrying influences the subjective effectiveness of CBT-I. METHODS: Patients with a complaint of insomnia received CBT-I. At the beginning of the therapy, patients completed a sleep evaluation list, the Beck Depression Inventory (BDI-II-NL, N = 92), and the Penn State Worry Questionnaire (PSWQ, N = 119). Based on the BDI and the PSWQ, the sample was divided into different groups: patients with low versus high depression scores, low worriers versus high worriers, and patients without depressive symptoms who were also classified as low worriers and patients with depressive symptoms who were also classified as high worriers. The sleep evaluation list was completed directly after the treatment and 6 months later. RESULTS: Sleep evaluation scores, subjective total sleep time, subjective sleep onset latency, and subjective wake after sleep onset all changed in a positive way after CBT-I and remained that way over the next 6 months for all patients. These positive effects of CBT-I did not differ between the subgroups. CONCLUSIONS: Results suggest that CBT-I improves subjective sleep experiences, regardless of depressive symptom severity or worrying.

Respiratory and sleep disorders in female children with atypical Rett syndrome caused by mutations in the CDKL5 gene.

AIM: In female children with drug-resistant seizures and developmental delay from birth, atypical Rett syndrome caused by mutations in the CDKL5 gene should be considered. Several clinical features resemble classic Rett syndrome. Respiratory and sleep abnormalities are frequently present in Rett syndrome, whereas little is known in patients with CDKL5 mutations. METHOD: In four genetically confirmed female patients with CDKL5 mutations (age range 2-15 y), the presence of breathing and sleep abnormalities was evaluated using the validated Sleep Disturbance Scale for Children and polysomnography (PSG). RESULTS: The Sleep Disturbance Scale for Children indicated disorders of initiating and maintaining sleep, daytime somnolence, and sleep breathing disorders. In one patient, PSG showed central apnoeas during sleep: her total apnoea-hypopnoea index (AHI) was 4.9, of which the central AHI was 3.4/h. When awake, central apnoeas were present in two of the four female children (central AHI 28/h and 41/h respectively), all preceded by hyperventilation. PSG showed low rapid eye movement (REM) sleep (9.7-18.3%), frequent awakenings, and low sleep efficiency (range 59-78%). INTERPRETATION: Episodic hyperventilation followed by central apnoeas was present while awake in two of four patients. This may indicate failure of brainstem respiratory centres. In addition, low REM sleep, frequent arousals (not caused by apnoeas/seizures), and low sleep efficiency were present. Similar to Rett syndrome, in patients with CDKL5 mutations PSG seems warranted to evaluate breathing and sleep disturbances.

Effects of daytime secondarily generalized epileptic seizures on sleep during the following night.

Nocturnal seizures may disturb sleep, but the effect of an epileptic seizure during daytime on sleep during the next night has been under investigated. In this proof-of-principle study, the sleep of 425 patients with epilepsy, who underwent long-term video-electroencephalography recordings, was analyzed. The sleep recordings were retrospectively divided into two groups: Seizure Free, no seizure occurred at least 24 h before the start of the night sleep recording, and Daytime Seizure, at least one (secondarily) generalized seizure occurred during the day before. In Daytime Seizure, longer time in bed and latency to first REM and more NREM II were seen as well as a decrease of deep sleep and REM sleep compared to Seizure Free. As many participants underwent long-term recordings over a period longer than 48 h, we had the opportunity to compare in individual patients the sleep architecture during nights with and without seizures on the day before the recording. Time in bed and WASO were longer, and sleep efficiency was less in the nights after a seizure on the day before the recording. These differences were statistically significant, but their clinical relevance is doubtful.

Morningness and eveningness: when do patients take their antiepileptic drugs?

Almost one-third of epilepsy patients continue to have seizures despite adequate drug treatment. Chronotherapy (based on dynamic changes in drug pharmacology and disease-related processes) could be a promising treatment option. We aimed to explore whether different circadian types adjust administration times of anti-epileptic drugs (AEDs) as a step in exploring chronotherapeutic possibilities. We performed a questionnaire-based study to compare behavior of different circadian types in relation to times of taking drugs. Circadian type was determined by the Morningness-Eveningness Questionnaire. Results clearly show that morning types are taking their AEDs significantly earlier than do evening types on free days. Times of taking AEDs in the morning on work days also differ significantly between morning and evening types. Regardless of circadian type, drugs on free days are taken later than on working days. In conclusion, our study shows that patients adapt times of taking medication to their circadian type.

