The burden of parenting children with frontal lobe epilepsy.

OBJECTIVE: Caring for a child with a chronic illness adds stress to the typical parenting stress in healthy developing children. This stress can place a heavy burden on parents and may increase when a child displays problem behavior. In general, parenting and child's behavior problems are associated. Furthermore, externalizing (more outgoing) behavior is reported frequently in children with frontal lobe epilepsy (FLE). Therefore, in this study, we first investigated the burden of parents of children with FLE, and second, we investigated the relation between the experienced burden and reported behavioral problems. The validity of parents' reports on proxy measures as well as duration of epilepsy is taken into account. METHODS: Thirty-one parents of children with FLE completed validated questionnaires about behavioral problems and burden of parenting. To examine if parents tend to be inconsistent or unusually negative, we used the two validity scales of the Behavioral Rating Inventory of Executive Function (BRIEF) (Negativity and Inconsistency). RESULTS: Only parents of children with FLE who have had epilepsy for 5 years or longer report more problems on the Nijmeegse Vragenlijst voor de Opvoedingssituatie (NVOS) subscales 'Able to manage', 'Child is a burden', and 'Good Interaction' compared with the healthy controls. The subscale 'Child is a burden' significantly predicts scores in about 20% to 49% on the main scales of the Child Behavior Checklist (CBCL), the Global Executive Composite (GEC), and Behavioral Regulation Index (BRI) of the BRIEF. Only 6% of parents scored in the clinical range of the negativity scale of the BRIEF. For the inconsistency scale, this was 45%. CONCLUSION: Parents of children with FLE do not report excessive parental burden. Longer duration of epilepsy might be a risk factor in experiencing burden. The findings suggest a link between parental burden and behavioral problems in children with FLE. Externalizing behavioral problems are the most marked behavioral problems, which relate to the parental burden. Parents tend to be inconsistent in their ratings.

Headache in epilepsy patients: the (un)awareness of this phenomenon among Dutch neurologists.

PURPOSE: Headache is a frequently heard complaint that can strongly influence quality of life. This is probably even more so in people with a chronic illness. Knowing that headache, and especially migraine, is more frequent among epilepsy patients, the knowledge concerning this problem has been studied among Dutch neurologists. METHODS: Seven hundred and seventy two neurologists, working in 89 hospitals and two tertiary epilepsy clinics were asked to participate. Using a questionnaire, neurologists were surveyed on different subjects, e.g. whether they thought current headaches are more frequent in people with epilepsy than in the general population, their interest for epilepsy and how many patients with epilepsy visited their polyclinic per month. RESULTS: In total, 334 questionnaires were returned (response rate of 43%) of which 18 were excluded. One third of neurologists responded affirmatively that current headaches are more prevalent among people with epilepsy and eight percent knows that this is, more specified, migraine. The number of years of experience does not influence knowledge on headaches in epilepsy patients. The interest in epilepsy and the number of epilepsy patients per month on the polyclinic does. CONCLUSIONS: These results show that the occurrence of headache in people with epilepsy is underestimated by Dutch neurologists. This leaves an often bothersome and potentially treatable condition underexposed.

Periictal and interictal headache including migraine in Dutch patients with epilepsy: a cross-sectional study.

As early as in 1898, it was noted that there was a need to find "a plausible explanation of the long recognized affinities of migraine and epilepsy". However, results of recent studies are clearly conflicting on this matter. In this cross-sectional study, we aimed to define the prevalence and characteristics of both seizure-related and interictal headaches in patients with epilepsy (5-75years) seeking help in the tertiary epilepsy clinic SEIN in Zwolle. Using a questionnaire, subjects were surveyed on the existence of headaches including characteristics, duration, severity, and accompanying symptoms. Furthermore, details on epilepsy were retrieved from medical records (e.g., syndrome, seizure frequency, and use of drugs). Diagnoses of migraine, tension-type headache, or unclassifiable headache were made based on criteria of the International Classification of Headache Disorders. Between March and December 2013, 29 children and 226 adults were evaluated, 73% of whom indicated having current headaches, which is significantly more often when compared with the general population (p<0.001). Forty-nine percent indicated having solely interictal headache, while 29% had solely seizure-related headaches and 22% had both. Migraine occurs significantly more often in people with epilepsy in comparison with the general population (p<0.001), and the occurrence of tension-type headaches conforms to results in the general population. These results show that current headaches are a significantly more frequent problem amongst people with epilepsy than in people without epilepsy. When comparing migraine prevalence, this is significantly higher in the population of patients with epilepsy.

