bluuebunny/crossref_metadata_2025_split huggingface dataset:
Active surveillance of 2017 seasonal influenza vaccine safety: an observational cohort study of individuals aged 6 months and older in Australia
Authors: Pillsbury Alexis J , Glover Catherine , Jacoby Peter , Quinn Helen E , Fathima Parveen , Cashman Patrick , Leeb Alan , Blyth Christopher C , Gold Michael S , Snelling Thomas , Macartney Kristine K 
Published: October 2018

ObjectiveTo actively solicit adverse events experienced in the days following immunisation with quadrivalent inactivated influenza vaccine using Australia’s near real-time, participant-based vaccine safety surveillance system, AusVaxSafety.Design and settingObservational cohort study conducted in 194 sentinel surveillance immunisation sites (primary care, hospital and community-based clinics) across Australia.ParticipantsIndividuals aged ≥6 months who received a routine seasonal influenza vaccine at a participating site (n=102 911) and responded to a survey (via short message service or email) sent 3 days after vaccination about adverse events experienced (n=73 892; 71.8%).Main outcome measureNear real-time and cumulative participant-reported rates of any adverse event, fever or medical attendance experienced within 3 days after vaccination overall, by brand, age, pregnancy status and concomitant vaccine receipt.ResultsParticipant median age was 57 years (range: 6 months to 102 years); 58.1% (n=42 869) were female and 2.7% (n=2018) were pregnant. Near real-time fast initial response cumulative summation and Bayesian analyses of weekly event rates did not demonstrate a safety signal. Children aged 6 months to 4 years had higher event rates (522/6180; 8.4%) compared with older ages; participants aged ≥65 years reported fewer events (1695/28 154; 6.0%). There were no clinically significant differences in safety between brands, by age group or overall. Cumulative data analysis demonstrated that concomitant vaccination was associated with increased rates of fever (2.1% vs 0.8%) and medical attendance (0.8% vs 0.4%), although all rates were low and did not exceed expected levels.ConclusionsNovel, postmarketing AusVaxSafety surveillance demonstrated comparable and expected safety outcomes for the 2017 quadrivalent inactivated influenza vaccine brands used in Australia. These near real-time, participant-reported data are expected to encourage confidence in vaccine safety and promote uptake.
281 Predicting neurodegeneration after traumatic brain injury
Authors: SN Graham Neil , E Jolly Amy , J Bourke Niall , Gregory Scott , H Cole James , J Sharp David 
Published: October 2018

BackgroundDementia rates are elevated after traumatic brain injury (TBI) and a subgroup develops chronic traumatic encephalopathy. Post-traumatic neurodegeneration can be measured by brain atrophy rates derived from neuroimaging, but it is unclear how atrophy relates to the initial pattern of injury.ObjectivesTo investigate the relationship between baseline TBI patterns and subsequent neurodegeneration measured by progressive brain atrophy.Methods55 patients after moderate-severe TBI (mean 3 years post-injury) and 20 controls underwent longitudinal MRI. Brain atrophy was quantified using the Jacobian determinant defined from volumetric T1 scans approximately one year apart. Diffuse axonal injury was measured using diffusion tensor imaging and focal injuries defined from T1 and FLAIR. Neuropsychological assessment was performed.ResultsAbnormal progressive brain atrophy was seen after TBI (~1.8%/year in white matter). This was accompanied by widespread reductions in fractional anisotropy, in keeping with the presence of diffuse axonal injury. There was a strong negative correlation between FA and brain atrophy, whereby areas of greater white matter damage showed greater atrophy over time.ConclusionsThe results show a strong relationship between the location of diffuse axonal injury and subsequent neurodegeneration. This suggests that TBI triggers progressive neurodegeneration through the long-lasting effects of diffuse axonal injury.
283 Quantifying muscle amyloid in inclusion body myositis using pet
Authors: B Lilleker James , Richard Hodgson , E Roberts Mark , Karl Herholz , James Howard , Rainer Hinz , Hector Chinoy 
Published: October 2018

