bluuebunny/crossref_metadata_2025 huggingface dataset:
180 NeuroHealth designing a well-being app for people with neuroscience conditions using co-production principles
Authors: King Rachel , Wu Kit , Symeon Chris , Wellington Sharon , Larcombe Karen , Dilley Mike
Published: August 2022

Introduction

The Neurological Alliance Patient Experience Survey 2019/2020 has highlighted the delay to access of neurological care, infrequent monitoring by specialists and a wide regional variation in referral to support services for chronic disease management in patients with neurological diagnosis.

Aim

To design a patient-held wellbeing app to support longitudinal symptom monitoring and provide signposting of support services for patients.

Methods

The project is led by the South London Neurosciences network, co-designed with its patient advisory group, King’s Health Partners and a medical technology firm (BMI Inc). Together, we identified key attributes which were beneficial to symptom monitoring. The app went through four cycles of devel- opment, and has been launched for public use.

Results

The NeuroHealth app consists of 4 key sections: mood, sleep, diet and exercise, identified by the patient group as beneficial for daily symptom monitoring. Each section uses illustrations and can be customised to individual needs. Such longitudinal recordings can be shared with health professionals and may help improve joint decision-making.

NeuroHealth app is a co-designed and freely available digital resource which can benefit chronic condition monitoring and self-management.
186 Risdiplam through the UK early access to medicines scheme for patients with spinal muscular atrophy
Authors: Scoto Mariacristina , Galtrey Clare , Hewamadduma Channa , Keane Paul , Lilleker James B , Marini-Bettolo Chiara , Phillips Zoe , Sharp Becky , Wenham Andrew , Baranello Giovanni
Published: August 2022

Background

Risdiplam is the first oral spinal muscular atrophy (SMA) treatment and was made available in the UK through an Early Access to Medicines Scheme (EAMS), which enrolled patients between September 2020–July 2021.

Methods

Risdiplam’s EAMS indication was for patients with Type 1/2 SMA, ≥2 months old and not suitable for authorised SMA treatments. Patient characteristics and risdiplam-provision data from treating physi- cians, and patient/caregiver feedback, were collected.

Results

Of 242 patients enrolled, 92 were children (<18 years, 38.0%) and 150 were adults (62.0%). Most had Type 2 SMA (90.1%; Type 1: 9.9%). Most commonly reported comorbidities and clinical management characteristics were scoliosis (99/242; 40.9%), previous spinal surgery (82/242; 33.9%) and ventilatory support (79/242; 32.6%). Risdiplam was the first disease-modifying SMA therapy that most patients had received (155/242; 64.0%), including 7 (aged 15–33) with the more severe Type 1 form. Most patients who received risdiplam did so through sponsor-funded home delivery vs. hospital pharmacy collection (226/234; 96.6% vs. 6/234; 2.6% [not reported: 2/234]).

Conclusions

Risdiplam was made available to a varied SMA population through the EAMS, targeting patients otherwise unable to access disease-modifying treatment. Many patients received disease- modifying treatment for the first time, addressing a significant unmet need.
198 Should we thrombolyse cognitively impaired patients with acute ischaemic stroke?
Authors: Ramnarine Isabela , Rasheed Omar , Harkness Kirsty , Majid Arshad , Bell Simon
Published: August 2022

Introduction

Underlying pathological mechanisms of cerebral vessel damage in dementia patients have been suggested to increase the risk of intracranial haemorrhage following thrombolysis for acute ischaemic stroke. This has not been demonstrated in earlier literature. Our study compared the throm- bolysis outcomes of stroke patients with and without cognitive impairment.

Methods

A retrospective cohort analysis of stroke patients receiving thrombolysis was conducted. Cognitive impairment was defined as: a diagnosis of dementia, mild cognitive impairment, or clinical evidence of mild cognitive impairment. Outcome measures included haemorrhagic complications, NIHSS and mRS scores, and mortality.

Results

Statistical analysis included data from 428 ischaemic stroke patients, 62 of whom had cognitive impairment. Following thrombolysis, cognitively impaired stroke patients had greater increments in dis- ability level, an increased risk of haemorrhagic complications, and a lower likelihood of 90-day survival. Logistic regression modelling revealed the main predictors of these poorer outcomes to be age, time to thrombolysis, and NIHSS on admission. Two thirds of the variance in stroke outcomes remained unex- plained by our models.

Conclusion

Confounding variables were the main predictors of poorer thrombolysis outcomes in cogni- tively impaired stroke patients. A large portion of these poorer outcomes remains unexplained, leaving potential for further investigation.
200 Covid-19 and acute ischaemic strokes in CADASIL: a systematic review of the literature
Authors: Noui Yassine , Williams Owain , Chan Nathan , Chandra Julie , Sztriha Laszlo
Published: August 2022

Introduction

Acute ischaemic stroke (AIS) has been reported as a relatively infrequent complication of Covid-19. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the commonest inherited stroke disorder. Our aim was to determine the clinical and radio- logical features of Covid-19 associated AIS in patients with CADASIL.

Methods

We performed a systematic review of publications of patients with Covid-19 and CADASIL and included one unpublished case from two such presentations to our center.

Results

We identified a total of 6 cases; average age 46 years, all female. Mean time from Covid-19 to AIS was 9.8 days, with no severe infection. Imaging demonstrated multiple infarcts in 66%, mean number of DWI lesions 9.6, 83% sustained infarcts located in the internal borderzones. An initial diagnosis of neuro- inflammation was revised in 50% following genetics, with 83% unaware of the CADASIL diagnosis.

