bluuebunny/crossref_metadata_2025_split huggingface dataset:
SPINAL STENOSIS IN FAMILIAL TRANSTHYRETIN AMYLOIDOSIS
Authors: Carr Aisling , Evans Matthew , Pelayo-Negro Ana , Shah Sachit , Choi David , Blake Julian , Phadke Rahul , Gilmore JD , Hawkins PN , Reilly Mary 
Published: October 2015

BackgroundTransthyretin-associated amyloidosis (ATTR) is the commonest cause of hereditary amyloidosis. Age at onset is 50–60 years with average life-expectancy of 7–14 years. Lumbar spinal stenosis also presents in late middle-age; related to disc degeneration, spondylolisthesis and age-related degenerative processes, such as WT-TTR amyloid deposition. Mutant-TTR has not been described in this scenario. Recent therapeutic developments in ATTR, with disease modifying potential, make histological evaluation clinically relevant.CaseFamily history prompted screening in an asymptomatic female carrier (heterozygous I84S) at age 42. At age 47 she developed sensory disturbance and pain in the left leg, brought on by exercise and relieved by rest. One year on, exercise tolerance <100 meters and she had sphincter disturbance. Examination revealed decreased pinprick sensation in the L5 dermatome and asymmetry of ankle and knee jerks, pulses were present. A disc bulge at L2/3 with superimposed narrowing of the lumbar canal due to ligamentum flavum hypertrophy was seen on MRI. Immunohistochemistry labelled ATTR deposits in the excised connective tissue. There was neurological improvement post-operatively but amyloid-symptoms qualified her for inclusion in ATTR treatment trials.DiscussionWe recommend histological examination for amyloid on tissue from spinal decompression and TTR gene analysis if this is positive.
A PROSPECTIVE CLINICAL AND IMMUNOLOGICAL STUDY OF LATE ONSET MYASTHENIA GRAVIS
Authors: Sadalage Girija , Jacob Saiju , Buckley Camilla , Hilton-Jones David , Vincent Angela , Maddison Paul 
Published: October 2015

There is an increasing incidence and prevalence of MG in older patients (>50 years) – defined as Late Onset Myasthenia Gravis (LOMG). This study aims to define the demographics, clinical features, response to treatment and immunological features which may distinguish LOMG from the early onset patients (who will also be recruited as controls).Who to refer?▸ All patients should be over the age of 18 years, able to provide informed consent.▸ The study aims to recruit patients who are willing to travel to one of the myasthenia clinics in Birmingham, Nottingham or Oxford▸ All patients with a new diagnosis of MG (or diagnosed in the last 12 months) prior to immunosuppression.▸ Diagnosis of MG based on typical clinical features and the presence of AChR or MuSK Abs, or evidence of neuromuscular transmission defect on single fibre EMG.What it involves for patients▸ Management will be as per usual practice▸ Annual serum samples at follow up with QoL and MG composite scores▸ Follow up for 5 years (3 years by the clinical research fellow and 2 years optionally by the local participating neurologists).
5 YEARS EXPERIENCE OF PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA (PCNSL): A RETROSPECTIVE PILOT AUDIT
Authors: Datta Sayan , Lee Rebecca , Webb Tom , Ismail Azzam , Jung Agam 
Published: October 2015

