bluuebunny/crossref_metadata_2025_split huggingface dataset:
11.15 MS incidence rates vary threefold by health board in scotland, persistently
Authors: Kearns Patrick , Connick Peter , Paton Martin , O’Neill Martin , O’Riordan Jonathon , McDonald James , Colville Shuna , Ascherio Alberto , MacDougall Niall , Chandran Siddharthan 
Published: March 2023

Fifteen regional studies of multiple sclerosis rates have been published in Scotland; spanning some six decades and surveying prevalence, mortality and hospital admissions. However, only one national study has attempted to compare regional rates (using admissions data).We used the Scottish Multiple Sclerosis Register to investigate incidence rates (IR). We present crude and age-adjusted IRs, cumulative incidences, and the sex ratio of cases and rates, for all regions of Scotland: 01/01/2010 to 31/12/2017.The overall IR for Scotland was 8.76/100,000 person-years (standardized: 8.54), which is high, but likely a conservative estimate of the total burden, including only definite cases. The female-male ratio was 2.3. Regional rates and sex ratios varied significantly – up to threefold – between Health Boards (p<10–13), well correlated with the 1997–2009 admissions data (r=0.85, p<0.001). Significant variation was observed on the mainland, not just on the islands. Lifetime risk ranged from 19.9/1000 for females in Orkney to 1.6/1000 for males in the Borders.This study is the first attempt to analyse the patterns of incidence for the whole of Scotland from prospectively collected data and benefits from a high level of completeness and ascertainment. The wide variation in incidence rates and sex-ratios is unexplained.
100 Progressive MRI and EEG changes in a patient with Creutzfeldt-Jakob disease
Authors: Johnson Naomi , Thura Myat , Tun Shwe , Mowafi Walied 
Published: March 2023

We report the case of a 56 year old male, presenting approximately 6 weeks after his first symptoms of Sporadic Creutzfeldt-Jakob Disease. The patient demonstrated classical magnetic resonance imaging (MRI) and electroencephalographic (EEG) changes reflecting clinical progression.Creutzfeldt-Jakob Disease was suspected and later confirmed by positive biochemical markers approximately 10 days after presentation; including positive protein 14-3-3, S-100b and positive rtQUIC assay.Serial MRI and EEG findings correlated with clinical symptoms witnessed on presentation (nystagmus, mild myoclonus and lability of mood) and disease progression through to coma before deathMRI changes were seen as initial localised cortical ribboning progressing to more widespread ribboning and areas of high signal in the basal ganglia (striatum). This correlated with the findings on his earliest EEG of periodic frontal and right side dominant sharp waves, to the last EEG showing more widespread periodic sharp waves.MRI and EEG findings are discussed as well as differentials that may appear with similar MRI findings.The presentation provides an accessible learning case for neurologist, neuro-radiologists and neurophysiologists at all levels.
104 Utility and incidental findings on CT chest, abdomen and pelvis scans in neurology patients
Authors: Monaghan Ian , Fernandes Peter 
Published: March 2023

CT scanning of the chest, abdomen and pelvis (CT-CAP) is often used by neurologists to identify underlying disease processes such as malignancy or sarcoidosis. However, the utility of CT-CAP, and the rate of incidental findings, remains uncertain.This retrospective study investigated all CT-CAP requests by the neurology department in Edinburgh over a 5-year period (n=261), combining CT-CAP reports with electronic patient records. Incidental findings were classified by need for further investigations. Category 1 findings required further investigations (e.g. pulmonary nodule >4 mm). Category 2 findings required extra tests depending on clinical context (e.g. dilated biliary tract; liver function and surgical history determining need for further investigations). Category 3 findings did not require further investigations (e.g. simple diverticular disease).20% (n=51) of CT-CAP scans were reported as showing an underlying malignancy, with half (n=26) confirmed as cancer by subsequent investigations. CT-CAP identified three cases of sarcoidosis and one of tuberculosis. There were 191 category 3 incidental findings, 57 category 2 incidental findings, and 27 category 1 findings.Overall, CT-CAP shows an underlying cause of neurology symptoms in 11.5% of cases. The proportion of patients needing further tests due to category 1 or concerning category 2 incidental findings (12/57) was higher at 14.9%.
107 Secondary intracranial hypertension in nephropathic cystinosis
Authors: Harijanto Chloe , Pegg Emily , Biswas Sumito , Lavin Tim 
Published: March 2023

