1,031 results match your criteria: "Heemstede; Leiden University Medical Center S.C.; Huygens & Versteegh M.V.[Affiliation]"

An expert committee was formed to reach consensus on the use of Tilt Table Testing (TTT) in the diagnosis of disorders that may cause transient loss of consciousness (TLOC) and to outline when other provocative cardiovascular autonomic tests are needed. While TTT adds to history taking, it cannot be a substitute for it. An abnormal TTT result is most meaningful if the provoked event is recognised by patients or eyewitnesses as similar to spontaneous ones.

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Epilepsy in China: major progress in the past two decades.

Lancet Neurol

April 2021

Institute of Neurology, Huashan Hospital, Fudan University, Shanghai, China.

China has approximately 10 million people with epilepsy. There is a vast epilepsy treatment gap in China, mainly driven by deficiencies in health-care delivery and social discrimination resulting from cultural beliefs about epilepsy. WHO's Global Campaign Against Epilepsy project in China showed that it was possible to treat epilepsy in primary care settings, which was a notable milestone.

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An expert committee was formed to reach consensus on the use of tilt table testing (TTT) in the diagnosis of disorders that may cause transient loss of consciousness (TLOC) and to outline when other provocative cardiovascular autonomic tests are needed. While TTT adds to history taking, it cannot be a substitute for it. An abnormal TTT result is most meaningful if the provoked event is recognised by patients or eyewitnesses as similar to spontaneous events.

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Purpose Of Review: Malaria, Chagas Disease and Human African Trypanosomiasis are vector-borne protozoan illnesses, frequently associated with neurological manifestations. Intriguing but ignored, limited mainly to resource-limited, tropical settings, these disorders are now coming to light because of globalisation and improved diagnosis and treatment. Enhanced understanding of these illnesses has prompted this review.

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Aims: We describe five patients with syncope caused by a complete atrioventricular block (AVB) while they were bending forward, not rising after bending, and aim to describe the occurrence and the association between bending forward and AVB.

Methods And Results: In two patients, bending forward was the exclusive trigger for syncope, while in the remaining three, other postural changes (sitting down, standing up, and exertion) could also provoke syncope. Complete AVB as the cause of syncope was documented using ECG monitoring in two cases and an implantable loop recorder in the other three.

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Aims: Syncope care is often fragmented and inefficient. Structuring syncope care through implementation of guidelines and Syncope Units has been shown to improve diagnostic yield, reduce costs and improve quality of life. We implemented the European Society of Cardiology (ESC) 2018 syncope guidelines at the Emergency Departments (ED) and established Syncope Units in five Dutch hospitals.

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Identifying patients with epilepsy at high risk of cardiac death: signs, risk factors and initial management of high risk of cardiac death.

Epileptic Disord

February 2021

Stichting Epilepsie Instellingen Nederland - SEIN, Achterweg 5, 2103 SW, Heemstede, The Netherlands, NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK, Department of Neurology, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.

People with epilepsy have a three-fold increased risk of dying prematurely, and a significant proportion is due to sudden cardiac death or acute myocardial infarctions. The causes of increased cardiovascular morbidity and mortality in epilepsy are manifold and include acute or remote effects of epileptic seizures, the longstanding epilepsy itself or antiseizure treatments. Seizure-related cardiac arrhythmias are common and comprise bradyarrhythmia and asystole, atrial fibrillation and ventricular tachycardia.

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Dravet syndrome (DS) is a rare severe epilepsy syndrome associated with slowed psychomotor development and behavioral disorders from the second year onward in a previously seemingly normal child. Among cognitive impairments, visuospatial, sensorimotor integration, and expressive language deficits are consistently reported. There have been independent hypotheses to deconstruct the typical cognitive development in DS (dorsal stream vulnerability, cerebellar-like pattern, sensorimotor integration deficit), but an encompassing framework is still lacking.

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Objective: In people with low-grade intrinsic brain tumors, an epileptic focus is often located close to the lesion. High-frequency oscillations (HFOs) in electrocorticography (ECoG) might help to delineate this focus. We investigated the relationship between HFOs and low-grade brain tumors and their potential value for tumor-related epilepsy surgery.

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Background: In the EORTC 1410/INTELLANCE 2 randomised, phase II study (NCT02343406), with the antibody-drug conjugate depatuxizumab mafodotin (Depatux-M, ABT-414) in patients with recurrent EGFR-amplified glioblastoma, the primary end-point (overall survival) was not met, and the drug had ocular dose-limiting toxicity. This study reports results from the prespecified health-related quality of life (HRQoL) and neurological deterioration-free survival (NDFS) exploratory analysis.

Patients And Methods: Patients (n = 260) were randomised 1:1:1 to receive either Depatux-M 1.

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Climate change and epilepsy: Insights from clinical and basic science studies.

Epilepsy Behav

March 2021

Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK and Chalfont Centre for Epilepsy, Bucks, UK. Electronic address:

Climate change is with us. As professionals who place value on evidence-based practice, climate change is something we cannot ignore. The current pandemic of the novel coronavirus, SARS-CoV-2, has demonstrated how global crises can arise suddenly and have a significant impact on public health.

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Article Synopsis
  • Pure autonomic failure (PAF) and multiple system atrophy (MSA) are rare disorders that cause severe autonomic system issues, and distinguishing between them can take years.
  • The study aimed to see if new non-invasive tests could help differentiate between PAF and MSA using skin and eye assessments.
  • The findings showed some differences in skin tests between affected individuals and controls, but the tests were ineffective in distinguishing between PAF and MSA specifically.
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On-the-road driving performance of patients with central disorders of hypersomnolence.

