bluuebunny/crossref_metadata_2025_split huggingface dataset:
298 Neurosarcoidosis – review of 91 patients from three UK neuroscience centres
Authors: Reem Farwana , Alex Murley , Lauren Ankrah , Dan Whittam , Tom Hayton , Anu Jacob , Cris Constantnescu , Saiju Jacob 
Published: October 2018

BackgroundNeurosarcoidosis is a rare neuro-inflammatory disease with a wide variety of clinical presentations.MethodsRetrospective audit was conducted independently at Birmingham, Liverpool and Nottingham to identify patients with neurosarcoidosis and pooled data was analysed.Results88% of the 91 patients identified presented over 40 years of age. From the data available, 70% were White Caucasians, 21% Afro-Caribbeans and 8% were Asians. Optic neuritis (31%), limb weakness (30%), headaches (20%) and facial palsy (19%) were the most common clinical presentations with nearly a third of patients (27/91) having moderate or severe disability (modified Rankin scale ≥3) at onset. 27/58 (46.6%) of patients had raised serum ACE levels and 37/44 (84%) patients had raised CSF protein with 28/41 (68.3%) demonstrating CSF lymphocytosis (mean – 54, range 5 to 380 cells). 35/91 patients had radiological changes affecting the brain parenchyma with 23% showing leptomeningeal enhancement. 75% of the 36 patients with data available for CT chest showed evidence of pulmonary sarcoidosis. Steroids were administered in 84.9% (73/86) patients with 21 receiving steroid sparing drugs, mainly Azathioprine and Mycophenolate.ConclusionWe discuss the varied clinical presentation of neurosarcoidosis across three major UK neuroscience centres and attempt to risk stratify patients prior to immunomodulatory therapy.
THUR 037 Identification of biofluid markers of TDP-43 pathology
Authors: Rachelle Shafei , Martha Foiani , Carolin Heller , Amanda Heslegrave , Ione Woollacott , Katrina Dick , Jason Warren , Jonathan Schott , Henrik Zetterberg , Jonathan Rohrer 
Published: October 2018

IntroductionFrontotemporal dementia (FTD) is usually caused pathologically by either tau or TDP-43. Previous biofluid assays of TDP-43 have not so far proved to be sensitive or specific for identifying those cases with TDP-43 pathology.Material and methodsWe set out to investigate the novel TDP-43 Simoa assay (Quanterix) assay in both plasma and CSF in a cohort of patients recruited from the University College London FTD observational studies with known or likely TDP-43 pathology (17), non-TDP-43 pathology (13), and healthy controls (10).ResultsThe mean [standard deviation] plasma TDP-43 concentration was higher in those with likely TDP-43 pathology (155.1 [223.4] pg/ml) than those with non-TDP pathology (112.39 [252.9] pg/ml), and healthy controls (50.0 [23.1] pg/ml), but the differences between groups was non-significant, with substantial overlap in concentrations between all three groups. The mean CSF TDP-43 concentration was 2.9 [0.3] pg/ml in those with likely TDP-43 pathology, 2.8 [0.4] pg/ml in those with non-TDP pathology, and 3.1 [0.5] pg/ml in healthy controls. DiscussionThe assay tested in this study does not accurately distinguish between those with likely TDP-43 pathology and either disease controls or healthy individuals. There remains an urgent need to develop a better biofluid assay for pathological TDP-43.
THUR 052 Epilepsy in the land of ice and fire
Authors: L Wojtowicz Alex , H Thomas Rhys 
Published: October 2018