A 21-year-old woman presented with bilateral papilloedema and a 2-week history of high-pressure headaches. Medical history was remarkable for Infantile Cystinosis and a renal transplant.Her Body Mass Index was 26.7 kg/m2. Visual acuity (VA) was 6/12 in the left eye and 6/6 in the right. Cerebrospinal Fluid (CSF) opening pressure was 30cmH20 in the left decubitus position.Following lumbar puncture and Acetazolamide 250 mg bd, VA improved but the latter was stopped due to renal side effects. She was asymptomatic on Topiramate 50 mg bd. VA was 6/6 bilaterally, with decreased optic disc swelling on Optical Coherence Tomography and a normal CSF opening pressure.DiscussionInfantile Cystinosis is a rare autosomal recessive lysosomal storage disease where multisystem accumulation of cysteine leads to renal failure, hypothyroidism and corneal deposits.Intracranial Hypertension occurs in around 5% of patients with cystinosis (Dogulu 2004). Meningeal cysteine accumulation could reduce CSF absorption by the arachnoid villi. Other risk factors include renal transplant and immunosuppressant therapy.Neurologists should be aware of this rare but important complication of cystinosis. Low dose topiramate demonstrated benefit here and lacks the diuretic effects of acetazolamide. Early shunting or optic nerve decompression should be considered in severe cases.
111 An audit of headache referrals from primary care, and subsequent management in the outpatient clinic
Authors: Fernandes Linford , Tanti Matthew , Randall Marc , Cosgrove Jeremy 
Published: March 2023

BackgroundHeadache is the most common reason for referral to a neurology outpatient clinic but many headaches can be managed in primary care.MethodsWe audited 100 consecutive headache referrals against Leeds Teaching Hospitals NHS Trust (LTHT) referral criteria and the Neuro Network Vanguard (NNV) headache pathway.ResultsThe mean age was 41 years (range 16 -90), 67% were female and 31% had previously seen a neurologist for headaches. Referrals were appropriate in 44% and 49% according to LTHT and NNV guidelines, respectively. Migraine was the suspected diagnosis in 50% of referrals - half of these tried two or more preventative medications. The clinic consultations of 89 of the 100 referrals were reviewed: 72% were diagnosed with migraine (of which 53% were chronic). Following consultation, migraine was the final diagnosis in 49% of referrals that proposed an alternative diagnosis; 9% referred as suspected migraine received a different diagnosis.ConclusionLess than half of the referrals received were appropriate according to LTHT and NNV criteria. Migraine was the major referral reason and ultimate diagnosis. Empowering primary care to manage migraine is an urgent local priority and may reflect a national issue.
112 Spontaneous intracranial hypotension presenting as postural head tremor and gait ataxia
Authors: Bangalore Priyadarshini , Jadun Changez , Davies Brenden , Abraham Roby 
Published: March 2023

We present a 65 year old lady with 30 year history of chronic daily headache without postural changes after a road traffic accident presented with three months history of postural head and upper limb tremor, progressive ataxia, dizziness, mild confusion and few falls. She had worsening of epilepsy with partial status prompting acute hospital admission. Symptoms exacerbated on standing and she preferred lying down most of the time. Previous history of temporal lobe epilepsy and Anterior Cervical Decompression and Fusion.Examination: She had a head tremor which exaggerated on upright posture along with truncal ataxia, gait ataxia and dysmetria bilaterally. Head and upper limb tremor improved on lying down position without any change to her headache. She did not have any other pyramidal or extrapyramidal features. Increasing Lamotrigine dosage and adding Levetiracetam helped control seizures.CT head and two MRI head scan showed radiological features of low CSF pressure syndrome. There were small bilateral subdural hygromas, uniform meningeal enhancement thought the cranium extending to cervical spinal cord dura, and dural-venous-sinus engorgement. MRI spine did not show any CSF leak. CSF opening pressure was 12.0 cm of H20 with normal constituents.A blind blood patch was performed with excellent outcome, her postural tremors, chronic headache and gait ataxia improved when reviewed at week one.Low CSF pressure syndrome presenting as Movement disorder is rare and recognising atypical presentation is cardinal.
113 Incidence of migraine and treatment patterns in UK primary care
Authors: Yelland Eleanor , Ghosh Rebecca , Murray-Thomas Tarita , Williams Alison , Hacking Victoria 
Published: March 2023