Conclusions

This systematic review describes the pattern of COVID-19 associated AIS in CADASIL. Such multiple infarcts, primarily affecting the internal borderzones, have been described in patients with Covid-19 without CADASIL. We discuss possible pathophysiological mechanisms, which may be extrapolated to patients with cerebral small vessel disease, including endothelial injury causing microvascular thrombosis and hypoperfusion due to cerebral blood flow dysautoregulation.
202 Frequency of inter-specialty consensus decisions and adherence to advice following a weekly neurovascular multidisciplinary meeting
Authors: Offiah C , Tierney S , Egan B , Collins DR , Ryan DJ , McCarthy AJ , Smith DR , Boyle E , Delaney H , McCabe DJH ,   on behalf of the TUH/AMNCH NV MDT Team
Published: August 2022

Background/Aims

Data are limited on the frequency of ‘consensus’ between sub-specialists attending a neurovascular multidisciplinary meeting (MDM) regarding management of patients with extracranial carotid/vertebral stenoses, and post-MDM ‘adherence’ to advice. This prospective audit/quality improve- ment project collated data at a Neurovascular/Stroke Centre.

Methods

Data from a weekly MDM were prospectively-recorded to document the proportion of extrac- ranial carotid/vertebral stenosis patients in whom ‘consensus management decisions’ were reached by Neurologists/Vascular Surgeons/Stroke Physicians-Geriatricians. Adherence to MDM advice was analysed in patients with asymptomatic, symptomatic and ‘indeterminate symptomatic status [ISS]’ stenoses, including intervals between symptom onset-MDM discussion +/- intervention.

Results

115 patients were discussed (September/2017-February/2020). Consensus regarding manage- ment was 96.5% (111/115) overall; 100% (29/29) with asymptomatic carotid stenosis [ACS], 96.5% (55/57) with symptomatic carotid stenosis [SCS], and 93.1% (27/29) with ISS. Overall adherence to MDM advice was 93% (107/115); 100% (29/29) with ACS, 89.5% (51/57) with SCS, 93.1% (27/29) with ISS. Median interval from index TIA/stroke to intervention was 12.5 days (IQR:9-18d), and MDM discussion-to-intervention was 5.5d (IQR:1-7d) in patients with 50-99% SCS.

Conclusions

High-frequency inter-specialty consensus regarding management/adherence to proposed treatment supports a collaborative, multidisciplinary model-of-care in patients with extracranial arterial stenosis. Service development should shorten intervals between symptoms-MDT discussion-intervention to optimise secondary prevention.
206 Stroke and sociodemographic factors: a review
Authors: Vidot Micha , Wu Kit , Mitchell James , Nitkunan Arani , Carr Georgina
Published: August 2022

Background

To 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 stroke.

Aims

To describe the relationship between sociodemographic factors and the epidemiology, clinical factors, diagnosis, treatment, patient experience and outcomes for people with stroke.

Methods

We conducted a focused review of published literature from 2000 to 2021 looking at stroke and each of the patient protected characteristics, as outlined in the Equality Act 2010, and the relationship to epidemiology, treatment and patient outcomes. Additional patient factors such as socioeconomic status, education and employment were also explored.

Results

There is plenty of evidence published exploring the relationship of stroke with age, pregnancy, race, sex and gender. There is a lack of evidence exploring that relationship with disability, gender reas- signment, marriage and civil partnership, religion and beliefs, sexual orientation, geographical variation, socioeconomic status, settlement status, education and employment.

Discussion

Certain areas within stroke medicine are well researched, with cause and effect relationships established. However, there are certain protected patient characteristics with minimal/no evidence which require further research to ensure equal access for all patients to stroke care.
Longitudinal clinical study of patients with white matter iron rims lesions in multiple sclerosis
Authors: Altokhis Amjad , Hibbert Aimee , Allen Christopher , Mougin Olivier , Alotaibi Abdulmajeed , Lim Su-Yin , Constantinescu Cris , Abdel-Fahim Rasha , Evangelou Nikos
Published: August 2022

Introduction

Identifying accurate prognostic imaging biomarkers in multiple sclerosis (MS) is crucial. Iron rims (IR) surrounding white matter lesions (WML) are suggested to predict a more severe disease course. Only small longitudinal cohorts of patients with and without IRL have been reported so far.

Objective

To assess if the presence and number of IRL in patients with CIS and MS are associated with worse long-term clinical disability.

Methods

Ninety-one CIS/MS patients were recruited between 2008 and 2013 and scanned with 7T MRI. Age-Related Multiple Sclerosis Score (ARMSS), a validated measure of disease severity, was calculated for patients at the time of scan and at the latest clinical follow-up after 9 years. WMLs were assessed for the presence of IRL using SWI-filtered phase images.

Results

132 IRLs were detected in 42 patients (46%). 9% of WMLs had IR. 54% of the cohort had no rims, 30% had 1-3 rims and 16% had ≥4. Patients with IRL at baseline had a higher EDSS and ARMSS. Presence of IRL was also a predictor of long-term disability especially in those patients with ≥4 IRL. The effect of IRL on disability was greater than the baseline WML numbers, another known prognostic biomarker.

