bluuebunny/crossref_metadata_2025_split huggingface dataset:
001  Machine learning accurately determines the population prevalence of Alzheimer’s disease based on microbiome profile
Authors: Basgaran Amedra , Lymberopoulos Eva , Reis-Dehabadi Maryam , Sharma Nikhil 
Published: May 2022

The human microbiome is a complex and dynamic community of microbes, thought to have symbiotic benefit to its host. Influences of the gut microbiome on brain microglia have been identified as a potential mechanism contributing to neurodegenerative diseases such as Alzheimer’s disease (AD), motor neurone disease and Parkinson’s disease. We hypothesise that machine learning applied to the population level gut microbiome will predict the prevalence of AD.Analyses were performed in R, using two large, open-access microbiome datasets (n=1006 & n >2000) (Lahti et al., 2014; Pasolli et al., 2017). Countries in these datasets were grouped based on AD prevalence and the microbiome profiles compared.In countries with a high prevalence of AD, there is a significantly lower diversity of the gut microbiome (p<0.001). A PERMANOVA (p<0.05) revealed significant differences between several taxa in countries with high vs low prevalence of AD. Additionally, using machine learning, we were able to predict the preva- lence of AD within a country based on the microbiome profile (Mean AUC 0.935 & 0.870).We conclude that differences in the microbiome can predict the varying prevalence of Alzheimer’s disease between countries. Our results support a key role of the gut microbiome in neurodegeneration at a population level.a.basgaran@nhs.net
009  Language decline following white matter tract damage during anterior temporal lobe resection in language dominant hemisphere
Authors: Binding Lawrence , Taylor Peter , Thompson Pamela , Baxendale Sallie , Tisi Jane de , McEvoy Andrew , Miserocchi Anna , Winston Gavin , Duncan John , Vos Sjoerd B 
Published: May 2022

Anterior temporal lobe resection (ATLR) for temporal lobe epilepsy (TLE) has remission rates up to 80%, however is underutilised, in part because of the risk of language decline following surgery. Language decline occurs in up to 50% of ATLR in the language dominant side. This can occur despite careful planning with functional MRI (fMRI) language mapping. Several white matter bundles are close to the resection area which could contribute to language decline. Diffusion MRI-based fibre tracking to map the arcuate, uncinate, inferior fronto-occipital, inferior, and middle longitudinal fasciculus was performed on 43 patients. We extracted the left-sided bundles in those temporal lobe epilepsy patients who had left-dominant language based on verbal fluency functional MRI (fMRI) and a left-sided resection. Resection masks were manually drawn and used as exclusion regions. Changes from pre- to post-operative tractography and language ability were measured as percentages. Linear regression revealed that the McKenna Graded Naming test decline was predicted by the arcuate and middle longitudinal fasciculus resection (F(2,41)=5.562, p=0.007) with an adjusted R2 of 0.175. These findings demonstrate that damage to anterior arcuate extensions and the middle longitudinal fasciculus affects picture naming ability.lawrence.binding.19@ucl.ac.uk
016  Long-term efficacy and safety of perampanel in elderly patients from phase III open-label extension studies
Authors: Marawar Rohit , Leppik Ilo E , Wechsler Robert T , Patten Anna , Ngo Leock Y , Malhotra Manoj 
Published: May 2022

BackgroundThe long-term efficacy and safety of adjunctive perampanel was evaluated in patients aged≥60 years with focal-onset seizures (FOS) with/without focal to bilateral tonic-clonic seizures (FBTCS) who participated in open-label extension Studies 307 (NCT00735397) and 335 (NCT01618695).MethodsPatients received perampanel 2–12 mg/day during Studies 307 (16-week blinded Conversion; 256-week Maintenance) and 335 (4-week Pre-conversion; 6-week Conversion; ≥46-week Maintenance). Assessments included median per cent reduction in seizure frequency/28 days vs pre-perampanel baseline, 50% responder rates and treatment-emergent adverse events (TEAEs).ResultsThe Safety Analysis Set included 71 patients (mean age, 64.0 years). Seizure frequency reductions during Years 1 and 2 were 31.7% (n=71) and 44.5% (n=38) (total FOS), and 95.5% (n=19) and 100.0% (n=9) (FBTCS), respectively. Seizure frequency reductions were observed during Years 3/4 (low patient numbers); over one-third of patients achieved a ≥50% seizure reduction each year. During Years 1, 2, 3 and 4, TEAE incidence was 87.3% (n=62/71), 60.4% (n=29/48), 47.4% (n=9/19) and 57.1% (n=8/14), respectively; most common were dizziness and fall during Years 1/2 and 3/4, respectively.ConclusionPerampanel was associated with reductions in seizure frequency over 4 years in elderly patients. The safety profile was consistent with the overall populations.FundingEisai Inc. stella_ngo@eisai.com33
017  PROVE study 506: perampanel in real-world clinical care of patients based on seizure type
Authors: Wheless James , Patten Anna , Ngo Leock Y , Salah Alejandro , Malhotra Manoj 
Published: May 2022