ObjectivesTo estimate the incidence of migraine and describe treatment patterns for an indicative year.MethodsPatients ≥18 years old in the Clinical Practice Research Datalink with an incident diagnosis of migraine between 01/04/2007–31/07/2017. The incidence of migraine was estimated. Treatment groups were categorised as none, acute only, prophylactic only, acute and prophylactic. 12-month treatment patterns amongst patients initiating prophylactic treatment with amitriptyline, propranolol or topiramate were classified as continuous use, augmentation, switching or discontinuation. Treatment adherence was defined as medication possession ratio (MPR); patients were ‘adherent’ if their MPR was ≥0.80.Results89,442 patients with incident migraine were included. Mean age at diagnosis was 40 years [SD 15]; 76.6% were females. The cumulative incidence of migraine rate was 25.8/10,000 person-years [95%CI: 25.6–26.0]. 86.7% of patients received prophylactic and/or acute treatment; 37.6% and 20.1% had received ≥2 and ≥3 prophylactic treatments, respectively.1,636 patients initiated prophylactic treatment in 2015. Propranolol (47.8%) and amitriptyline (47.4%) were most commonly prescribed. Although 80–85% of patients were adherent while on treatment, only 20–30% continued treatment up to 12 months.ConclusionsMigraine treatment remains a challenge. Most patients receive one or more pharmacotherapies but the majority discontinue prophylactic treatment within 12 months of initiation.
115 Economic burden among migraine patients: United Kingdom perspective
Authors: Martelletti Paolo , Schwedt Todd , Lanteri-Minet Michel , Diener Hans-Christoph , Torre Elena Ruiz de la , Craven Audrey , Evans Simon , Walsh Donna , Dumas Paula , Laflamme Annik 
Published: March 2023

ObjectivesAs part of the worldwide My Migraine Voice survey, this study evaluated the real-world healthcare resource utilization (HRU) and impact on work and activity impairment among people living with migraine in the United Kingdom (UK).MethodsAn analysis was conducted using UK subset data from My Migraine Voice survey: a worldwide, cross-sectional, online study including respondents (≥18 years), with ≥4 monthly migraine days, with 90% having used preventive migraine treatment in the preceding three months. HRU (Emergency room [ER] visits and hospitalisations) and impact of migraine on work and daily activities were evaluated and compared amongst different preventive treatment failure (TF) patient subgroups.ResultsOf the 690 respondents, 17% had visited ER (average 2.8 times/year), and 13% were hospitalised (average 3.0 days). Participants reported 13% missed working time (absenteeism), 52% reduction in productivity (presenteeism), and 58% reduction in both overall work productivity and daily activities due to migraine in last 7-days. HRU, absenteeism and presenteeism was higher in those with ≥2 TF vs patients with no TF.ConclusionThis study demonstrates the burden among patients with migraine in terms of HRU, work productivity loss, and activity impairment. The impact is increased in patients who have had ≥2 preventive treatment failures.
119 Double jeopardy or VOMIT syndrome in trigeminal autonomic cephalalgias
Authors: Thaller Mark , Davies MB 
Published: March 2023