Conclusion

The presence and number of perilesional IR at a baseline scan hold prognostic value for long term clinical disability in MS.
Long term outcomes of deep brain stimulation for treatment refractory chronic cluster headache and SUNHA
Authors: Cheema Sanjay , Parras Olga , Ferreira Francisca , Lagrata Susie , Hyam Jonathan , Zrinzo Ludvic , Akram Harith , Matharu Manjit
Published: August 2022

Objective

To present long term outcomes of ventral tegmental area (VTA) deep brain stimulation (DBS) for medically refractory chronic cluster headache (CCH) and short-lasting unilateral neuralgiform headache attacks (SUNHA)

Methods

Open label study using prospectively collected clinical data in consecutive patients treated by VTA DBS between 2009 and 2019 in a single tertiary neuroscience centre, treated by a multidisciplinary team.

Results

Between 2009 and 2019, 71 patients (43 CCH and 28 SUNHA) were treated with VTA DBS. Mean duration of follow up was 5.6 years (range 1.0-11.7 years).

Overall, there was a significant improvement in attack frequency, attack severity, and attack duration in both groups (all p<0.001). Twenty nine (67.4%) CCH patients and 20 (71.4%) SUNHA patients were classed as responders (a greater than 50% improvement in attack frequency).

Adverse events were rare

Four non-responders had the DBS system explanted, two of them secondary to infection and one due to post-surgical neuropathic pain around the head wound site. Four patients died from unrelated conditions during the study period.

Conclusions

This study provides open label evidence than VTA DBS is a safe and effective treatment for refractory CCH and SUNHA, an effect which is maintained at long term follow up.
004 Dermato myo myelitis: a case of concurrent anti-synthetase syndrome and NMOSD
Authors: Heller Simon , Sanna Giovanni , Williams Victoria
Published: August 2022

A 50 year old Afro-Caribbean gentleman with a background of Ro52 and MDA5 antibody positive anti- synthetase syndrome on prednisolone and mycophenolate mofetil presented with a tight band like pain around his thorax and abdominal pain. Examination identified severe dysaesthesia with a T6 spinal level, as well as lower limb and truncal weakness. MRI showed an expansile T2 hyperintense lesion from C7 to T8 with patchy enhancement in keeping with a NMOSD. He had no optic nerve involvement, confirmed by OCT. The weakness and dysaesthesia responded rapidly to high dose methylprednisolone however he developed constipation, recurrent neuropathic pain and tonic spasms. Serum AQP4 antibodies were present. At presentation serum CK was also raised.

Up to 25% of patients with NMOSD have co-existing autoimmunity. However, there are very few reports of anti-synthetase syndrome and NMOSD. Ro-antibodies are well documented in studies on Sjögren’s disease and transverse myelitis. Patients with autoimmune connective tissue diseases who develop neurological symptoms should be investigated for neurological autoantibodies too as they are at risk of multiple autoimmune disorders. Furthermore, neurologists should be observant to other systems when presented with a patient with NMOSD as they may have other diseases which are currently asymptomatic or minimally symptomatic.
013 The community neuroscience project: transforming the delivery of neuroscience care across North Central London
Authors: Balaratnam Michelle , Okin Rachel , Powell Freya , Prior Stephen , Tasnim Sabiha , Baulk Ryan , Capp Adrian , Turner Patricia , Gunn Fergus Rugg , Turner Chris
Published: August 2022

The Community Neuroscience Project has brought together clinicians from community services, secondary care and tertiary neuroscience to understand the existing neuroscience services in each region of North Central London (NCL), assess where there may be inequalities in accessing neuroscience services across boroughs, and harmonise neuroscience care across the sector.

The team are working closely with NCL CCG, exploring ways to support the existing primary care and community services. All GP practices across NCL have been given the opportunity to feedback on their experience of NCL neurological services. Neurology pathways for primary care are being developed. A neurological seminar series will be delivered for community-based clinicians.

Two virtual MDT clinics commenced in January 2022. A Community Neuroscience MDT clinic and a Community Neuroscience MDT Clinic for FND enable clinicians discuss their patients with a specialist multi-disciplinary team. These clinics aim to support community services provide care to their patients. We anticipate this will help build a cohesive neurological network across NCL, raising the profile of commu- nity-based services, and ensuring that patients receive the right care in the right place at the right time.

The Community Neuroscience Project is an innovative step in transforming the delivery of neuroscience care across NCL.
014 Medical student and consultant surveys assessing educational value of telephone and video neurology clinics
Authors: Balfour Katharine , Lavin Tim
Published: August 2022

Introduction

The COVID-19 pandemic affected medical students experience of clinical neurology. Outpatient clinics reverted to telephone or video consultations. We sought to assess the impact of this change via a survey of both students and consultant neurologists.

Methods

An online 14-part questionnaire using Likert scales and free text options was distributed to all Year 4 medical students and consultant neurologists via email.

Results

366 students received the survey and 39 responded (an 11% response rate). 41 consultants received the survey and 9 responded (a 22% response rate). 83% of consultants felt the student’s inter- action with the patient was affected by the telephone/video software. 29% of students strongly agreed and 36% agreed that technological barriers impacted their learning experience.

The free text student feedback highlighted good practice. We developed best practice guidance which included use of video consultation software, student engagement and clinic debrief. This was distributed as digestible checklists for students and clinicians. The limitations of this survey are the small response rates of both consultant and student groups.

