bluuebunny/crossref_metadata_2025_split huggingface dataset:
FRACTURE RISK IN INCIDENT PARKINSONIAN COHORT VERSUS CONTROLS
Authors: Sleeman Isobel , Che Zhu-Chung , Counsell Carl 
Published: October 2015

BackgroundPostural instability and resultant fractures are common in parkinsonism but no studies have analysed fracture rates in prospective incident cohorts of parkinsonian patients and controls.MethodsData from the PINE study (326 incident parkinsonian patients, 261 age-gender matched controls) were used. The study database included prospectively collected data on falls and fractures from annual reviews supplemented by hospital and GP records. Major fractures were defined as skull, long bone or pelvis. Kaplan-Meier curves for time-to-first fracture from diagnosis were analysed by diagnostic groups. Multivariate Cox regression analysis was used to identify factors that independently predicted fractures.ResultsThe fracture rates in patients with PD, Parkinson's plus syndromes, dementia with Lewy Bodies and vascular parkinsonism were similar (log rank p value=0.89) and so these groups were combined. The percentage of frequent fallers among the patients (24.2%) was higher than the controls (1.3%). The incidence of any fractures and major fractures was about three times higher in patients (5.51 and 4.25 per 100 patient-years respectively) than in controls (1.96 and 1.42 respectively). Parkinsonism, female gender, frequent fallers and development of dementia were independent predictors of major fractures.ConclusionPatients with parkinsonism have a much higher risk of falls and fractures.
PREDICTORS OF INSTITUTIONALISATION IN PARKINSON'S DISEASE
Authors: Macleod Angus , Counsell Carl 
Published: October 2015

BackgroundInstitutionalisation (entry to nursing home, or other place of residential care) is an important outcome in PD. We investigated frequency of, and baseline predictors of, institutionalisation in Parkinson's disease (PD).MethodsWe identified all new cases of PD in Aberdeen, UK, over 4.5 years using multiple, community-based ascertainment methods. Age-sex matched community-based controls were also recruited. Participants were seen annually and place of residence at each follow-up or before death were ascertained, together with date of entry to institution. Kaplan–Meier institution-free survival probabilities were plotted. Potential baseline predictors were investigated using Cox regression.Results198 PD patients and 260 controls were recruited. One patient and one control were institutionalised before diagnosis. One patient and four controls were lost to follow-up. 44 (22%) PD patients and 19 (7%) controls were institutionalised during mean 6.1 years follow-up. Hazards of institutionalisation were higher in PD than controls (HR 3.72 [2.16–6.39]). Older age (HR 1.05 [1.01–1.09]), lower MMSE score (HR 0.88 [0.79–0.98]), and baseline dependence (HR 2.48 [1.18–5.25]) were independently associated with institutionalisation.ConclusionsInstitutionalisation was higher in PD than controls. Older age, poorer cognition and being dependent at diagnosis led to increased institutionalisation.
MORTALITY IN PARKINSON'S DISEASE AND ATYPICAL PARKINSONIAN DISORDERS
Authors: Macleod Angus , Counsell Carl 
Published: October 2015

BackgroundWe evaluated the mortality associated with Parkinson's disease (PD), Lewy body dementia (LBD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and vascular parkinsonism (VP) using a community-based incident cohort.MethodsAll incident parkinsonism cases identified over 4.5 years (2002-4, 2006-9) were tagged to the NHS central register for regular death notifications. Kaplan-Meier survival probabilities were plotted. Standardised mortality ratios (SMRs) and life expectancy, adjusted for age, sex and calendar year, were calculated using regional mortality data.ResultsUntil June 2014, 90 deaths occurred in 198 PD patients, and 107 deaths in 117 patients with other syndromes. Median survival in PD, LBD, PSP, MSA, and VP was 7.8 (6.7–9.4), 3.3 (2.3–4.1), 2.6 (1.1–3.8), 5.1 (1.3–NA), 2.1 (1.5–3.4) years, respectively. SMRs were 1.5 (1.2–1.9), 4.2 (3.0–5.9), 3.8 (2.6–5.5), 1.8 (0.9–3.4), 4.2 (3.0–6.0) respectively. In PD, median survival was lower than life expectancy, but more so in under 65s.ConclusionsMortality in PD was increased by 50% over expected population mortality. Younger patients have proportionally more to lose. Survival was much poorer in other syndromes.
ABNORMALITIES OF FIXATION, SACCADE AND PURSUIT IN POSTERIOR CORTICAL ATROPHY
Authors: Shakespeare Timothy , Kaski Diego , Yong Keir , Patterson Ross , Slattery Catherine , Ryan Natalie , Schott Jonathan , Crutch Sebastian 
Published: October 2015