On-the-road driving performance and driving-related skills in older untreated insomnia patients and chronic users of hypnotics.

RATIONALE: Many older adults report sleep problems and use of hypnotics. Several studies have shown that hypnotics can have acute adverse effects on driving the next morning. It is unclear however whether driving of chronic hypnotic users is impaired. Therapeutic effects on insomnia and development of tolerance may reduce the residual effects on driving. OBJECTIVES: The present study aimed to compare actual driving performance and driving-related skills of chronic hypnotic users to good sleepers. To determine whether insomnia itself affects driving performance, driving and driving-related skills were compared between insomnia patients who do not or infrequently use hypnotics and good sleepers. METHODS: Twenty-two frequent users of hypnotics (using hypnotics ≥ 4 nights per week for more than 3 months), 20 infrequent users (using hypnotics ≤ 3 nights per week), and 21 healthy, age-matched controls participated in this study. On the night before testing, all subjects were hospitalized for an 8-h sleep recorded by polysomnography. Frequent hypnotic users used their regular medication at bedtime (2330 hours), while infrequent users and controls received no medication. Cognitive performance (word learning, digit span, tracking, divided attention, vigilance, and inhibitory control) was assessed 8.5 h and driving performance between 10 and 11 h after bedtime and dosing. RESULTS: Polysomnographic recordings did not significantly differ between the groups, but the insomnia patients, treated or untreated, still reported subjective sleep complaints. Results show no differences in driving performance and driving-related skills between both groups of insomnia patients and controls. CONCLUSIONS: Driving performance in chronic users of hypnotics and untreated insomnia patients is not impaired. For chronic users, this may be due to prescription of relatively safe drugs and low doses. For untreated insomniacs, this corroborates previous findings showing an absence of neuropsychological deficits in this group of patients.

Residual effects of zopiclone 7.5 mg on highway driving performance in insomnia patients and healthy controls: a placebo controlled crossover study.

RATIONALE: Residual effects of hypnotics on driving performance have been mainly determined in studies using a standardized driving test with healthy good sleepers. Responses to effects may differ, however, between insomniacs and healthy volunteers due to the underlying sleep disorder. In addition, a majority of insomniacs uses hypnotics chronically resulting in the development of tolerance to impairing effects. Impaired driving performance in healthy volunteers may then be an overestimation of the actual effects in insomniacs. OBJECTIVES: The present study aims to compare the residual effects of zopiclone 7.5 mg on on-the-road driving performance of 16 middle-aged insomniacs chronically using hypnotics (chronic users), 16 middle-aged insomniacs not or infrequently using hypnotics (infrequent users), and 16 healthy, age matched, good sleepers (controls). METHODS: The study was conducted according to a 3 × 2 double-blind, placebo controlled crossover design, with three groups and two treatment conditions. Treatments were single oral doses of zopiclone 7.5 mg and placebo administered at bedtime (2330 hours). Between 10 and 11 h after administration subjects performed a standardized highway driving test. RESULTS: Zopiclone 7.5 mg significantly impaired on-the-road driving performance in both insomnia groups and healthy controls. The magnitude of impairment was significantly less in the chronic users group as compared with the controls. CONCLUSIONS: The smaller magnitude of effects suggests that investigating residual effects of hypnotics in healthy volunteers may yield a minor overestimation of the actual effects in insomnia patients.

Subjective sleep disturbances in children with partial epilepsy and their effects on quality of life.