IntroductionPrimary Central Nervous System lymphoma (PCNSL) is responsible for 5% of all primary brain tumours within the UK. Barriers to diagnosis include steroid-responsiveness/false-negative biopsy and poor CSF sensitivity. We performed a pilot audit reflecting 5 years experience of PCNSL diagnosis based on criteria derived from 2011 British Neuro-Oncology guidelines.MethodsRetrospective case note and electronic patient record review for patients between 2008 to 2013 with biopsy-proven label of PCNSL. Imaging and staging results were reviewed to exclude secondary/metastatic disease.Results20 patients were identified; median age of 58 and 60% were male. Top three clinical presentations were cognitive, paresis and ataxia. Median duration of symptoms was 21 days (range 3 to 180) and median time to biopsy was 14 days.Pre-biopsy5 patients had negative CSF, 20% had steroids with documented clinical reason, 5% had record of HIV status checked and 88% had CT staging.ConclusionDespite limitations, this audit identified that the majority of these patients received steroids pre-biopsy, but in some cases, this may have reasonably been withheld. There is scope for a diagnostic algorithm to highlight safe withholding of steroids, early HIV testing and appropriate staging investigations, downplaying the importance of CSF sampling.
LEVETIRACETAM IS INDEPENDENTLY ASSOCIATED WITH FATIGUE IN ADULT GLIOMA OUTPATIENTS
Authors: Zienius Karolis , Rooney Alasdair , Grant Robert 
Published: October 2015

AimsTo identify the frequency and severity of fatigue in adults with cerebral glioma, and its association with anti-epileptic drugs (AED).MethodsCross-sectional study. Stable glioma patients attending Edinburgh Neuro-Oncology clinic completed a fatigue Visual Analogue Scale (range 0-100mm, “high” >=60 mm). AED type and dose were recorded.ResultsOne hundred and sixty six patients were enrolled (61% males; mean age 49 yrs; 70% high-grade glioma). Among all patients, 43% reported high fatigue (95% CI 35–50%). Sixty eight percent were taking AEDs (58% of which were non enzyme inducing). Levetiracetam (LEV) was the most common AED. LEV monotherapy correlated with higher fatigue levels (mean=57 mm, SD 5.2, 95% CI 46–67 mm), compared with other AEDs (mean=43 mm, SD 3.9, 95% CI 35–50 mm, ANCOVA p=0.04). Patients taking LEV had poorer Karnofsky Performance Scores (KPS, Mann–Whitney p=0.03). The effect of LEV on fatigue was independent of KPS (LR p<0.0001, R2= 27.7, OR 2.7 [1.1–6.2]).ConclusionsGlioma patients on levetiracetam therapy are more fatigued than patients on other or no anti-epileptic therapy. Changes to AED medications may benefit patients with troublesome fatigue.
A CASE OF ENLARGED VIRCHOW ROBIN SPACES CAUSING PROGRESSIVE HEMI SENSORY SYMPTOMS
Authors: Agrawal Amit , Zaidi S , Rathi B , Codony M 
Published: October 2015

IntroductionVirchow Robin Spaces (VRS) or perivascular spaces are usually an incidental finding but rarely they can enlarge and causes neurological symptoms. We report a case of symptomatic VRS with striking appearance on MRI Scan.Case descriptionA 33 year lady presented with progressive sensory disturbances which initially affected her right arm and later on right side of face. Her MRI Brain scan showed enlarged Virchow Robin Spaces in the left corona radiata corresponding to her symptoms.ImportanceOur case highlights a common MRI finding, typically considered incidental but which can be symptomatic in some cases. The typical scan characteristic and location of Virchow Robin spaces can help to differentiate from close mimics like cystic mass lesion. Its awareness can avoid frequent repeated imaging and interventions.
IMPACT & TREATMENT OF NON-MOTOR SYMPTOMS IN EARLY PARKINSON'S DISEASE
Authors: Baig Fahd , Lawton Michael , Rolinski Michal , Ruffmann Claudio , Nithi Kannan , Evetts Samuel , Fernandes Hugo , Ben-Shlomo Yoav , Hu Michele 
Published: October 2015

