bluuebunny/crossref_metadata_2025_split huggingface dataset:
WED 253 An atypical presentation of sneddon syndrome
Authors: Cleaver Jonathan , Renowden Shelley , Teo Mario , Clatworthy Philip 
Published: October 2018

A 50-year-old female was admitted following a subacute and increasing headache, numbness in both hands and feet, generalised weakness and confusion.An MRI brain revealed an acute left temporal infarct with multi-focal established infarcts. MR angiography demonstrated marked occlusive disease affecting terminal internal carotid artery and both middle cerebral and posterior cerebral arteries, in a potential Moyamoya pattern.Lumbar puncture, extensive blood tests and echocardiography were unremarkable. A skin biopsy showed intimal thickening of the deep dermal arteries compatible with a diagnosis of Sneddon Syndrome. Livedo reticularis was absent and antiphospholipid antibodies negative. Antiplatelet therapy only was commenced given her seronegativity and Moyamoya.DiscussionSneddon syndrome is an uncommon disorder, characterised as generalised livedo reticularis with stroke (Sneddon, 1965). It is an increasingly recognised cause of ischaemic stroke in young adults, however, its clinical course remains poorly defined in the literature (Boesch et al. 2003). It is increasingly associated with Moyamoya syndrome, posing a challenge in terms of anticoagulation in these patients (Fierini et al. 2015). To our knowledge, this is only the second reported case without livedo reticularis (Marianetti et al. 2011) - highlighting the importance of skin biopsy - and the first with this clinical and radiological combination.
WED 255 SSRIS and risk of intracranial haemorrhage
Authors: Jensen Melanie P , Ziff Oliver J , Banerjee Gargi , Ambler Gareth , Werring David J 
Published: October 2018

The abstract has also been presented as an oral presentation at the ESOC conference.Background and aimsObservational studies have suggested increased risk of intracranial haemorrhage (ICrH) in patients receiving selective serotonin reuptake inhibitors (SSRI). We sought to clarify the impact of SSRI on ICrH, accounting for study methodology.MethodA comprehensive search of Medline, Embase and Cochrane from 1960 to December 2017 comparing SSRI with control. ICrH was meta-analysed using a random-effects model and the review was prospectively registered (PROSPERO:CRD42017084513).Results25 observational studies, but no randomised trials, were available for meta-analysis, with a combined total of 4,843,857 patient-years follow-up. Those treated with SSRI were more likely to have depression (p<0.001) and be female (p=0.04). Compared to control, SSRI were significantly associated with first-ever ICrH (RR: 1.31; 95% CI: 1.15 to 1.49); however, in survivors of ICrH there was no association between SSRI and recurrence (0.95, 0.83–1.09). Sensitivity analyses revealed a greater association between SSRI and ICrH in studies with a high risk of bias (p<0.001) than those with a lower risk of bias (p=0.10).ConclusionSSRI are associated with increased risk of first-ever ICrH, but not with recurrence. These findings, based solely on observational data, should be taken with caution due to fundamental differences in patients receiving treatment, highlighting the need for randomised trials.
THUR 264 Multimodal intervention to improve lumbar puncture
Authors: Vanessa Tobert , James Allen , Evan Edmond 
Published: October 2018

