bluuebunny/crossref_metadata_2025_split huggingface dataset:
Future approaches to seizure source localisation using optically pumped magnetoencephalography and electrical impedance tomography
Authors: Vivekananda U , Mellor S , Tierney T , Aristovich K , Barnes G , Walker M 
Published: August 2022

A third of patients with epilepsy are drug resistant, and epilepsy surgery (i.e. removal of the brain epilepsy focus) may present a powerful alternative in achieving seizure freedom. Magnetoencephalography (MEG) has been increasingly used in identifying the epilepsy focus due to its inherent properties of high spatial resolution and relative immunity to muscle artefact. However current MEG systems are cumbersome and restrictive for the patient, meaning that recording sessions are usually brief (1-2 hours). Here we demon- strate the first use of Optically Pumped Magnetoencephalography (OP-MEG) worldwide in three epilepsy patients with unrestricted head movement. We use a different type of sensor, which operates at room temperature and can be placed directly on the patient’s scalp, permitting free head movement. We show that OP-MEG could reliably identify abnormal epileptiform activity, even in cases where EEG could not, and localise this activity to the abnormal brain region.In patients who undergo intracranial EEG i.e. electrodes surgically placed within the brain, we have also demonstrated the first use of Electrical Impedance Tomography (EIT), a method for producing images of the internal electrical impedance of a subject due to nerve cell swelling. This method can produce images of seizure propagation similar to fMRI, but can be recorded continuously at any time during presurgical video-telemetry. Both methods have the potential to revolutionise how functional imaging is performed in epilepsy.
A novel neurodegenerative disease with multi-system features
Authors: Holm-Mercer Leah , Rudge Peter , Coysh Thomas , Brandner Sebastian , Jaunmuktane Zane , Ramadan Haw- raman , Ostler Jo , Smith Colin , Reisz Zita 
Published: August 2022

Inherited prion diseases (IPD) are caused by mutations in the prion protein gene (PRNP) leading to accu- mulation of misfolded prion protein (PrP). Here we describe three novelPRNPmutations.A patient with gradually progressive cognitive decline, peripheral neuropathy, diarrhoea and autonomic dysfunction, starting in the 6th decade, whose father suffered a similar illness with neuropathology showing PrP-cerebral amyloid angiopathy, PrP-amyloid plaques and abundant tau disease with co-localising TDP-43, had a truncation mutation at codon 157.A missense mutation at codon 107 ofPRNPcausing an amino acid change from threonine to isoleucine was observed in a patient with a 9-year illness starting with cognitive decline followed by gait disturbance. Neuropathology shows multi-centric PrP-amyloid plaques.Substitution of proline to serine at codon 105 has previously been reported in one patient, however our case exhibited a different phenotype: dysphasia in the absence of neurological signs, with subsequent rapid cognitive then motor decline with myoclonus. Neuropathology showed predominantly synaptic deposition of PrP and sparse cerebellar plaques.These cases highlight the remarkable heterogeneity which can be seen within IPD, and the importance of genetic screening ofPRNPin cases where there is an unexplained phenotype, particularly when there is a family history.
Multi-centre audit of myotonic dystrophy care in UK: is it the time for national recommendations?
Authors: Litesh Mohit , Campbell Paul R , Mehboob Sadaf , Vonberg Frederick , Koh Woon Senn , Kelly Phillip , Shanmugarajah Priya , Viegas Stuart , Lilleker James B , Hewamadduma Channa 
Published: August 2022

Myotonic Dystrophy Type 1 (DM1) is the most common muscular dystrophy in adults. Structured multi- disciplinary care is required to manage the multi-system involvement, to improve patient safety, and to reduce mortality and morbidity. Previous audits based on the 2018 consensus-based care recommenda- tions have been conducted at individual UK centres. In this study a standardised audit tool was created and deployed to 3 tertiary UK neuroscience centres.In total, data regarding 375 patients (197 (53%) female, mean age 47 years (range 1-84)) with DM1 were analysed. 62% had adult-onset disease. Neurology teams reviewed 64% of patients at least once a year. Key findings included: Cardiology review was performed annually for only 30%; of patients with respiratory needs, only 12% were reviewed six-monthly by specialist teams; Swallowing function was assessed in only 44% of those required; Clinical assessment for cognitive impairment was conducted in 12%.In conclusion, this multi-centre UK audit identifies several significant areas of care that need to be optimised in DM1, even within dedicated neuromuscular clinics. This highlights the need for a national initiative to establish minimum care standards. Such initiative can help drive resource allocation resulting in positive change.
007 Functional neurological disorders and sociodemographic factors: a review
Authors: Vidot Micha , Wu Kit , Mitchell James , Nitkunan Arani , Carr Georgina 
Published: August 2022