ObjectiveTo delineate treatment and quality of life of non-motor symptoms (NMS) in early Parkinson's disease (PD) and first-degree relatives.BackgroundNon-motor symptoms (NMS) are an important prodromal feature of Parkinson's disease (PD). However, their frequency, treatment rates and impact on health-related quality of life (HRQoL) in the early motor phase is unclear.Methods769 population-ascertained PD subjects within 3.5 years of diagnosis and 287 control subjects were assessed. Validated severity questionnaires were employed to assess NMS symptoms across the following domains: (1) neuropsychiatric (2) gastrointestinal (3) sleep (4) sensory (5) autonomic (6) sexual. Health related quality of life (HRQoL), functional status and management were also evaluated.ResultsNMS were common in early PD. More than half of the PD cases had hyposmia, pain, fatigue, sleep disturbance or urinary dysfunction. PD cases had worse HRQoL scores than controls (OR 4.1, p<0.001) with depression, anxiety and pain being stronger drivers than MDS-UPDRS motor scores. Quality of life is affected in early PD, although 23% of participants reported no problems. NMS were rarely treated in routine clinical practice.ConclusionsDespite their major impact on HRQoL, NMS are usually under-recognised and treated. The use of screening tools could improve recognition and treatment of NMS in early PD.
VISUOSPATIAL TESTS TO SCREEN FOR IMPAIRED COGNITION IN PARKINSON'S
Authors: Cosgrove Jeremy , Jamieson Stuart , Smith Stephen , Alty Jane 
Published: October 2015

ObjectiveTo determine which test of visuospatial function – copying a wired cube (‘cube’) or interlocking pentagons (‘pentagons’) – is the best screening tool for detecting cognitive impairment in Parkinson's, as defined by abnormal Montreal Cognitive Assessment (MoCA) score.Methods107 Parkinson's subjects completed the MoCA and copied pentagons from the Mini-Mental State Examination (MMSE). They were classified into two groups based on MoCA score: <26 (cognitive impairment (CI)) or ≥26 (normal cognition (NC)). Cube and pentagons were scored using MoCA and MMSE criteria.ResultsThe CI group (n=57) was older (p 0.032) but disease duration, stage and medication were not different. 28% of CI and 72% of NC correctly copied cube. 69% of CI and 92% of NC correctly copied pentagons. Inability to correctly copy cube (p<0.001) or pentagons (p 0.003) was associated with CI. Age adjusted odds ratio for predicting cognitive impairment was 6.85 (2.97–16.39, p 0.001) for incorrect cube and 4.61 (1.41 – 14.93, p 0.011) for incorrect pentagons.ConclusionsIncorrect cube was the most predictive visuospatial marker of cognitive impairment in Parkinson's. This is potentially useful when assessing cognitive function in a busy outpatient clinic, for example. Larger numbers are required for validation.
FINGER TAPPING AND COGNITION IN PARKINSON'S
Authors: Cosgrove Jeremy , Lacy Stuart , Jamieson Stuart , Smith Stephen , Alty Jane 
Published: October 2015

ObjectiveTo investigate separable components of finger tapping (FT) of the thumb and index finger in Parkinson's (PD) with normal (PD-NC) and impaired cognition (PD-CI) and in healthy controls (HC).Methods58 PD and 29 HC performed FT for 30 seconds whilst attached to electromagnetic movement sensors sampling at 60 Hz. All subjects completed the Montreal Cognitive Assessment (MoCA); PD-NC defined as MoCA score ≥26, PD-CI as MoCA score <26.ResultsPD and HC were age-matched. Mean disease duration of PD 6.2 years, Hoehn and Yahr stage 1.9. Mean MoCA score PD 23.1, HC 26.3 (P<0.001). PD had significantly (P<0.01) smaller mean velocity and amplitude of FT, greater decrement of speed and greater variability of rhythm (speed and amplitude coefficient of variation).Age, disease duration, motor score and levodopa dose were not significantly different between PD-CI (n=36) and PD-NC (n=22). PD-CI had significantly less rhythmic FT movements than PD-NC (P<0.01) but other parameters were similar. MoCA score was negatively correlated with rhythm in PD (P 0.017).ConclusionAs expected PD have slower, less rhythmic FT movements with greater decrement than HC. Those with PD-CI have significantly less rhythmic movement than those with PD-NC – this finding is currently being investigated.
REWARD SENSITIVITY DEFICITS UNDERLIE APATHY IN PARKINSON'S DISEASE
Authors: Muhammed Kinan , Manohar Sanjay , Tofaris George , Lennox Graham , Bogdanovic Marko , Hu Michele , Husain Masud 
Published: October 2015