IntroductionLumbar puncture (LP) is a routine procedure performed for diagnostic and therapeutic indications for more than 120 years.1 For as long, post-dural puncture headache (PDPH) has been a complication that can be persistent and severe.2 Finer gauge needles and ‘atraumatic’ needle designs3 significantly reduce the incidence of PDPH.4–6MethodsA multimodal intervention consisting of teaching session on atraumatic needle usage, simulation training with 25G Sprotte needles, and electronic LP proforma was introduced in October 2017.Records for all patients having LP in the Neurology day-case unit at the John Radcliffe Hospital, Oxford, in the months of November 2016 and Nov/Dec 2017 were retrospectively reviewed for documentation and atraumatic needle usage.Results39 records were reviewed from Nov/Dec 2017 and 16 from November 2016. Documentation was significantly improved across all criteria assessed except for documentation of informed consent. Atraumatic needle usage increased from none documented pre-intervention to 38% (n=11 out of 29 where atraumatic needle indicated) post intervention.ConclusionProforma use was associated with improved LP documentation. Atraumatic needle usage increased significantly post-intervention. These results are consistent with previous studies on changing behaviour in LP technique in neurology.7 Large scope for further improvement exists.Abstract THUR 264 Figure 1References1. Quincke H. Die Lumbalpunction des Hydrocephalus -Heinrich Quincke -Google Books 1891. Klin. Wochenschrist. pp. 929–965.2. Greene HM. Lumbar puncture and the prevention of postpuncture headache. JAMA J. Am. Med. Assoc 1926;86:391.3. Hart JR, Whitacre RJ. Pencil-point needle in prevention of postspinal headache. J. Am. Med. Assoc 1951;147:657.4. Vallejo MC, Mandell GL, Sabo DP, Ramanathan S. Postdural puncture headache: A randomized comparison of five spinal needles in obstetric patients. Anesth. Analg 2000;91:916–20.5. Thomas SR, Jamieson DR, Muir KW. Randomised controlled trial of atraumatic versus standard needles for diagnostic lumbar puncture. BMJ 2000;321:986–90.6. Halpern S, Preston R. Postdural puncture headache and spinal needle design. Metaanalyses. Anesthesiology 1994;81:1376–83.7. Davis A, et al. Change practice now! Using atraumatic needles to prevent post lumbar puncture headache. Eur. J. Neurol 2014;21:305–11.
WED 270 Optician’s diagnosis of papilloedema impact hospital admissions
Authors: Sarah Williamson , David Whiteside , Thomas Coysh , Vinod Metta , Fatima Geranmayeh , Alex Everitt , Carolyn Gabriel , Barry Seemungal , Joanna Ball , Richard Nicholas 
Published: October 2018

BackgroundIn a landmark case in August 2016 an optometrist was found guilty of missing papilloedema. We examined the current referral pathway for managing papilloedema to determine whether initial assessment by an optician impacted resource use.MethodsDischarge summaries from St Marys Hospital (SMH) between 1/1/17 and 16/5/17 the referral centre for the Western Eye Hospital (WEH) accident and emergency (A and E) were searched to identify subjects with suspected papilloedema. Analysis of the patients journey was performed.ResultsOf 76 patients identified, 26 were excluded either due to being<18 years old or were treated elsewhere. Of the 50 included, 25/50 were seen by an optician prior to a WEH A and E ophthalmologist, 75% of these were referred for urgent hospital assessment with an admission rate of 45.8%. Of 19/50 assessed by WEH A and E only there was a significantly lower urgent assessment referral rate (75% vs 52.6%, p=0.02) and admission rate (45.8% vs 21%, p=0.02). Of the 6/50 patients finally found to have normal discs, 5 were initially referred by opticians.ConclusionReferral by optician prior to ophthalmology A and E assessment is more likely to result in rapid referral for urgent hospital assessment and inpatient admission.
276 A new face for an old foe?
Authors: Gavinda Sangha , Anthony Bron , Michael Donaghy , Cerys Evans , Alison Hardcastle , Kevin Talbot 
Published: October 2018

BackgroundMutations in the protein gelsolin result in hereditary systemic amyloidosis, characterised by onset of corneal lattice dystrophy in the third decade, a slowly progressive cranial neuropathy and cutis laxa. To date four dominantly inherited gelsolin mutations have been identified, two mutations resulting in the classical syndrome and two mutations resulting in renal-predominant amyloidosis.MethodsWe identified a family in which four individuals in three generations presented with corneal lattice dystrophy and neurological symptoms. Detailed clinical neurological and ophthalmological assessment and investigations including neurophysiology and imaging were performed. Whole exome sequencing was undertaken in three family members.ResultsWhole exome sequencing revealed a novel variant in the gelsolin gene (c. G1738A; p. E580K), dominantly inherited and predicted to be pathogenic. Examination, neurophysiological testing and imaging revealed the presence of distal upper limb weakness and wasting, corneal lattice dystrophy and cervical myelopathy in all affected family members.ConclusionThe E580K mutation described in this family is in a conserved calcium-sensitive actin-binding domain that displays sequence homology with other actin-depolymerising proteins. Mutations in this domain may result in abnormal gelsolin-actin interactions and changes in calcium sensitivity may render the protein susceptible to the same aberrant proteolytic cascade as with other known mutations.
288 Challenges in reducing hospital deaths in multiple sclerosis
Authors: Richard Nicholas , Emma Nicholas , Jonathan Koffman 
Published: October 2018