BackgroundPROVE (NCT03208660) was a retrospective, non-interventional Phase IV study of perampanel during real-world clinical care of patients with epilepsy; we report the efficacy and safety of perampanel in PROVE.MethodsData from patients initiating perampanel after 01-Jan-2014 were included. Endpoints included retention rate at 3/6/12/18/24 months (primary), efficacy and safety (secondary). Outcomes were strati- fied by baseline seizure type: focal-onset seizures (FOS) only; generalised seizures only; generalised tonic- clonic seizures (GTCS) only; myoclonic; absence. Patients with FOS and generalised seizures (n=718) were excluded.ResultsIn the Safety Analysis Set (n=1703), 24-month retention rates were: 48.1% (FOS only, n=164/341); 47.8% (generalised only, n=120/251); 42.9% (GTCS only, n=27/63); 50.3% (myoclonic, n=100/199); 47.7% (absence, n=84/176). Of 51 patients with seizure data at Months 22–24, median reductions in total seizure frequency/28 days were: 75.0% (FOS only, n=20); 91.6% (generalised only, n=10); 40.0% (GTCS only, n=3); 70.0% (myoclonic, n=7); 100.0% (absence, n=4). Treatment-emergent adverse events occurred in 231/545 (42.4%; FOS only), 185/440 (42.0%; generalised only), 50/110 (45.5%; GTCS only), 117/328 (35.7%; myoclonic) and 154/301 (51.2%; absence) patients; most common were dizziness and aggression.ConclusionPerampanel retention rates and safety were generally similar across seizure types. Seizure frequency reductions were observed across subgroups.Funding.Eisai Inc. stella_ngo@eisai.com
019  Reduced activations within right mesial temporal lobe for visual episodic memory in right temporal lobe epilepsy
Authors: Ramaswamy Arjun , Sablik Maria , Duncan John , Buck Sarah , Sidhu Meneka 
Published: May 2022

Rationale: Epilepsy surgery is an effective treatment for Temporal lobe epilepsy (TLE), however memory decline occurs in up to 40% of people. Functional MRI (fMRI) can be used to determine the pre-operative functional anatomy of memory representations to guide surgical decision making and reduce the risk of memory decline.Methods22 healthy controls, 8 patients with right TLE (RTLE) and 10 with left TLE (LTLE), performed an overt, word and faces memory-encoding, recognition and recall fMRI paradigm with a baseline of odd-even number decisions. Statistical Parametric Mapping (SPM) was used to investigate specific brain regions associated with encoding and retrieval processes contrasted with the baseline task. One sample and two sample t-Tests were performed between controls and patient groups. Group activations are reported at p<0.001 for extratemporal and p<0.05 for mesial temporal lobe (MTL) activations.ResultsA more specific memory fMRI paradigm showed distinct activations for verbal and visual memory among controls, LTLE and RTLE groups. Particularly for visual memory, the paradigm elicited greater acti- vations in controls within the right anterior MTL and right posterior MTL for face encoding and recognition respectively. These regions have been widely associated with strong encoding and retrieval of visual memory. The reduced activations in RTLE within these regions are indicative of potential visual memory decline after surgery.arjun.ramaswamy.19@ucl.ac.uk
021  Tics in patients with encephalitis
Authors: Badenoch James , Searle Tamara , Watson Iona , Cavanna Andrea 
Published: May 2022

Movement disorders have been described in the context of different types of encephalitis. Among hyperkinetic manifestations, tics have sporadically been reported in cases of encephalitis resulting from a range of aetiologies. This review aimed to assess the prevalence and characteristics of tics in patients with encephalitis. We conducted a systematic literature review of original studies on the major scientific databases, according to the standards outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In addition to the established association between tics and encephalitis lethargica, our literature search identified reports of tics in patients with immune-mediated pathologies (including autoimmune encephalitides affecting the N-methyl-d-aspartate receptor, voltage gated potassium channels, and glycine receptors) and infective processes (ranging from relatively common viral pathogens, such as herpes simplex, to prions, as in Creutzfeldt-Jakob disease). Tics were most commonly reported in the post-encephalitic period and involvement of the basal ganglia was frequently observed. The association of new-onset tics and encephalitis, in the background of other neuropsychi- atric abnormalities, has practical implications, potentially improving the detection of encephalitis based on clinical features. Future research should focus on the categorisation and treatment of hyperkinetic movement disorders associated with encephalitis.jxb752@student.bham.ac.uk
Bad altitude
Authors: Kalam Sabrina , Davenport Richard 
Published: May 2022