BackgroundEpilepsy is often explained through allegory; from magical thinking to misfiring neurons. It is important to appreciate pervasive media portrayals which influence lay attitudes toward epilepsy. George R. R. Martin’s series of fantasy novels A Song of Ice and Fire, and the HBO television adaptation A Game of Thrones, introduce characters with ‘mundane’ and ‘mystical’ epilepsy to millions. Cases are presented to highlight these varying portrayals.CasesRA, 8 years old, has ‘shaking sickness’. Triggered by stress, his hands ‘shake’ with subsequent involvement of all limbs. He loses consciousness, is incontinent of urine, and demonstrates post-ictal confusion. He is enmeshed with his mother and his condition is viewed as a manifestation of his unsuitability to rule.BS fell at 9 years old, resulting in a coma. Subsequently he remains paraplegic with dialeptic episodes. During absences, he can ‘possess’ animals or people, and experiences impossible hallucinations. These abilities are portrayed as empowering for a boy who suffers heavy stigma against physical disability.ConclusionFans will probably not adopt ‘magical’ views but may internalise stigma weighed against characters with both ‘mundane’ and ‘magical’ epilepsy. Due to the large audience, clinicians working with epilepsy patients might benefit from awareness of these portrayals.
WED 061 ‘real life’ experience with brivaracetam as add-on treatment for epilepsy
Authors: Rhiannon Hughes , Melissa Maguire , Jo Geldard , Markus Reuber 
Published: October 2018

BackgroundBrivaracetam received its UK licence as an adjuvant drug for focal epilepsy in adults in 2016. There is little data on its efficacy and tolerability in ‘real life’ settings. We present an interim analysis of a multicentre service evaluation of brivaracetam.MethodsData was retrospectively collected from clinical records at two UK centres of consecutive patients treated with brivaracetam and at least one follow up. Data were also extracted on previous levetiracetam use. Seizure frequency was categorised at baseline and follow-up (daily/weekly/monthly/yearly/none).Results: Of the 44 patients identified (17 male, mean age 39, range 19–66), 75% had a history of levetiracetam exposure (LE+) and 25% did not (LE−). Mean brivaracetam exposure was 10.3 months (2–21), the mean daily dose was 200 mg (50–400). Retention was 91% vs 100% in LE+ versus LE− groups at 3 months and 82% in both groups at 6 months. Seizure category improvements were seen in 20%/36.4% in the LE+ vs LE− groups, seizure category deteriorations in 0% vs 9%. There were no serious adverse events.ConclusionBrivaracetam emerges as a potentially useful adjuvant medication for focal epilepsy. It may be better tolerated by some patients than Levetiracetam and more effective than this drug.
WED 066 Web-based media as a self-management tool for people with epilepsy
Authors: Alison McKinlay , Leone Ridsdale 
Published: October 2018

BackgroundThere is little evidence on how people with epilepsy (PWE) use web-based media in self-management of their condition. This study focused on the benefits/risks for PWE using social networking sites and web-based media.MethodsWe recruited 14 PWE who had volunteered after seeing information provided by Epilepsy Action, UK. We asked open-ended questions about online media use. Interviews lasted 60–90 min. Sessions were recorded, transcribed, and thematically analysed using Nvivo.ResultsSeven men and seven women participated, age range: 33–73, average diagnosis length was 25 years. Twelve participants used web-based media to gather information about their epilepsy. Seven used apps to manage their epilepsy by logging seizures or medication reminders. Six participants were hesitant to use web-based media due to privacy concerns. Four participants felt that epilepsy was underrepresented or misrepresented online. Three participants preferred traditional sources of epilepsy-related information.DiscussionResults show the value of web-based media in providing information and support to PWE, with the caveat that concerns around privacy and disclosure can undermine potential benefits. Health service providers and advocacy groups can assist by ensuring online information is accurate and up-to-date. Further research may help in developing understanding and future services.
THUR 075 Evaluating a personal information pack in epilepsy
Authors: Evan Edmond , Arpan Mehta , Alison Taylor , John Jacob 
Published: October 2018

A mainstay of epilepsy management is patient education and engagement. Previous educational interventions have varied greatly in number of sessions, teaching methodology and cost. This study assessed the impact of a low-cost intervention consisting of a personal information pack.MethodsForty-six consecutive patients with an existing epilepsy diagnosis attending clinic at MKUH NHS Trust were enrolled and pack provided. A baseline questionnaire assessed patient understanding and service utilisation (GP, A and E, specialist nurse). The questionnaire was repeated 3 months later. Patient engagement with the pack was assessed (completeness of information in pack, patient rating of usefulness).ResultsFourteen patients were lost to follow-up - thirty-two completed the second interview. There was a trend towards reduced service utilisation. Specialist nurse visits were significantly reduced (2.75 visits/year pre-intervention vs 1.11 post-intervention, p=0.01). Patient understanding was not significantly changed. Patient rating of usefulness was positive (2.4/3 on Likert scale). Two thirds of patients had not filled out the pack or added basic details only.DiscussionThis simple, low cost intervention may reduce some types of service utilisation and be found helpful by patients. A planned redesign involves the provision of a low-effort pre-filled information card together with the larger pack.
WED 078 ‘not just a headache’: social media use in people with migraine
Authors: Leone Ridsdale , Rosanna Swindale , Peter Keighley , Carly Pearson 
Published: October 2018