Associating executive dysfunction with behavioral and socioemotional problems in children with epilepsy. A systematic review.

As children with epilepsy may have a number of learning and behavioral problems, it is important that insight into the underlying neurocognitive differences in these children, which may underlie these areas of challenge is gained. Executive function (EF) problems particularly are associated with specific learning abilities as well as behavioral problems. We aim to review systematically the current status of empirical studies on the association between EF problems and behavior and socioemotional problems in children with epilepsy. After search, 26 empirical studies were identified, most of them of moderate quality. Overall, attention problems were the most reported cognitive deficit in test assessment and the most reported problem by parents. In 54% of the studies, children with epilepsy scored below average compared to controls/normative samples on different aspects of EF. Most studies reported behavior problems, which ranged from mild to severe. Forty-two percent of the studies specifically reported relationships between EF deficits and behavioral problems. In the remaining studies, below average neuropsychological functioning seemed to be accompanied by above average reported behavioral problems. The association was most pronounced for cognitive control and attention in relation to externalizing behavior problems. This cognitive control is also associated with social functioning. Relevant epilepsy variables in this relationship were early age at onset and high seizure frequency.Future research should distinguish specific aspects of EF and take age into account, as this provides more insight on the association between EF and behavior in pediatric epilepsy, which makes it possible to develop appropriate and early intervention.

Temporal distribution of clinical seizures over the 24-h day: a retrospective observational study in a tertiary epilepsy clinic.

PURPOSE: Very few studies have evaluated seizure occurrence in humans over the 24-h day; data from children are particularly scarce. Circadian patterns in seizure occurrence may be of importance in epilepsy research and may have important implications in diagnosis and therapy. METHODS: We have analyzed clinical seizures of 176 consecutive patients (76 children, 100 adults) who had continuous electroencephalography (EEG) and video monitoring lasting more than 22 h. Several aspects of seizures were noted, including classification, time of day, origin, and sleep stage. RESULTS: More than 800 seizures were recorded. Significantly more seizures were observed from 11:00 to 17:00 h, and from 23:00 to 05:00 h significantly fewer seizures were seen. The daytime peak incidences were observed in seizures overall, complex partial seizures (in children and adults), seizures of extratemporal origin (in children), and seizures of temporal origin (in adults). Incidences significantly lower than expected were seen in the period 23:00 to 05:00 h in seizures overall, complex partial seizures (in children and adults), and in tonic seizures (in children). In addition, significantly fewer seizures of temporal (in children and adults) and extratemporal origin (in children) were observed in this period. DISCUSSION: The results suggest that certain types of seizures have a strong tendency to occur in true diurnal patterns. These patterns are characterized by a peak during midday and a low in the early night.

Diurnal rhythms in seizures detected by intracranial electrocorticographic monitoring: an observational study.

Few studies have evaluated human seizure occurrence over the 24-hour day, and only one group has employed intracranial electrocorticography monitoring to record seizures. Circadian patterns in seizures may have important implications in diagnosis and therapy and provide opportunities in research. We have analyzed spontaneous seizures in 33 consecutive patients with long-term intracranial EEG and video monitoring. Several aspects of seizures were noted, including time of day, origin, type, and behavioral state (sleeping/awake). We recorded 450 seizures that showed an uneven distribution over the day, depending on lobe of origin: temporal lobe seizures occurred preferentially between 1100 and 1700 hours, frontal seizures between 2300 and 0500 hours, and parietal seizures between 1700 and 2300 hours. In the awake state, larger proportions of clinical seizures were seen from 0500 to 1100 hours and from 1700 to 2300 hours. During sleep, larger proportions occurred from 1100 to 1700 hours and from 2300 to 0500 hours. Our results suggest that seizures from different brain regions have a strong tendency to occur in different diurnal patterns.

Prevalence of sleep disturbances in people with epilepsy and the impact on quality of life: a survey in secondary care.