ObjectivesInclusion body myositis (IBM) shares some histopathological features with polymyositis (PM). Current investigations have low sensitivity for identification of amyloid deposits that are characteristic of IBM, contributing to frequent misdiagnosis. We compared muscle amyloid content, quantified using a novel positron emission tomography (PET) technique, in IBM and PM.MethodsTen cases with IBM and six controls with PM underwent clinical review, [18F]florbetapir PET/computed tomography, and magnetic resonance imaging (MRI) of whole-body skeletal musculature. [18F]florbetapir standardised uptake value ratios (SUVRs, reference=lumbar fat pad) in skeletal muscle were compared between cases and controls. The relationship in IBM of [18F]florbetapir SUVRs to clinical and MRI-derived measures of disease severity were also investigated.Results[18F]florbetapir SUVRs were significantly higher in those with IBM for all muscle regions assessed (total SUVR 1.45 [IQR 1.28–2.05] versus 1.01 [IQR 0.80–1.22], p=0.005). Strong negative correlation between MRI-derived muscle inflammation levels and [18F]florbetapir SUVRs were observed only in calf muscles bilaterally (right −0.73, p=0.02; left −0.68, p=0.03). No significant relationship between [18F]florbetapir SUVRs and clinical measures of disease severity were identified.ConclusionMuscle amyloid imaging using [18F]florbetapir PET may be useful in the diagnostic workup of IBM, particularly when differentiating from PM.
286 Hyperacute stroke unit and hyperacute neurology unit under one roof
Authors: Das Joyutpal , McKenzie Stefan , Gozzard Paul , Pattrick Michael , O’Malley Ronan , Payne Thomas , Grunewald Richard , Harkness Kirsty 
Published: October 2018

The Acute neurology unit (ANU), located at the Royal Hallamshire Hospital, Sheffield is a regional centre for a population of approximately 1.5 million. We are unusual, as our neurologists manage both hyperacute stroke and hyperacute neurology. HASU is located within our ANU. We had 4026 neurology ward admissions, 3265 emergency cases and 761 elective cases last year. We receive acute admissions from Trust A and E, direct admissions from general practitioners and urgent transfers from DGHs.We provide 24 hour stroke thrombolysis and a weekday working hours thrombectomy service. Overnight thrombolysis is supervised through a regional ‘Telemedicine’ system. A 2 week survey in July 2017 showed that only 59% patients, who were referred with suspected stroke, had stroke/TIA. Neurological conditions, such as migraine, seizure, syncope, acute vestibulopathy, Bell’s palsy and medically unexplained symptoms were common ‘stroke mimics’. 46% of patients with ‘stroke mimic’ were discharged on the day of admission. Average length of stay was 3 days. A separate survey of acute neurology referrals showed that 58% of patients did not require admission and were managed through an ambulatory care pathway. Patient satisfaction regarding their rapid assessment and diagnosis was high. Co-location of ANU and HASU allows rapid and effective management of ‘stroke mimics’.
292 Mapping patient pathways for acute functional neurological symptoms
Authors: Clea Southall , Samantha Haley , Stefan Williams , D Graham Christopher , Jane Alty , Owen Johnson , Melissa Maguire 
Published: October 2018

IntroductionPatients with Functional Neurological Symptoms (FNS) can present acutely, but many hospitals lack specific pathways or services for them. Outpatient FNS cohorts have been studied, but there is little data regarding acute FNS to inform service improvement.MethodOver an 8 month period at Leeds Teaching Hospitals NHS Trust (UK), the on-call neurology and stroke teams were telephoned regularly. Acute referrals with possible FNS were recorded. Electronic documentation was searched for the following events: symptoms, first presentation, diagnosis, A and E, outpatient clinic, inpatient admission, investigation, and psychological/psychiatric referral. This data was analysed with process mapping software (Fluxicon disco).ResultsWe present a dynamic care pathway map using real data, showing 205 patients with possible FNS moving through hospital services over time. Our map visualises the temporal relationships between healthcare utilisation, first presentation, diagnosis and therapy. The picture shows high healthcare burden, with incomplete and slow movement towards appropriate therapy (e.g. clear diagnosis documented in only 66%; referral to psychological therapy in 26%).ConclusionPatients with FNS are regularly referred to acute neurological services in Leeds. Our dynamic map shows a high healthcare burden, and slow or incomplete movement to appropriate care, which suggests potential targets for service improvement.
296 Longitudinal measurement of serum NFL in early familial AD
Authors: Philip Weston , Teresa Poole , Natalie Ryan , Yuying Liang , Simon Mead , Kaj Blennow , Martin Rossor , Jonathan Schott , Henrik Zetterberg , Nick Fox 
Published: October 2018