BackgroundTo improve equity of service, in line with the aspirations of public policy across the UK, it is imperative that we understand how sociodemographic factors affect the management and care of functional neurological disorders (FND).AimsTo describe the relationship between sociodemographic factors and the epidemiology, clinical factors, diagnosis, treatment, patient experience and outcomes for people with FND.MethodsWe conducted a focused review of published literature from 2000 to 2021 investigating the rela- tionship between FND and each of the patient protected characteristics, as outlined in the Equality Act 2010, with additional factors such as socioeconomic status, education and employment also included.ResultsThere is limited but some published data looking at age, disability, marriage/civil partnership, race, sex and gender, education and employment. However, minimal data is found discussing gender reassignment, pregnancy and maternity, religion and beliefs, sexual orientation, geographical variation, socioeconomic status and settlement status/immigrants.DiscussionFND is a relatively new term used to describe a condition that has had many aliases in the past, therefore leading to a lack of consistency in terminology and research methodology. Further research is needed to explore FND in relation to patient protected characteristics, and the effect on patient outcomes.
011 A case of a neurological immune-related adverse event associated with ipilimumab/nivolumab
Authors: Walker Josh , Farag Mena , Josephs Debra , Williams Victoria , Holmes Paul 
Published: August 2022

AbstractWe present an unusual movement disorder due to immune checkpoint inhibitors which responded rapidly to prednisolone.BackgroundImmune checkpoint inhibitors (ICIs) have revolutionised the scope of cancer therapy but can induce autoimmune effects on healthy organs, termed immune-related adverse events (irAEs). Their pathophysiology relies on the same mechanisms that confer anti-tumour activity and can affect any organ in the body. Neurological irAEs (n-irAEs) although rare, are potentially fatal. Differentiating n-irAEs from paraneoplastic syndromes and other neurological disorders can be challenging.CaseWe present a case of n-irAE in a patient with metastatic renal cell carcinoma treated with two cycles of ipilimumab/nivolumab. Following the second cycle, the patient developed subacute onset of postural and rest tremor, predominantly affecting her upper limbs, head and tongue, of variable amplitude, with bradykinesia of foot tapping. Her gait was broad-based. MRI brain was unremarkable. CSF was lymphocytic (WBC 103 cells, 90% lymphocytes) with elevated protein (1.30g/L), normal CSF:serum glucose ratio, negative CSF culture and viral PCR. She was treated for ICI-associated neurotoxicity and responded rapidly to high-dose steroids.ConclusionPhysicians should be aware of the diverse presentations of n-irAEs, including unusual movement disorders. Close collaboration between neurologists and oncologists is imperative in the management of these patients.
017 Neurology virtual clinic: quality improvement project
Authors: Ashjaei Shayan , Varmpompiti Kyriakoula , Alexandratou Anna , Wu Kit , Stanton Biba , Theochari Evangelia , Weeks Robert 
Published: August 2022