PURPOSE: The purposes of this study were to explore the prevalence of sleep disturbances in a large cohort of school-aged children with partial epilepsy, to compare the findings with those in children without epilepsy of the same age and gender, and to evaluate the relationship between sleep disturbances and health-related quality of life (HRQoL). METHODS: One hundred thirty children with partial epilepsy aged 4 to 10years, who were treated in the outpatient setting of a Dutch epilepsy clinic, and 161 age- and sex-matched controls participated in this study. In addition to providing information about their child's demography and health, parents of both groups of children completed three questionnaires to measure their child's sleep [Sleep Disturbance Scale for Children (SDSC), Medical Outcomes Study-Sleep Scale (MOSS-S), and Groningen Sleep Quality Scale (GSQS)] and one questionnaire to measure quality of life (Kidscreen-27). Parents of children with epilepsy also completed the Hague Scales to measure the severity of epilepsy. The prevalence of sleep disturbances and scores on HRQoL in children with and without epilepsy were compared. Additionally, the HRQoL scores were compared between children with and without sleep disturbances in children both with and without epilepsy. RESULTS: The answers for all three questionnaires suggested worse sleep in children with epilepsy than in children of the same age and gender without epilepsy. Pathological scores (T-value>70) for total SDSC were seen twelve times more frequently in children with epilepsy (36.92% vs. 3.01%, p<0.001). Children with epilepsy also scored significantly lower for all dimensions of HRQoL. Between subgroups of children with and without disturbed sleep, insignificant differences in quality of life were found, with the lowest scores in children with sleep disturbances in both groups. CONCLUSION: This study confirms the high prevalence of disturbed sleep, as well as its effect on quality of life, in a large group of children with partial epilepsy. The abnormalities are both more prevalent and more severe than in children without epilepsy.

Sleep monitoring by actigraphy in short-stay ICU patients.

Sleep deprivation is common in intensive care unit (ICU) patients. The criterion standard for sleep monitoring, polysomnography, is impractical in ICU. Actigraphy (a wrist watch indicating amount of sleep) proved to be a good alternative in non-ICU patients, but not in prolonged mechanically ventilated patients, probably due to ICU-acquired weakness. Short-stay ICU patients do not suffer from ICU-acquired weakness. However, the accuracy of actigraphy is unknown in these patients. Therefore, we compared actigraphy to polysomnography in short-stay ICU patients. Sleep measurements were conducted in 7 postcardiothoracic surgery patients. The sensitivity (percentage of actigraphy data that agreed with sleep determined using polysomnography) and specificity (percentage of actigraphy data that agreed with awake determined using polysomnography) were calculated. The result showed that actigraphy underestimated the amount of wake time and overestimated the amount of sleep. The median specificity for actigraphy was always less than 19% and sensitivity more than 94%. Therefore, actigraphy is not reliable for sleep monitoring in short-stay ICU patients.

Predicting success of vagus nerve stimulation (VNS) from interictal EEG.

PURPOSE: Vagus nerve stimulation (VNS) has shown to be an effective treatment for drug resistant epilepsy in numerous patients, however, not in all. It is still not possible to predict which patients will profit from VNS. In this pilot study, we explore predictive interictal EEG features for seizure reduction after VNS. METHODS: 19 Patients with medically refractory epilepsy and an implanted VNS system were included. Interictal EEG registrations, recorded before implantation, were retrospectively analysed. A quantative symmetry measure, the pair wise derived brain symmetry index (pdBSI), was tested to predict VNS outcome. Reduction in seizure frequency was used to define the responders. RESULTS: 10 Patients did respond to VNS, of whom 7 patients had a seizure reduction of at least 50% in a follow-up period of 2 years. On average, we find higher pdBSI values for delta, theta, alpha and beta bands for non-responders than for responders. The average pdBSI of the theta and alpha bands could significantly discriminate between responders and non-responders. CONCLUSION: In this study, quantifying EEG symmetry using the pdBSI shows promising results in predicting the reduction of seizure frequency after VNS treatment.

[Interactions of epilepsy and psychiatric problems: life-threatening situations]. / Interactie van epilepsie en psychiatrische problematiek: levensbedreigend.

