bluuebunny/crossref_metadata_2025_split huggingface dataset:
020 Increased risk of an abnormal cervical screening test in women with MS exposed to high-efficacy disease-modifying treatments
Authors: Walt Anneke Van der , Yuvaraj Jeremy , Stankovich Jim , McGuinn Nicola , Rath Louise , Skibina Olga , Nesbitt Cassie , Wesselingh Robb , Monif Mastura , Richards Janene , Kalincik Tomas , Taylor Lisa , Baker Josephine , Nguyen Ai-Lan , Wrede David C , Butzkueven Helmut , Brotherton Julia , Jokubaitis Vilija 
Published: July 2019

IntroductionLong-term exposure of women with Multiple sclerosis (MS, wwMS) to immunomodulatory or immunosuppressive treatments may increase the risk of cervical dysplasia. However, little is known about cervical dysplasia risk and Human Papillomavirus (HPV)-vaccine coverage in wwMS.MethodsAdult wwMS were recruited from two tertiary MS clinics. To explore the association between MS treatments (DMTs) and abnormal cervical screening tests (CSTs), we linked individual data from MSBase, the Victorian Cervical Screening Registry, and National HPV vaccination program registry (NHPVPR).ResultsTo date, we have recruited 208 wwMS of whom 102 had complete data (vaccination status, cervical screening tests, MSBase data) and no previous history of abnormal CST at MS onset for this interim analysis. The average age was 33.8 (18 to 59 yrs) and most (n=58, 88%) were unvaccinated. 19 wwMS (19%) had an abnormal CST after MS onset (incidence rate 20.6 cases/1000 person-years, 95% confidence interval 12.4–32.1) over average 9.0 years of follow-up. 57 wwMS were treated with lower-efficacy therapies (56%), 73 with a high-efficacy therapy (72%), and 44 were exposed to both. Eight abnormal CSTs were detected before starting high-efficacy therapy (rate 12.6, 95% CI (5.4–24.8)) and 11 were detected after starting high-efficacy therapy (rate 38.6, 95% CI (19.3–69.0), p=0.022.ConclusionWe provide preliminary data that high efficacy DMTs may increase the risk of abnormal CSTs over time. A larger cohort and inclusion of additional cervical dysplasia risk factors are required to fully elucidate risk in wwMS.
021 Profiles of benign positional vertigo tested on the epley omniax chair
Authors: Argaet Emma C , Lechner Corinna , Bradshaw Andrew P , Michael Halmagyi G , Welgampola Miriam S 
Published: July 2019

IntroductionBenign positional vertigo (BPV) has a characteristic pattern of nystagmus specific to the affected semicircular canal and the underlying mechanism of canalithiasis (where otoconia float freely) or cupulolithiasis (where otoconia are adherent to the cupula).MethodsWe analysed the nystagmus slow-phase velocity (SPV) profiles of 100 subjects with posterior-canalithiasis, 30 with lateral-canalithiasis, 10 with lateral-cupulolithiasis and 3 with anterior-canalithiasis. Subjects were examined on the Epley Omniax Rotator, a mechanical chair with real-time video-oculography. Video data was analysed using custom-made LabVIEW software. Nystagmus onset, duration, peak-velocity, peak-latency and time taken for the peak-velocity to halve (t50), were measured.ResultsIn posterior-canalithiasis, nystagmus occurred within 14.2 seconds of positioning and lasted 2.5–34.5 seconds. The median vertical peak-SPV was 37.3°/s. The median peak-latency was 2.9 seconds and the median t50 was 3.4 seconds. In lateral-canalithiasis, nystagmus onset was mostly immediate. With the affected ear down, the median peak-SPV was 52.2°/s and the median peak-latency was 3.6 seconds; the t50 was 7 seconds (median) and the paroxysms lasted 9.9–48.5 seconds. In lateral-cupulolithiasis, nystagmus onset was instantaneous. With the unaffected ear down, the median peak-SPV was 69.6°/s. The peak-latency (median 18.6 s) and t50 (median 34.5 s) were significantly prolonged compared to canalithiasis. For anterior-canalithiasis, the onset was 0–2.9 seconds, the peak-latency was 3–5.4 seconds, the t50 was 6.4–10.5 seconds and the duration was 13.4–23.1 seconds.ConclusionsCanalithiasis and cupulolithiasis produce distinct SPV profiles, which enable their identification and the separation of BPV from other causes of positional nystagmus.
024 Resistance exercises with blood flow restriction in patients with sporadic inclusion body myositis
Authors: Liang Christina , Burk Melanie , Wall Abby , Davis Ryan , Augustine Libby , Bright Sarah , Brown Whitney , Harris Anneliese , Holmes Sarah , Jeyalingam Sayanthinie , Large Sarah , Mai Sylvia , Raper Eleanor , Judtih Wong Hang Wah , Wong Pok Man , Sue Carolyn M , Lucas Barbara 
Published: July 2019