Background and aimsThe clinico-neuroradiological syndrome posterior cortical atrophy (PCA) is the cardinal ‘visual dementia’. We evaluated oculomotor function in patients with PCA.Methods20 PCA patients, 17 typical Alzheimer's disease (tAD) patients and 22 healthy controls completed tests of fixation, saccade and smooth pursuit eye movements using an infrared pupil tracking system.ResultsEye movement abnormalities occur in 80% of PCA patients (compared to 17% tAD, 5% controls). PCA saccades were significantly hypometric, especially for distant targets. PCA patients were slower to reach saccadic targets whose onset overlapped with fixation, consistent with ‘sticky fixation’. Time to reach saccadic targets was also significantly associated with parietal and occipital cortical thickness measures. On fixation stability tasks, tAD patients showed more square wave jerks whose frequency was associated with lower cerebellar grey matter volume, whilst PCA patients showed characteristically large saccadic intrusions whose frequency correlated significantly with generalised reductions in cortical thickness. Smooth pursuit was also impaired, with lower gain in both PCA and tAD patient groups compared to controls.ConclusionsEye movement abnormalities are near-ubiquitous in PCA, and may help distinguish PCA from tAD. We suggest the PCA oculomotor profile reflects weak input from degraded occipito-parietal spatial representations of stimulus location into a superior collicular spatial map for eye movement regulation. The results have implications for other patients with dementia-related visual impairment.
FEATURES IN IDIOPATHIC RBD MIRROR THOSE OBSERVED IN PD
Authors: Rolinski Michal , Zokaei Nahid , Lawton Michael , Evetts Samuel , Mackay Clare , Quinnell Timothy , Zaiwalla Zenobia , Ben-Shlomo Yoav , Husain Masud , Hu Michele 
Published: October 2015

IntroductionPatients with idiopathic RBD have an increased risk of developing a defined neurodegenerative disorder, the majority developing PD. Is it possible to detect features of PD in RBD, before a diagnosis of PD is established?MethodsFifty-seven patients with polysomnography-proven idiopathic RBD and seventy-four control and drug-naïve PD subjects were recruited. All participants underwent a thorough motor and non-motor assessment, and were screened for mutations in glucocerebrosidase (GBA) genes. Visual working memory was separately assessed in 21 RBD, 15 drug-naïve PD and 21 controls using a serial order task testing both recall precision and the pattern of impairment.ResultsRBD patients had increased motor and postural impairment compared to controls. Non-motor deficits (hyposmia, constipation, depression, anxiety) were similar between RBD and PD cases. Furthermore, there was a significant deficit of working memory memory recall precision in PD and RBD, with the pattern of deficit being similar in both groups.ConclusionRBD is associated with motor and non-motor impairment often seen in early PD. The pattern of visual working memory impairment in RBD is equivalent to that observed in early PD. These results support the hypothesis that idiopathic RBD is representative of prodromal sporadic PD.
KNOWLEDGE OF PRESCRIBING IN PARKINSONISM AMONG JUNIOR DOCTORS
Authors: Bedford Jonathan , Whiteside David , Dassan Pooja 
Published: October 2015

Junior doctors are primarily responsible for prescribing medications for patients with parkinsonism attending a district general hospital. We assessed levels of confidence and knowledge of appropriate prescribing in a variety of circumstances among 30 junior doctors at Ealing Hospital, using a 5 part questionnaire. Only 10% felt confident in prescribing a regimen for patients with parkinsonism who are unable to take their usual medications orally. 60% could not select a suitable anti-emetic, and just 2 respondents answered all assessment questions correctly. Confidence in prescribing improved with seniority but overall scores in the assessment section did not.We have designed a protocol to assist doctors when managing patients with parkinsonism on the ward, and are conducting teaching sessions to improve understanding. The impact of these interventions will be assessed and presented at the Annual Meeting.
THROMBOSED BRAINSTEM DEVELOPMENTAL VENOUS ANOMALY CAUSING HYDROCEPHALUS
Authors: Dodd Katherine , Pegg Emily , Mathur Sachin , Suresh Chhetri 
Published: October 2015

