bluuebunny/crossref_metadata_2025_split huggingface dataset:
035  Vestibular syndrome: a single centre experience of acute dizziness
Authors: Mahmud Mohammad , Prendergast Mabel , Dixon Luke , Seemungal Barry 
Published: May 2022

Dizziness accounts for 2–5% of emergency department presentations. Vestibular syndrome, one cause of dizziness, can present secondary to an acute stroke of the brainstem and cerebellar (posterior circula- tion), a life-threatening condition that has potential time dependent treatments.We conducted a retrospective case note analysis using Imperial NHS Trust patient records from January to September 2020. We looked at presentations of all presentations of vestibular syndrome requiring MRI head imaging. Retrospective ethics were obtained from local ethics committee.116 patient encounters (54% female) were included with a mean (SD) age of 62 (±18). DWI-positive stroke was confirmed in 23% of patients presenting with acute dizziness. Of these DWI-positive stroke patients, a further 23% presented with isolated vertigo. The mean NIHSS score of isolated vertigo patients was 0 and none underwent thrombolysis. Only 14% of patients had a complete HINTS examination and none were referred to the Neuro-Otology Unit. The isolated vertigo patients who were DWI-negative had no definitive diagnosis in 45% of the patient encounters.Major deficiencies exist in the care of patients presenting with dizziness, especially stroke patients. There is a need for stroke clerking to include dizziness and for doctors to be aware of stroke presenting as isolated vertigo.mabel.prendergast18@imperial.ac.uk
037  New insight into dysautonomia in GFAP astrocytopathy
Authors: Goh Yee Yen , Koay Shiwen , Goodall Jack , Oommens Grace , Ingle Gordon , Parris Victoria , Iodice Valeria , Swanton Josephine , McNamara Patricia 
Published: May 2022

A 17yo Filipino man presented with a rapid onset meningoencephalitic illness, dysautonomia (cardiovas- cular and genitourinary), neuropsychiatric, myoclonus and cerebellar symptoms. Examination revealed lower limb proprioceptive loss and areflexia.CSF showed lymphocytosis, hypoglycorrachia and glial fibrillary acidic protein (GFAP) antibodies. MRI head was normal on D4 and D11, but repeat imaging (D25) showed a reversible splenial T2 hyperintensity, which improved by D40. Neurophysiology showed a sensory neuronopathy. Autonomic function testing confirmed sympathetic cardiovascular failure. Pupillometry showed bilateral Horner’s indicating further sympathetic deficit. Dynamic sweat testing showed evidence of post-ganglionic sudomotor dysfunction.He received treatment with IV methylprednisolone followed by a prednisolone taper with good response.GFAP is an intracytoplasmic protein, found in astrocytes, unmyelinated nerve fibres as well as dorsal root ganglia, in keeping with our patient’s clinical presentation. However, literature review shows signifi- cant heterogenicity in presentation and pathogenicity of the GFAP antibody is unclear, as it could be a bystander protein.Dysautonomia is reported in 24–57% of GFAP astrocytopathy, but has not been well-characterised. We describe the first in-depth autonomic testing in GFAP astrocytopathy which shows sympathetic cardio- vascular failure and evidence of sudomotor post-ganglionic dysfunction.yeeyen.goh@nhs.net|ABN Bursary
043  A case of cerebral air embolism from giant pulmonary bulla during air travel
Authors: Matthews Rachael , Ahmed Rohan , Mohanraj Rajiv 
Published: May 2022

Cerebral air embolism following pulmonary barotrauma during flight in patients with giant pulmonary bullae is rare, with only five reported cases. We aim to highlight and better understand this unusual condition.A 47-year-old male airline passenger with a primary lung bulla experienced an inflight cerebral air embolism resulting in hypoxic brain injury. On landing, he was found unconscious and developed seizures. He was admitted to intensive care, requiring invasive ventilation. CT brain demonstrated diffuse oedema. Sub- sequent MR brain imaging demonstrated bilateral hypoxic injury, characteristic of air embolism. Chest CT revealed a giant pulmonary bulla.Air embolism occurs in patients with pulmonary bullae during flight due to pulmonary barotrauma. Pressure change during ascent causes expansion of bullae, resulting in tears in the pulmonary vasculature, with air leakage and embolisation to the arterial cerebral circulation. No British Thoracic Society air travel guide- lines extend to advice regarding risk of rupture of bulla given the rarity of this presentation.Neurological deficit in cerebral air embolism is variable, and some cases can be reversible. Our patient had modest improvement in clinical status and was discharged to a neurorehabilitation facility. Patients with known giant pulmonary bullae should be counselled on the risks associated with air travel.rachaelmatthews@doctors.org.uk
053  Intracranial pressure determines headache morbidity in idiopathic intracranial hypertension
Authors: Mollan Susan , Wakerley Ben , Alimajstorovic Zerin , Mitchell James , Ottridge Ryan , Yiangou Andreas , Thaller Mark , Grech Olivia , Brock Kristian , Sinclair Alex 
Published: May 2022