PURPOSE: Studies in adults with epilepsy, mainly in specialized epilepsy clinics, have shown that sleep disturbances were twice as prevalent in people with epilepsy as in healthy controls. Our aim was to determine the prevalence of sleep disturbances in people with epilepsy treated in district hospitals, as well as the impact of it on Quality of Life. METHOD: Adults with epilepsy, attending outpatient clinics in three district hospitals were invited to participate. Those who accepted (N = 122) provided their own controls matched for age and sex. Both groups completed four questionnaires (Groningen Sleep Quality Scale (GSQ), Medical Outcomes Study-Sleep scale (MOSS), Sleep Diagnosis List (SDL) and Epworth Sleepiness Scale) to measure their sleep over different periods and the 36-Item Short Form Health Survey (SF-36) to measure Quality of Life (QoL). The prevalence of sleep disturbances and scores on QoL were compared between both groups. RESULTS: Sleep quality, measured by the SDL, was in the pathological range 50% more often in the epilepsy group than in controls. This was confirmed by the MOSSINDEX and GSQ. People with epilepsy experienced excessive daytime sleepiness more often than controls. The lowest scores on nearly all domains of the SF-36 were seen in people with epilepsy and associated sleep disturbances. CONCLUSION: We confirmed the higher prevalence of sleep disturbances in people with epilepsy compared to controls as previously reported from specialized settings. The (co-morbid) sleep disturbances result in lower QoL scores, in both people with epilepsy and in controls, but more in people with epilepsy.

Sleep-Related Breathing Disorders in Young Adults With Prader-Willi Syndrome: A Placebo-Controlled, Crossover GH Trial.

CONTEXT: Sleep-related breathing disorders (SRBD) are common in people with Prader-Willi syndrome (PWS). Young adults with PWS benefit from GH continuation after attaining adult height by maintaining the improved body composition obtained during childhood. There are, no studies about the effects of GH on SRBD in young adults with PWS who were treated with GH during childhood. OBJECTIVE: Investigate the effects of GH vs placebo on SRBD in young adults with PWS who were treated with GH during childhood and had attained adult height. DESIGN: Two-year, randomized, double-blind, placebo-controlled, crossover study in 27 young adults with PWS, stratified for sex and body mass index. SETTING: Dutch PWS Reference Center. INTERVENTION: Crossover intervention with GH (0.67 mg/m2/d) and placebo, both over one year. MAIN OUTCOME MEASURES: Apnea hypopnea index (AHI), obstructive apnea index (OAI), central apnea index (CAI), measured by polysomnography. RESULTS: Compared with placebo, GH did not increase AHI, CAI, or OAI (P > 0.35). The effect of GH vs placebo was neither different between men and women, nor between patients with a deletion or maternal uniparental disomy/imprinting center defect. After two years, there was no difference in AHI, CAI, or OAI compared with baseline (P > 0.18). Two patients (7%) fulfilled the criteria of obstructive sleep apnea regardless of GH or placebo. CONCLUSIONS: GH compared with placebo does not cause a substantial increase in AHI, CAI, or OAI in adults with PWS who were treated with GH during childhood and have attained adult height. Our findings are reassuring and prove that GH can be administered safely.

Efficacy of rotigotine for treatment of moderate-to-severe restless legs syndrome: a randomised, double-blind, placebo-controlled trial.

BACKGROUND: Continuous administration of a dopamine agonist could be used to treat patients with restless legs syndrome. Our aim was to investigate the efficacy of transdermal rotigotine in the treatment of idiopathic restless legs syndrome. METHODS: In this randomised, double-blind, placebo-controlled trial, 458 patients with moderate-to-severe idiopathic restless legs syndrome (average baseline International Restless Legs Syndrome Study Group severity rating scale [IRLS] sum score of 28.1) were randomly assigned to receive transdermal rotigotine 1 mg over 24 h (n=115), 2 mg over 24 h (n=112), or 3 mg over 24 h (n=114), or to receive placebo (n=117). Study medication was delivered via patches, applied once a day for 6 months. Randomisation was done with a computer-generated randomisation list, stratified by centre. Primary efficacy outcomes were absolute change from baseline to end of maintenance in IRLS sum score and in the clinical global impressions (CGI) item 1 score, assessed by analysis of covariance in the intention-to-treat population. This trial is registered with ClinicalTrials.gov, number NCT00136045. FINDINGS: Efficacy analyses were done on 112 patients in the 1 mg group, 109 in the 2 mg group, 112 in the 3 mg group, and 114 in the placebo group. Mean change in IRLS sum score from baseline at the end of the maintenance phase was -13.7 (SE 0.9) in the 1 mg group, -16.2 (0.9) in the 2 mg group, -16.8 (0.9) in the 3 mg group, and -8.6 (0.9) in the placebo group (p<0.0001 for treatment difference vs placebo with each dose). Mean change in CGI item 1 score from baseline at the end of the maintenance phase was -2.09 (0.14) in the 1 mg group, -2.41 (0.14) in the 2 mg group, -2.55 (0.14) in the 3 mg group, and -1.34 (0.14) in the placebo group (p<0.0001 for treatment difference vs placebo with each dose). Skin reactions, mostly mild or moderate, were seen in 145 (43%) of 341 patients who received rotigotine and in two (2%) of 117 who received placebo. Ten patients had serious adverse event that were deemed to be related to rotigotine: elevation of liver enzymes (one patient), worsening of tinnitus (one patient), non-response to anticoagulation (one patient), electrocardiogram changes (one patient), and application-site reactions (six patients). No admissions to hospital were needed for the application-site reactions, and they all resolved within a short time of patch removal without any other therapeutic intervention. The rate of typical dopaminergic side-effects in patients who received rotigotine was low; no signs of augmentation were noted. INTERPRETATION: 24 h transdermal delivery of low-dose rotigotine could be used to relieve the night-time and daytime symptoms of restless legs syndrome. FUNDING: Schwarz Biosciences.