A blood-based biomarker able to track early neurodegeneration in Alzheimer’s disease would be valuable. Serum neurofilament-light (NfL) is elevated in familial Alzheimer’s disease (FAD) mutation carriers prior to symptom onset, but exactly how early NfL becomes abnormal and whether it can track change within individuals is uncertain.We recruited 18 symptomatic carriers of autosomal dominant FAD mutations, 19 presymptomatic carriers, and 11 non-carriers. Blood was taken at baseline, and 26 participants also gave at least one follow-up sample (mean interval=2.5 years). Serum NfL was measured on the SIMOA platform. A longitudinal mixed effects framework was used to model change in NfL over time.Serum NfL was increased (p<0.05) in mutation carriers compared with non-carriers 11 years before the estimated time of symptom onset, with rate of change in NfL becoming significantly different 12 years before. However, there was high variability in the inter-individual rate of change in NfL between participants.Serum NfL concentration, and its rate of change, are sensitive, at the group level at least, to very early AD-neurodegenration. However, the high variability between individuals in NfL rate of change may make it difficult at present to use this measure to track early change in individual patients.
THUR 051 Ictal asystole: return to the history
Authors: Gashirai Mbizvo , Chris Derry , Richard Davenport 
Published: October 2018

In these two adult cases of transient loss of consciousness (TLoC), careful history-taking revealed a stereotyped prodrome of déjà vu suggestive of focal seizures. One patient had an implantable cardiac monitor fitted, the other a 48 hour Holter monitor. Each patient subsequently had a typical event of déjà vu followed by TLoC with rapid recovery and for both, the monitor showed cardiac asystole corresponding with this event. A permanent pacemaker (PPM) was inserted in both patients. This abolished TLoC. In its absence, déjà vu became more frequent, intense, and prolonged for one patient, resolving with levetiracetam. The other patient had a post-PPM episode of déjà vu followed by a generalised tonic-clonic seizure. It transpired that their lamotrigine had been withdrawn following PPM insertion. Seizure-freedom was achieved by restarting lamotrigine.Ictal asystole, a rare complication of focal epilepsy, has a mean ventricular standstill duration of 20 s. This can be benign or progress to post-ictal asystole −fatal in 54% of cases. Careful attention should be paid to prodromal symptoms suggestive of focal seizures (including ‘auras’) in patients presenting with what might otherwise sound like syncope. These patients may have epilepsy presenting with ictal asystole and require antiepileptic drugs alongside their cardiac investigations/interventions.
THUR 054 The heart holds answers the brain cannot to see
Authors: Owain Williams , Mo Hu , Deepthi Changaradil , Andrew Fry , Robert Powell 
Published: October 2018

Neuronal ceroid lipofuscinosis (NCL) is a range of inherited lysosomal storage disorders. This case highlights the cardiac pathology associated with NCL and reports improved seizure control following correction of cardiac arrhythmias.A 26-year-old woman presented with episodes of altered awareness with head and eye deviation on a background of a progressive neurodegenerative disorder. She initially presented at 7 years of age with progressive retinal dystrophy. As a teenager she developed seizures with prominent myoclonus, psychosis, cognitive impairment and immobility. Her epilepsy was refractory to treatment and seizures had increased in frequency prior to admission.ECG demonstrated sinus bradycardia of 30bpm and episodes of 20 s sinus arrest. This was managed by the insertion of a pacemaker, resulting in a dramatic reduction in the frequency of her seizures. Investigation into the underlying disorder was revisited identifying vacuolated lymphocytes and homozygous CLN3 gene deletions. These findings are consistent with a diagnosis of juvenile NCL (Batten disease).This case demonstrates the importance of cardiac monitoring in investigating a change in seizure pattern. We hypothesise that the patient presented with reflex hypoxic seizures secondary to asystolic episodes. It also highlights the value of securing a diagnosis to enable appropriate cardiac screening in NCL patients.
WED 097 Diagnostic lumbar punctures in IIH: what is the patient experience?
Authors: Scotton William J , Mollan Susan , Walters Thomas , Doughty Sandra , Nightingale Peter , Botfield Hannah , Markey Keira , Yiangou Andreas , Williamson Shelly , Sinclair Alexandra 
Published: October 2018