Epilepsy and psychiatric problems can occur simultaneously and can, quite unexpectedly, trigger interactions that may lead to life-threatening situations. This became obvious in the case of a female patient who suddenly developed a status epilepticus while admitted to hospital. The seizure was probably connected with secret self-induced vomiting which led to a low intake of antiepileptic medication. She used the vomiting to control emotionally charged post-traumatic intrusions. Both the vomiting and the number of seizures were triggered by a life event (in this case emigration). In case of epilepsy, early screening for psychopathology seems advisable because there may be interactions with epileptic variables.

[The restless legs syndrome can be treated with dopamine agonists]. / Het 'restless legs'-syndroom, te behandelen met dopamineagonisten.

In three patients, a man aged 46 years and two women aged 74 and 38 years, respectively, restless legs syndrome (RLS) was diagnosed. The second patient had a low serum ferritin concentration. The patients were successfully treated with ropirinol, iron suppletion and gabapentine, and pramipexol, respectively. With a prevalence of 7% in the general population, RLS is a frequent disease. Patients complain about unpleasant feelings in the legs and have an urge to move the legs, causing discomfort during rest. Sleep is disturbed significantly, which interferes with normal functioning of the patients during the day. RLS may be caused by anaemia, uraemia or as a side effect of drugs such as selective serotonin uptake inhibitors (SSRIs), lithium and tricyclic antidepressants. RLS can be treated successfully by dopaminergic agents, especially dopamine agonists.

Sleep-related breathing disorders in prepubertal children with Prader-Willi syndrome and effects of growth hormone treatment.

CONTEXT: Recently, several cases of sudden death in GH-treated and non-GH-treated, mainly young Prader-Willi syndrome (PWS), patients were reported. GH treatment in PWS results in a remarkable growth response and an improvement of body composition and muscle strength. Data concerning effects on respiratory parameters, are however, limited. OBJECTIVE: The objective of the study was to evaluate effects of GH on respiratory parameters in prepubertal PWS children. DESIGN: Polysomnography was performed before GH in 53 children and repeated after 6 months of GH treatment in 35 of them. PATIENTS: Fifty-three prepubertal PWS children (30 boys), with median (interquartile range) age of 5.4 (2.1-7.2) yr and body mass index of +1.0 sd score (-0.1-1.7). INTERVENTION: Intervention included treatment with GH 1 mg/m2.d. RESULTS: Apnea hypopnea index (AHI) was 5.1 per hour (2.8-8.7) (normal 0-1 per hour). Of these, 2.8 per hour (1.5-5.4) were central apneas and the rest mainly hypopneas. Duration of apneas was 15.0 sec (13.0-28.0). AHI did not correlate with age and body mass index, but central apneas decreased with age (r = -0.34, P = 0.01). During 6 months of GH treatment, AHI did not significantly change from 4.8 (2.6-7.9) at baseline to 4.0 (2.7-6.2; P = 0.36). One patient died unexpectedly during a mild upper respiratory tract infection, although he had a nearly normal polysomnography. CONCLUSIONS: PWS children have a high AHI, mainly due to central apneas. Six months of GH treatment does not aggravate the sleep-related breathing disorders in young PWS children. Our study also shows that monitoring during upper respiratory tract infection in PWS children should be considered.

ECG abnormalities in predicting secondary cerebral ischemia after subarachnoid haemorrhage.