This is a rare presentation of a cluster-like headaches which poses diagnostic and management dilemmas in light of two secondary headache pathologies being identified.A female in her seventies presented with a 12 month history of worsening intensity and frequency left-side locked, excruciating, searing and stabbing craniofacial pain with associated eye watering and orbital oedema. Duration was 1–2 hours up to 4 times per week. There was never more than 3 weeks between attacks and was pain free in-between. There was a long term milder infrequent but similar headache and migraine with aura in her twenties reported.The atypical features (older age of onset, female, frequency and limited autonomic features) as well as pattern change and lack of remission triggered further investigation. Her MRI head showed both a left cavernous sinus aneurysm and a pituitary adenoma.Both cavernous sinus aneurysm and pituitary pathologies have individually been shown to ‘mimic’ cluster headache but dual pathology hasn’t been reported. Several authors suggest screening for pituitary pathology in patients with TACs and others for vascular lesions. However this can add an additional management dilemma when the case could be a Victim of Modern Imaging Technology (VOMIT) (incidentalomas).
14.57 Expanding the phenotypic spectrum of PSP: Findings from the PROSPECT-UK study
Authors: Jabbari Edwin , Woodside John , Costantini Alyssa , Lees Andrew , Morris Huw ,   on behalf of the PROSPECT-UK consortium
Published: March 2023

BackgroundProgressive supranuclear palsy (PSP) is a common cause of atypical parkinsonism.ObjectiveTo study the genetic and clinico-pathological profile of various PSP phenotypes using patient data from the PROSPECT-UK study.MethodsClinical data from PSP patients in the PROSPECT-UK study was analysed to assess the rates of various PSP phenotypes, as defined by the 2017 Movement Disorder Society diagnostic criteria. A subset of patients are currently undergoing 5 year longitudinal follow-up, including genetics, video clinical assessments and post-mortem confirmation of diagnosis.ResultsWe have established a cohort of 345 patients with PSP. The longitudinal sub-cohort consists of 100 PSP patients: 58 patients with classical Richardson’s syndrome (RS) and 42 patients with a variety of non-RS presentations including PSP-Parkinsonism and Pure Akinesia with Gait Freezing. We found significant differences in the mean disease duration of deceased RS and non-RS cases (5.6 vs 9.2 years) but similar rates of PSP pathology in cases (n=28) with post-mortem confirmation (94% and 100%). Our PROSPECT-UK data has contributed to a GWAS which identified that common variation at the TRIM11 locus determined PSP phenotype (Jabbari et al 2018). In addition, we have identified 1 case with a MAPT L284R mutation and 1 case with genetic and biochemically confirmed Niemann-Pick type C disease.ConclusionsThe PROSPECT-UK study has furthered our knowledge on the clinical profile of PSP and the biological determinants which drive phenotypic variation.
127 Audit of poor response to botulinum toxin in cervical dystonia
Authors: Tucker Harry , Osei-Poku Foster , Ashton Diane , Lally Rachael , Alty Jane , Kobylecki Christopher 
Published: March 2023

BackgroundBotulinum toxin (BoNT) is an effective first-line treatment for cervical dystonia. However, secondary non responsiveness to BoNT treatment remains a key reason for a discontinuation rate of 20%.In 2016 the British Neurotoxin Network (BNN) published recommendations for the management of cervical dystonia patients with poor response to BoNT treatment.AimsTo compare management of patients with secondary non responsiveness in two regional neuroscience centres with the BNN guidelines.MethodsWe retrospectively analysed 68 patients with cervical dystonia who met criteria for secondary non responsiveness to BoNT-A treatment.ResultsSuboptimal response to BoNT-A was recorded in 37 patients (54%), whilst 31 (46%) had no therapeutic response.In the ‘suboptimal response’ group, 21 (57%) had a subsequent good therapeutic response with adjustment of dose, muscle selection and injection technique and continued BoNT-A treatment. Of the remainder, 6 (38%) were switched to BoNT-B and 7 (44%) were referred for Deep Brain Stimulation surgery.In the ‘no response’ group 6 patients (19%) had a good therapeutic response with adjustments of dose, muscle selection and injection technique and continued BoNT-A treatment. In this group 22 patients (71%) were assessed for BoNT-A resistance, which was confirmed in 8 (36%).ConclusionOur audit shows the importance of careful assessment of patients with cervical dystonia presenting with secondary non-reponsiveness to BoNT-A therapy. The BNN recommendations provide a useful framework for improving dystonia treatment.
128 Efficacy and safety of deep brain stimulation in Parkinson’s disease: systematic review and meta-analysis
Authors: Bratsos Sosipatros , Karponis Dimitrios , Saleh Sohag 
Published: March 2023