Aims and methodsPatients with idiopathic intracranial hypertension (IIH) often report significant morbidity associated with lumbar punctures. We therefore aimed to assess the patient’s experience of diagnostic lumbar puncture (LP) in IIH using an online survey designed in collaboration with IIH: UK (a leading UK charity).Results463 patients responded to the survey, and were eligible for analysis, over a 2 month period in 2015. 40% of patients described severe pain during the LP (VRS≥8), and the median pain score during the LP was 7 (VRS, IQR 5–7). The majority of patients felt they received insufficient pain relief (85%). Levels of anxiety about future LP’s were high (median VRS 7, IQR 4–10), with 47% being extremely anxious (VRS≥8). LPs performed as an emergency were associated with significantly greater pain scores compared to elective procedures (median 7, IQR 5–7 vs 6, IQR 4–8, p=0.012). Higher LP pain scores (VRS) were significantly associated with poorly informed patients (Spearman correlation, r=−0.32, p<0.001).ConclusionsThis study has shown that a significant number of these patients are experiencing significant morbidity from pain and anxiety. This morbidity is associated with both inadequate pre-procedural information, as well as the setting the LP is performed in.
THUR 006 A case of GABA-B antibody mediated encephalopathy
Authors: Omar Abdel-Mannan , Seeedharan Harikrishnan , Khaled Abdel-Aziz 
Published: October 2018

A previously well 17 year old female presented with a self-limiting episode of derealisation and depersonalisation lasting 24 hours. Initial MRI demonstrated new left peri-trigonal white matter hyperintensity. Initial bloods were normal and serum was negative for white cell enzymes, VLCFA and broad autoimmune screen but positive for GABA-b antibodies (Ab).Six months later, an acute cognitive decline, intermittent upper limb tremor and emotional lability led to readmission. On examination she had an ACE-R of 88/100 and mildly slowed saccades. Repeat MRI was stable and EEG was diffusely slow, particularly the right temporal lobe. She had a mild CSF lymphocytosis (8 cells). An underlying malignancy was excluded with CT chest, abdomen, pelvis and PET CT. She was treated with PLEX and pulsed intravenous methylprednisolone, which led to an improvement of her symptoms and cognitive status. She was discharged on oral steroid taper. One month later she had a milder relapse and was retreated with PLEX and started on mycophenolate. She has now been in remission for 18 months.ConclusionsOur case highlights that neurologists should consider GABA-b Ab mediated encephalitis as a differential in patients presenting with derealisation and depersonalisation, in addition to previously described presentations of seizures and ataxia.
WED 129 Improving community exercise provision in parkinson’s
Authors: Palin Madeleine , Rideout Jane , Carroll Camille , Evans Valerie 
Published: October 2018

ObjectiveTo improve community-based exercise provision for people with Parkinson’s (PwP).BackgroundRegular exercise of moderate-high intensity is beneficial to PwP, significantly reducing impact of symptoms and improving quality of life.AimTo increase exercise opportunity for PwP by:Development, delivery and evaluation of a programme to increase community exercise providers’ knowledge and understanding of Parkinson’s disease (PD).Development of a ‘tool kit’ to facilitate national dissemination of the course.MethodsFunding was achieved from Active Devon and Parkinson’s UK. Course content and evaluation methodology were developed by the PenPEN training co-ordinator (VE), and project team. The 3 hour pilot course was delivered in November 2017. A pre- and post-course questionnaire was administered to participants.Results25 Delegates attended the course, representing a range of activities including dance, swimming and football. 100% completed questionnaires. Evaluation demonstrated significant improvement in comfort communicating with PwP (p=0.002), understanding of PD and the benefits of exercise, and suggesting exercise to PwP (p<0.001).ConclusionsWe have developed a means of increasing community exercise provision for PwP. Details of trained exercise providers are disseminated via clinics and Parkinson’s UK branches. An Exercise Provider Training Toolkit will be made available via the Parkinson’s Excellence Network.
THUR 153 Antibodies against the voltage-gated potassium channel complex
Authors: Das Joyutpal , Chauhan Vanisha , Keh Ryan , Mills Daniel , Nicholas Johal , Matthews Rachael , Lilleker James , Gosal David , Sharaf Nazar 
Published: October 2018

