Newborn With Refractory Seizures Due To Hemimegalencephaly And Tuberous Sclerosis Complex: Case Report and Literature Review.

Background Hemimegalencephaly (HME) is a rare congenital disorder that is initiated during embryonic development with abnormal growth of one hemisphere. Tuberous sclerosis complex (TSC), a genetic disorder, is rarely associated with HME. Methods We present a case of a newborn with HME with a confirmed mutation in the TSC-1 gene and describe the clinical course, findings on (amplitude integrated) electroencephalography (aEEG), cranial ultrasound (CUS), MRI, and the postmortem evaluation.

Expert level of detection of interictal discharges with a deep neural network.

Objective: Deep learning methods have shown potential in automating the detection of interictal epileptiform discharges (IEDs) in electroencephalography (EEG). We compared IED detection using our previously trained deep neural network with a group of experts to assess its potential applicability.

Methods: First, we performed clinical validation on an internal data set.

Quantitative Characterization of Rhythmic and Periodic EEG Patterns in Patients in a Coma After Cardiac Arrest and Association With Outcome.

Objectives: Rhythmic and periodic patterns (RPPs) on EEG in patients in a coma after cardiac arrest are associated with a poor neurologic outcome. We characterize RPPs using qEEG in relation to outcomes.

Methods: Post hoc analysis was conducted on 172 patients in a coma after cardiac arrest from the TELSTAR trial, all with RPPs.

Quantitative electroencephalography in cerebral amyloid angiopathy.

Objective: We investigated whether quantitative electroencephalography (qEEG) correlates with cognition and cortical superficial siderosis (cSS) in cerebral amyloid angiopathy.

Methods: We included patients with sporadic (sCAA) and hereditary Dutch-type CAA (D-CAA). Spectral measures and the phase lag index (PLI) were analyzed on qEEG.

Efficacy of Levetiracetam as Add-On Therapy in the Treatment of Seizures in Neonates.

Introduction: There is no consensus regarding the efficacy of add-on therapy with levetiracetam (LEV) in the treatment of seizures in neonates. The aim of this study was to evaluate the efficacy of add-on therapy with LEV for achieving >80% seizure reduction after phenobarbital (PB) treatment.

Methods: Retrospective cohort study of near term neonates admitted to the neonatal intensive care unit with EEG-confirmed seizures despite treatment with PB as first-line therapy and using LEV as 2nd-, 3rd- or 4th-line treatment.

Cognition, emotional state, and quality of life of survivors after cardiac arrest with rhythmic and periodic EEG patterns.

Aim: Rhythmic and periodic patterns (RPPs) on the electroencephalogram (EEG) in comatose patients after cardiac arrest have been associated with high case fatality rates. A good neurological outcome according to the Cerebral Performance Categories (CPC) has been reported in up to 10% of cases. Data on cognitive, emotional, and quality of life outcomes are lacking.

Using Existing Clinical Information Models for Dutch Quality Registries to Reuse Data and Follow COUMT Paradigm.

Background: Reuse of health care data for various purposes, such as the care process, for quality measurement, research, and finance, will become increasingly important in the future; therefore, "Collect Once Use Many Times" (COUMT). Clinical information models (CIMs) can be used for content standardization. Data collection for national quality registries (NQRs) often requires manual data entry or batch processing.

Perioperative cerebral perfusion in aortic arch surgery: a potential link with neurological outcome.

Objectives: The aim of this study was to examine whether perioperative changes in cerebral blood flow (CBF) relate to postoperative neurological deficits in patients undergoing aortic arch surgery involving antegrade selective cerebral perfusion (ASCP).

Methods: We retrospectively analysed data from patients who underwent aortic arch surgery involving ASCP and perioperative transcranial Doppler assessments. Linear mixed-model analyses were performed to examine perioperative changes in mean bilateral blood velocity in the middle cerebral arteries, reflecting changes in CBF, and their relation with neurological deficits, i.

Myoclonus in comatose patients with electrographic status epilepticus after cardiac arrest: Corresponding EEG patterns, effects of treatment and outcomes.

Objective: To clarify the significance of any form of myoclonus in comatose patients after cardiac arrest with rhythmic and periodic EEG patterns (RPPs) by analyzing associations between myoclonus and EEG pattern, response to anti-seizure medication and neurological outcome.

Design: Post hoc analysis of the prospective randomized Treatment of ELectroencephalographic STatus Epilepticus After Cardiopulmonary Resuscitation (TELSTAR) trial.

Setting: Eleven ICUs in the Netherlands and Belgium.

[The importance of professional guidelines for Appropriate Care].

'Appropriate Care' forms the cornerstone of the Integral Care Agreement, composed and signed by several Dutch health care organizations and the government. Professional guidelines play an important role to fulfill the four principles of 'Appropriate Care'. Guidelines contribute to value based care by describing effective and efficient treatments.

Heterozygous NOTCH1 Variants Cause CNS Immune Activation and Microangiopathy.