Conclusion

Remote consultations are likely to remain part of neurological practice. Ongoing evaluation of its effect on medical student education and specific guidance for educators is required.
Tofersen in adults with SOD1-ALS: phase 3 VALOR trial and open-label extension results
Authors: Shaw Pamela , Miller Timothy , Cudkowicz Merit , Genge Angela , Sobue Gen , Nestorov Ivan , Graham Dan- ielle , Fanning Laura , Fradette Stephanie , McNeill Manjit
Published: August 2022

VALOR was a Phase 3, placebo-controlled trial to evaluate the clinical efficacy and safety of tofersen in adults with ALS and a confirmed SOD1 mutation (SOD1-ALS). Participants were randomised 2:1 to tofersen 100 mg (3 doses ~2 weeks apart, then 5 doses every 4 weeks) or placebo administered intrathecally. The primary endpoint was change from baseline to Week 28 in the ALS Functional Rating Scale-Revised (ALSFRS-R) total score. Key secondary endpoints included CSF total SOD1 concentration and other clinical outcome measures. Participants could continue in an open-label extension (OLE) upon completion of VALOR. 108 total participants were enrolled (tofersen [n=72], placebo [n=36]). VALOR did not achieve statistical significance on the ALSFRS-R at Week 28, the primary endpoint. Tofersen administration led to robust reductions in CSF total SOD1 protein and plasma neurofilament light chain (NfL) at Week 28 compared to baseline. Trends favoring tofersen were seen across clinical outcome measures of respira- tory function, muscle strength, and quality of life. These effects became more apparent with longer-term follow-up in the OLE, particularly with earlier tofersen initiation. Most adverse events were mild to moderate in severity. Serious neurologic events, including myelitis (2%), were seen in tofersen-treated participants.

Study sponsored by Biogen.
024 Croydon neurology: assessing the impact of the introduction of patient initiated follow-up
Authors: Sutton Fiona , Balakrishnan Kamal , Nitkunan Arani
Published: August 2022

NHS England identified Patient Initiated Follow Up (PIFU) as part of NHS COVID-19 recovery, in line with the personalised-care agenda. We aimed to assess how the introduction of PIFU for neurology patients at Croydon University Hospital (CUH) has impacted patient outcomes, capacity and non-elective admissions.

Data were extracted from electronic records on all patients booked into a CUH neurology PIFU appoint- ment from 1st June to 29th October 2021. Data were collected on the number of admissions or outpatient attendances for 5 years pre-PIFU appointment and compared with 0-6 months post-PIFU.

355 patients had a PIFU appointment at CUH neurology between 1st June and 29th October 2021. The most common neurological conditions prompting a PIFU appointment were epilepsy (40%), multiple sclerosis (22%) and Parkinson’s disease (12%). The average number of outpatient appointments reduced from 11.0 pre-PIFU to 1.5 post-PIFU. The average number of ED attendances reduced from 3.1 pre-PIFU to 0.4 post-PIFU.

Although there are significant limitations in this study (inadequate time since appointment, the pandemic), we remain keen on PIFU and had been running an advice line service before it was labelled as PIFU. Further follow-up data and patient satisfaction data is needed.
025 Croydon neurology: 5 years of novel service developments
Authors: Nitkunan Arani , Bagtas Creselda , Boodhoo Ajay , Grant Charmaine , Lastrilla Glenda , MacDonald Bridget , Mastrolilli Francesca , Rashid Waqar , Southam Medina , Schon Fred
Published: August 2022

5 years ago we embarked on a series of novel developments to improve our DGH neurology services. The key was ever closer integrated working between consultant neurologists and specialist nurses with support from our Trust and commissioners.

We highlight 6 very diverse ongoing projects:

1. Acute neurology ward referrals have increased by 49% between 2016-2021 with nurses triaging referrals prior to consultant review.

2. Post discharge nurse telephone review for selected patients since 2020 to try and reduce readmission rates.

3. Consultant delivered helpline for all local GPs Monday to Friday since 2020 to discuss urgent cases of concern.

4. Nurse delivered lumbar puncture service, since 2019 138 procedures carried out.

5. Diagnostic coding for all neurology outpatients started in 2021. Within 8 months all 5 consultants were coding over 70% of attendances.

6. Patient initiated follow up (PIFU) – 2020 NHS England initiative. Specialist nurse Helpline for patients with epilepsy, multiple sclerosis and Parkinson’s running successfully since 2018.

Service improvements need not be expensive or onerous but require imagination and team cohesion. We strongly recommend others to try what seems most appropriate to their service.
030 Post COVID-19 syndrome dysautonomia: a tertiary referral centre experience
Authors: Mangion Sean Apap , Oommen Grace , VIchayanrat Ekawat , Heightman Melissa , Hillman Toby , Bell Robert , Hagen Ellen Merete , Ingle Gordon , McNamara Patricia , Iodice Valeria
Published: August 2022

Post-coronavirus disease 19 (COVID-19) syndrome has substantial health and economic implications. It is multi-systemic, with prevalent autonomic symptoms. Understanding presentations and potential autonomic causes may help guide treatment strategies and recovery.

All patients with a suspected or confirmed history of COVID-19 infection who underwent autonomic testing between May 2020 and October 2021 were reviewed retrospectively.

We evaluated 62 patients (20 male, 42 female, mean age of 41.38 ±11.52). COVID-19 was PCR confirmed in 15 patients (26%), and five (8%) required acute hospital intervention. Most common symptoms included palpitations (81%), light-headedness/dizziness (62%), dyspnoea (48%), fatigue (46%), or cognitive symptoms

(33%)

Autonomic testing showed normal blood pressure responses to pressor stimuli, a mean respiratory sinus arrhythmia of 18.89b/m, and Valsalva ratio of 2.09. Postural tachycardia syndrome (PoTS) was diagnosed in 12 patients, autonomically mediated syncope (AMS) in 11, neurogenic orthostatic hypotension (NOH) in two, and initial orthostatic hypotension (IOH) in seven.

