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Objective: To assess the performance of a multimodal seizure detection device, first tested in adults (sensitivity 86%, PPV 49%), in a pediatric cohort living at home or residential care., Methods: In this multicenter, prospective, video-controlled cohort-study, nocturnal seizures were detected by heartrate and movement changes in children with epilepsy and intellectual disability. Participants with a history of >1 monthly major motor seizure wore Nightwatch bracelet at night for 3 months. Major seizures were defined as tonic-clonic, generalized tonic >30 s, hyperkinetic, or clusters (>30 min) of short myoclonic or tonic seizures. The video of all events (alarms and nurse diaries) and about 10% of whole nights were reviewed to classify major seizures, and minor or no seizures., Results: Twenty-three participants with focal or generalized epilepsy and nightly motor seizures were evaluated during 1511 nights, with 1710 major seizures. First 1014 nights, 4189 alarms occurred with average of 1.44/h, showing average sensitivity of 79.9% (median 75.4%) with mean PPV of 26.7% (median 11.1%) and false alarm rate of 0.2/hour. Over 90% of false alarms in children was due to heart rate (HR) part of the detection algorithm. To improve this rate, an adaptation was made such that the alarm was only triggered when the wearer was in horizontal position. For the remaining 497 nights, this was tested prospectively, 384 major seizures occurred. This resulted in mean PPV of 55.5% (median 58.1%) and a false alarm rate 0.08/h while maintaining a comparable mean sensitivity of 79.4% (median 93.2%)., Significance: Seizure detection devices that are used in bed which depend on heartrate and movement show similar sensitivity in children and adults. However, children do show general higher false alarm rate, mostly triggered while awake. By correcting for body position, the false alarms can be limited to a level that comes close to that in adults., (© 2022 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

A 19-year-old man presented on the Emergency Room on New Year's Eve with a 2 cm-wide black burn located on his lower arm. Earlier that evening he was hit by a piece of firework. Even though he immediately felt a sharp burning pain, he did not realize the severity of the injury. He presented with numbness in the digiti 4 and 5 and the Wartenberg sign, which are signs of an ulnar nerve injury. An exploration of the ulnar nerve was performed. Extensive burns and gunpowder were seen on the flexor carpi radialis and ulnaris muscle, the fascia and the epineurium of the ulnar nerve. However, the ulnar nerve was intact. A week later a babysitting procedure was performed because of absent intrinsic muscle activity, with an end-to-side anastomosis of the anterior interosseous nerve to the motor branch of the ulnar nerve. Hand physiotherapy was started after the operation.

Objective: To develop and prospectively evaluate a method of epileptic seizure detection combining heart rate and movement., Methods: In this multicenter, in-home, prospective, video-controlled cohort study, nocturnal seizures were detected by heart rate (photoplethysmography) or movement (3-D accelerometry) in persons with epilepsy and intellectual disability. Participants with >1 monthly major seizure wore a bracelet (Nightwatch) on the upper arm at night for 2 to 3 months. Major seizures were tonic-clonic, generalized tonic >30 seconds, hyperkinetic, or others, including clusters (>30 minutes) of short myoclonic/tonic seizures. The video of all events (alarms, nurse diaries) and 10% completely screened nights were reviewed to classify major (needing an alarm), minor (needing no alarm), or no seizure. Reliability was tested by interobserver agreement. We determined device performance, compared it to a bed sensor (Emfit), and evaluated the caregivers' user experience., Results: Twenty-eight of 34 admitted participants (1,826 nights, 809 major seizures) completed the study. Interobserver agreement (major/no major seizures) was 0.77 (95% confidence interval [CI] 0.65-0.89). Median sensitivity per participant amounted to 86% (95% CI 77%-93%); the false-negative alarm rate was 0.03 per night (95% CI 0.01-0.05); and the positive predictive value was 49% (95% CI 33%-64%). The multimodal sensor showed a better sensitivity than the bed sensor (n = 14, median difference 58%, 95% CI 39%-80%, p < 0.001). The caregivers' questionnaire (n = 33) indicated good sensor acceptance and usability according to 28 and 27 participants, respectively., Conclusion: Combining heart rate and movement resulted in reliable detection of a broad range of nocturnal seizures., (Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

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., (Copyright © 2014 Elsevier Inc. All rights reserved.)

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., (Copyright © 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Wnts are secreted, lipidated proteins that regulate multiple aspects of brain development, including dopaminergic neuron development. In this study, we perform the first purification and signaling analysis of Wnt2 and define the function of Wnt2 in ventral midbrain precursor cultures, as well as in Wnt2-null mice in vivo. We found that purified Wnt2 induces the phosphorylation of both Lrp5/6 and Dvl-2/3, and activates beta-catenin in SN4741 dopaminergic cells. Moreover, purified Wnt2 increases progenitor proliferation, and the number of dopaminergic neurons in ventral midbrain precursor cultures. In agreement with these findings, analysis of the ventral midbrain of developing Wnt2-null mice revealed a decrease in progenitor proliferation and neurogenesis that lead to a decrease in the number of postmitotic precursors and dopaminergic neurons. Collectively, our observations identify Wnt2 as a novel regulator of dopaminergic progenitors and dopaminergic neuron development.