BACKGROUND: Electrocardiographic (ECG) abnormalities frequently occur after subarachnoid haemorrhage (SAH), and have been linked with poor outcome. The pathogenesis behind this relation is unclear. We hypothesized that cardiac dysfunction may contribute to the development of delayed cerebral ischemia (DCI) and investigated if electrocardiographic repolarization abnormalities on admission, representing this cardiac dysfunction, are related to DCI. We also assessed the additional value of ECG characteristics to establish prognosticators for clinical outcome (WFNS, age and Hijdra score). METHOD: In a series of 121 consecutive patients with aneurysmal SAH we related individual repolarization-like ECG changes (ST and T-wave changes, QTc prolongation, a U-wave) to the occurrence of DCI by means of Cox proportional hazard modelling and to poor outcome (death or dependence) with logistic regression analysis. We used ROC curves to assess the additional prognostic value of the most important ECG characteristics to established prognosticators. FINDINGS: Only ST segment depression had a statistically significant relationship with the occurrence of DCI (HR 2.4 [95%CI 1.2-4.9]) in univariate analysis. In a similar analysis ST-elevation (OR 4.9; [95%CI 0.99-24.0]), ST-depression (OR 10.6; [95%CI 2.3-48.8]), T-wave inversion (OR 2.5; [95%CI 1.1-5.5]) and ischemic like ECG abnormalities (OR 8.3; [95%CI 3.0-22.2]) were significantly related to poor outcome. In multivariate models with extension of these ECG characteristics for establishing prognosticators the AUC of the ROC improved from 0.81 to 0.84. CONCLUSIONS: ECG abnormalities did not contribute to the prediction of DCI and have limited value in prognosticating poor outcome. The occurrence of DCI is not the explanation of this relationship between ECG characteristics and outcome.

Abnormal H-reflexes in periodic limb movement disorder; impact on understanding the pathophysiology of the disorder.

OBJECTIVE: To get more insight in the pathophysiological basis of periodic limb movement disorder (PLMD) with or without restless legs syndrome (RLS), we investigated whether these patients have spontaneous changes in H-reflexes or show altered reflex patterns after (external) inhibition or excitation of the relevant spinal segment. METHODS: The ratio of the peak-to-peak values of the maximal soleus H-reflex and the maximal direct muscle potential (H/M ratio), H-reflex recruitment curves, vibratory inhibition and recovery curves of the soleus H-reflex in double stimulus experiments were measured in 9 PLMD patients and 11 controls. RESULTS: In comparison to controls the vibratory inhibition, predominantly reflecting pre-synaptic inhibitory action, was depressed in PLMD patients. The soleus H-reflex recovery curves showed increased late facilitation and depressed late inhibition, both reflecting diminished inhibition due to post-synaptic central activity. CONCLUSIONS: Our data indicate diminished inhibition at spinal level in PLMD patients. This is probably due to altered function of the descending spinal tracts, peripheral influence or changes at the inter-neural circuitry at spinal level itself, or combinations of these 3 possibilities. SIGNIFICANCE: The results of this study give further insight in the pathophysiology of PLMD and RLS by stressing the importance of diminished central inhibition.

Clinical and electrophysiological follow-up after local steroid injection in the carpal tunnel syndrome.

OBJECTIVE: Local steroid injections are used for treatment for the carpal tunnel syndrome (CTS). Study of changes in neurophysiological parameters after such treatment for idiopathic CTS might be a supportive argument for the effectiveness of steroid treatment. METHODS: Twenty-one patients with CTS were included and evaluated before and at 1, 3 and 6 months after treatment. At the inclusion date patients received a single 1 ml local steroid injection. Various electrophysiological tests were used. For clinical evaluation, we used the Boston Carpal Tunnel Questionnaire (BCTQ) and the General Outcome Score. RESULTS: The mean nerve conduction parameters had significantly improved at 1 month, except the SNAP4 and CMAP. This improvement was still present at 3 months. At 6 months follow-up, the improvement in the SDL4, SNAP4, M-U, DML and CMAP remained significant. In 6 patients (29%), the nerve conduction parameters normalized and remained so until the end of the study. The BCTQ and the General Outcome Score significantly improved as well. However, there was no correlation between the electrophysiological data, the BCTQ and the General Outcome Score. CONCLUSIONS: The improvements of nerve conduction parameters independently support the ideas on effectiveness of steroid injection therapy in CTS.

[The teenager who finds it difficult to wake up in the morning: aberrant behavior, misperception or an underlying sleep disorder?]. / 's Morgens moeilijk wakker worden bij tieners: gedragsprobleem, misperceptie of slaapstoornis?

