Publications by authors named "Al W De Weerd"

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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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The interaction between epilepsy and sleep is thoroughly studied and is very complex. This review focuses on prevalence, impact on quality of life and effects of treatment of sleep disorders on the course of epilepsy. Self-reported sleep disturbances in people with epilepsy are about twice as prevalent as in healthy controls.

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There is strong evidence that epileptic seizures occur in diurnal or 24-h patterns. A study in rat models of partial epilepsy showed circadian seizure patterns, and in humans circadian rhythmicity in interictal discharges has been found, suggesting that circadian rhythm may play a role in epilepsy. Circadian influences on human seizure patterns have not been investigated.

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Accumulating evidence suggests epilepsy and seizures may influence circadian rhythms and that circadian rhythms may influence epilepsy. It is also conceivable that seizure timing influences the timing of daily activities, sleeping, and wakefulness (i.e.

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Temporal lobe epilepsy has a subtle and diverse symptomatology, and therefore temporal lobe seizures can initially be misdiagnosed. Here we discuss 3 patients with this type of epilepsy, but with completely different presentations. The first, a 67-year-old woman suffered from episodes of confusion, and later she developed tonic-clonic seizures.

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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.

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Knowledge on the interaction between circadian rhythm and human epilepsy is relatively poor, although if it exists, this interaction may be of value for better knowledge of pathophysiology and for timing of diagnostic procedures and therapy. It appears that human seizure occurrence may have 24-h rhythmicity, depending on the origin. These findings are endorsed by animal studies.

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Background: The annual death rate of patients with Prader-Willi syndrome (PWS) is high (3%). Many deaths of children are sudden and unexplained. Sleep apneas have been suggested to play a role in sudden deaths.

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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.

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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.

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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.

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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.

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Prader-Willi syndrome (PWS) is a neurogenetic disorder with hypotonia, psychomotor delay, obesity, short stature, and sleep-related breathing disorders. The aim of this study was to evaluate the association between psychomotor development and sleep-related breathing disorders in PWS infants. Bayley Scales of Infant Development were performed in 22 PWS infants, with a median (interquartile range, IQR) age of 1.

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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.

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