Normal supine and upright plasma noradrenaline levels were measured in 34 patients (mean 283.38 pg/ml supine; 472.43pg/ml tilted).

Autonomic testing was reassuring (PoTS and syncope) in the majority with abnormal testing (n=32, or 52%). Further phenotyping of PoTS to exclude neuropathic pathology may be needed. IOH and OH are important considerations.
032 Supine hypertension and cardiovascular autonomic failure in patients with alpha-synucleinopathies
Authors: Vichayanrat Ekawat , Koay Shiwen , Ingle Gordon , Hagen Ellen Merete , McNamara Patricia , Watson Laura , Iodice Valeria
Published: August 2022

Introduction

Orthostatic hypotension (OH) and supine hypertension (SH) are prevalent in alpha-synucle- inopathies, posing a therapeutic dilemma as OH treatment may worsen SH. We aimed to characterise SH in pure autonomic failure (PAF), multiple system atrophy (MSA), and Lewy body disorders (LBD: Parkinson’s disease and Dementia with Lewy bodies).

Methods

166 patients (72 PAF, 59 MSA, 35 LBD) underwent cardiovascular autonomic testing and 24hr- ambulatory blood pressure monitoring (24hr-ABPM). Demographic, clinical features, medications and cardiovascular autonomic biomarkers were compared.

Results

SH was present in more than half of patients with PAF, MSA and LBD (56%, 51% and 50%, respec- tively) without anti-hypotensive medications. Supine pre-stand BP during 24hr-ABPM detected SH with 60% sensitivity and 86% specificity [area under the curve 0.73 (95%CI 0.66-0.81)]. Supine noradrenaline levels were significantly higher in MSA vs PAF and LBD (268 vs 183 and 210 pg/ml, p<0.01). There was a strong correlation between OH, SH and nocturnal hypertension after adjusting for age, anti-hypotensive medications and supine noradrenaline levels (R2=0.48, p<0.01).

Conclusions

SH and nocturnal hypertension commonly co-exist and are independently associated with OH in alpha-synucleinopathies. 24hr-ABPM is useful in detecting SH in these patients. The pathophysiology of SH is likely to be heterogeneous and not entirely explained by residual sympathetic tone.
035 Brain tumours and sociodemographic factors: a review
Authors: Vidot Micha , Wu Kit , Mitchell James W , Nitkunan Arani , Carr Georgina
Published: August 2022

Background

To 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 management and care of brain tumours.

Aims

To describe the relationship between sociodemographic factors and the epidemiology, clinical factors, diagnosis, treatment, patient experience and outcomes for people with brain tumours.

Methods

We conducted a focused review of published literature from 2000 to 2021, exploring brain tumours and each of the patient protected characteristics, as outlined in the Equality Act 2010, in addition to other factors such as socioeconomic status, education and employment. Paediatric CNS tumours were excluded.

Results

There is much research exploring the characteristics age, sex and gender, and race. However, minimal data is found discussing disability, gender reassignment, marriage and civil partnership, pregnancy, religion and beliefs, sexual orientation, socioeconomic status, settlement status, education and employment.

Discussion

Incidence and prevalence of brain tumours is based upon the tumour type, with better diag- nostic imaging and testing we are also seeing an increase in diagnosis due to better detection. Some modifiable and non-modifiable risk factors have been identified, however a better understanding of all characteristics may help improve patient outcomes and access to care.
036 A GWAS of LGI1-antibody encephalitis
Authors: Binks Sophie , Elliott Kate , Sempere Vicente , Castrillo Sergio Muñiz , Ambati Aditya , Harper Andrew , Honnorat Jerome , Mignot Emmanuel , Knight Julian , Irani Sarosh
Published: August 2022

Introduction

Patients with encephalitis with antibodies to leucine-rich glioma-inactivated 1 (LGI1-Ab-E) are typically elderly males with a distinct phenotype, and ~90% carry the class II major histocompatibility (MHC) allele, DRB1*07:01. This allele is found in ~25% of healthy controls, suggesting other genetic and environmental disease factors operate in patients with LGI1-Ab-E. Yet, a previous genome-wide associa- tion study did not find variants attaining genome-wide significance outside the MHC region.

Methods

LGI1-Ab-E patients were genome-wide genotyped with standard arrays. Missing variants were imputed using Minimac4 and the Haplotype Reference Consortium panel. Population-matched controls were selected from UK Biobank. Genetic association with LGI1-Ab-E was determined with PLINK, SNPTEST and GWAMA and processed using bespoke bioinformatics pipelines.

The discovery cohort of 131 French patients (92 men; 70%) was population-matched with 2613 controls (957 men; 36.6%): >6 million SNPs remained after quality control (lambda 1.04). The validation cohort comprises 97 US/UK cases (66 men; 68%) and 1940 matched controls (882 men; 45%), >5 million variants and lambda of 1.