IntroductionSporadic inclusion body myositis (sIBM) is the most common muscle disease affecting older adults with no disease-modifying treatment. Resistance exercises increase muscle hypertrophy, but weakness prevents exercising with higher resistance. In healthy subjects, augmentation of light-load training with blood flow restriction improved muscle strength1; and similar exercises were safe in the elderly.2 We therefore investigate whether resistance exercises with blood flow restriction is safe and helpful in sIBM patients. We explored methods for exercising weak leg muscles, and options for better outcome measures.MethodsA matched-control pilot study, with 12-week treatment and 4-week follow-up periods, where participants concentrated on lower limbs resistance exercises with 50% blood flow restriction 3 times/week, at 20%-30% of their repetition maximum. Patients are reviewed 4-weekly for muscle strength, blood biomarkers, 2-minute walk test (2MWT), timed up-and-go (TUG) test, minimal chair height standing ability test (MCHSAT), thigh girths, and quality of life scales.Results4 patients with varying abilities were on the exercise arm, and 3 acted as controls. Muscle groups with MRC score ≥2 were able to be exercised. We found strength testing by hand-held dynamometer was unreliable, whereas the 2MWT, TUG test and MCHSAT showed less variability. All patients could perform their exercises at significantly increased repetitions or weights by week 4, without concerning adverse events, with trend towards continued improvement over the 4-month period.ConclusionResistance exercises with blood flow restriction appear safe, and may be helpful in sIBM patients, even in weakened muscles, enabling improvement in muscle strength and endurance.ReferencesHughes L, Paton B, Rosenblatt B, Gissane C, Patterson SD. Blood flow restriction training in clinical musculoskeletal rehabilitation: a systematic review and meta-analysis. Br J Sports 2017.Vechin FC, Libardi CA, Conceicao MS, et al. Comparisons between low-intensity resistance training with blood flow restriction and high-intensity resistance training on quadriceps muscle mass and strength in elderly. J Strength Cond Res 2015.
036 The impact of a clinical support tool on stroke key performance indicators
Authors: Alanati Khaled , Evans James 
Published: July 2019

IntroductionAdherence to key performance indicators (KPIs) in stroke care is associated with better outcomes.1–6 The complexity in management of acute strokes, however, has created barriers towards delivering best care with plateauing of KPIs as measured by The National Stroke Foundation Clinical Audit.We examined the impact on stroke KPIs in our local health district of a web-based decision support stroke platform which provides clinicians with up-to-date information about the patient’s management flagging potential areas for improvement, allowing treatment to be optimised in real time.MethodsSix months following the introduction of the platform we performed a retrospective analysis of Electronic medical records of patients admitted to Gosford hospital with acute stroke between June 2018 and September 2018 assessing access to the stroke unit as well as being discharged on appropriate secondary prophylactics, including antihypertensives and correct antithrombotic therapy. Patients whose direction of care was palliative and patients with documented contraindication to secondary prophylactics were excluded.ResultsOver four months, 136 patients presented with acute ischaemic stroke and 11 patients had a haemorrhagic stroke. 49 ischaemic stroke patients had atrial fibrillation. Stroke unit access was higher following its introduction in 2018 compared to 2017 (97% vs 76%, respectively). Similar findings were noted for patients with atrial fibrillation who received oral anticoagulants on discharge (90% vs 50%) and patients discharged on antihypertensives (95% vs 80%).ConclusionUse of a clinical support platform in managing acute stroke is an intervention that improves stroke care.ReferencesUrimubenshi G, Langhorne P, Cadilhac DA, Kagwiza JN, Wu O. Association between patient outcomes and key performance indicators of stroke care quality: A systematic review and meta-analysis. European Stroke Journal 2017;2(4):287–307. https://doi.org/10.1177/2396987317735426Sandercock P, Gubitz G, Foley P. Antiplatelet therapy for acute ischaemic stroke. Cochrane Database Syst Rev 2003;2: CD000029. Google ScholarKwan J, Sandercock P. In-hospital care pathways for stroke. Cochrane Database Syst Rev2004;4: CD002924. Google ScholarSaxena R, Koudstaal PJ. Anticoagulants for preventing stroke in patients with nonrheumatic atrial fibrillation and a history of stroke or transient ischemic attack. Cochrane Database Syst Rev 2004;4: CD000187. Google ScholarGoyal M, Menon BK, van Zwam WH. Endovascular thrombectomy after large-vessel ischaemic stroke: a meta-analysis of individual patient data from five randomised trial. Lancet 2016;387:1723–1731. Google Scholar | Crossref | Medline | ISIMiddleton S, McElduff P, Ward J. Implementation of evidence-based treatment protocols to manage fever, hyperglycaemia, and swallowing dysfunction in acute stroke (QASC): a cluster randomised controlled trial. Lancet 2011;378:1699–1706. Google Scholar | Crossref | Medline | ISI
039 Estimating the health and economic burden of investigating tumefactive demyelination compared to conventional multiple sclerosis
Authors: Silsby Matthew , Lopez Pedro S , Spies Judy , Reddel Stephen , Frith Jane , Barton Joshua , Beadnall Heidi , Barnett Michael , Hardy Todd 
Published: July 2019