Developmental venous anomaly (DVA) is a commonly encountered congenital abnormality of the venous drainage system. Spontaneous thrombosis of DVA is rare. We present a case of thrombosed brainstem DVA leading to venous infarction and oedema within the posterior fossa.A 49 year old, previously fit gentleman presented to the local hospital with a one day history of headache, slurred speech and incoordination. Examination demonstrated GCS of 13/15, cerebellar dysarthria, horizontal nystagmus to the left, mild right sided pyramidal weakness, right sided cerebellar ataxia and bilateral extensor plantars.CT venogram revealed a cerebellar DVA with thrombosis of one of the veins. There was surrounding venous infarction and oedema within the posterior fossa, causing compression of the fourth ventricle and dilatation of the lateral ventricles. MRI demonstrated extensive T2 change in the right cerebellar hemisphere, dorsal pons and right cerebral peduncle. No underlying thrombotic tendency was identified. He was treated successfully with intravenous heparin infusion. He improved over the next 3 weeks, and was discharged on warfarin with mild right sided ataxia and cerebellar dysarthria.Our case demonstrates that DVAs, generally considered as common insignificant anatomical variants, can uncommonly lead to significant complications.
ORAL ANTICOAGULANT PRESCRIBING IN A HYPERACUTE STROKE UNIT
Authors: Samani Amit , Kayani Mahaz , Asokanathan Asaipillai 
Published: October 2015

BackgroundNon-vitamin K antagonist oral anticoagulants provide a valuable alternative option to warfarin for thromboprophylaxis for non-valvular atrial fibrillation. NICE has recently released a national consensus statement which will guide local protocol/guideline development. Currently, their use is often governed by physician preference combined with patient factors. We explored the pattern of prescribing in the pre-guideline era.MethodRetrospective analysis of admissions from June–October 2014. Student T-test and Fischer's exact test with Bonferroni corrections were used to compare demographics between groups. A creatinine clearance of <60 umol/L defined renal impairment.Results61 patients, 37 female, mean age 80.8 years were audited. 35 were prescribed rivoroxaban 20 mg, 7 rivoroxaban 15 mg, 11 apixaban 5 mg and 8 apixaban 2.5 mg. None were prescribed dabigatran. Age, CHADS2 and HAS-BLED scores did not show statistically significant variability between subgroups. Renal impairment was signficantly commoner in the apixaban 2.5 mg group compared to the 5mg group (p<0.001) and the rivoroxaban 15mg compared to the 20mg group (P<0.01). There was no difference between the rivoroxaban and apixaban groups.ConclusionCurrently, renal impairment is the main determinant for thromboprophylaxis choice. Local guidelines have been created with physician education underway and re-audit planned in 3 months. The authors will present the full data.
MOTOR NEURON DISEASE: A CASE OF MISGUIDED THERAPEUTIC NIHILISM
Authors: Rossor Alexander , Josifova Dragana , Howard Robin , IsaacsItua Alifa 
Published: October 2015

We report the case of a 62 year old gentleman with sensorineuronal hearing loss beginning in the second decade. At the age of 40 he collapsed and was unable to walk more than a few metres. He was admitted to hospital and diagnosed with a motor neuron disease on the basis of an EMG. Examination at the time revealed bilateral deafness, facial weakness, dysphagia, dysphonia and both proximal and distal weakness.The family history was remarkable for two sisters who died of a similar condition in the second decade. In view of the combination of sensory neuronal deafness and a motor neuronopathy, a diagnosis of Brown Violetta Van Laera (BVVL) syndrome was made.A PEG tube was inserted and the patient was discharged to a nursing home for palliation. After several months, our patient discharged himself, reintroduced thickened fluids resulting in removal of the PEG tube. His clinical condition has continued to improve although he intermittently requires a wheelchair.Genetic testing of the SLC52A3 gene revealed two missense mutations (p.Glu36Lys and p.Val413Ala) confirming the clinical diagnosis of BVVL. SLC52A3 encodes a riboflavin transporter and in case series, riboflavin supplementation has halted or reversed the disease.
INDOMETHACIN TEST USAGE AT UNITED KINGDOM TERTIARY CENTRES OVER 20 YEARS-AN AUDIT
Authors: Bose Pyari , Palethorpe DG , Marin J , Nesbitt A , Lowe J , Goadsby PJ 
Published: October 2015