A previously well 15 year old girl presented with recurrent facial weakness associated with air travel. The first event occurred the year before. Mid-flight she developed right facial numbness and weakness with auricular pain resolving within 30 minutes. She remained well and multiple subsequent flights were uneventful. She had an identical episode a year later on the same route, although had flown without incident several times in between. In clinic her neurological examination was unremarkable. Structural imaging was normal and we diagnosed recurrent barotrauma induced transient right facial nerve palsy.Only 12 cases of facial baroparesis associated with air travel have been reported. These describe transient facial paresis at high altitude with resolution of symptoms within several hours of onset. This phenomenon was previously described as a rare occurrence in divers. The suggested aetiology in both is increased pressure on the tympanic segment of facial nerve within the facial canal, leading to temporary pressure- induced ischaemic neuropraxia. Equalisation of middle-ear pressure relieves symptoms. Most cases require only reassurance although two reported cases underwent surgical management. This case highlights a rare phenomenon that could be mistaken for more sinister disease and is perhaps another (if less com- pelling) reason to reduce air travel…sabrina_k2005@hotmail.co.uk
041  Posterior reversible encephalopathy syndrome (PRES) in mild and severe COVID-19
Authors: Hayton Philip , Hamdalla Hisham 
Published: May 2022

As the COVID-19 pandemic progresses neurological complications are increasingly being reported. Posterior reversible encephalopathic syndrome (PRES) is a clinico-radiological syndrome characterised by headache, visual loss, encephalopathy and seizures, and the development of vasogenic white matter lesions in a classically parieto-occipital distribution. The pathophysiology of PRES is incompletely understood, but both hyperperfusion secondary to hypertension, and endothelial dysfunction leading to vasogenic oedema have been implicated. Here we present a case series of 2 hospitalised COVID-19 patients with markedly different disease severity, both of whom developed PRES. Patient 1 presented with confusion and headache without significant systemic features, on a background of known hypertension. Patient 1 had a single generalised seizure and was managed with levetiracetam and antihypertensives, and showed complete clinical recovery. In contrast, patient 2 presented with respiratory distress, metabolic disturbance and encephalopathy requiring critical care admission. Patient 2 had a protracted admission, developing marked visual disturbance and generalised seizures requiring multiple agents. In both cases initial CT/MRI showed characteristic posterior PRES-like leukoencephalopathy with resolution on follow-up imaging, and CSF biochemistry, cytology and virology were normal. This case series highlights the potential for neurological complications in COVID-19 patients across the spectrum of disease severity.Philip.hayton@nhs.net
058  Tolosa-Hunt syndrome with features of trigeminal autonomic cephalagia, pituitary enlargement, and non-lateralised headache
Authors: Sim Nicholas Keyi , Liew Sze Yii , Warren Daniel , Hassan Ahamad 
Published: May 2022

We present a case of Tolosa-Hunt syndrome with a headache phenotype mimicking a right-sided trigemi- nal autonomic cephalagia (hemicrania continua), and serial MRI imaging showing a stable enlarged pituitary. She was diagnosed initially with common migraine, revised to hemicrania continua, then chronic daily headache, before finally arriving at her diagnosis. She tried multiple anti-migraine therapies and her best response was to indomethacin, discontinued due to gastrointestinal side effects. Her diagnosis was achieved after her headache changed to a left sided headache with a left sided cavernous sinus syndrome, 4 years into her disease course, despite her serial surveillance scans. She responded well to steroids.This case demonstrates Tolosa-Hunt syndrome may not be side-locked, and the need to consider inflam- matory causes of headache when faced with a patient with persistent headaches and ‘incidental’ pituitary adenoma.nchlasim@doctors.org.uk54
063  Improving service provision for patients with suspected idiopathic intracranial hypertension in a joint neuro-ophthalmology clinic
Authors: Dokal Kitt , O’hanlon Ciaran , Castelhano Rute , Yiin Gabriel , Hinze Stephan , Pandit Jyotin , Morrish Paul , Paul Nicola 
Published: May 2022