Traffic Inj Prev

June 2021

Department of Neuropsychology and Psychopharmacology, Faculty of Psychology and Neuroscience, Maastricht University, Maastricht, The Netherlands.

Introduction: Excessive Daytime Sleepiness is a core symptom of narcolepsy and idiopathic hypersomnia, which impairs driving performance. Adequate treatment improves daytime alertness, but it is unclear whether driving performance completely normalizes. This study compares driving performance of patients with narcolepsy and idiopathic hypersomnia receiving treatment to that of healthy controls.

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Background: Orexin-A and -B are neuropeptides involved in sleep-wake regulation. In human narcolepsy type 1, this cycle is disrupted due to loss of orexin-producing neurons in the hypothalamus. Cerebrospinal fluid (CSF) orexin-A measurement is used in the diagnosis of narcolepsy type 1.

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GABA receptors containing the α5 subunit mediate tonic inhibition and are widely expressed in the limbic system. In animals, activation of α5-containing receptors impairs hippocampus-dependent memory. Temporal lobe epilepsy is associated with memory impairments related to neuron loss and other changes.

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Purpose: Being able to function independently in society is an important aspect of quality of life. This ability goes beyond self-care, requires higher order cognitive functioning, and is typically measured with instrumental activities of daily living (IADL) questionnaires. Cognitive deficits are frequently observed in brain tumour patients, however, IADL is almost never assessed because no valid and reliable IADL measure is available for this patient group.

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Seizure detection devices: Exploring caregivers' needs and wishes.

Epilepsy Behav

March 2021

Stichting Epilepsie Instellingen Nederland (SEIN) Heemstede, PO Box 540, 2130 AM Hoofddorp, The Netherlands; Department of Neurology, Leiden University Medical Center (LUMC), Albinusdreef 2, 2333 ZA Leiden, The Netherlands. Electronic address:

Introduction: User preferences for seizure detection devices (SDDs) have been previously assessed using surveys and interviews, but these have not addressed the latent needs and wishes. Context mapping is an approach in which designers explore users' dreams and fears to anticipate potential future experiences and optimize the product design.

Methods: A generative group session was held using the context mapping approach.

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Article Synopsis
  • ALDH1A3 is an enzyme involved in converting aldehydes to carboxylic acids and is linked to cancer stem cells, which are associated with higher cancer relapse risks and poor outcomes.
  • Researchers characterized NR6, a new selective inhibitor of ALDH1A3, showing it effectively targets cancer cells and binds to a unique site in the enzyme.
  • NR6 exhibits anti-metastatic properties and reduces cancer stem cell markers, suggesting it could be a valuable candidate for further research in treating glioblastoma and colorectal cancers.
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GATOR1-related focal cortical dysplasia in epilepsy surgery patients and their families: A possible gradient in severity?

Eur J Paediatr Neurol

January 2021

Department of Paediatric Neurology, Motol Epilepsy Center, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, 15006, Prague, Czech Republic. Electronic address:

Article Synopsis
  • Variants of GATOR1-genes are linked to focal cortical dysplasia (FCD), a common cause of drug-resistant epilepsy in children, but data on familial cases and surgical outcomes are scarce.
  • The study involved four unrelated children with severe epilepsy and FCD, whose parents also had epilepsy, and each child was genetically tested; results showed more severe symptoms in children compared to their parents and poor seizure outcomes after surgery.
  • The findings highlight that while GATOR1-associated FCD patients are often seen as good candidates for surgery, the complexity of their condition may complicate the identification of the epileptogenic zone, leading to less favorable surgical results.
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Article Synopsis
  • Guillain-Barré syndrome (GBS) has two main subtypes: acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), with early nerve excitability changes being key indicators of subtype.
  • A study involving 19 patients from The Netherlands and 22 from Bangladesh used compound muscle action potential (CMAP) scans within days of weakness onset to assess nerve function.
  • Results showed distinct CMAP scan patterns between AMAN and AIDP patients, particularly in stimulus intensity, suggesting that early CMAP scans can help identify GBS subtype early on.
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Tuberous sclerosis complex (TSC) is a genetic multisystem disease due to the mutation in one of the two genes TSC1 and TSC2, affecting several organs and systems and carrying a significant risk of early onset and refractory seizures. The pathogenesis of this complex disorder is now well known, with most of TSC-related manifestations being a consequence of the overactivation of the mammalian Target of Rapamycin (mTOR) complex. The discovery of this underlying mechanism paved the way for the use of a class of drugs called mTOR inhibitors including rapamycin and everolimus and specifically targeting this pathway.

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Objective: Diagnosing autoimmune encephalitis (AIE) is difficult in patients with less fulminant diseases such as epilepsy. However, recognition is important, as patients require immunotherapy. This study aims to identify antibodies in patients with focal epilepsy of unknown etiology, and to create a score to preselect patients requiring testing.

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Seizure activity and brain damage in a model of focal non-convulsive status epilepticus.

Neuropathol Appl Neurobiol

August 2021

Epilepsy Unit, Fondazione Istituto Neurologico Carlo Besta, Milan, Italy.

Aims: Focal non-convulsive status epilepticus (FncSE) is a common emergency condition that may present as the first epileptic manifestation. In recent years, it has become increasingly clear that de novo FncSE should be promptly treated to improve post-status outcome. Whether seizure activity occurring during the course of the FncSE contributes to ensuing brain damage has not been demonstrated unequivocally and is here addressed.

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