Introduction‘Invisible’ conditions like migraine may leave individuals seeking support and information. We aimed to describe how people with migraines use and benefit from social media and to identify harms of social media use.MethodsTwenty participants were recruited via migraine charities. Semi-structured interviews were conducted with questions based on a topic guide. Interviews were transcribed and analysed using thematic analysis.ResultsWe found people with migraine use social media to better understand their condition and treatment options. It offers instant access to continuous information and social support from empathic others. Participants viewed social media as an outlet to vent frustrations and to validate their migraine experience. They referred to the invisible and episodic nature of migraine which contributes to misunderstanding of the impact and/or severity of the condition. Some masked their online migraine-related behaviour, using different sites or closed online groups, which sometimes changed their online behaviour in other areas. Harms of social media included inaccuracy of information, occasional negativity, and privacy issues.ConclusionsSocial media can provide people who experience migraines with instant, continuous access to social support and health information, from empathic others. This can validate their illness experience, reassure and help to reduce feelings of isolation.
WED 079 Epidural blood patch: impact of non-luer one-way butterfly needle
Authors: Asif Mahmood , Paul Sharpe 
Published: October 2018

The epidural blood patch (EBP) requires a syringe that connects to the intravenous and epidural route. Thereby contravening Department of Health guidance, which recommends adoption of equipment that, prevents wrong route drug administration. To comply with these recommendations a non-Luer butterfly needle with one-way valve has been produced by GBUK. The one-way valve and length of tubing has the potential of activating the clotting cascade. This could reduce the time clinicians have to utilise the blood in the syringe. Also any alteration in clotting could affect the therapeutic value of the EBP. The primary objective of this research was to determine if phlebotomy using this new 21G needle altered blood clotting, determined by thromboelastograph analysis, compared to a standard 21G hypodermic needle.We performed paired phlebotomy on 10 healthy volunteers; producing a study capable of revealing a 25% change in R-time (power 80%). Mean R-time was increased with the new needle, however remaining within normal range (7.3 vs 6.6 min (p=0.04)). This would not limit the time to utilise the blood before clot formation in the syringe. With no clinically relevant difference in MA or LY30, it is unlikely to have any impact on the therapeutic value of the EBP.
WED 080 Experiences of specialist referral and GP access to MRI for headache
Authors: Alison McKinlay , Raphael Underwood , Asif Mazumder , Rachael Kilner , Leone Ridsdale 
Published: October 2018

IntroductionWhen General Practitioners (GPs) refer patients with headache to neurologists, it is often because the patient and/or doctor want imaging. Some GPs now access Magnetic Resonance Imaging (MRI) directly. We aimed to describe patients’ experience of GPs using direct access, compared to patients who saw a specialist first.MethodsWe invited participants to semi-structured Interviews about two months after imaging. Interviews were audio-recorded, transcribed, and analysed using thematic analysis in Nvivo.ResultsWe interviewed 10 patients from each pathway in South London, eleven women, median age 41, range 20–72. We found more similarities than differences between groups. Ten said they received a clear scan result explanation, while six had difficulty understanding results. Eleven participants felt relief once results were received, while five still wanted an answer on the underlying cause for symptoms. Seven felt the specialist appointment wait time was long. Those using the direct-access pathway were more likely to report MRI results delay.ConclusionPatient reassurance was linked closely with results receipt and worry linked with wait times. Some felt MRI results did not provide sufficient explanation for symptoms. Improvement in both pathways can be achieved, providing results are delivered in a clear, timely manner.
THUR 084 Cranial autonomic symptoms in cluster headache induced by nitroglycerin
Authors: Wei DY , Goadsby PJ 
Published: October 2018