IntroductionCare of patients with suspected Idiopathic Intracranial Hypertension (IIH) involves neurolo- gists and ophthalmologists, however this can be difficult to co-ordinate. Consensus guidelines suggest all patients with suspected IIH should have weight, visual fields, neuroimaging and a CSF opening pressure. The aim of our study was to determine adherence to consensus guidelines.MethodsWe assessed medical notes of consecutive patients attending the new joint neuro-ophthalmol- ogy clinic in the Great Western Hospital, Swindon between 1st September–31st December 2020. Due to the Covid-19 pandemic a neurologist was not always present.ResultsOf 38 patients attending the clinic with suspected IIH, 35(92%) were female. 29(76%) had weight recorded; 14(37%) patients had BMI recorded, 12(32%) had BP recorded; 36(95%)had visual fields recorded, 12(32%) and 17(45%) respectively had whether neuroimaging or lumbar puncture were required. 23(61%) had weight management discussed, 22(58%) had a medication review discussed.ConclusionDocumentation of weight, BMI, BP, investigation results and management discussions could be improved in the joint neuro-ophthalmology clinic. We aim to address this by introducing an “IIH clinic checklist” for use by clinicians and patients to help improve adherence to consensus guidelines.kitt.dokal@kcl.ac.uk
075  Secondary syphilis presenting as acute unilateral visual loss in an immunocompetent elderly patient
Authors: Paluri Srinivasa , Arun Tarunya , Prasadh Balaji 
Published: May 2022

A 75-year-old lady with a history of hypertension, hypothyroidism and posterior vitreal detachment was admitted with acute reduction of vision and floaters in the left eye.Fundoscopy revealed optic disc swelling left eye and visual acuity was reduced to finger counting. MRI head and orbits showed micro angiopathic changes only with no optic nerve lesion. A CT Thorax, abdomen and pelvis did not show any abnormalities. Bloods investigations including ESR, CRP ANA, ANCA, dsDNA, ACE, aquaporin and MOG antibodies and serum electrophoresis were normal. Cardiolipin anti- bodies were raised at 101.0 (<10.1). CSF results were bland. Serological investigations revealed a positive RPR, 1:256, we performed a TPPA test to confirm the diagnosis. Fluorescein fundus angiography showed “Leopard spots” suggestive of posterior placoid chorioretinopathy. Subsequent sexual history confirmed recent contact with a new partner. She was treated with doxycycline due to severe penicillin allergy. Visual acuity on the left improved to 6/7.5 in two months’ time.We report an infrequent case of unilateral optic neuritis as a presenting manifestation of syphilis in an immunocompetent elderly patient. An infectious cause of optic neuropathy needs to be considered at all age groups and it is often completely reversible if intervention is timely.s.paluri@nhs.net
080  Listen to learn: podcast-series for IMT induction at National Hospital for Neurology and Neurosurgery
Authors: Iqbal Sabahat , Levee Viva , Goh Yee Yen , Parmar Sunjay , Carr Aisling 
Published: May 2022

BackgroundPodcasts are increasingly used as a learning resource in medicine. However, evidence illustrating their impact is limited.ObjectiveTo assess use and utility of podcasts in IMT induction/introduction to specialist neurology services in NHNN.Method: A survey of the 2019/20 IMT cohort revealed 85.7% to be ‘not confident’ in the management of patients with neurological disorders at induction. The same proportion had no previous neurology clinical experience. Podcast format, topics and content were derived from this feedback.We developed 5 open-ended questions on 10 topics (neuropathy, PLEX, DBS, seizure management, IVIg, CSF analysis, MG, GBS, myelopathy and demyelination) and recorded live Q&A sessions with specialist consultants and nurses after approval by UCLH comms team. The podcasts were launched 1 week pre- induction August 2020 on the NHNN education website via a link sent to all new starters. The website is accessible by IMT and specialist trainees after registration with a nhs.net email.A repeat survey was performed 1month post-induction and download data accessed from the website in January 2021.Results/Conclusion85.71% felt ‘confident’ in the management of neurological conditions. The podcasts have been played 560 times in 6 months, 453 times outside London suggesting transferable utility beyond NHNN.siqbal2482@gmail.com|ABN Bursary65
094  Measuring neutralising antibodies (NAbs) to interferon-beta (IFNB) for multiple sclerosis (MS): a neglected practice?
Authors: McKee JD , Spence RW , Hughes S , Gray O , Campbell J , Droogan A , McDonnell GV 
Published: May 2022