IntroductionTumefactive demyelinating lesions, defined as demyelinating lesions > 2cm in diameter, occur most commonly in association with multiple sclerosis (MS), and can pose a diagnostic challenge. The aim of this study was to estimate the cost and morbidity associated with the diagnostic investigation of patients with tumefactive demyelination (TD) compared to patients with conventional relapsing-remitting MS.MethodsRetrospective review of patients seen between 2013–2018 in clinics at the Brain and Mind Centre, Sydney; a centre with tertiary referral expertise in MS. Records were searched for the terms ‘tumefactive’ and ‘pseudotumour’. All patients diagnosed with TD were included and paired with a patient of similar age diagnosed in the same year with MS according to 2010 McDonald criteria.ResultsThere were 31 patients with TD and 31 patients with conventional relapsing remitting MS. The estimated cost of investigating TD was more than 7.5 times higher per patient than in MS ($18,300 AUD vs $2,418 AUD, p<0.001). Brain biopsy was performed in 6/31 TD patients and 0/31 MS patients and was the cause of more adverse outcomes in the TD versus MS group. More patients in the TD group were admitted to hospital (22/31 vs 10/31) and ICU admissions only occurred in the TD group (10/22 vs 0/10).ConclusionThe cost and adverse outcomes associated with investigating TD are higher than in conventional MS. Improvements in the diagnosis of TD have the potential to improve health and economic outcomes.
004 Vestibular event monitoring in the emergency department
Authors: Nham Benjamin , Reid Nicole , Argaet Emma , Young Allison , Bein Kendall , Halmagyi Gabor M , Welgampola Miriam S 
Published: July 2019

IntroductionAcute vertigo is often accompanied by ictal-nystagmus which may assist with diagnosis. We examine the merits of a structured assessment combined with vestibular event-monitoring in the Emergency Department (ED).MethodsWe undertook a structured clinical assessment and video-nystagmography in 220 non-consecutive patients presenting to a public-hospital ED with acute vertigo, during a 10-month period. The records of 115 consecutive vertiginous patients who underwent standard-assessment were compared.ResultsFor the structured assessment group: 54% presented with acute vestibular syndrome (AVS), 24% with episodic spontaneous vertigo (EVS), and 20% with recurrent positional-vertigo (RPV).For AVS (n=119), most common diagnoses were vestibular neuritis (34%), stroke (34%) and vestibular migraine (13%). Nystagmus slow-phase velocity (SPV) for VN, stroke and VM were 11±5.5o/s, 5.6±2.5o/s, 5.4±5.9o/s; Mean ipsilesional video-head impulse gains were 0.51±0.29, 0.89±0.20 and 0.96±0.13. For EVS(n=53), diagnoses included vestibular migraine (63%), Meniere’s Disease (11%) and others (26%). Nystagmus SPV was 5.4±3.6o/s, 7.6±6.3o/s, 4.1±1.5o/s. In RPV (n=43), common diagnoses were posterior-canal BPPV (66%), horizontal-canal BPPV (23%), migraine (7%). Positional nystagmus SPV profile showed Peak SPV of 42.5o/s, 77.6o/s, 20.64o/s and Time-constants of 6.52s, 22.51s, 34.56s for Posterior-canal BPPV, Horizontal-canal BPPV and Atypical Positional-Vertigo. A final diagnosis was reached in 96% of patients.In the ED control group, only 77% were separated into spontaneous or positional-vertigo. A diagnosis was provided in 57% and was concordant with the history and examination in 34%.ConclusionVestibular event-monitoring and structured clinical assessment secured a diagnosis in 96% of cases compared with 34% for the control group, reinforcing its merit.
045 Primary cutibacterium acnes central nervous system infection causing focal seizures: an unusual presentation of a rare disease
Authors: Waller Sophie E , Browning Sarah , Pepper Elizabeth 
Published: July 2019

