Disrupting the transmembrane domain interface between PMP22 and MPZ causes peripheral neuropathy.

Peripheral Myelin Protein 22 (PMP22) and MPZ are abundant myelin membrane proteins in Schwann cells. The MPZ adhesion protein holds myelin wraps together across the intraperiod line. PMP22 is a tetraspan protein belonging to the Claudin superfamily.

Sensitivity and Costs of Intraoperative Trans-Impedance Matrix Recordings, Spread of Excitation Functions, and X-ray Imaging in Detecting Cochlear Implant Tip Foldovers.

Objective: Evaluate the sensitivity and financial costs of Trans-Impedance Matrix recordings, Spread of Excitation functions, and x-rays in detecting cochlear implant tip foldovers.

Setting: Tertiary academic medical center.

Patients: 113 ears of 108 patients.

Interictal blood-brain barrier dysfunction in piriform cortex of people with epilepsy.

Objective: The piriform cortex is considered to be highly epileptogenic. Its resection during epilepsy surgery is a predictor for postoperative seizure freedom in temporal lobe epilepsy. Epilepsy is associated with a dysfunction of the blood-brain barrier.

Associations between short-term exposure to airborne carbonaceous particles and mortality: A time-series study in London during 2010-2019.

Exposure to ambient particulate matter (PM) has been identified as a major global health concern; however, the importance of specific chemical PM components remains uncertain. Recent studies have suggested that carbonaceous aerosols are important detrimental components of the particle mixture. Using time-series methods, we investigated associations between short-term exposure to carbonaceous particles and mortality in London, UK.

"Close to My Community": A Qualitative Study of Community Health Worker-Supported Teleaudiology Hearing Aid Services.

Objectives: Given well-documented disparities in rural and minority communities, alternative service delivery models that help improve access to hearing care are needed. This article reports on a study of older Latino adults with hearing loss who received hearing aid services with Community Health Workers (CHWs) providing support via teleaudiology. The present study used qualitative data to explore perceptions of this novel service delivery model.

Long-term neuropsychological trajectories in children with epilepsy: does surgery halt decline?

Neuropsychological impairments are common in children with drug-resistant epilepsy. It has been proposed that epilepsy surgery might alleviate these impairments by providing seizure freedom; however, findings from prior studies have been inconsistent. We mapped long-term neuropsychological trajectories in children before and after undergoing epilepsy surgery, to measure the impact of disease course and surgery on functioning.

Functional brain connectivity in children with focal epilepsy: A systematic review of functional MRI studies.

Epilepsy is increasingly recognised as a brain network disorder and many studies have investigated functional connectivity (FC) in children with epilepsy using functional MRI (fMRI). This systematic review of fMRI studies, published up to November 2023, investigated profiles of FC changes and their clinical relevance in children with focal epilepsy compared to healthy controls. A literature search in PubMed and Web of Science yielded 62 articles.

A new species of Ecitonides Wasmann, 1894 (Coleoptera: Staphylinidae: Paederinae) from Peru and phylogenetic placement of the genus based on molecular data.

Ecitonides constanceae sp. nov., a new species of the myrmecophilous genus Ecitonides Wasmann, 1894, which is associated with army ants (Hymenoptera: Formicidae: Ecitoninae), is described from Peru.

Disrupting the transmembrane domain interface between PMP22 and MPZ causes peripheral neuropathy.

PMP22 and MPZ are abundant myelin membrane proteins in Schwann cells. The MPZ adhesion protein holds myelin wraps together across the intraperiod line. PMP22 is a tetraspan protein belonging to the Claudin superfamily.

An embedded intracranial seizure monitor for objective outcome measurements and rhythm identification.

Providing clinicians with objective outcomes of neuromodulation therapy is a key unmet need, especially in emerging areas such as epilepsy and mood disorders. These diseases have episodic behavior and circadian/multidien rhythm characteristics that are difficult to capture in short clinical follow-ups. This work presents preliminary validation evidence for an implantable neuromodulation system with integrated physiological event monitoring, with an initial focus on seizure tracking for epilepsy.

Deep Brain Stimulation for Children with Generalized Epilepsy.

Intracranial neuromodulation is an evolving therapy for patients with drug-resistant epilepsy (DRE). Deep brain stimulation (DBS) is now available as a therapy for patients with DRE and focal-onset seizures in select health care systems; however, there remains a substantial need of efficacy data before DBS can be more widely adopted into routine clinical practice. This review and commentary focuses on a particular shifting paradigm: DBS as a therapy for children with generalized-onset seizures.

Multiobjective Optimization of Silver-Nanowire Deposition for Flexible Transparent Conducting Electrodes.

Optimizing the spin coating of silver nanowires to form transparent conducting electrodes (TCE) is guided by machine learning (ML). A good TCE has two competing characteristics: high transmittance and high conductance. Optimization using a scalar figure of merit, as often done in the field, cannot satisfy the independent requirements for transmittance and conductance imposed by specific applications.