BackgroundIndomethacin-responsive trigeminal autonomic cephalalagias (TACS), paroxysmal hemicrania (PH) and hemicrania continua (HC), are unique, important syndromes to understand.AimEvaluation of the role of the placebo-controlled indomethacin test (P-Indotest) in the diagnosis of primary headache disorders.MethodPatients notes, clinic letters and headache diaries of patients under our care (PJG) over a 20 year period across various UK Neurology tertiary care centres were examined to obtain data against an audit collection tool. A pre-determined set of data were extracted, and the test procedure reviewed for compliance against a standard with minimum data set requirement by an unblinded observer. The original test score was noted, and prospectively re-scored by a blinded observer.ResultsPatients (n=7; 4 female) were aged from 24 to 51 years. The majority (85%) had side locked headache. P-Indotest was positive in 78% of the cases with patients having either HC or PH. Of all patients tested, 42% had unilateral photophobia and phonophobia and all these cases had a positive response. No serious adverse events were noted. Sleepiness was often reported on the active treatment as was transient worsening of headache in unilateral chronic migraine. Further data is being compiled.ConclusionThe P-IndoTest is a reliable and safe test and should in the diagnosis of patients with possible indomethacin sensitive TACs. While far from ideal, a positive indomethacin test does identify a particular biology and should be retained as a diagnostic marker of PH and HC until its basis is understood.
A FRAMEWORK FOR MANAGING AND MONITORING NEUROLOGY REFERRALS
Authors: Ng Melissa J , Anderson Jane M , Joannides Alexis J 
Published: October 2015

ObjectivesManagement of emergency referrals is an important component of neurology inpatient practice. On this background, we have developed and implemented a robust electronic framework for referral recording and analysis.DesignCodes representing all possible referral outcomes were defined and refined in response to user feedback. Further datasets for presenting symptoms, candidate anatomical syndrome and working diagnosis were developed to enable further epidemiological analysis.MethodsAggregate referral data from the first year of implementation (1/7/13 to 30/6/14) were analysed with respect to demographics, underlying pathology and management outcome.ResultsA total of 1896 referrals were recorded during the study period, with a mean of 5.9±0.22 per day and a constant activity level throughout the year. Weekly and daily variations were observed, with lower levels of activity outside working hours. Age distribution showed a peak at 50–69 years. The commonest diagnostic categories included cerebrovascular pathology, epilepsy/loss of consciousness, and headache/facial pain, which together represented 49.8% of referrals. Of these, headache-related disorders most often required further neurological management (61% of cases), thus representing a greater area of clinical activity.ConclusionsOverall, our results demonstrate the feasibility of efficient referral analysis through structured electronic data capture, with potential implications for service evaluation and epidemiological research.
USING NHS PRIMARY CARE DATA TO IDENTIFY UNDIAGNOSED DEMENTIA
Authors: Jammeh Emmanuel , Carroll Camille , Pearson Stephen , Escudero Javier , Anastasiou Athanasios , Zajicek John , Ifeachor Emmanuel 
Published: October 2015

BackgroundUp to 50% of patients with dementia may not receive a formal diagnosis, limiting access to appropriate services. It may be possible to build a picture of ‘underlying undiagnosed dementia’ from a profile of symptoms recorded in routine clinical practice.AimTo develop a machine learning tool to identify patients who may have underlying dementia but have not yet received formal diagnosis from analysis of routinely collected NHS data.MethodRoutinely collected NHS READ-encoded data were obtained from 18 consenting GP surgeries across Devon, UK, totalling 26,483 patient records of those aged >65 years. 539 Patients were identified as having dementia within the 2 year study period (June 2010 to June 2012). We determined other codes assigned to these patients that may contribute to dementia risk. The dataset was used to train a supervised classifier (Naives Bayes) to discriminate between patients with underlying dementia and healthy controls using a ten-fold cross-validation approach.ResultsThe model obtained a sensitivity of 72.31% and a specificity of 83.06% for identifying dementia.ConclusionRoutinely collected NHS data can be used to identify patients who are likely to have undiagnosed dementia. This type of methodology is promising for increasing dementia diagnosis within primary care.
DURAL AVF CAUSING A TREATABLE CJD LIKE COGNITIVE DYSFUNCTION
Authors: Randall Anna , Ellis Richard James Booth , Hywel Brython , McCoy Christopher Andrew , Davies Rhys , Eldridge Paul Richard , Putharan Mani , Radon Mark , Alusi Sundus 
Published: October 2015