IntroductionFifty-five percent of people with multiple sclerosis (pwMS) will die in hospital, above the national average. This study aimed to determine factors that impact place of death for pwMS.MethodsRetrospective sequential notes review from the UK MS Tissue Bank.ResultsThirty notes were reviewed from 18/06/12% to 08/09/16. 53% died in hospital, 23% in nursing homes, 13% at home and 10% in hospices. Health interventions increased in year of death versus year prior to death in community (p=0.0369) and primary care (p=0.002) but not in secondary care but were not associated with a non-hospital death (NHD). Recognition of dying (73%, p=0.0024) and having an advanced care plan (ACP) (67%, p=0.0003) were associated with NHD. Family involvement was associated with recognition of dying (p=0.0146) but not with NHD. Multivariable analysis found recognition a person is dying and having an ACP were independently predictive of NHD (R2=0.52, p=0.034). The mean time prior to death of having an ACP (9.6±8.1 months) was months prior from when dying was recognised (17.4±27 days, p=0.0004).ConclusionHaving an ACP and recognition a pwMS is dying are key factors to achieving a NHD. This study highlights the challenges to reducing hospital deaths in MS.
293 Autoimmune autonomic ganglionopathy: the NHNN experience
Authors: Shiwen Koay , Bethan Lang , Gordon Ingle , Merete Hagen Ellen , Christopher Mathias , Laura Watson , Michael Lunn , Fion Bremner , Angela Vincent , Valeria Iodice 
Published: October 2018

BackgroundAutoimmune Autonomic Ganglionopathy (AAG) is a rare, immune-mediated condition characterised by subacute pandysautonomia. 50% have auto-antibodies to the ganglionic nicotinic acetylcholine receptor (gAChR), affecting synaptic transmission at autonomic ganglia.MethodsWe describe 13 patients (5 female, median age 47) presenting with widespread autonomic failure, confirmed on cardiovascular, sudomotor and pupillometry testing at the National Hospital for Neurology and Neurosurgery (NHNN), and high gAChR antibody levels (>200 pm) measured by radioimmunoprecipitation assay at Oxford University.ResultsOf the 13 patients, 8 had other autoimmune conditions, 3 had antecedent infection and 3 were paraneoplastic. All had orthostatic hypotension, gastro-intestinal and urinary symptoms, 11 had documented pupillary abnormalities (9 mixed sympathetic and parasympathetic deficits, 2 subclinical sympathetic deficits), 11 had secretomotor dysfunction, 8 had generalised/partial anhidrosis, 8 had sexual dysfunction and 7 had evidence of small fibre dysfunction on neurophysiology.Ten received immunomodulatory treatment; 6 plasma exchange and 4 combination treatment including intravenous immunoglobulin, mycophenolate and rituximab. Earlier treatment was associated with greater clinical response.DiscussionPatients with AAG and high gAChR antibody levels have widespread dysfunction of the autonomic nervous system, which can respond dramatically to immunomodulation. Further research is needed to develop robust clinical biomarkers to guide treatment and monitor response.
294 Neurofilament light protein as a biomarker for huntington’s disease
Authors: Lauren Byrne , Filipe Rodrigues , Eileanoir Johnson , Rana Solyu Kucharz , Sarah Gregory , Rachael Scahill , Maria Björkqvist , Henrik Zetterberg , Sarah Tabrizi , Edward Wild 
Published: October 2018

BackgroundNeurofilament light protein (NfL), a component of the axonal cytoskeleton, has been shown to be increased in cerebrospinal fluid (CSF) and blood and to respond to successful treatment in several neurological diseases. We set out to investigate NfL as a potential biomarker for Huntington’s disease (HD).MethodsWe studied NfL in plasma from 298 participants, in plasma and CSF in 37 participants, and in the R6/2 HD mouse model.ResultsNfL concentration was increased in plasma at every stage of HD including premanifest mutation carriers, rose with progression and had a striking relationship with HTT CAG repeat length. In premanifest HD, baseline plasma NfL predicted subsequent motor onset even after adjustment for age and CAG repeat length. NfL predicted clinical, cognitive and neuroimaging progression, and CSF and plasma levels were strongly associated (Byrne et al, Lancet Neurology 2017). VBM analysis revealed that NfL level predicted atrophy throughout the white matter and in the occipital grey matter (Johnson et al, Neurology 2018). In the R6/2 mouse model, NfL was increased in plasma and CSF and associated with brain volume and clinical measures (Soylu Kucharz et al, Scientific Reports 2017).ConclusionsNfL is a promising clinical and translational biomarker for HD.
WED 027 Acute neurology in glasgow
Authors: M Brennan , A Tyagi , JP Leach 
Published: October 2018