ObjectiveHeadache is the predominant disabler in idiopathic intracranial hypertension (IIH). The aim was to characterise headache and investigate the association with intracranial pressure (ICP).MethodsIIH:WT was a randomised controlled parallel group multicentre trial investigating weight manage- ment methods in IIH. Participants with active IIH (evidenced by papilloedema) and a body mass index (BMI) ≥35kg/m2 were recruited. At baseline, 12 months and 24 months headache characteristics and quality of life outcome measures were collected and lumbar puncture measurements were performed.ResultsSixty-six women were included (mean age 32.0 years (SD ± 7.8)), and mean body mass index of43.9 ± 7.0 kg/m2. The headache phenotype was migraine-like in 86%. Headache severity correlated with ICP) at baseline (r=0.285; p=0.024); change in headache severity and monthly headache days correlated with change in ICP at 12 months (r=0.454, p=0.001 and r=0.419, p=0.002 respectively). Cutaneous allodynia was significantly correlated with ICP at 12 months. (r=0.479, p<0.001). Boot strap analysis noted a positive association between ICP at 12 and 24 months and enabled prediction of change in headache severity and monthly headache days. ICP was associated with significant improvements in quality of life (SF-36).ConclusionsWe demonstrate a positive relationship between ICP and headache and cutaneous allodynia, which has not been previously reported in IIH. Those with the greatest reduction in ICP had the greatest reduction in headache frequency and severity.soozmollan@doctors.org.uk
054  Tolerability of fremanezumab in migraine patients with cardiovascular medica- tion use at baseline: a pooled analysis
Authors: Coppola Gianluca , Cohen Joshua , Ning Xiaoping , Kessler Yoel , Janka Lindsay , Riaz Sajjad , Barbanti Piero 
Published: May 2022

ObjectiveTo evaluate adverse events (AEs) in a subgroup of migraine patients with baseline cardiovas- cular (CV) medication use in this pooled analysis of three phase 3 trials of fremanezumab.MethodsThis analysis included data from the HALO EM, HALO CM, and FOCUS studies, in which patients were randomized 1:1:1 to subcutaneous quarterly or monthly fremanezumab or matched placebo over 12 weeks. AEs were evaluated for patients with baseline CV medication use.ResultsOverall, 280 of 2,842 patients (9–11% across treatment groups) in this pooled population were receiving CV medications at baseline, most commonly agents acting on the renin-angiotensin system (3–4%) and beta-blockers (3–4%). In the placebo, quarterly fremanezumab, and monthly fremanezumab groups, respectively, 53%, 66%, and 64% of patients reported at least 1 AE, and the most common AEs were injection-site-related (pain [19%, 29%, and 20%], erythema [10%, 20%, and 13%], and induration [8%, 18%, and 24%]). Cardiovascular AEs (CVAEs) were infrequent; no individual CVAE was reported for >1 patient in any treatment group. No safety signals were identified.ConclusionIn this pooled analysis, fremanezumab treatment was well tolerated over 12 weeks, with low incidences of CVAEs, comparable to placebo, in patients with migraine using CV medications at baseline.JMarzella@cellohealth.com52
062  A case of autosomal dominant optic atrophy plus syndrome
Authors: Brennan Declan , Langan Yvonne , Redmond Janice 
Published: May 2022

Autosomal dominant optic atrophy plus syndrome is a rare cause of blindness and sensorineural deafness, usually presenting with insidious onset in childhood. Other clinical manifestations are variable but include, peripheral neuropathy, myopathy, opthalmoplegia, ataxia and spastic paraparesis.We present a 50-year-old gentleman brought to clinic with a history of a slowly progressive gait difficulty. He was blind and deaf since childhood but no diagnosis had been made. He had no relevant family history. Examination revealed bilateral optic atrophy on fundoscopy. He was unable to stand unassisted and had reduced reflexes in the lower limbs. MRI brain and spine, together with routine bloods including neuropathy screen were unremarkable. Nerve conduction studies/EMG showed evidence of a large fibre sensorimotor axonal peripheral neuropathy, which was worse in the lower limbs. Given his constel- lation of symptoms, genetic testing was arranged and revealed a heterozygous pathogenic variant in OPA1 gene. This was consistent with a diagnosis of autosomal dominant optic atrophy plus syndrome.This condition is rare and often first detected in childhood. Diagnosis can be challenging due to variable clinical phenotypes and markedly variable expression of disease within families. There is a need for strong clinical suspicion for the disease in any patient with a history of blindness.declanbrennan.1@ucdconnect.ie56
076  What is the cost? An audit of investigations sent before and during lumbar puncture (LP)
Authors: Andrews Michael , Delaney Milo , Schmierer Klaus , Dobson Ruth , Marshall Charles 
Published: May 2022