Cognition and behavior in pre-pubertal children with Prader-Willi syndrome and associations with sleep-related breathing disorders.

Prader-Willi syndrome (PWS) is characterized by hypotonia, hypogonadism, obesity, and short stature. Neurobehavioral abnormalities, cognitive impairment, and sleep-related breathing disorders (SRBD) are common. In the general population associations between neurobehavioral and cognitive abnormalities and SRBD have been found. We investigated cognition, behavior, and SRBD in children with PWS. Thirty-one pre-pubertal PWS children were evaluated (5 with paternal deletion, 14 with maternal disomy, 4 with imprinting-center mutation, and in 8 the defect was not specified). Cognition was assessed by Wechsler scale subtests, and behavior by parent-questionnaires. Polysomnography was performed. Cognition, behavior, and associations with SRBD were evaluated. All cognitive subtests were significantly below O SDS, with the lowest median (interquartile range) scores for the Block design subtest (-2.7 SDS (-3.0 to -0.3)). In 60%, verbal subtests were less affected than performance subtests. Parents reported problem behavior related to "emotions/behavior not adapted to the social situation" and "insensitivity to social information." All children had SRBD, with an Apnea Hypopnea Index of 4.1/hr (2.6-7.9). One performance subtest score was significantly higher in children with better sleep efficiency, and daytime sleepiness was associated with more autistic-like social impairment. In contrast to our expectations, behavior was worse in children with better sleep-related breathing. In pre-pubertal PWS children, cognition is impaired. Neurobehavioral abnormalities are common, particularly autistic-like social impairment. Sleep efficiency was associated with better performance on one of the performance subtests, and neurobehavioral abnormalities were associated with daytime sleepiness. In contrast, we could not confirm a positive association of neurobehavioral abnormalities with SRBD in PWS.

How to assess circadian rhythm in humans: a review of literature.

It is well known that seizures of some types of epilepsy tend to occur in patterns. The circadian rhythm may play a significant role in this phenomenon. In animal studies it has been found that seizures in experimental partial epilepsy are probably under the influence of the biological clock. In this review an introduction to the influence of the human circadian rhythm in epilepsy is given. Furthermore, the methodology of measuring the circadian rhythm in humans is explored. An overview of widely used methods includes protocols used to desynchronize circadian rhythm, and sleep-wake and biological markers such as the dim light melatonin onset, core body temperature, and cortisol that are employed to determine the phase of the circadian rhythm. Finally, the use of sleep parameters, actigraphy, and questionnaires is discussed. These are also important in assessment of the circadian rhythm.

Safety and efficiency of medication withdrawal at home prior to long-term EEG video-monitoring.