IntroductionCutibacterium acnes is a Gram positive, anaerobic bacterium of low pathogenic potential that forms part of the normal cutaneous flora. Although most often identified as a contaminant in culture of microbiological specimens, it is commonly implicated in both postoperative wound and implantable device infection. Neurosurgical device infections secondary to C. acnes are well recognised and are likely secondary to bacterial contamination from the skin during surgery. Indolent infection characterised by delayed presentation of weeks to months following intervention is common. C. acnes infection involving the central nervous system (CNS) in the absence of previous neurosurgical intervention is rare, but has been described following dental or mastoid infections and following facial trauma. A further case series has reported de novo C. acnes CNS infection occurring in the absence of these recognised risk factors, but with clinical features of meningitis being common to all.Methods and resultsWe describe a unique case of primary C. acnes extra-dural collection in a previously well patient with no neurosurgical history presenting with sub-acute focal seizures and progressive focal leptomeningeal thickening on MRI.ConclusionC. acnes CNS infection can occur in the immunocompetent and in the absence of neurosurgical intervention.
053 GLUT-1 deficiency presenting as dopa-responsive dystonia: an atypical phenotype of a rare disease
Authors: Waller Sophie E , Blackie Jeffrey , Goel Himanshu 
Published: July 2019

IntroductionThe SLC2A1 gene encodes the glucose transporter GLUT1, responsible for normal glucose transport across the blood-brain barrier. Mutations in this gene have classically been associated with an epileptic encephalopathy referred to as GLUT-1 Deficiency Syndrome, which typically presents with early-onset refractory epilepsy, developmental delay and complex movement disorders.1 2 More recently, SLC2A1 variants have been identified in patients with paroxysmal exercise-induced dyskinesia (PED), with or without a history of epilepsy.3 4 Response to medication is typically poor; however, both seizures and dyskinesia may improve following implementation of a ketogenic diet.5 A single case of levodopa responsiveness has previously been described in a subject with SLC2A1 mutation and PED.5Methods and resultsWe describe a 47-year-old female with mild intellectual disability since childhood but no history of epilepsy, who developed episodic dystonia affecting the lower limbs in her early 20’s. A clinical diagnosis of dopa-responsive dystonia was made following a marked, sustained response to levodopa. There was no significant family history. In her 40’s she developed breakthrough dystonia with exertion and choreiform movements affecting the fingers and face. A subsequent dystonia panel identified a heterozygous variant c.[1199G>T];[=] (p.Arg400Leu) in the SLC2A1 gene. Cerebrospinal fluid glucose concentration was low (2.0 mmol/L). She declined a trial of a ketogenic diet.SLC2A1 variants are associated with PED; however, response to levodopa has not been widely reported.ConclusionIt is becoming increasingly evident that the phenotypic presentations of GLUT1 deficiency are diverse, and SLC2A1 testing should be considered in a broader range of patients.ReferencesWang, D, Kranz-Eble P, De Vivo DK. Mutational analysis of GLUT1 (SLC2A1) in Glut-1 Deficiency Syndrome. Human Mutation 2001;16:224–231.Klepper J, Leiendecker B. GLUT1 deficiency syndrome – 2007 update. Developmental Medicine & Child Neurology 2007;49:707–716.Schneider SA, Paisan-Ruiz C, Garcia-Gorostiaga I, Quinn NP, Weber YG, et al. GLUT1 gene mutations cause sporadic paroxysmal exercise-induced dyskinesias. Mov Disord 2009;24(11):1684–1696.Suls A, Dedeken P, Goffin K, Van Esch H, Dupont P. Paroxysmal exercise-induced dyskinesia and epilepsy is due to mutations in SLC2A1, endoding the glucose transporter GLUT1. Brain 2008;131:1831–1844.Baschieri F, Batla A, Erro R, Ganos C, Cordivari C, Bhatia KP. Paroxysmal exercise-induced dystonia due to GLUT-1 mutation can be responsive to levodopa: a case report. J Neurol 2014;261:615–616.
059 Takotsubo cardiomyopathy and myasthenic crises: a case series
Authors: Katz Matthew , Walsh Stephen , Tsang Benjamin , McCombe Pamela , Sabet Arman 
Published: July 2019