IntroductionCluster headache (CH) is characterised by attacks of unilateral excruciating headache, ipsilateral cranial autonomic symptoms (CAS) and/or agitation. Studying of CAS can further our understanding of CH pathophysiology, but is limited by the episodic nature of the disease. Nitroglycerin (NTG) is known to induce CH. The aim of this study is to characterise CAS induced by NTG.MethodsCH patients received intravenous NTG 0.5 mcg/kg/min over 20 min. CAS and headache phenotype were recorded. The study was approved by the NHS Research Ethics Committee.ResultsTwenty-three patients participated: 83% male and 61% episodic cluster headache. The most common spontaneous CAS reported were lacrimation, nasal congestion and conjunctival injection. Agitation was reported in 96%. Nitroglycerin induced ipsilateral CAS in 91% of the patients, with 74% with ipsilateral pain. Most commonly induced CAS were nasal congestion, lacrimation and periorbital swelling. Agitation was reported in 61%. The majority of the CAS (80%) induced by NTG presented before the onset of severe pain.ConclusionWe demonstrate that NTG effectively triggers ipsilateral cranial autonomic symptoms in CH patients and that they often present in a phase before the onset of pain reflecting the underlying pathways during a cluster headache attack.
THUR 101 SCA-1 with unusual early dysautonomia and delayed cerebellar signs
Authors: Eleanor d’Ersu , NurAizaan Anwar , Talal Al-Mayhani , Meneka Sidhu 
Published: October 2018

A 59-year-old right-handed bus driver presented with gradually worsening erectile dysfunction, urinary frequency and nocturia over 18 months. He had a past medical history of hypertension with regular medications including amlodipine, aspirin and atorvastatin. Initial examination revealed a mild bilateral intention tremor.The patient had undergone brain imaging showing brainstem and cerebellar atrophy with a ‘hot cross bun sign’. The patient was then referred for autonomic function tests that demonstrated mild cardiovascular autonomic changes, and suggested sympathetic dysfunction. A working diagnosis of multiple system atrophy (MSA) was made.Over the next two years, his tremor worsened with the development of slurred speech and unsteady gait. On examination, he had prominent fasciculations and wasting in the right shoulder, severe bilateral intention tremor with past-pointing. In addition he had Parkinsonism and signs of a sensorimotor neuropathy.This atypical clinical evolution with prominent neuropathy and cerebellar signs led to investigations for spinocerebellar ataxia (SCA), particularly type 2 or 3 (given the autonomic dysfunction). However, the genetic testing showed a mutation in ATXN1, confirming a diagnosis of SCA type 1.To our knowledge, this is the first reported case of prominent early autonomic dysfunction associated with SCA type 1.
THUR 104 Global MD registry and dystonia non-motor symptoms study
Authors: Zehra Yilmaz , Kathryn Peall 
Published: October 2018

Myoclonus Dystonia (MD) is a rare hyperkinetic movement disorder with an early childhood onset. Estimates of MD prevalence among European populations places this at 1 in 5 00 000 individuals, however no worldwide estimates currently exist. MD patients experience alcohol-responsive myoclonus predominantly affecting the trunk and upper limbs, and dystonic posturing typically involving the neck and hands (writer’s cramp) but may also involve other parts of the body (e.g. lower limbs and trunk). Individuals with MD also frequently report non-motor symptoms (NMS) such as psychiatric co-morbidity, significant pain that impacts function and daily living, and disturbance to sleep.This study aims to develop a global register of MD patients of all ages, and further characterise the NMS in 500 adult MD patients via validated online psychiatric, sleep and pain questionnaires and cognitive tests. The register will also be used to establish a research hub to direct future research into elucidating disease mechanisms, and develop novel therapeutic targets for MD. The online questionnaires will be developed using the Bristol Online Surveys platform. All data will be encrypted to ensure patient confidentiality, and then analysed using Microsoft Excel and R statistical software package. P values less than 0.05 will be deemed as statistically significant.
WED 114 Pathways to diagnosis in PSP, CBD and PD
Authors: Diane Swallow , Carl Counsell 
Published: October 2018