PURPOSE: Long-term video-EEG monitoring (LTM) is frequently used for diagnostic purposes and in the workup of epilepsy surgery to determine the seizure onset zone. Different strategies are applied to provoke seizures during LTM, of which withdrawal of anti-epileptic drugs (AED) is most effective. Remarkably, there is no standardized manner of AED withdrawal. For instance, the majority of clinics taper medication during clinical admission, whereas we prefer to taper medication at home prior to admission. Our aim was to study the advantages (efficiency and diagnostic yield) and disadvantages (safety and complication rates) of predominantly tapering of medication at home. METHOD: We report a retrospective observational cohort of 273 patients who had a LTM at our tertiary epilepsy center from 2005 until 2011. Provocation methods to induce seizures were determined on individual basis. Success rate (duration of admittance, time to first seizure, efficiency and diagnostic yield) and complications and serious adverse events were assessed. RESULTS: AED were tapered in 180 (66%) patients, in 93 (24%) of these patients with additional (partial) sleep deprivation. In all of these patients tapering started at home one to four weeks prior to admission. In the other patients, only (partial) sleep deprivation or none provocation method at all was applied. Seizure recordings were successful in 79,9% of patients. Complications occurred in 19 patients (10.9%) of which 3 had (1.7%) serious adverse events (status epilepticus (SE)) with AED withdrawal. These complications only occurred during admittance, not at home. CONCLUSIONS: AED withdrawal at home prior to LTM is an efficient and convenient method to increase the diagnostic yield of LTM and appears relatively safe.

Validation of the Augmentation Severity Rating Scale (ASRS): a multicentric, prospective study with levodopa on restless legs syndrome.

BACKGROUND: Augmentation is the main complication during long-term dopaminergic treatment of restless legs syndrome (RLS) and reflects an overall increase in RLS severity. Its severity varies considerably from a minor problem to a devastating exacerbation of disease. Despite its clinical relevance, systematic evaluations have rarely been undertaken and there has been no development of methods to assess the severity of augmentation. To fill this gap, the European RLS Study Group (EURLSSG) has developed the Augmentation Severity Rating Scale (ASRS), using three items that assess the degree of change in three specific dimensions of augmentation. The changes in each dimension are summed to give an ASRS total score. METHODS: The ASRS was developed to cover the basic dimensions defining RLS augmentation. The items were developed by an interactive process involving professional and patient input. The ASRS that was evaluated included four major items and two alternative forms of one item. The validation was conducted using 63 (85%) mostly untreated RLS patients from six centers, who were treated for six months with levodopa (L-Dopa) (up to 500 mg/day, as clinically needed). Two consecutive assessments before and at baseline measured test-retest reliability. Consecutive ASRS ratings by two independent raters on a subsample of patients evaluated inter-rater reliability. Comparison with clinical severity ratings of two independent experts provided external validation of the ASRS. Comparison of patients with and without augmentation with regard to the items and the total score of the ASRS added discriminant validity. RESULTS: Sixty patients (63% females, mean age: 53 years, baseline International RLS Severity Rating (IRLS) score 24.7+/-5.2) were treated with a median daily dose of 300 mg L-Dopa (range: 50-500 mg). Thirty-six patients (60%) experienced augmentation. Item analyses indicated that one item could be removed as it did not contribute significantly to the test score and only one form of the duplicated item needed to be used. The final ASRS then included three items. Test-retest reliability for the total score was rho=0.72, and inter-rater reliability was rcc=0.94. Cronbach's alpha was 0.62. Validity as assessed by the correlation between the worst ASRS total score during the trial and the expert rating was rho=0.72. ASRS total score differed between patients without versus with augmentation (mean: 7.4 (standard deviation (SD)=4.0) vs. 2.0 (2.7) (P<0.0001). CONCLUSIONS: The ASRS is a reliable and valid scale to measure the severity of augmentation. Due to the need to systematically quantify augmentation for both long-term efficacy and tolerability, the ASRS may become a useful tool to monitor augmentation in future clinical trials.

Disorders of sleep and wake in patients after subarachnoid hemorrhage.

BACKGROUND AND PURPOSE: To determine the frequency and severity of disorders of sleep and wake and their relation to the quality of life (QoL) in patients who have survived an episode of subarachnoid hemorrhage (SAH). METHODS: In a prospectively collected, consecutive series of 89 patients, 83 patients completed validated and frequently used questionnaires for the assessment of disorders of sleep and wake (SDL and Epworth Sleepiness Score) at least 1 year (range, 1 to 3.4 years) after the SAH. We used the modified Rankin scale for functional outcome and Short Form 36 (SF-36) to assess QoL. We related the occurrence of severe problems with sleep (insomnia or excessive daytime sleepiness score or both > or =3 on SDL) to functional outcome and to the QoL scores and compared the latter scores with data from a Dutch reference population. In a subset of 20 patients with severe problems with sleep, we performed polysomnographic and actigraphic studies at home during 48 hours. RESULTS: Twenty eight (34%) patients had severe problems with sleep. Frequently reported problems are initiating (25%) or maintaining (31%) sleep, difficulty returning (28%) asleep, tiredness (31%), and excessive sleepiness during the day (6%). QoL was considerably reduced in patients with severe problems with sleep. During the sleep monitoring studies, severe sleep fragmentation, sleep apnea, restless legs syndrome/periodic limb movement disorder, or a combination of these disorders of sleep and wake occurred in 19 of 20 patients. CONCLUSIONS: Many patients who have survived an episode of SAH have disorders of sleep and wake, which are related to the QoL.