BackgroundDeep brain stimulation (DBS) is a neurosurgical procedure indicated in patients with advanced Parkinson’s disease (PD). This systematic review and meta-analysis aimed to investigate the efficacy and safety of DBS in comparison to best medical therapy (BMT) in the treatment of PD.MethodsA systematic search was performed in Medline, Embase and Central. Randomised controlled trials (RCTs) comparing DBS to BMT in PD patients were included. Outcome measures were impairment/disability (UPDRS), quality of life (PDQ-39), levodopa equivalent dose (LED) and rates of serious adverse events (SAE).ResultsEight eligible RCTs (n=1189) were included in the meta-analysis. Regarding efficacy outcomes, there were significant improvements in UPDRS, PDQ-39 and LED in favour of DBS (P<0.00001). The risk of SAE was significantly higher in the DBS group (P=0.005), as was the total number of SAE (P<0.0018).ConclusionsOverall, DBS was found superior to BMT at improving impairment/disability, quality of life and reducing medication doses, but these benefits need to be weighed against the higher risk of SAE.
136 Mitochondrial biomarkers in parkinson’s disease
Authors: Davis Ryan L , Wong Siew L , Carling Phillipa J , Payne Thomas , Sue Carolyn M , Bandmann Oliver 
Published: March 2023

BackgroundThere is strong evidence of mitochondrial dysfunction in both familial and sporadic Parkinson’s disease (PD). A biomarker reliably identifying mitochondrial dysfunction would be particularly important for future stratified/personalized medicine trials in Parkinson’s disease. A previous comparison of serum biomarkers in mitochondrial disease established that serum growth differentiation factor 15 (GDF-15) outperforms fibroblast growth factor-21 (FGF-21) and distinguishes patients with mitochondrial diseases from those without them.ObjectiveTo systematically assess GDF-15, FGF-21 and mitochondrial DNA copy number (mtDNA) levels in the serum of patients with sporadic PD and controls to determine their utility as a suitable mitochondrial biomarker panel for PD.Methods120 patients with PD and 102 age-matched controls were recruited from a single centre. GDF-15, FGF-21 and mtDNA copy number were quantified using previously established assays.ResultsFGF-21 concentrations did not significantly differ between groups. GDF-15 serum levels were significantly raised in late onset PD compared to controls and early onset PD. There was no difference in mtDNA between groups. None of the biomarkers tested showed a significant association with disease following multivariate logistic regression analysis.ConclusionSerum FGF-21, GDF-15 and blood mtDNA could not accurately differentiate between PD and controls when assessed as candidate biomarkers.
137 Usability testing of a non-motor symptom app in PD
Authors: Dominey Thea , Mullin Stephen , Edwards Emma , Whipps John , Whipps Sue , Carroll Camille 
Published: March 2023

BackgroundNon-motor symptoms (NMS) are a significant cause of morbidity in Parkinson’s, with major impact on quality of life. There is an urgent need to empower people with Parkinson’s (PwP) to self-manage NMS, and facilitate timely intervention. A mobile application (NMS Assist) was developed, which enables regular assessment of NMS and provides self-help information in the form of animations.AimTo evaluate key areas for NMS Assist design improvement.MethodPwP and carers were recruited from local Parkinson’s UK groups. NMS Assist user journeys were designed and carried out, filmed with ‘Mr Tappy’, using ‘think aloud’ methodology. Satisfaction was evaluated by the System Usability Scale (SUS).Results13 Participants were recruited (PwP, n=9 and carers, n=4) with varying age, cognition, disease severity and smartphone experience. Amendment need was identified in three key areas: navigation, content and accessibility. There was no difference in SUS ratings between experienced and never smartphone users. However, the experienced group had a significantly higher error free task completion rate than never users (p=.01).ConclusionsRefinements to NMS Assist design have been identified and implemented to improve its usability. The effectiveness of these interventions will be tested in future rounds of usability testing, leading to in-service evaluation.