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.

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.

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.

[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.

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.

[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.

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.

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.

Computed tomography in acute cerebral multiple sclerosis. A report of two cases.

In two cases of acute cerebral multiple sclerosis, computed tomographic (CT) scans with contrast disclosed several enhanced foci, mainly situated in the periventricular white matter and, in one patient, in the cerebellum. Administration of dexamethasone sodium phosphate in one patient and prednisone with cyclophosphamide in the other was followed by considerable clinical improvement. Successive CT scans with contrast enhancement showed a close correlation between improvement of symptoms and the subsidence of contrast-enhanced foci during treatment. Perhaps clinical improvement reflected restoration of the impaired blood-brain barrier.

Secondary periodic limb movement disorder and restless legs syndrome.

Periodic limb movement disorder (PLMD) and restless legs syndrome (RLS) are well-known entities from a clinical and polysomnographic point of view. PLMD and RLS are seen mostly as primary or hereditary diseases, but may occur in conjunction to other diseases such as uremia, polyneuropathy, Parkinson's disease, and deficiencies of iron and magnesium. This review will discuss the prevalence, etiology and pathophysiology of secondary PLMD and RLS.

Critical illness polyneuropathy in multiple organ dysfunction syndrome and weaning from the ventilator.

BACKGROUND: Acute axonal polyneuropathy has been found in patients with multiple organ dysfunction syndrome. This 'critical illness polyneuropathy' (CIP) has been associated with difficult weaning from the ventilator in retrospective studies. OBJECTIVE: To test the hypothesis that CIP is related to the degree and number of organ dysfunctions, and to weaning problems. DESIGN: Prospective study of 18 months. SETTING: A multidisciplinary intensive care unit in a general hospital SUBJECTS: Thirty-eight patients under 75 years of age who had been mechanically ventilated for more than 7 days, without previous signs of or risk factors for polyneuropathy. MEASURES: Organ dysfunctions were quantified using a dynamic scoring system (0-12 points). Electromyography studies were performed during mechanical ventilation to identify patients with and without CIP. RESULTS: CIP was present in 18 out of 38 patients and associated with an increased organ dysfunction score (5.3 +/- 1.8 vs. 3.6 +/- 1.5; p = 0.003) and number of organs involved [median (range): 4 (3-5) vs. 2 (1-4); p = 0.009], in particular cardiovascular (p = 0.003), renal (p = 0.04), and hematopoietic failure (p = 0.04). Patients with polyneuropathy were ventilated longer, but this was not clearly due to more difficult weaning [median: 16.5 (1-48) vs. 9.5 (1-38) days; p = 0.26]. Polyneuropathy was present in 2 of 4 patients with normal weaning. CONCLUSIONS: Axonal polyneuropathy is related to the severity of multiple-organ-dysfunction syndrome. Its presence does not necessarily implicate difficult weaning from artificial ventilation.

The prognosis of intraventricular hemorrhage.

A series of 40 patients with blood in the cerebral ventricular system demonstrated by computed tomography (CT) is presented. Overall prognosis was found to be better than expected from the literature. Nearly half of the patients are still alive; 12 of the 40 patients in this study had no significant residual disability. The results suggest that the rupture into the ventricular system by itself is of minor importance. However the clinical condition of the patient on admission, site and origin of the ruptured intracerebral hematoma and possibly the age of the patient seem to play an important role in predicting the outcome.

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.

Long-latency somatosensory evoked potentials.

Theoretically, long-latency somatosensory evoked potentials (SEPs) provide information on the function of somatosensory associative cortical structures. Their potential role in clinical studies and research has been hampered by the lack of standardized methodology in the use of these SEPs. Other factors, such as drugs, simultaneous stimuli, and state of consciousness, also have far-reaching influences on the various parameters of long-latency SEPs. The knowledge of the origin of most SEP components is at best fragmentary; studies on clinical-electrophysiological correlations seem to be hopeful in this respect. As yet, clinical applications of long-latency SEPs are limited; for future research, studies of disturbances of SEPs are most promising, mainly with regard to diseases of the gray matter, the influence of drugs on the cerebral function, and psychopathology.

The validity of the Dutch Sleep Disorders Questionnaire (SDQ).

The Sleep Disorders Questionnaire (SDQ) is a 176-item questionnaire designed to diagnose the presence of common sleep disorders. This study set out to assess the validity of a Dutch translation of the SDQ. Scores on 145 questionnaires were analyzed. A cluster analysis of these scores revealed the following clusters: healthy, depression, insomnia, narcolepsy, and apnea. The cluster classification proved correct for 67% of the subjects, as determined on the basis of polysomnography. These results show that the Dutch SDQ is a reasonably valid instrument for diagnosing sleep disorders.

Chemonucleolysis. Predictive factors.

The aim of this study was to identify factors in the patient's history and parameters of the neurologic, roentgenographic, and electromyographic examinations as well as cerebrospinal fluid analysis before chemonucleolysis that might be used to predict the results of this treatment after 1 year. On the basis of a combination of three factors, that is, the presence or absence of a narrowed intervertebral space on the lumbosacral survey film, the localization of the disc herniation (medial, mediolateral or lateral) on the computerized tomographic scan and the number of cells in the cerebrospinal fluid, a satisfactory result could be predicted for 92% of the patients.

Coactivation of the ulnar nerve in motor tests for carpal tunnel syndrome.

Coactivation of the ulnar nerve at the wrist can be a source of error in tests for carpal tunnel syndrome (CTS). We compared the effects of coactivation in two tests for CTS: the abductor pollicis brevis-distal motor latency (APB-DML) and lumbrical-interosseus-distal motor latency difference (2LI-DML). We studied 33 hands of 25 consecutive patients referred for suspected CTS. In severe CTS, when selective supramaximal stimulation of the median nerve was impossible, all APB-compound muscle action potentials (CMAP) showed abnormalities, indicating volume conduction of ulnar muscle activation. 2LI-DML in these hands led to falsely normal test results, as two identical CMAP were obtained after median and ulnar stimulation. In less severe CTS, warning signs indicating coactivation were observed in APB-DML virtually as often as in 2LI-DML. Undetected coactivation was more likely to be associated with false normal test results in 2LI-DML than in APB-DML.

Variability of central conduction in the course of multiple sclerosis. Serial recordings of evoked potentials in the evaluation of therapy.

In 27 normal subjects evoked potentials (EPs) were serially measured across an interval of 3.5 months. The variation in latencies of the P100 component of the VEP and of the components N13 and N20 of the SEP did not exceed 12% of the absolute values of these latencies on the first recording. The VEP and SEP were also serially recorded over the same interval in 35 patients with 'clinically definite' multiple sclerosis. Changes of the latencies exceeding those of normal subjects were noted in 30% of the cases. The changes in the EPs showed no correlation with the variations in the overall clinical disability. It is argued that EPs are probably more sensitive to changes in function of parts of the white matter than the clinical examination and are useful in the quantification of these changes. As a model for future studies, EPs were used in the 'evaluation' of ACTH therapy. It appeared, that therapy did not have effect on the EPs.

Computerized tomography in patients with multiple sclerosis.

In 13 of 23 patients with multiple sclerosis, studied with computerized tomography (C.T.) the lateral ventricles were found to be enlarged. In 10 cases sharply demarcated areas of low density were seen in the periventricular region, presumably representing 'plaques'. Cerebral atrophy was more frequent in those patients with a longer duration of the disease, with more exacerbations and with a greater degree of disability. Some areas of low density on C.T. scans correlated with clinical symptoms.