A 67-year-old woman presented with a seven-month history of progressive psychiatric disturbance and sub-acute cognitive decline mimicking Creuztfeldt-Jakob Disease.She presented initially to Psychiatry with worsening anxiety, depression, lassitude, impaired concentration and insomnia. She was given a diagnosis of agitated depression.She subsequently experienced episodes of disorientation, indecision and inability to complete routine, learned tasks as well as visual hallucinations. Initial investigations for causes of subacute dementia were unremarkable including a non-diagnostic MRI. However, five months into her presentation, there was further rapid deterioration with fluctuating consciousness and deteriorating mobility. She became bedbound, incontinent and cortically blind. She had right hemiparesis, hemineglect, extrapyramidal features, and extensor plantars.A 4D computerised tomography angiogram demonstrated a dural arteriovenous (AV) fistula with retrograde filling of superior sagittal sinus and cortical venous congestion. She underwent emergency Onyx embolisation. Over a few weeks, there was gradual improvement in her anxiety, cognition and mobility. She had a mild residual right hemiparesis.Intracranial dural AV fistulae can present with a spectrum of neurological symptoms, including cognitive decline. A lowhigh index of suspicion combined with close liaison between Neuroradiology, Neurology and Neurosurgery ensures prompt diagnosis and maximises the potential for cognitive recovery.
CONFUSION IN CLINICAL TRIALS OF ALZHEIMER'S DISEASE
Authors: Hobart Jeremy , Cleanthous Sophie , Zajieck John , Cano Stefan 
Published: October 2015

BackgroundNeurology clinical trials frequently use clinical outcome assessment instruments (COAs) as outcome measures. We question the extent to which measurement limitations of COAs contribute to clinical trials failures in Alzheimer's disease.ObjectivesWe conducted a series of literature reviews to: identify the concepts assessed in AD clinical trials, and how these concepts were defined and measured; identify which COAs have been used in AD clinical trials, and on which grounds they were selected. We examined published measurement properties of COAs used in AD clinical trials in accordance with FDA guidance.ResultsA set of literature reviews identified >6500 publications from PUBMED/EMBASE inception. In the extracted 850 articles, 984 uniquely named concepts were assessed and 1283 COAs were used. However, few trials provided definitions of the concepts they measured; very different COAs were used to measure the same (named) concepts; the same COAs were used to measure different concepts; COA selection was rarely justified or evidence-based. Further reviews of COA development papers (n=174) indicated most fail to meet recommended criteria.ConclusionsFindings imply substantial measurement confusion in AD clinical trials and suggest they have provided weak evaluations of treatment effectiveness. Findings were replicated in Parkinson's disease (n366 articles).
MINI-ADDENBROOKE'S COGNITIVE EXAMINATION (M-ACE): PRAGMATIC STUDY
Authors: Larner Andrew 
Published: October 2015

ObjectiveTo test diagnostic accuracy of the mini-Addenbrooke's Cognitive Examination (m-ACE) compared to the MMSE for the diagnosis of dementia and MCI in consecutive referrals to a dedicated cognitive disorders clinic.Results: Of 135 consecutive new outpatients seen over 6 months (June–November 2014) administered the mini-ACE (F:M=64:71, 47% female; age range 18–88 years, median 60), 24 were diagnosed with dementia (DSM–IV–TR criteria) and 39 had MCI (Petersen criteria). Using the cutoffs defined in the index paper (≤25/30 and ≤21/30), m-ACE was sensitive (1.00, 0.92) but not specific (0.28, 0.61) for dementia diagnosis; it also proved useful for MCI diagnosis (sensitivities 1.00, 0.77; specificities 0.43, 0.82). Area under the ROC curve was 0.86. Effect size (Cohen's d) for m-ACE for dementia vs. no dementia was 1.53 (large) and for MCI vs no cognitive impairment was 1.59 (large); for MMSE the corresponding figures were 1.56 and 1.26. Weighted comparison suggested a small net loss for m-ACE vs MMSE for dementia diagnosis (–0.13) but a large net benefit for MCI diagnosis (0.38).Conclusions: In this pragmatic study, m-ACE proved quick, easy to use, and acceptable to patients, with metrics comparable to MMSE for dementia diagnosis and better for MCI diagnosis.