Misdiagnosis and delayed diagnosis are common in PSP/CBD. To inform approaches to reduce diagnostic delay, it is essential to systematically evaluate patient pathways to diagnosis.Cases with a final diagnosis of PSP (n=28), CBD (n=2), and 30 age-sex matched PD controls, were identified from the Parkinsonism Incidence in North-East Scotland study. Using general practitioner, hospital and research records, referral and diagnostic time intervals from symptom onset to death were recorded.Comparing PSP/CBD to PD, the median (interquartile range) time intervals from index symptom to first secondary care referral [0.7 (0.01, 2.53) vs 0.02 (0.00, 0.73) years] and review [0.84 (0.18, 2.56) vs 0.13 (0.07,0.88) years], and, first neurologist referral [1.67 (0.70, 4.45) vs 0.12 (0.00, 1.56) years] and review [1.72 (0.88, 4.53) vs 0.23 (0.11, 1.65) years], were significantly longer in PSP/CBD (p=0.001 to 0.031). The average time intervals from index symptom to a parkinsonian syndrome diagnosis [2.26 (0.85, 5.41) vs 0.10 (0.00, 0.90) years], inclusion of the final diagnosis amongst differential diagnoses [3.62 (2.06, 7.21) vs 0.16 (0.00, 1.53) years], and the final diagnosis as primary diagnosis [4.22 (2.28, 7.63) years vs 0.67 (0.10, 3.04) years] were similarly longer in PSP/CBD (p≤0.001).Referral and diagnostic time intervals are significantly longer in PSP/CBD when compared to PD. Identifying factors that both improve the timing and destination of referral decision-making, and the accuracy and timeliness of diagnosis is necessary.
THUR 116 Vascular prevention in patients with parkinson’s disease
Authors: Isla Kempe , A Grosset Katherine , G Grosset Donald 
Published: October 2018

BackgroundVascular prevention is appropriate for patients with a vascular history (secondary prevention) and increased risk (primary prevention). Cerebrovascular disease adds to gait and cognitive problems in patients with Parkinson’s disease (PD).MethodsA convenience cross-sectional sample of consecutive PD patients attending the Neurology Movement Disorder clinic was assessed, and QRISK3 scored when appropriate (cases without vascular events, age <85 years).ResultsOf 100 cases, mean age 66.5 (SD 9.0) years, 52.0% male, with PD duration 8.3 (SD 5.5) years, 15 had a vascular history meriting statin therapy, of whom 12 (80.0%) were prescribed statins. 22 had a high vascular risk (QRISK3 >20%), mean QRISK3 28.6 (SD 7.7) of whom 2 (9.1%) were prescribed statins. We are now actively assessing QRISK3 and recommending statin therapy where appropriate.ConclusionsSecondary vascular prevention with statins is more commonly implemented than primary prevention, in patients with PD. In patients without a vascular diagnosis, vascular risk should be assessed and statin therapy offered where appropriate, noting that around one-fifth of patients have a high vascular risk and are not on statin treatment.
THUR 122 Parkinson’s kinetigraph improves movement disorder service provision
Authors: Christopher Kobylecki , Lucy Partington-Smith , Monty Silverdale 
Published: October 2018

IntroductionObjective evaluation of symptoms of Parkinson’s disease (PD) can be challenging. There is increasing interest in technological solutions to assess, monitor and manage people with PD.ObjectiveTo evaluate the effect of the Parkinson’s Kinetigraph (PKG) on management of patients with PD in a large tertiary movement disorder service.MethodsWe retrospectively reviewed the notes of 47 patients with PD (22 female, 25 male) who underwent PKG recording over a six month period. The indications and PKG findings, and the subsequent effect on clinical decision making and service provision were recorded.ResultsManagement was significantly altered in 25 patients (53%), while in 13 patients (28%) PKG confirmed the use of advanced therapies such as deep brain stimulation. Significant effects were seen with regard to service provision. Outpatient appointments could be deferred with advice following PKG in 15 (32%), advanced therapies assessment was improved in 16 (34%), while inpatient admission was avoided in six patients (13%).ConclusionThe use of PKG has enhanced service provision in our movement disorder service. In particular, it enhances our assessment of patients considered for high-cost advanced therapies, allows more efficient use of clinic appointments, and has the potential to reduce hospital admissions.