Four children of Turkish origin, three boys aged 12, 8 and 7 years, and a girl aged 5 years, presented with clinical symptoms of familial Mediterranean fever. They had the characteristic episodes of fever combined with abdominal pain, thoracic pain, general malaise or arthralgia. Familial Mediterranean fever is an autosomal recessive genetic disorder restricted to people originating from the Middle East. The causative gene (MEFV) and many missense mutations have been identified. The clinical syndrome is characterised by self-limiting febrile episodes accompanied by inflammation of the serous membranes, resulting in peritonitis, pleuritis or synovitis. In untreated patients systemic amyloidosis may develop, which manifests as renal insufficiency. The diagnosis is based on the characteristic medical history and is confirmed by DNA analysis. Meanwhile, treatment with colchicine can be started. This is effective in 90% of affected patients. Being aware of the prevalence of familial Mediterranean fever in immigrant populations can improve the quality of life and prevent long-term complications.

When treating bronchial hyperresponsiveness to so-called direct and indirect stimuli, distinct pathophysiological mechanisms might require differences in dose and duration of inhaled corticosteroid therapy. To test this hypothesis in children with asthma, we investigated the time- and dose-dependent effects of 2 doses of fluticasone propionate (FP, 100 or 250 microg bid.) in improving exercise- (EIB) and methacholine-induced bronchoconstriction during 6 months of treatment, using a placebo-controlled parallel group study design. Thirty-seven children with asthma (aged 6 to 14 years; forced expired volume in 1 sec (FEV(1)) >/=70% predicted; EIB >/=20% fall in FEV(1) from baseline; no inhaled steroids during the past 4 months) participated in a double-blind, placebo-controlled, 3-arm parallel study. Children receiving placebo were re-randomized to active treatment after 6 weeks. Standardized dry air treadmill exercise testing (EIB expressed as %fall in FEV(1) from baseline) and methacholine challenge using a dosimetric technique (expressed as PD(20)) were performed repeatedly during the study. During FP-treatment, the severity of EIB decreased significantly as compared to placebo within 3 weeks, the geometric mean % fall in FEV(1) being reduced from 34.1% to 9.9% for 100 microg FP bid, and from 35.9% to 7.6% for 250 microg FP bid (P < 0.05). These reductions in EIB did not differ between the 2 doses and were sustained throughout the treatment period. PD(20) methacholine improved significantly during the first 6 weeks as compared to placebo (P < 0.04) and steadily increased with time in both treatment limbs (P = 0.04), the difference in improvement between doses (100 microg FP bid, 1.6 dose steps; 250 microg FP bid, 3.3 dose steps) approaching significance after 24 weeks (P = 0.06). We conclude that in childhood asthma, the protection afforded by inhaled fluticasone propionate against methacholine-induced bronchoconstriction is time- and dose-dependent, whereas protection against EIB is not. This suggests different modes of action of inhaled steroids in protecting against these pharmacological and physiological stimuli. This has to be taken into account when monitoring asthma treatment., (Copyright 2000 Wiley-Liss, Inc.)

At the present time, there is still controversy concerning the presence of a late asthmatic response (LAR) to exercise challenge in asthma. We have, therefore, investigated the occurrence of a LAR after exercise in asthmatic children visiting an out-patient clinic, using time-matched baseline and histamine control days, and a statistical analysis according to recently published recommendations. After a screening exercise day, 17 children (aged 7-14 yrs) randomly performed, on three subsequent study days, either: a second standardized exercise challenge; or a histamine challenge whilst matching the bronchoconstriction after exercise; or measurement of baseline lung function without any challenge. Measurements of forced expiratory volume in one second (FEV1) were made repeatedly during 8 h. Analysis was performed using multiple regression analysis for each patient, with FEV1 as the dependent, and test day (exercise or control) and clock time as independent variables during the period 2-8 h after exercise. A significant interaction (p < 0.05) between test day and clock time was considered to be indicative of a LAR. Fifteen children completed the study. All children showed an early asthmatic reaction to exercise (range 14-62% fall in FEV1). In two children, a significant interaction (p < 0.05) was found between test day and clock time. However, the difference in FEV1 between exercise and control days for each clock time did not exceed the 99.6% confidence limits of normal diurnal variation in any of the children. We conclude that, in children with mild-to-moderate asthma, a LAR to exercise does not occur. This suggests that exercise is only a symptomatic trigger of asthma. Whether exercise is capable of inducing inflammation needs to be further investigated.