Results

We replicated the MHC association (rs2858869, p=3.371e-52 in the discovery cohort;rs2858870, p=1.085e-54 in the validation cohort) and will report the extent of non-MHC associations currently under- going bioinformatic assessment and validation.
038 Cerebellar cognitive affective syndrome in ataxia-telangiectasia patients
Authors: Tiet May Yung , Rittman Timothy , Horvath Rita , Hensiek Anke
Published: August 2022

Background

Cognitive function in Ataxia telangiectasia (AT) has been reported in few studies. The cer- ebellum is increasingly recognised as a pivotal structure in cognition and Cerebellar Cognitive Affective Syndrome (CCAS) has been observed in AT. Here, we report on cognitive studies in adult AT patients.

Methods

We performed cognitive assessments on an adult AT cohort and healthy volunteers. We assessed patients using the CCAS Scale, Mini Linguistic State Examination (MLSE) and the Test Your Memory (TYM) assessment. A nominated carer completed the Cambridge Behavioural Inventory Revised (CBI-R). AT patients were also assessed for severity of neurological symptoms using Scale for Assessment and Rating of Ataxia (SARA) and Inventory of Non-Ataxia Signs (INAS).

Results

Our interim analysis confirms Cerebellar Cognitive Affective Syndrome in the adult AT cohort. AT patients showed deficits primarily in tasks relying on executive function: semantic and phonemic fluency, category switching, digit span and affect. There were language deficits in motor speech and phonologi- cal errors greater than syntax or semantic errors.

Conclusions

Our large-scale cross-sectional study demonstrates the presence of cognitive dysfunction in AT affecting executive function, motor language function, and affect. We advocate routine cognitive assessment in AT patients as part of their clinical care.
039 Solriamfetol real world experience study (SURWEY): safety and effectiveness for patients with narcolepsy from Germany
Authors: Kallweit Ulf , Winter Yaroslav , Kotterba Sylvia , Benes Heike , Burghaus Lothar , Koch Andreas , Girfoglio Daniela , Setanoians Melinda , Mayer Geert
Published: August 2022

Introduction

Solriamfetol is a dopamine/norepinephrine reuptake inhibitor approved in the EU for excessive daytime sleepiness (EDS) associated with narcolepsy. This real-world study characterises outcomes following solriamfetol initiation.

Methods

SURWEY is an ongoing retrospective chart review (Germany, France, Italy). Patients (≥18 years old, EDS due to narcolepsy, stable solriamfetol dose, ≥6 weeks of treatment) were classified by solriamfe- tol initiation: changeover (from existing EDS medications), add-on (to current EDS medication), or new- to-therapy (no current/prior EDS medication). Epworth Sleepiness Scale (ESS) scores, patient/physician impressions of improvement, and adverse events (AEs) were assessed.

Results

Among 78 German patients (36.9±13.9 years old; 56% female, 57% with cataplexy), changeo- ver was most common (n=43), followed by add-on (n=19) and new-to-therapy (n=8). Final follow-up was 15.9±7.0 weeks after initiation. Overall, ESS scores were 17.6±3.1 (n=61) at initiation and 13.6±3.8 at follow-up (n=51), indicating improvement of EDS (improvements similar across subgroups). Most patients perceived slight/strong improvements in their condition (physician report, 94%; patient report, 91%; results similar across subgroups). Common AEs: headache (9%), decreased appetite (6%), insomnia (6%).

Conclusion

In this real-world cohort of German patients with narcolepsy, EDS improved across all subgroups with solriamfetol treatment. AEs were consistent with those reported in clinical trials.

Support

Jazz Pharmaceuticals.
044 At what resolution does the brain perform computations?
Authors: Potter Aneirin
Published: August 2022

Computation is the transformation of inputs into outputs through logical operations such as AND, OR, and NOT. This literature review compares models of computation at different physiological resolutions, whole brain networks, multi-cell circuits, individual synapses and individual molecular interactions and discusses if these models might be useful for bridging between functional neuroimaging with molecular models of disease. While resolution in functional neuroimaging such as EEG, MEG, PET, and fMRI is of groups of neurons pharmacotherapy alters the brain at a molecular level. Bridging this resolution gap presents many difficulties for modellers and wider connectome projects. Sufficiently detailed models can quickly outstrip computational capacity while not including sufficient detail leads to models lacking physiological validity. This is particularly problematic when connectome projects overpromise in their capacity to understand brain disorders without basis in valid physiological models. Examples of computation at network and molecular levels suggests a lack of consensus about what resolution the brain performs computations and how these computations interact. This interaction should be a goal for further research, especially given its role in linking functional neuroimaging diagnostics and pharmacological treatments in neurology.
048 Impact of the COVID-19 pandemic on Creutzfeldt-Jakob disease surveillance and patient care in the UK
Authors: Watson Neil , Kirby Jack , Kurudzhu Hatice , Leitch Margaret , MacKenzie Janet , Smith-Bathgate Blaire , Smith Colin , Summers David , Green Alison , Pal Suvankar
Published: August 2022

Background

Creutzfeldt-Jakob disease (CJD) is lethal and transmissible. We assessed the impact of the COVID-19 pandemic on UK CJD surveillance. We hypothesised: i) disruptions prolonged diagnostic latency, ii) autopsy rates declined, and iii) COVID-19 infection negatively affected diagnosis, care and survival.

Methods

We retrospectively investigated the first year of the pandemic, using the preceding year as a comparator, quantifying numbers of individuals assessed by the UK National CJD Research & Surveillance Unit (NCJDRSU) for suspected CJD, time-to-diagnosis, disease duration and autopsy rates. We evaluated the impact of COVID-19 status on diagnosis, care and survival in CJD.