BackgroundUnnecessary investigations are an important source of wasteful healthcare spending. We studied rates of inappropriate tests performed for patients having elective lumbar punctures in a large regional neurosciences centre.MethodsWe analysed 127 consecutive elective lumbar punctures during a four-month period. We audited whether pre-procedure blood tests were performed in each patient, and whether these were indicated according to ABN guidelines. We then compared the CSF tests requested at the time of procedure to the tests specified by the referring consultant.Results114/127 (90%) patients had pre-procedure blood tests for platelets and/or clotting, whereas only 5.8% of these were required according to established guidelines. The total cost of these unnecessary blood tests was £1766. 55 unnecessary CSF tests were sent in 36 patients, at a total cost of £5,233.00. The combined annual cost of unnecessary blood and CSF tests for this patient group at our centre was estimated at £15,699.ConclusionsUnnecessary blood and CSF tests are a significant source of wasteful spending in our centre. We have designed a quality improvement project comprising an educational component and redesign of electronic documentation. We will present the results of a reaudit after this intervention.mikeandrews91@hotmail.co.uk63
084  Reducing the environmental impact of junior doctors at Queen Square
Authors: Wilkins Dominic 
Published: May 2022

ObjectiveTo demonstrate that realistic, targeted interventions can reduce the negative environmental impact of the neurology SHO cohort (12 ST1/2-grade doctors) at the National Hospital for Neurology and Neurosurgery (NHNN), using QI methodology.Method: Improvement priorities were identified using UCLH Sustainability Steering Group guidance, and refined with a focus group of stakeholders (NHNN SHOs). 3 sub-projects were planned in parallel, using PDSA cycles. A SMART framework generated predictions for outcome and process measures. Data was collected via surveys at three timepoints. Results were used to plan further intervention.ResultsThe waste reduction sub-project demonstrated a 35% reduction in paper use for patient lists, saving 72 sheets a month or 8% of one tree a year.The emission reduction sub-project demonstrated a change in commute behaviour, leading to an estimated 54% reduction in commute-related emissions.The culture change sub-project demonstrated increased confidence in reducing environmental impact, with 85.7% of SHOs taking action to do so.ConclusionsThis project demonstrates the potential for meaningful benefit through scalable interven- tions. The most encouraging aspect was the engagement of the SHO cohort, representative of a gen- eration of young healthcare professionals with the potential to lead neurology and the NHS to a more sustainable future.dominicwilkins.14@ucl.ac.uk67
086  A UK multicentre audit on the use of MRI in multiple sclerosis
Authors: Allen Christopher , Fernandes Linford , Williams Thomas , Chataway Jeremy , Evangelou Nikos , Tallantyre Emma , Ford Helen ,   UK MS Registries,   Trials Group
Published: May 2022

The contemporary use of MRI in the care of 2567 persons with multiple sclerosis (MS) was audited in 25 MS centres, from all four UK nations. Variation in practice has an impact on patient care and implica- tions for service design.Routine monitoring of all MS patients accounted for 45% of MRI scans, and the mean inter-scan interval varied by centre from 12 to 32 months, a wide variation in practice. For routine monitoring scans, the detection of one new MS lesion was associated with 30% of patients subsequently changing disease- modifying treatment (DMT); this increased to 52% when ≥2 new lesions were reported.Unfortunately, only half of patients requiring progressive multifocal leukoencephalopathy surveillance were scanned at the recommended frequency.Most MRI scans are performed within ten years of diagnosis. With DMT now available for all stages of MS, this will lead to significant additional MRI utilisation. Spinal imaging was included in half of routine moni- toring scans; this had no measureable impact on DMT decision-making. Neither did it have any impact on detection of radiological dissemination in time for diagnostic scans. This suggests that its use could be safely restricted to help meet the additional capacity required.christopherallen@nottingham.ac.uk|ABN Bursary68
087  Paramagnetic rims are a specific biomarker in multiple sclerosis
Authors: Allen Christopher , Meaton Isobel , Altokhis Amjad , Clarke Margareta , Sinnecker Tim , Wuerfel Jens , Evangelou Nikos ,   MAGNIMS study group
Published: May 2022