IntroductionTakotsubo cardiomyopathy (TCM) is an acute, reversible cardiomyopathy that can mimic acute coronary syndrome.1 It is characterised by left ventricular dysfunction, electrocardiogram (ECG) changes and transient apical ballooning in the absence of significant coronary artery disease.1 It is usually triggered by acute stress with catecholamine surge but the exact pathogenesis is not known.1 Takotsubo cardiomyopathy has been described in patients with myasthenic crisis. We present the first and largest case series of four patients with TCM in the setting of myasthenic crisis and discuss possible causes.MethodsTwo patients from each tertiary neurologic centre were identified by their treating neurologist for inclusion in the series. We performed a review of their case notes with respect to history, examination, investigations and management. A brief literature review was also completed.ResultsThe mean age was 78 with a 1:1 female to male ratio. Three of the patients were newly diagnosed with myasthenia gravis (MG) at the time of their TCM. All patients were AChRab positive. One patient had a previous thymectomy but the others had no evidence of thymoma.On review of the literature most cases of TCM in myasthenic crisis occurred in older females. Abnormalities of the ECG were universal. Most cases did not have a thymoma or history of thymectomy.ConclusionTakotsubo cardiomyopathy may be easily overlooked in those presenting with myasthenic crises as they share overlapping clinical features. Rigorous attention to the cardiac status of these patients, especially the ECG, may help to avoid missing this important diagnosis.ReferenceAkashi YJ, Goldstein DS, Barbaro G, Ueyama T. Takotsubo cardiomyopathy: a new form of acute, reversible heart failure. Circulation 2008;118:2754–2762.
070 Burden of migraine is australia: a systematic literature review
Authors: Benhaddi Hicham , McCabe Sophie , Lau Diana T 
Published: July 2019

IntroductionMigraine is a disabling neurological disease characterised by recurrent attacks of moderate to severe headaches. This systematic literature review (SLR) aimed to investigate the clinical, humanistic, and economic burden of chronic migraine (CM), episodic migraine (EM), and of current preventive migraine treatments in Australia.MethodsThe methodology of this SLR was aligned with the National Institute for Health and Care Excellence (NICE) guidelines. An electronic database search was conducted in Embase, MEDLINE and the Cochrane Library, with a time frame of 2008 to 2018.ResultsIn total, 1,122 records were identified and 168 of these were included for data extraction. The prevalence of migraine in Australia is estimated at 18.9%. Of those, 44% of people with EM and 86% of people with CM reported moderate-to-severe disability. Over one-third (36%) of people with EM and nearly two-thirds (64%) of people with CM reported visiting a healthcare provider in the previous three months. No data relating to the economic burden of migraine were returned by the searches. In people with EM and CM, anti-calcitonin gene-related peptide (anti-CGRP) preventive treatments for migraine safely, effectively and significantly reduced the mean number of monthly migraine and/or headache days from baseline compared with placebo.ConclusionsMigraine is associated with a substantial burden, and people living with migraine feel the impact in their day-to-day lives. Anti-CGRPs are a promising class of preventive treatments for all people with migraine. Longer-term studies are needed to determine if the positive effects of anti-CGRPs are sustained over greater time periods.
080 The incidence, diagnosis and outcomes of idiopathic intracranial hypertension in the southern tasmanian catchment
Authors: Krishnadas Natasha , Taylor Bruce 
Published: July 2019

IntroductionThis study aimed to identify the incidence of idiopathic intracranial hypertension (IIH) in Southern Tasmania, Australia. Secondary aims were to elucidate demographics, current approaches to investigation, treatment and outcomes. To our knowledge, similar regional studies have not been performed.MethodsThe study was approved by the University of Tasmania Human Ethics Committee. Patients presented between June 2016-June 2018 to Royal Hobart Hospital, the single tertiary Neurology service in Tasmania. Cases were identified by screening lumbar punctures (LP) performed by Neurology services (inpatient, outpatient, Radiologically-assisted) and by surveying all regional Neurologists. Medical records were used to corroborate LP results and determine whether patients met Modified Dandy Criteria (MDC) (used to define IIH in current literature). Regional population statistics were obtained from the Australian Bureau of Statistics (ABS). Duplicate records were excluded. Exclusion criteria included age <18 at data collection, LP opening pressure <20cm of water or secondary causes for IIH.Results45 cases identified; 30 within the Greater Hobart region, population of 229,088 (June 2016-June 2017). Cumulative incidence was 6.55/100,000 (incidence rate 0.06/1000) with classification based on Neurologist diagnosis and 5.46/100,000 (0.05/1000) with classification according to MDC. 100% of the cohort were female. Mean age was 26.7 (range 17–45) and mean weight was 105.3kg (range 78–170). Headache was the most commonly reported symptom. 8.9% (4/45) of the total cohort were medically refractory (requiring VP/LP shunting). Ophthalmology services initiated 51% (23/45) of the referrals.ConclusionsOur incidence rates are higher than rates in previous studies for population subsets of young women.