Provision of acute liaison in-patient neurology reduces demand on neurology out patient services, reduces unnecessary investigations and use of medical beds by patients waiting on in-patient neurology review and allows speedier access to necessary neurological services for those with a neurological illness.The pressure on acute neurology beds at QEUH Glasgow is immense and there is considerable delay in patients waiting for admission to the ward. An audit of the acute on call service in mid 2015 showed a 100% increase in the number of phone calls received by the on call registrar when compared to a similar audit in 2008. The number of requests for ward visiting to review medical inpatients at the Queen Elizabeth University hospital increased by more than 100% over the previous year.In June 2016 an Acute Neurology rota was introduced whereby a Consultant Neurologist supervised and delivered patient care for the acute neurology wards, referrals from medical wards and acute receiving, as well as twice a week acute neurology clinics. This has led to a significantly improved care for patients referred with neurological problems as perceived by trainees, consultants and referring medical physicians, as evident on a survey carried out in 2017.
WED 033 Minimum prevalence of non-compliance recorded in an audit of antenatal care in a district general hospital joint obstetric epilepsy
Authors: C Smyth , A Gornall , J Bowen 
Published: October 2018

Epilepsy is among the leading indirect causes of death in pregnancy. A retrospective case review of 143 DGH patients with seizures (50 Idiopathic Generalised, 72 Focal 19 Dissociative) seen in a joint obstetric neurology antenatal clinic from 2015–2017 was undertaken. Systematic data collection was not possible owing to the retrospective nature of the audit, however, salient findings were: Of the cohort of 143, 25 were not taking any AED at booking (around 12 weeks). Of those on AEDs over 80% were on lamotrigine or Levetiracetam monotherapy. Most (87/143) had been seizure free pre-conception for at least 1 year. 11 of this group had breakthrough seizures. AED levels were checked on 57 patients (Lamotrigine – 48:Levetiracetam 9) and were undetectable in 10/57 (17.5%) at booking. Reasons for AED testing refusal and non-concordance (when present) were unknown. Of 11 patients losing seizure control during pregnancy 3 were non-concordant at booking.ConclusionNon concordance at booking was documented in 1 in 6. Loss of previous seizure control occurred in 11/87 (12.6%) of previously seizure free patients of whom 3/11 were nonconcordant. AED monitoring revealed significant non-concordance and may relate to subsequent loss of seizure control. A prospective study is planned.
THUR 058 Eslicarbazepine acetate as monotherapy in clinical practice
Authors: Rob McMurray , Norman Delanty , Vicente Villanueva 
Published: October 2018

PurposeTo assess eslicarbazepine acetate (ESL) as monotherapy in everyday clinical practice.MethodEuro-Esli was a pooled analysis of 14 European studies. In a subanalysis, data were compared for patients treated initially with ESL monotherapy versus adjunctive therapy, and for patients treated at last visit with ESL monotherapy versus adjunctive therapy.Assessments included responder rate (≥50% seizure frequency reduction), seizure freedom rate (seizure freedom at least since prior visit) and incidence of adverse events (AEs).ResultsESL was used as monotherapy in 88/2045 and 229/1340 patients initially and at last visit, respectively. At 12 months, responder and seizure freedom rates were significantly higher in patients treated initially with ESL monotherapy versus adjunctive therapy (responder: 94.1% versus 74.8%; seizure freedom: 88.2% versus 39.0%), and in patients treated at last visit with ESL monotherapy versus adjunctive therapy (responder: 93.2% versus 70.4%; seizure freedom: 77.4% versus 25.9%). Overall incidence of AEs was similar in patients treated initially with ESL monotherapy and adjunctive therapy (29.4% versus 34.4%), and in patients treated at last visit with ESL monotherapy and adjunctive therapy (27.1% versus 30.8%).ConclusionESL was significantly more effective when used as monotherapy compared with adjunctive therapy; safety/tolerability was generally comparable.Supported by Eisai
WED 063 Eslicarbazepine acetate switch from carbamazepine or oxcarbazepine
Authors: Jukka Peltola , Rob McMurray , Vicente Villanueva 
Published: October 2018