Three teenagers, two girls aged 14 years and one boy aged 10 years, had had difficulty with getting to sleep in the evening and waking up in the morning from a very early age. These difficulties led to underperformance at school and/or tension in the household. The anamneses and a sleep study led to the following conclusions: 'delayed sleep phase syndrome', 'sleep state misperception' and 'aberrant interaction between the child and its parents'. Treatment with phototherapy and regulation of the bedtimes, information and regulation of the bedtimes, and pedagogic advice, respectively, was successful within a few months. Teenagers often complain about problems with sleeping, in particular about late onset of sleep and difficulties with awakening. These problems are self-limiting in most cases, but can have a disastrous influence on social life and education.

Activity patterns of leg muscles in periodic limb movement disorder.

The movements of leg muscles in reference to periodic limb movement disorder (PLMD) have only been described in global terms. The sequences of contracting muscles that cause the PLMs are said to be stereotypical. There is, however, doubt about this fixed sequencing in PLMD. Our goal was to define the sequence of muscle movements in PLMs and then analyse their patterns. We recorded with surface EMG all movements of the muscles said to be involved in PLMs (extensor digitorum brevis, EDB; tibialis anterior, TA; biceps femoris, BF; tensor fasciae latae; TFL) as well as the quadriceps (Q) and soleus (S) muscles in 12 patients with restless legs syndrome combined with PLMD. Accompanying polysomnography provided the sleep parameters. In total, 469 movements were analysed. In only 12% was there the appearance of the classic movement (EDB-TA-BF-TFL) or its direct variants. The most frequent sequences were characterised by contraction of only the TA, TA-EDB only, or TA-EDB followed by all other combinations (32%). The pattern EDB only, EDB-TA, or EDB-TA followed by contraction of one or more other muscles, was seen in 18%. All other combinations appeared in much smaller numbers or only once. Eight patients had specific patterns. Three consistently started with the same muscle. One patient always contracted all six muscles. Six patients never contracted more than three muscles. The number of muscles contracted correlated positively with the appearance of arousal from sleep. The interval between onset of contractions within the PLMs varied randomly in a range of 0-1 s. Within PLMs many variations of muscle movements were documented. Patterns were recognisable, individually determined, and related to arousal from sleep.

Ropinirole in the treatment of restless legs syndrome: results from the TREAT RLS 1 study, a 12 week, randomised, placebo controlled study in 10 European countries.

OBJECTIVE: To assess the efficacy, safety, and tolerability of ropinirole in the treatment of patients with restless legs syndrome. METHODS: A 12 week, prospective, double blind, randomised comparison involving 284 patients from 10 European countries. All participants had a score of > or =15 on the international restless legs scale (IRLS). Patients were randomised (1:1) to receive either ropinirole 0.25-4.0 mg once daily or placebo. The primary efficacy end point was mean change from baseline to week 12 in total IRLS score. Global improvements (clinical global impression (CGI) scale) and improvements in sleep, health related quality of life (QoL; using generic and disease specific measures), work, and other activities were also assessed. RESULTS: 112/146 patients (76.7%) taking ropinirole and 109/138 (79.0%) taking placebo completed the study. Improvement in IRLS at week 12 with ropinirole (mean (SD) dose, 1.90 (1.13) mg/day) was greater than with placebo (mean (SE): -11.04 (0.719) v -8.03 (0.738) points; adjusted difference = -3.01 (95% confidence interval (CI), -5.03 to -0.99); p = 0.0036). More patients in the ropinirole group (53.4%) showed improvement on the CGI scale at week 12 than in the placebo group (40.9%; adjusted odds ratio = 1.7 (1.02 to 2.69); p = 0.0416). Significant differences on both IRLS and CGI scales favouring ropinirole were apparent by week 1. Ropinirole was also associated with significantly greater improvements in sleep and QoL end points. The most common adverse events were nausea and headache. CONCLUSIONS: Ropinirole improves restless legs syndrome compared with placebo, with benefits apparent by week 1. It is generally well tolerated.