Beta-subunit-eliminated eHAP expression (BeHAPe) cells reveal subunit regulation of the cardiac voltage-gated sodium channel.

Voltage-gated sodium (Na) channels drive the upstroke of the action potential and are comprised of a pore-forming α-subunit and regulatory β-subunits. The β-subunits modulate the gating, trafficking, and pharmacology of the α-subunit. These functions are routinely assessed by ectopic expression in heterologous cells.

Homomeric interactions of the MPZ Ig domain and their relation to Charcot-Marie-Tooth disease.

Mutations in MPZ (myelin protein zero) can cause demyelinating early-onset Charcot-Marie-Tooth type 1B disease or later onset type 2I/J disease characterized by axonal degeneration, reflecting the diverse roles of MPZ in Schwann cells. MPZ holds apposing membranes of the myelin sheath together, with the adhesion role fulfilled by its extracellular immunoglobulin-like domain (IgMPZ), which oligomerizes. Models for how the IgMPZ might form oligomeric assemblies has been extrapolated from a protein crystal structure in which individual rat IgMPZ subunits are packed together under artificial conditions, forming three weak interfaces.

Extent of piriform cortex resection in children with temporal lobe epilepsy.

Objective: A greater extent of resection of the temporal portion of the piriform cortex (PC) has been shown to be associated with higher likelihood of seizure freedom in adults undergoing anterior temporal lobe resection (ATLR) for drug-resistant temporal lobe epilepsy (TLE). There have been no such studies in children, therefore this study aimed to investigate this association in a pediatric cohort.

Methods: A retrospective, neuroimaging cohort study of children with TLE who underwent ATLR between 2012 and 2021 was undertaken.

Epileptogenic Tubers Are Associated with Increased Kurtosis of Susceptibility Values: A Combined Quantitative Susceptibility Mapping and Stereoelectroencephalography Pilot Study.

Background And Purpose: Prior studies have found an association between calcification and the epileptogenicity of tubers in tuberous sclerosis complex. Quantitative susceptibility mapping is a novel tool sensitive to magnetic susceptibility alterations due to tissue calcification. We assessed the utility of quantitative susceptibility mapping in identifying putative epileptogenic tubers in tuberous sclerosis complex using stereoelectroencephalography data as ground truth.

Pediatric epilepsy surgery from 2000 to 2018: Changes in referral and surgical volumes, patient characteristics, genetic testing, and postsurgical outcomes.

Objective: Neurosurgery is a safe and effective form of treatment for select children with drug-resistant epilepsy. Still, there is concern that it remains underutilized, and that seizure freedom rates have not improved over time. We investigated referral and surgical practices, patient characteristics, and postoperative outcomes over the past two decades.

Endoscopic third ventriculostomy for the management of children with cerebrospinal fluid disorders, ventriculomegaly, and associated Chiari I malformation.

Objectives: Our objective was to review the outcomes of children with CIM and associated cerebrospinal fluid (CSF) disorders and ventriculomegaly undergoing endoscopic third ventriculostomy (ETV) as a primary intervention.

Materials And Methods: A retrospective, single-center, observational cohort study was conducted of consecutive children with CIM with associated CSF disorders and ventriculomegaly treated first by ETV between January 2014 and December 2020.

Results: Raised intracranial pressure symptoms were the most frequent in ten patients, followed by posterior fossa and syrinx symptoms in three cases.

Sustained year-round oceanographic measurements from Rothera Research Station, Antarctica, 1997-2017.

Oceanographic changes adjacent to Antarctica have global climatic and ecological impacts. However, this is the most challenging place in the world to obtain marine data due to its remoteness and inhospitable nature, especially in winter. Here, we present more than 2000 Conductivity-Temperature-Depth (CTD) profiles and associated water sample data collected with (almost uniquely) full year-round coverage from the British Antarctic Survey Rothera Research Station at the west Antarctic Peninsula.

Predicting seizure outcome after epilepsy surgery: Do we need more complex models, larger samples, or better data?

Objective: The accurate prediction of seizure freedom after epilepsy surgery remains challenging. We investigated if (1) training more complex models, (2) recruiting larger sample sizes, or (3) using data-driven selection of clinical predictors would improve our ability to predict postoperative seizure outcome using clinical features. We also conducted the first substantial external validation of a machine learning model trained to predict postoperative seizure outcome.

Variation in postoperative outcomes of patients with intracranial tumors: insights from a prospective international cohort study during the COVID-19 pandemic.

Background: This study assessed the international variation in surgical neuro-oncology practice and 30-day outcomes of patients who had surgery for an intracranial tumor during the COVID-19 pandemic.

Methods: We prospectively included adults aged ≥18 years who underwent surgery for a malignant or benign intracranial tumor across 55 international hospitals from 26 countries. Each participating hospital recorded cases for 3 consecutive months from the start of the pandemic.