PurposeTo investigate eslicarbazapine acetate (ESL) in patients transitioning from carbamazepine or oxcarbazepine in clinical practice.MethodEuro-Esli was a pooled analysis of 14 European studies. Data were analysed for patients transitioning from carbamazepine and oxcarbazepine to ESL due to lack of efficacy or poor tolerability. Responder rate (≥50% seizure frequency reduction) and seizure freedom rate (seizure freedom at least since prior visit) were assessed after 3, 6 and 12 months of ESL treatment, and at last visit. Safety/tolerability analysis evaluated adverse events (AEs) and ESL discontinuation due to AEs.ResultsEuro-Esli included 2058 patients; 233 (11.3%) transitioned from carbamazepine and 134 (6.5%) transitioned from oxcarbazepine. After 12 months, responder and seizure freedom rates for patients transitioning from carbamazepine due to lack of efficacy (n=163) were 70.0% and 30.9%, respectively. Corresponding values for patients transitioning from oxcarbazepine due to lack of efficacy (n=90) were 57.1% and 25.0%, respectively. Among patients who transitioned from carbamazepine (n=64) and oxcarbazepine (n=61) due to poor tolerability, 26.6% and 39.5% experienced AEs; 8.3% and 6.8% discontinued ESL due to AEs, respectively.ConclusionESL may be a useful option for patients experiencing intolerable AEs or not achieving adequate control with carbamazepine or oxcarbazepine.Supported by Eisai.
THUR 090 Idiopathic intracranial hypertension – towards a streamlined pathway
Authors: Vivek Vijay , Laura Grant , Anindyt Nagar , Anish Bahra 
Published: October 2018

Recent studies have yielded the first evidence-based treatments for idiopathic intracranial hypertension (IIH) and shed light on many aspects of this condition, providing an opportunity to streamline the assessment and management of patients with IIH.This study highlighted the inefficiency of most patient pathways, especially when via outpatients, with resulting delays in diagnosis and /or management. Of note, 37% of patients did not have venography. Up to a third of patients with cerebral venous sinus thrombosis present with isolated signs of elevated intracranial pressure, thus clinically mimicking IIH and emphasising the importance of venography acutely.Acetazolamide was appropriately commenced but rarely titrated to the maximum dose of 4 g/day as supported by the IIHTT. Over 40% of our cohort had a diagnosable co-morbid primary headache disorder which persisted and required additional headache-specific medication in almost half of these. This is in keeping with studies showing that headache is unrelated to raised intracranial pressure in IIH patients, and headache-specific management is a key aspect to the management of IIH patients.In summary, this study highlights significant delays and missed investigations when not performed acutely, emphasising the importance of implementing a clear and visible pathway for the assessment and management of IIH patients.
THUR 103 Chorea associated with atrial myxoma
Authors: Sarah Healy , Booth Ellis Richard James , Malcolm Steiger 
Published: October 2018

BackgroundChorea is a hyperkinetic movement disorder characterised by involuntary, unpredictable muscle contractions and is associated with a broad range of primary and secondary aetiologies.CaseA 73-year-old gentleman presented with a 12 month history of progressive behavioural change and involuntary movements. His family had become concerned about periods of erratic behaviour and confusion. He appeared agitated and restless. On examination he had choreiform movements predominantly affecting his upper limbs. An abnormal diastolic heart sound was detected on auscultation.MRI brain and CSF analysis were normal; ASO titre, paraneoplastic, anti-NMDAr antibodies and Huntington’s genetics were negative. A blood film was normal. Transoesophageal echocardiogram demonstrated a variegated mobile mass within the left atrium.An immune-mediated mechanism was suspected. Intravenous immunoglobulins and oral corticosteroids were trialled with initial major benefit but he relapsed several months later and did not respond to a further trial. The atrial mass was resected and histological analysis confirmed an atrial myxoma. The chorea and cognitive symptoms significantly improved after surgery and he remains well.DiscussionAtrial myxoma is the most common primary tumour of the heart and is rare with an incidence of 0.5/1,000,000 population/year. There has only been one other case in the paediatric literature.