BackgroundNeurology patients with complex diagnoses in a busy tertiary centre can undergo multiple investigations to reach a diagnosis and are usually discharged with pending results, as tests can take a considerable amount of time to be processed (and released).AimWe set up a Virtual Clinic to follow-up patients’ results post discharge to avoid loss to follow up and improve safety of patient care.MethodsWe identified a significant proportion of neurology inpatients who were not followed-up efficiently (30.8% over 3-month-period) and addressed this issue by initiating a Virtual Clinic. The clinic was run weekly by neurology junior doctors, with protected clinic hours. The outcome of the clinic was communicated to the referring neurology consultant. We compared the follow up rates in the three months pre-clinic (October to December 2019) to the three months after setting up the clinic (January to March 2020).ResultsUsing the PDSA methodology, we successfully managed to increase the follow-up for outstanding investigations to 68.8% from 30.8% (pre-clinic setup), via the implementation of the Neurology Virtual Clinic. We re-audited for another three-month period using the same methodology. For the period September to November 2020, this percentage increased further to 73.68%.ConclusionThis new service was effective at improving test result monitoring and decreased the number of investigations that were not followed up promptly. Most importantly improved the safety of our patients’ care.
022 Preliminary results from a consultant-led acute neurology service based in the emergency department
Authors: Alim-Marvasti Ali , See Isaiah , Kandasamy Rohan , Hutchinson Joseph , Lane Charlie , Baruah Tim , Balaratnam Michelle , Chandratheva Arvind , Simister Robert , Haider Salman 
Published: August 2022

Background and aim5-10% of emergency department (ED) presentations are primarily neurological. We investigated the impact of the introduction of an acute neurology service to the ED, using the same day emergency care (SDEC) model.MethodsWe performed a retrospective review of consecutive referrals to a consultant-led service at University College London Hospital during weekday afternoons from 5th May 2021 to 20th Jan 2022.ResultsOf 664 Neurology referrals, female sex was more common than male (60% vs 35.8%, p<0.0001, Fig. 1).Most referrals were from ED Majors (30%, Fig. 2). The most common presenting complaints were headache(n=187), weakness(n=34), dizziness(n=28), and numbness(n=26). Referrers’ working diagnoses included no diagnosis (n=69), unspecified headaches(n=62), migraines(n=42), and stroke(n=23) (Fig. 3). The most common diagnoses after Neurology review were migraines(n=160), vascular events(n=21), functional(n=16), and seizures(n=12) (Fig 4).Following review, working diagnosis changed in 307(46.2%), and the following planned actions were cancelled: hospital admission in 204(30.7%); stroke referral in 190(28.6%); imaging in 45 and lumbar puncture in 33. 170(25%) cases were fully managed in SDEC which would otherwise have followed the urgent 2-week-wait pathway.ConclusionsAcute Neurology input in the ED had major impacts on working diagnoses, hospital admis- sions, urgent outpatient referrals, and emergency investigations.
027 Patient reported experience of acute neurology telephone clinics during COVID-19 pandemic
Authors: Wu Kit , Alexandratou Anna , Beyrouti Rahma , Amarasena Pubudu , King Rachel , Weeks Rob 
Published: August 2022

BackgroundThe COVID-19 pandemic has changed delivery of care worldwide where face to face clinics had to adapt to telephone consultations. Management of neurological symptoms have traditionally relied on detailed physical assessments which poses challenges for remote consultations.AimTo evaluate patient-reported experience of acute neurology telephone clinics to better understand areas for improvement of this ongoing service.MethodWe formulated a voluntary, confidential, thirteen-question survey on patient experience. The questionnaire was devised based on the previously published King’s Patient Experience Measure in Neuro- oncology Questionnaire (Ashkan et al, 2021). Four domains were measured: clinic environment, patients’ questions/queries, follow-up and feedback.ResultsOur data showed that telephone clinics can achieve a high-degree of satisfaction, efficiency in communication and engagement (100%), addressing patients’ concerns (100%) with clear clinical outcomes (100%) and over 95% of patients feeling satisfied with the care they have received. Despite this, over 35% of patients would prefer a face to face consultation. Feedback suggested patients would be open to a blended model of face to face and telephone consultations.DiscussionIncorporating telephone consultations as part of a new model of care can improve equity of access for patients, in line with the NHS Long Term Plan. It is an efficient way of bringing care closer to the patient, which would be well suited in the new Integrated Care System.
034 Revolutionary cancer treatment a pyrrhic victory?
Authors: Saeed Iman , Warner Graham 
Published: August 2022