Paramagnetic rims appear as a hypointense border around the edge of Multiple Sclerosis (MS)4lesions when visualised on phase sensitive imaging such as T2*, FLAIR* or SWI. They are likely due to immune cell uptake of liberated iron following the destruction of oligodendrocytes. While rarer than the central vein sign, initial reports suggest they are a specific marker of MS. However, disagreement remains with different groups reporting varying prevalence, location, persistence over time and association with expanding lesions, disease stage and disability.Eight centres in the MAGNIMS (Magnetic Resonance Imaging in Multiple Sclerosis) study group contrib- uted phase imaging of 562 people. They included patients with MS, clinically isolated syndrome, migraine, NMOSD, CNS vasculitis, small vessel ischaemia, diabetes mellitus, and cluster headache along with healthy controls. In patients with MS and clinically isolated syndrome 129 lesions with a paramagnetic rim were detected. The prevalence was 23% and 26% of patients respectively, suggesting this biomarker may be present across all MS stages. Only one paramagnetic rim lesion was found in the rest of the cohort, in a patient with diabetes mellitus, confirming its exceptionally high specificity of 99.7%.christopher.allen@nottingham.ac.uk|ABN Bursary
093  Improving care of multiple sclerosis-associated uveitis in a tertiary referral centre in Northern Ireland
Authors: Logan John , Ramsay Stephen , Kennedy Fiona , Hughes Stella , McDonnell Gavin , McAvoy Clara 
Published: May 2022

IntroductionMultiple sclerosis can be associated with uveitis. A cohort of patients were characterised using standard uveitis nomenclature and a series of recommendations to improve their care introduced.MethodsData was collected retrospectively for patients with consultant diagnosed multiple sclerosis from the local adult uveitis database.Results25 patients were identified according to inclusion criteria (71% female). The primary location of uveitis at presentation was 52% intermediate, 24% panuveitis, 12% posterior and 12% anterior. Subtypes of multiple sclerosis were 76% relapsing-remitting, 16% secondary progressive and 8% primary progressive. Average age of presentation to uveitis clinic was 39 (range 12–69). Average age of onset of demyelinating symptoms was 36 (range 16–68). Uveitis presented before demyelinating symptoms in approximately half (52%) of cases with a mean time difference of 7 years (range 0–29). Three patients (developed symptoms consistent with demyelination whilst on anti-TNFα agents for treatment of their uveitis (one intermediate, one posterior, one panuveitis).ConclusionsAfter collaboration between ophthalmology and neurology it was recommended to:Enquire about neurological symptoms in all patients with uveitis.Perform neurological imaging and referral where there is clinical suspicion of multiple sclerosis.Ensure no neurological symptoms have developed since first presentation of uveitis prior to commenc– ing anti-TNFα agents as a treatment for uveitis.Collaborate between departments in cases of multiple sclerosis-associated uveitis to use treatments which can be beneficial to both pathologies.johnlogan95@hotmail.co.uk
003  Systemic inflammation, erythrocyte fragility and multiple sclerosis
Authors: Stuart Charlotte M , Howard Sarah , Chouhan Joe , Varatharaj Aravinthan , Teeling Jessica L , Galea Ian 
Published: May 2022

In an unbiased proteomic assay, extracellular haemoglobin was the only serum protein which correlated with brain atrophy in secondary progressive MS. Increased erythrocyte osmotic fragility is reported in individuals with relapsing remitting multiple sclerosis (RRMS) versus control individuals and in active versus quiescent RRMS. Erythrocyte fragility has not been measured in progressive MS patients. Here we measure erythrocyte osmotic fragility in individuals with progressive MS versus control individuals. In a murine experimental model of systemic infection, we prove that systemic inflammation causes erythrocyte osmotic fragility. In summary, it is possible that extracellular hae- moglobin released during systemic infections contributes to MS pathology by entering the brain through a leaky blood-brain barrier.c.stuart@soton.ac.uk
096  COVID-19 in MS: clinically reported outcomes from the UK MS register
Authors: Middleton Rod , Craig Elaine , Rodgers William , Tuite-Dalton Katie , Garjani Afagh , Evangelou Nikos , Hunter Rachael , DasNair Roshan , Nicholas Richard 
Published: May 2022