Voltage-gated potassium channel (VGKC) complex antibodies have been associated with a spectrum of presentations including peripheral nerve hyperexcitability (PNH), Morvan’s syndrome, autoimmune encephalopathy, epilepsy and recently psychosis.We retrospectively reviewed the medical records of 70 patients from the Greater Manchester Neuroscience Centre, who had tested positive for VGKC-complex antibodies between 2012 and 2015 to identify the clinical relevance of positive results.The majority were diagnosed with autoimmune encephalopathy(19) followed by epilepsy(14), psychosis(10) and PNH(6). The remaining fifteen had other neurological presentations and six had no primary neurological disorder. 39/70 patients who had antibody titres>400 pM, were diagnosed with autoimmune encephalopathy(19), epilepsy(9), psychosis(4), PNH(3) and other disorders(4). 24/39 patients, who received treatment with one or a combination of corticosteroids, intravenous immunoglobulins, cyclophosphamide, plasma exchange, azathioprine or rituximab, had a diagnosis of autoimmune encephalopathy(18), epilepsy(2), psychosis(2) and malignancy(2). 16/24 were treatment responsive. 3/31 patients with lower titres were also treated, but only one with the classic phenotype (PNH) responded to treatment.The classic phenotype often had a titre >400 pM. PNH may have a titre ≤400 pM. The patients without classic presentations typically had titres≤400 pM. Consistent with previous studies, clinical phenotyping and antibody titre helped to determine the relevance of VGKC-complex antibodies.
THUR 155 Autonomic function testing in autoimmune autonomic ganglionopathies
Authors: Vinojini Vivekanandam , Ekawat Vichayanrat , Ian Skeavington , Nadine Almeida , Valeria Iodice , Merete Hagen Ellen , Gorgon Ingle 
Published: October 2018

IntroductionAutoimmune autonomic ganglionopathies (AAG) are an increasingly diagnosed cause of autonomic failure. In 50% of patients, nicotinic ganglionic acetylcholine receptor (α3-AchR) antibodies are found. The presentation includes orthostatic hypotension, gastrointestinal and bladder dysfunction, abnormal pupillary responses and sicca symptoms. Physiological changes are documented with serial autonomic function testing. Gold standard therapy is not well established; however treatment with repeated plasma exchange appears to alter disease course.We provide the first description of consecutive autonomic function tests of one patient with seronegative AAG versus one patient with seropositive AAG both treated with serial plasma exchanges.MethodsWe compare ten parameters – time tolerated in head up tilt (HUT), lowest tilt heart rate (HR), systolic and diastolic blood pressures (BP), HR Valsalva phase 2 and 4, Valsalva ratio, change in catecholamines, HR response to deep breathing and antibody titre.ResultsHUT time, Valsalva ratio and the lowest tilt diastolic BP correlate best with clinical course over time. The α3-AchR titre also correlates with clinical improvement in the seropositive AAG patient. ConclusionsWe propose that repeated HUT time, Valsalva ratio and lowest tilt diastolic BP should be recorded in patients with AAG to determine treatment response and to assist in deciding treatment continuation.
WED 011 Subacute combined degeneration of the cord: laugh until you are weak
Authors: Anna Francis , Maz Matar 
Published: October 2018

A 33 year old lady presented to her GP with a 2 week history of numbness affecting her thighs. Routine blood tests revealed normal haemoglobin and folate levels with a borderline B12 concentration of 103 ng/L. Over the subsequent 2 weeks the numbness spread to involve her feet and her hands. Despite commencing cobalamin injections her symptoms continued to progress. She was admitted to hospital 4 weeks after the onset of symptoms with disabling sensory ataxia.All reflexes were either absent or elicited only with reinforcement. Plantars were downgoing. There was impairment of all sensory modalities in a glove-and-stocking distribution, with proprioception accurate at the ankle on the right and the knee on the left. Her gait was broad-based. The remainder of the neurological examination was unremarkable.A full peripheral neuropathy screen revealed no abnormality. An MRI spine showed longitudinally extensive myelitis mainly affecting the dorsal cord extending from C4 to T3. MRI brain and lumbar puncture were pristine.Guided by the MRI findings direct questioning elicited a long history of frequent recreational nitrous oxide use. We review the accumulating literature regarding nitrous oxide-induced subacute combined degeneration of the cord and explore the pathophysiological mechanisms underlying it.