Two Siblings With a CDKL5 Mutation: Genotype and Phenotype Evaluation.

This is the second report of a family with a recurrence of a CDKL5 mutation (c. 283-3_290del) in 2 sisters. Both parents tested negative for the mutation in all tissues, but germline mosaicism is likely. Clinically CDKL5 patients resemble those with Rett syndrome, caused by a MECP2 mutation, who experience a regression, after an initial normal development. Even though both siblings showed a typical CDKL5 phenotype, their presentation is different. From birth, the oldest daughter had a severe developmental delay, feeding problems, and hypotonia and experienced daily refractory seizures. The youngest daughter appeared to be normal until age 3 months. At that age seizures started, deterioration and regression became evident, and an epileptic encephalopathy developed. This report of familial recurrence, with suspected germline mosaicism in a healthy parent, has important consequences for genetic counseling. Although it is not possible to predict an exact recurrence risk, it is likely to be increased.

The development of sleep during the first months of life.

The development of sleep during the first months of life is intriguing from a scientific point of view but is interesting to the clinician as well. This review aims at providing data on sleep in "low-risk" premature neonates, neonates born at term, and infants up to the age of 6 months. The various milestones in clinical and polygraphic features of sleep allow for week-to-week assessment of brain function in normal and sick children.

The prevalence of sleep disorders in patients with Parkinson's disease. A self-reported, community-based survey.

OBJECTIVE: Sleep disruption is common in patients with Parkinson's disease (PD). The goal of our survey was to gain insight into the causes of sleep disruption and into the prevalence of specific sleep disorders in PD. METHOD: A sleep questionnaire was mailed to 400 unselected PD patients. Analysis of the results was descriptive. RESULTS AND CONCLUSION: The results of earlier performed surveys for sleep disruptive factors in PD were confirmed and a high prevalence of a possible rapid eye movement-sleep behavior disorder and restless legs syndrome in PD was found.

[Nocturnal choking sensation]. / Stikgevoelens in de nacht.

There are many different types of sleep disorders. The majority of sleep-related breathing disorders can be attributed to sleep apnoea syndrome. Nocturnal choking sensation is a different symptom, for which we present two cases. Nocturnal choking sensation is a terrifying symptom for the patient and the diagnostic approach can be challenging. Aside from sleep choking syndrome, this symptom may appear with nocturnal laryngospasm, insular epilepsy and nocturnal gastro-oesophageal reflux. A thorough patient history and observation of the nocturnal event, sometimes supported by EEG findings, may provide the clue to the diagnosis. These kinds of nocturnal symptoms are best analyzed in a clinic specialized in both epilepsy and sleep disorders.

[Staring episodes in children with developmental disorders: epilepsy or behaviour?]. / Kinderen met ontwikkelingsproblemen die staren: epilepsie of gedrag?

Behavioural episodes of staring in children are difficult to distinguish from epileptic seizures, especially in children with developmental disorders such as ADHD, autism spectrum disorders and intellectual disabilities. We discuss two patients with staring episodes who were using anti-epileptic drugs. In both patients, EEG with video monitoring showed that the staring was non-epileptic. The first is an 8-year-old boy, who developed severe motor problems and ataxia during treatment with valproate. His staring episodes were behavioural, caused by his intellectual disability, and the motor problems resolved after discontinuation of valproate. The second patient is a 10-year-old boy with known autism, ADHD and infantile seizures, who developed staring for which he was using valproate. Again, video-EEG monitoring during staring showed no abnormalities and in this case the staring was caused by his intellectual disability and autism. We discuss the differential diagnosis of staring episodes in children with developmental disorders and present the pitfalls of the diagnostic process.