BackgroundThe UK MS Register (UKMSR) has been capturing longitudinal clinical and patient reported outcomes (PROs) since 2011. As the UK population ‘locked-down’ in March 2020 it became important that clinicians could record hospitalised MS patients due to COVID-19 and record outcome. The UKMSR provided an electronic case return form, designed collaboratively by the community.AimImpacts of disability, age and treatment on mortality in pwMS with COVID-19MethodLinear modelling and standardised hypothesis testing were performed on an outcome of died or not, impact of disability (EDSS), disease modifying therapies and age.ResultsN=132 PCR confirmed COVID-19 patients submitted, 14 missing EDSS, leaving n=118. Female n=80, n relapsing =74, n progressive = 44, mean age 49.2. Median EDSS = 5.0. Linear regression for age was found to be most significant for outcome (p=0.002). Univariate analysis found that the outcome was not independent of EDSS (ChiSq p=0.0008), DMT (ChiSq p=0.006) and MSType (ChiSq p=0.0006). In the multivariate model only, age remained significant.ConclusionsOnly age remained as a marker of poor outcome multivariate analysis. No MS Specific characteristics were found to be significant. We would encourage continued data collection from UK neurology centres to increase the utility of this data.r.m.middleton@swansea.ac.uk73
101  The importance of the multidisciplinary team (MDT) in the management of patients with multiple sclerosis
Authors: Ramsay Stephen , Spence Robert , Robinson Rebecca , Bhattacharya Debarata , Nelson Adam , Flynn Peter , Droogan Aidan , Hughes Stella , Kennedy Fiona , McDonnell Gavin 
Published: May 2022

BackgroundClinical decision-making for patients with MS relies heavily on the expertise of radiologists. In June 2018 we developed a monthly MDT meeting with neuroradiology to review complex cases. In this study, we assess the rate and clinical significance of variance between original MRI reports and those following MDT review.MethodsA 6-month retrospective study was performed. Data focused on the frequency of scan report revisions and subsequent impact on clinical management.ResultsWe analysed 157 cases. Of these, 68% had confirmed MS whilst 32% were under investigation. Of the 157, 64% were originally reported by a general radiologist and 36% by neuroradiology. Following neuroradiology review, 63 cases (40%) had the original report amended; of these, 81% were originally authored by a non-neuroradiologist. MDT review amended 64% of original reports from general radiology. The most frequently occurring revision was identification of additional, originally unreported inflammatory lesions. Of the 63 report revisions, 29% resulted in a change in clinical management. Overall, 4% of patients with a diagnosis of MS had this diagnosis questioned or reversed following MDT review of their imaging.ConclusionsThis study demonstrates a high rate of variance in MRI reporting between general and neuroradiologists. This has clear implications for patient management, confirming the importance of dedicated MDTs involving specialist neuroradiology.sramsay03@qub.ac.uk|ABN Bursary
106  (NOT) comparing apples with apples; MS hospital episode statistics dashboard highlights variation in trusts’ reporting
Authors: Rog David , Hobart Jeremy , Clements Jake , McGregor Jamie , Mazibrada Gordon , Rajkumar Arthi , Mathews Joela 
Published: May 2022

IntroductionGIRFT utilises comparator data to assess variation in practice, including HES data. Context and accuracy underpin meaningful data analysis. Dashboards enable similar components of service delivery to be compared and benchmarked.MethodsDR, JH and JM, worked with JC, to develop a bespoke MS HES dashboard, utilising IQVIA’s HES dataset (from 2014), in Tableau, for people with MS accessing hospital care in England. Multiple filters relevant to clinical practice were created.ResultsConsistent MS-related activities were very inconsistently reported across Trusts. Some Trusts report infusion-related activities as elective day cases, others as elective inpatients, some report as both. For the 2019/2020 financial year, the MS variances listed below were compared for 4 Trusts (Barts Health, Univer- sity Hospitals Birmingham, University Hospital Plymouth, Salford Royal). Elective waiting list: 42%, 57%, 9%, 8%; Elective planned admissions: 38%, 3%, 5%, 74%; Elective Booked admissions: 0%, 6%, 52%, 3%. These probably represent systematic differences in reporting admission methods, rather than significant differ- ences in types of admissions, but clarification is required.ConclusionTrusts report the same activity differently in HES. We recommend enabling MS services to examine, critique and benchmark their clinical practice routinely. Cautious interpretation of MS outcome comparisons is required until data are consistently reported. Acknowledgement Roche Products Ltd provided funding but had no control over dashboard content, maintenance or distribution.davidrog@srft.nhs.uk78