Results

148 individuals were diagnosed with CJD in the pandemic (from a total of 166 individuals assessed) compared to 141 in the comparator (from 145 assessed). No differences were identified in disease duration or time-to-diagnosis. Autopsy rates were unchanged. 20 individuals had COVID-19; 60% were symptomatic and 10% had severe disease. Disruptions to diagnosis and care were frequently identified. 40% of COVID-19 positive individuals died, however COVID-19 status did not significantly alter survival duration in CJD.

Conclusions

The COVID-19 pandemic has not impacted UK CJD case ascertainment or survival but diagnostic evaluation and clinical care of individuals have been affected.
Perceived impact of equality and equity in medical education by junior doctors in the UK
Authors: Massey Lisa , Siddiqui Muhammed Rafay , Shirazi Shalini , Hayes Catherine , Graham Yitka , Vig Stella
Published: August 2022

This exploratory study was undertaken to provide an insight into issues of equality and equity that UK junior doctors perceive in relation to being able to achieve a work–life balance within educational and clinical practice. A survey with 443 junior doctors was conducted between May 2018 and September 2019. Thematic analysis of open question responses alongside correlative analyses were used to highlight issues in equity and equality faced by junior doctors. The survey revealed 77% were junior doctors in Health Education England (HEE) posts. 59% were noti n personal relationships, 60% had no children, 38% perceived the national recruitment process as helpful and 70% perceived HEE did not impact on their training. 72% had no personal barriers and 77% felt the role eas not a barrier. 1% identified no barriers. The research raised important implications for redress of equality and equity issues for all within inclusive postgraduate training in the UK.

Key messages

What is already known on this topic

What this study adds

How the study might affect research, practice and policy

Main messages

Emergent research questions
Minimal embedding of the Standard Model into intersecting D-brane configurations with a bulk leptonic U(1)
Authors: Antoniadis I. , Rondeau F.
Published: August 2022

It has been recently shown that the discrepancy between the theoretical and experimental values of the anomalous magnetic moment of the muon can be fully accommodated by considering the contribution of few Kaluza–Klein (KK) states of the gauged lepton number with masses lighter than the LEP energy, consistently with present experimental limits. In this article, we construct the minimal embedding of the Standard Model (SM) into D-brane configurations with a gauged lepton number. In order to give rise to such KK modes, the lepton number gauge boson must live on an abelian $$U(1)_L$$

U

(
1
)

L

brane extended along at least one “large” extra dimension in the bulk, with a compactification scale $$M_L\sim {\mathcal {O}}(10{-}10^2~\mathrm{GeV})$$

M
L

∼
O

(
10
-

10
2

GeV
)

for a string scale $$M_s\gtrsim 10~\mathrm{TeV}$$

M
s

≳
10

TeV

. As a consequence, $$U(1)_L$$

U

(
1
)

L

cannot participate to the hypercharge linear combination. We show that the minimal realisation of this framework contains five stacks of branes: the SM color $$U(3)_c$$

U

(
3
)

c

, weak $$U(2)_w$$

U

(
2
)

w

and abelian U(1) stacks extended effectively only in four dimensions, the bulk $$U(1)_L$$

U

(
1
)

L

, as well as a fifth $$U(1)^{'}$$

U

(
1
)

′

brane. With these five abelian factors, one finds besides the hypercharge a second anomaly-free linear combination which does not couple to the SM spectrum, both in the non-supersymmetric case as well as in the minimal supersymmetric extension of the model. It is also shown how the right-handed neutrino can be implemented in the spectrum, and how fermions arising from the two non-SM branes and coupled to the SM through the $$U(1)_L$$

U

(
1
)

L

KK modes can provide Dark Matter candidates. Finally, the possibility of breaking Lepton Flavour Universality is studied by replacing $$U(1)_L$$

U

(
1
)

L

with a brane gauging only the muonic lepton number, avoiding most experimental constraints and enlarging the parameter space for explaining the discrepancy on the muon magnetic moment.
Redshift of photons emitted from the accretion disk of a regular black hole surrounded by dark matter
Authors: Narzilloev Bakhtiyor , Abdujabbarov Ahmadjon , Hakimov Abdullo
Published: August 2022

In this paper, we have investigated the motion of photons as well as the frequency shift of photons emitted by geodesic particles influenced by the central black hole (BH) described by Bardeen solution surrounded by perfect fluid dark matter (PFDM). It has been shown that in general there are two stable photon spheres formed due to the gravitational attraction of photons by the central gravitating compact object. It has been obtained that with the increase of the space–time parameters these photon spheres come close to each other until they merge forming unique photon sphere around central BH. Investigation of the frequency shift of photons radiated by the particles geodesically moving in the equatorial plane has shown that the former becomes stronger for the bigger values of the intensity of PFDM and smaller values of the magnetic charge of the Bardeen solution.
Fermionic vacuum polarization induced by a non-Abelian vortex
Authors: Bezerra de Mello E. R. , Santana Mota H. F.
Published: August 2022