ObjectiveTo determine frequency of testing for NAbs to IFNB in our MS population and adherence to international (EFNS) guidelines.BackgroundIFNB was the first approved MS disease modifying therapy (DMT). NAbs diminish efficacy regarding relapse rate, lesion load and disease progression.MethodsIn August 2019, the Northern Ireland (NI) DMT database was interrogated for patients currently or previously receiving IFNB. The NI Electronic Care Record and regional laboratory database were reviewed to verify if NAb testing ever undertaken, results, outcomes of positive results and reasons for treatment cessation.Results488 patients were currently on IFNB, 21.6% of the DMT population (IFNB1a intramuscularly - 210, pegylated IFNB1a - 71, IFNB1a subcutaneously - 175, IFNB1b subcutaneously - 32). Overall, 20.1% had NAbs checked (11.2% positive). Additionally, 288 patients had ceased treatment in the past 13 years, 273 having available records, 32 (11.7%) with NAb testing including 7 (21.9%) testing positive. 62/273 patients had discontinued due to relapse or disease progression - only 9 (14.5%) of these had ever had NAbs checked.ConclusionsThese data suggest that guidelines are poorly observed in this population. Improved testing could identify potential treatment failures earlier, avoiding adverse outcomes and facilitating more effective DMT decision making.jonmckee@doctors.org.uk72
095  Remyelination in people with MS due to an RXR agonist is age-dependent
Authors: McMurran Christopher , Mukherjee Trisha , William Brown J , Michell Andrew , Coles Alasdair , Cunniffe Nick 
Published: May 2022

Remyelination is a promising strategy to prevent axonal degeneration and progressive disability in people with multiple sclerosis (MS). In animal models, remyelination becomes inefficient with advancing age and much preclinical research is focused on interventions to reverse cellular hallmarks of ageing in remyelinat- ing lesions. However, there is currently limited evidence that human remyelination also declines with age.We investigated the effect of patient age on treatment response among participants of the CCMR One trial (ISRCTN14265371): a double-blind, placebo-controlled phase 2a study (n=52) that demonstrated the ability of bexarotene, a retinoid-X receptor agonist, to promote remyelination in people aged 25–50 with relapsing remitting MS. For eyes with chronic optic neuropathy (baseline latency >118ms), bexarotene shortened the full-field visual-evoked potential P100 latency maximally in younger patients. The treatment effect diminished by approximately 0.5ms per year, such that older patients receiving bexarotene had a similar P100 latency change to controls. Furthermore, MRI scans of the same patients demonstrated an age-dependent treatment effect on lesion magnetisation transfer ratio, a radiological correlate of remyelination.These results provide evidence that bexarotene promotes remyelination best in younger patients, rein- forcing the need to address the age-associated decline in remyelination capacity to develop successful remyelinating therapies.cem73@cam.ac.uk|NIHR Bursary
099  Safety and effectiveness of dimethyl fumarate in multiple sclerosis patients treated over 5 years
Authors: Giles Kathryn , Balashov Konstatin , Macdonell Richard , Windsheimer Joerg , Okai Annette , Martinez Mikel , Mokliatchouk Oksana , Lyons Jennifer , Kapadia Shivani 
Published: May 2022

IntroductionIn clinical studies, delayed-release dimethyl fumarate (DMF) demonstrated a favorable benefit-risk profile in patients with relapsing-remitting multiple sclerosis (MS). Real-world studies enable char- acterization of risks that may emerge with long-term exposure in clinical practice. ESTEEM (NCT02047097) is an ongoing 5-year study characterizing real-world long-term safety and effectiveness of routinely pre- scribed DMF in MS patients.MethodsPatients treated with DMF were recruited from ~380 sites. The primary objective was to determine incidence, type, and pattern of serious adverse events (SAEs), and AEs leading to DMF discontinuation.ResultsAs of April 3, 2019, 5804 patients had ≥1 dose of DMF. SAEs were experienced by 245 (4.8%) patients, with infections (n=64; 1.3%) and nervous system disorders (n=35; <1%) the most common. There were 1676 (33.0%) permanent treatment discontinuations. Annualized relapse rate over the period of up to 5 years was significantly lower than in the year prior to baseline (risk reduction 88.6% [95% confidence interval: 87.7–89.4]; P<0.0001).ConclusionsThese results reveal low risk of SAE and beneficial therapeutic effects over up to 5 years of real-world DMF use. Updated safety and efficacy results for DMF patients in the United Kingdom will be presented.Support.Biogen. Disclosures: Included on the poster. Ben.newth@biogen.com