Innovative immunotherapy in the form of Immune Checkpoint Inhibitors (ICIs) has significantly improved outcomes for patients with numerous metastatic cancers. A double-edged sword, we are increasingly learning about the immune-related adverse events (irAEs) associated with use of these novel therapeu- tic agents.Here we present the case of a 53 years old woman with background of malignant melanoma of the leg and metastatic lesions to the lung and brain. She commenced treatment with dual immunotherapy with Nivolumab/Ipilimumab with subsequent resolution of both metastatic lesions.Two years following the completion of immunotherapy, she developed left sided arm and leg weakness, hemianopia, left sided Epilepsia Partialis Continua (EPC) and cognitive decline. MRI showed diffusion res- triction along the active cortex during the period of EPC that settled as AEDs suppressed clinical seizure activity. However, while there was no tumour recurrence with interval oncology MRI, there was progressive asymmetrical atrophy of the right cerebral hemisphere with associated white matter signal abnormality in keeping with Rasmussen’s encephalitis scan changes.We propose that this represents a late delayed reaction to immunotherapy, beyond the recognised encephalitis that was the likely cause of EPC. Increasing use of ICIs will likely reveal more cases.
046 Sporadic Creutzfeldt-Jakob disease in the young: a 10 year review of United Kingdom national surveillance
Authors: Tam Johnny , Centola John , Kurudzhu Hatice , Watson Neil , MacKenzie Janet , Leitch Margaret , Green Alison , Summers David , Barria Marcelo ,   et al
Published: August 2022

IntroductionSporadic Creutzfeldt-Jakob Disease (sCJD) is the commonest human prion disease, with a median age of onset of 68 years. We aimed to characterise the clinical, investigation, and neuropatho- logical features in young individuals with sCJD using data derived from national CJD surveillance.MethodsWe interrogated the NCJDRSU database for individuals diagnosed with definite (post-mortem confirmed) or probable sCJD assessed from 2011-2021. We extracted data on clinical features, MRI, EEG, RT-QuIC, 14-3-3, and PRNP sequencing. Neuropathological findings were also studied where available. This data was compared in young individuals (≤50y) compared to all others.Results47 young individuals (4%) were identified (age at onset 25-50) from a total of 1178 cases. 14 (29.8%) were autopsy-confirmed. Psychiatric disturbance at presentation (36.2% vs 22%, p = 0.03) and longer disease duration (by 46 days, 95% CI 15-88, p < 0.01) were commoner in this group. CSF RT-QuIC showed lower sensitivity (81% vs 94%, p=0.01) in younger individuals. There was no difference in the sensi- tivity of MR brain, CSF 14-3-3, or EEG. There were no significant neuropathological differences in autopsy confirmed cases.ConclusionsYounger individuals with sCJD are more likely to present with neuropsychiatric symptoms, longer disease duration, and lower sensitivity of RT-QuIC.
054 Baseline characteristics of the Phase III GRADUATE studies: investigating subcu- taneous gantenerumab in early AD
Authors: Lane Christopher , Bullain Szofia , Thanasopoulou Angeliki , Delmar Paul , Boada Mercè , Grim-Mer Timo , Kerwin Diana , Baudler-Klein Monika , Kerchner Geoffrey A , Doody Rachelle S 
Published: August 2022

Gantenerumab is a fully human anti-amyloid beta (Aβ) monoclonal immunoglobulin G1 antibody in devel- opment for the treatment of Alzheimer’s disease (AD). Two ongoing multicentre, randomised, double-blind, placebo-controlled, Phase III studies, GRADUATE I (NCT03444870) and GRADUATE II (NCT03443973), are assessing the efficacy and safety of subcutaneous gantenerumab in early AD for 27 months.Eligible participants had prodromal AD or probable AD dementia; demonstrated abnormal memory using the Free and Cued Selective Recall Test; met criteria for the Mini-Mental State Examination (≥22) and Clinical Dementia Rating – Global Score (0.5-1); and had confirmed Aβ pathology. Participants were randomised 1:1 to receive subcutaneous gantenerumab or placebo, administered at study site or via home nursing. The study drug is titrated in three up-titration steps (120 mg, 225 mg, 510 mg monthly) over 9-months to the target 1,020 mg monthly dosage. All participants receive the same titration and target dose, regardless of apolipoprotein E β4 status.The GRADUATE primary endpoint is change from baseline to Week 116 in Clinical Dementia Rating scale – Sum of Boxes. Secondary efficacy measures, fluid and imaging biomarkers, and safety are also being assessed.Recruitment is complete. Participants were enrolled from 32 countries across 5 continents. Detailed baseline characteristics will be presented.
Parametrized black holes: scattering investigation
Authors: Magalhães Renan B. , Leite Luiz C. S. , Crispino Luís C. B. 
Published: August 2022