NOTCH1 belongs to the NOTCH family of proteins that regulate cell fate and inflammatory responses. Somatic and germline NOTCH1 variants have been implicated in cancer, Adams-Oliver syndrome, and cardiovascular defects. We describe 7 unrelated patients grouped by the presence of leukoencephalopathy with calcifications and heterozygous de novo gain-of-function variants in NOTCH1.

Case Report: A Highly Variable Clinical and Immunological Presentation of IKAROS Deficiency in a Single Family.

Here we describe a novel mutation in the gene encoding IKAROS, as the cause of common variable immunodeficiency (CVID). The identification of the same defect in the gene with manifestations of asymptomatic selective IgA deficiency and chronic ITP in the father and her younger brother, respectively, demonstrates the large variability of this genetic defect in one single family, while living in the same environment with a relatively similar genetic background. As discussed, clinical penetrance of the molecular defects identified by mutations in and other common gene defects in CVID in familial immune-related abnormalities makes genetic testing a necessary step for diagnosis, management, and counseling, as part of the routine immunological workup.

Treating Rhythmic and Periodic EEG Patterns in Comatose Survivors of Cardiac Arrest.

Background: Whether the treatment of rhythmic and periodic electroencephalographic (EEG) patterns in comatose survivors of cardiac arrest improves outcomes is uncertain.

Methods: We conducted an open-label trial of suppressing rhythmic and periodic EEG patterns detected on continuous EEG monitoring in comatose survivors of cardiac arrest. Patients were randomly assigned in a 1:1 ratio to a stepwise strategy of antiseizure medications to suppress this activity for at least 48 consecutive hours plus standard care (antiseizure-treatment group) or to standard care alone (control group); standard care included targeted temperature management in both groups.

Effects of targeted temperature management at 33 °C vs. 36 °C on comatose patients after cardiac arrest stratified by the severity of encephalopathy.

Objectives: To assess neurological outcome after targeted temperature management (TTM) at 33 °C vs. 36 °C, stratified by the severity of encephalopathy based on EEG-patterns at 12 and 24 h.

Design: Post hoc analysis of prospective cohort study.

Orbito-Cranial Gunshot Injuries with Retained Sinonasal Bullets.

Introduction: Gunshot injuries to the sino-orbital region are rare. In South Africa, where gunshot injuries are common, sino-orbital gunshot injuries are encountered. Sino-orbital gunshot injuries are associated with trauma to surrounding facial and intracranial structures.

An Elderly Woman With Recurrent Transient Loss of Consciousness Preceded by Hallucinatory Attacks.

We report a patient with transient loss of consciousness preceded by hallucinations caused by cardiac syncope. The diagnosis was confirmed by simultaneous video-electrocardiogram-electroencephalogram recording of an event. The attacks no longer occurred after pacemaker implantation.

Asthma-Associated Long TSLP Inhibits the Production of IgA.

Thymic stromal lymphopoietin (TSLP) contributes to asthmatic disease. The concentrations of protective IgA may be reduced in the respiratory tract of asthma patients. We investigated how homeostatic short TSLP (shTSLP) and asthma-associated long TSLP (loTSLP) regulate IgA production.

Transcranial Doppler 24 Hours after Carotid Endarterectomy Accurately Identifies Patients Not at Risk of Cerebral Hyperperfusion Syndrome.

Objectives: Intra-operative transcranial Doppler (TCD) is the gold standard for prediction of cerebral hyperperfusion syndrome (CHS) in patients after carotid endarterectomy (CEA) under general anaesthesia. However, post-operative cerebral perfusion patterns may result in a shift in risk assessment for CHS. This is a study of the predictive value of additional post-operative TCD measurements for prediction of CHS after CEA.

Propofol does not affect the reliability of early EEG for outcome prediction of comatose patients after cardiac arrest.

Objective: To quantify the effects of propofol on the EEG after cardiac arrest and to assess their influence on predictions of outcome.

Methods: In a prospective multicenter cohort study, we analyzed EEG recordings within the first 72 h after cardiac arrest. At six time points, EEGs were classified as favorable (continuous background), unfavorable (generalized suppression or synchronous patterns with ≥50% suppression), or intermediate.

Outcome Prediction in Postanoxic Coma With Deep Learning.

Objectives: Visual assessment of the electroencephalogram by experienced clinical neurophysiologists allows reliable outcome prediction of approximately half of all comatose patients after cardiac arrest. Deep neural networks hold promise to achieve similar or even better performance, being more objective and consistent.

Design: Prospective cohort study.

Early electroencephalography for outcome prediction of postanoxic coma: A prospective cohort study.

Objective: To provide evidence that early electroencephalography (EEG) allows for reliable prediction of poor or good outcome after cardiac arrest.

Methods: In a 5-center prospective cohort study, we included consecutive, comatose survivors of cardiac arrest. Continuous EEG recordings were started as soon as possible and continued up to 5 days.