In this paper, we analyze the fermionic condensate (FC) and the vacuum expectation value (VEV) of the energy–momentum tensor associated with an iso-spin-[Formula: see text] charged massive fermionic field induced by the presence of a [Formula: see text] vortex, taking into account the effect of the conical geometry produced by this object. We consider the vortex as an idealized topological defect, i.e. very thin, straight and carrying a magnetic flux running along its core. Besides the direct coupling of the fermionic field with the iso-vector gauge field, we also admit the coupling with the scalar sector of the non-Abelian vortex system, expressed as a vector in the three-dimensional iso-space. Due to this interaction, the FC is expressed as the sum of two contributions associated with the two different effective masses for the [Formula: see text] fermionic components of the iso-spin operator, [Formula: see text]. The VEV of the energy tensor also presents a similar structure. The vacuum energy density is equal to the radial and axial stresses. As to the azimuthal one, it is expressed in terms of the radial derivative of energy density. Regarding the magnetic flux, both the FC and the VEV of the energy–momentum tensor can be positive or negative. Another interesting consequence of the interaction with the bosonic sector, the FC and VEV of the energy–momentum tensor, presents different intensity for different values of the ratio between the scalar coupling constant and the mass of the fermionic field. This is a new feature presented by the system.
Mental Workload Artificial Intelligence Assessment of Pilots’ EEG Based on Multi-Dimensional Data Fusion and LSTM with Attention Mechanism Model
Authors: Jiang Guangyi , Chen Hua , Wang Changyuan , Xue Pengxiang
Published: August 2022

EEG has been proved to be an effective tool for researchers’ cognition and mental workload by detecting the changes of brain activity potential. The mental workload of pilots in aviation flight is closely related to the characteristics of flight tasks. The previous methods have problems such as lack of objectivity, low EEG analysis ability and lack of real-time analysis ability. In order to solve these problems, this paper proposes a multi-dimensional data fusion brain workload calculation method based on flight effect evaluation, which integrates vision, operational behavior and visual gaze, and classifies and analyzes them in combination with EEG data. This method evaluates the mental workload of pilots from three aspects: visual gaze behavior, control behavior and flight effect in the simulated flight experimental environment, and realizes a more objective mental workload analysis. Then, the synchronously collected EEG data are segmented and sampled to form a dataset, and an LSTM neural network model integrating attention mechanism is established, in which the attention mechanism is used to improve the feature processing ability of the network model for the classification of complex EEG data. After machine learning training, the final model can achieve 94% detection accuracy for 2-s EEG data, and has the ability of real-time analysis in the application environment. Compared with the previous similar LSTM model, the accuracy is improved by 6%, which also shows the effectiveness of the model.
CNS: Research on Data Security Technology and Network Data Security Regulations Driven by Digital Economy
Authors: Wang Yue
Published: August 2022

In order to improve data security and network data security in the digital economy-driven environment, this paper combines data security technology and network security technology to build a digital economy security management and control system. Moreover, this paper describes the data encryption of the data owners before the framework index and the composition and construction method of the corresponding EncIR tree, and analyzes the spatial keyword group query algorithm on the EncIR tree. In addition, this paper analyzes the experimental performance of index building and spatial query, and builds an intelligent digital economy security system on the basis of these algorithms. The experimental research results verify that the data security technology and network data system driven by the digital economy have good security performance, and on this basis, follow-up security regulations can be formulated.
A three-dimensional discrete model for approximating the deformation of a viral capsid subjected to lying over a flat surface in the static and time-dependent case
Authors: Piersanti Paolo , White Kristen , Dragnea Bogdan , Temam Roger
Published: August 2022

In this paper, we present a three-dimensional discrete model governing the deformation of a viral capsid, modelled as a regular icosahedron and subjected not to cross a given flat rigid surface on which it initially lies in correspondence of one vertex only. First, we set up the model in the form of a set of variational inequalities posed over a non-empty, closed and convex subset of a suitable space. Second, we show the existence and uniqueness of the solution for the proposed model. Third, we numerically test this model and we observe that the outputs of the numerical experiments comply with physics. Finally, we establish the existence of solutions for the corresponding time-dependent version of the obstacle problem under consideration.
Effective Adjustment of Crystallinity and Regular Anti-Corrosion Activity of Zinc Tungstate Pigments
Authors: Yuan Xiao-Yu , Guo Xiao-Jiao , Zhao Si-Rui , Lu Yi , Liu Jin-Ku , Tang Sheng , Gao Wen-Xiu , Hu Hua-Rong
Published: August 2022

It is important to reduce costs and improve performance by microstructure-control of materials. The direct precipitation method and hydrothermal method were adopted to adjust the crystallinity of zinc tungstate (ZnWO4) pigments. Correlation between the crystallinity of ZnWO4 and its anti-corrosion performance is explored. The results confirm that ZnWO4 crystals prepared by hydrothermal reaction for 12 h have high crystallinity, and the corrosion resistance is enhanced by 737.0% compared with blank coating in the epoxy resin system. The improvement in corrosion inhibition is due to the enhancement of the shielding effect of the multilayer passivation film and electronic suppression effect resulted from the high ZnWO4 crystallinity. The low ZnWO4 crystallinity can weaken the corrosion resistance because of the defect-enriched effect. Advancement in anticorrosive performance by crystallinity-control meets the reduction of costs and energy consumption in the coatings industry.
Competitive and exclusion and oscillation phenomena of an eco-epidemiological model: An invasive approach
Authors: Li Ke-Lu , Yang Jun-Yuan , Li Xue-Zhi
Published: August 2022

Eco-epidemiological model plays a crucial role in maintaining ecological balance and biological invasions. In this paper, we propose a predator–prey model where the predator population is infected by the disease. The non-negativity and boundedness of the solution are addressed. The existence and stability of equilibrium are discussed by an invasive approach. Numerical simulations are used to demonstrate the competitive and exclusion, and oscillation behaviors of the model. Interestingly, the predation rate of the infected predator is shown to have far-reaching implications for the structure and stability of ecological communities.