AbstractWe study the scattering of light-like geodesics and massless scalar waves by a static Konoplya–Zhidenko black hole, considering the case that the parametrized black hole solution contains a single deformation parameter. By performing a geodesic analysis, we compute the classical differential scattering cross section and probe the influence of the deformation parameter on null trajectories. Moreover, we investigate the propagation of a massless scalar field in the vicinity of the static Konoplya–Zhidenko black hole and use the plane waves formalism to compute the differential scattering cross section. We confront our numerical results in the backward direction with the glory approximation, finding excellent agreement. We compare the results for the deformed black hole with the Schwarzschild case, finding that the additional parameter has an important role in the behavior of the scattering process for moderate-to-high scattering angles.
Crystal-based pair production for a lepton collider positron source
Authors: Bandiera L. , Bomben L. , Camattari R. , Cavoto G. , Chaikovska I. , Chehab R. , De Salvador D. , Guidi V. , Haurylavets V. , Lutsenko E. , Mascagna V. , Mazzolari A. , Prest M. , Romagnoni M. , Ronchetti F. , Sgarbossa F. , Soldani M. , Sytov A. , Tamisari M. , Tikhomirov V. , Vallazza E. 
Published: August 2022

AbstractAn intense positron sources is a demanding element in the design of future lepton colliders. A crystal-based hybrid positron source could be an alternative to a more conventional scheme based on the electron conversion into positron in a thick amorphous target. The conceptual idea of the hybrid source is to have two separate objects, a photon radiator and a photon-to-positron converter target. In such a scheme an electron beam crosses a thin axially oriented crystal with the emission of a channeling radiation, characterized by a considerably larger amount of photons if compared to Bremsstrahlung. The net result is an increase in the number of produced positrons at the converter target. In this paper we present the results of a beam test conducted at the DESY TB 21 with 5.6 GeV electron beam and a crystalline tungsten radiator. Experimental data clearly highlight an increased production of photons and they are critically compared with the outcomes of novel method to simulate the number of radiated photons, showing a very good agreement. Strong of this, the developed simulation tool has been exploited to design a simple scheme for a positron source based on oriented crystal, demonstrating the advantages in terms of reduction of both deposited energy and the peak energy deposition density if compared to conventional sources. The presented work opens the way for a realistic and detailed design of a hybrid crystal-based positron source for future lepton colliders.
Deep ReLU neural networks overcome the curse of dimensionality for partial integrodifferential equations
Authors: Gonon Lukas , Schwab Christoph 
Published: August 2022

Deep neural networks (DNNs) with ReLU activation function are proved to be able to express viscosity solutions of linear partial integrodifferential equations (PIDEs) on state spaces of possibly high dimension d. Admissible PIDEs comprise Kolmogorov equations for high-dimensional diffusion, advection, and for pure jump Lévy processes. We prove for such PIDEs arising from a class of jump-diffusions on [Formula: see text], that for any suitable measure [Formula: see text] on [Formula: see text], there exist constants [Formula: see text] such that for every [Formula: see text] and for every [Formula: see text] the DNN [Formula: see text]-expression error of viscosity solutions of the PIDE is of size [Formula: see text] with DNN size bounded by [Formula: see text]. In particular, the constant [Formula: see text] is independent of [Formula: see text] and of [Formula: see text] and depends only on the coefficients in the PIDE and the measure used to quantify the error. This establishes that ReLU DNNs can break the curse of dimensionality (CoD for short) for viscosity solutions of linear, possibly degenerate PIDEs corresponding to suitable Markovian jump-diffusion processes. As a consequence of the employed techniques, we also obtain that expectations of a large class of path-dependent functionals of the underlying jump-diffusion processes can be expressed without the CoD.