Apathy is increasingly appreciated to be a major problem in Parkinson's disease (PD), with up to 70% of cases affected. The mechanisms underlying the condition are poorly understood and objective detection methods are lacking. We used eye movement and pupil modulation in response to rewards as metrics of motivation to assess the relation of reward sensitivity to apathy and influence of dopaminergic medication.30 patients with idiopathic PD, tested ON and OFF medication, and healthy age-matched participants made saccades for different monetary rewards. Saccadic peak velocity and pupil diameter were measured using an infrared eye-tracker, while apathy was indexed by Lille Apathy Rating Scale scores.PD patients ON demonstrated increased saccadic velocity and pupil diameter as reward magnitude increased, just like controls. This reward sensitivity was blunted in PD patients OFF dopaminergic medication. Crucially, apathetic PD patients exhibited significantly less pupillary reward sensitivity than more motivated PD cases.Saccadic velocity and pupil diameter are influenced by reward magnitude, with the degree of modulation varying with motivation levels across individuals. These indices provide novel, objective behavioural measures for assessing clinical apathy in PD. Dopaminergic medication may also be an effective treatment for apathy by increasing reward sensitivity, independent of effects on motor control.
NEUROIMAGING OF IDIOPATHIC REM SLEEP BEHAVIOR DISORDER
Authors: Rolinski Michal , Griffanti Ludovica , Szewczyk-Krolikowski Konrad , Menke Ricarda , Quinnell Timothy , Zaiwalla Zenobia , Mackay Clare , Hu Michele 
Published: October 2015

IntroductionResting-state functional magnetic resonance imaging (rs-fMRI) studies have previously shown significantly impaired connectivity in patients within the early motor phase of Parkinson's disease. Is it possible to detect the same imaging signature of Parkinson's in RBD subjects, before a clinical diagnosis of Parkinson's disease is established?MethodsTwenty-six patients with polysomnography-proven RBD and twenty-two age- and sex-matched healthy controls were recruited into the study. All subjects underwent a comprehensive structural and resting-state MRI protocol.ResultsVoxel-based morphometry analysis did not yield any significant grey matter differences between the two groups. Similarly, no significant differences of fractional anisotropy were found using white matter tract analysis. Rs-fMRI revealed decreased coactivation within the basal ganglia network (involving the caudate, putamen, globus pallidus bilaterally) and the sensorimotor network (precentral gyrus) of patients with RBD. A small area of increased coactivation was found in the default mode network of patients with RBD.ConclusionsOur findings support the presence of basal ganglia dysfunction in patients with RBD, likely representing the prodromal stages of Parkinson's disease. Clinical and neuroimaging follow up is necessary to assess the clinical utility of resting state fMRI to predict the onset of Parkinson's disease in RBD subjects.
THE EFFECT OF A NEW POLICY FOR INWARD TRANSFER OF PATIENTS DIAGNOSED WITH CEREBRAL VENOUS THROMBOSIS (CVT) ACROSS GREATER MANCHESTER TO THE TERTIARY NEUROSCIENCES CENTRE
Authors: Lavin Timothy , McMinn Jason , Punter Martin , Kellett Mark 
Published: October 2015

BackgroundFrom June 2014, Greater Manchester, Lancashire and Cumbria neurology network implemented regional guidelines on management of CVT. Central to this was an agreement to transfer where appropriate all radiologically confirmed CVT to the regional neurosciences unit within 24 hr. Given this change in practice we assessed the impact on our service in Greater Manchester Neurosciences Centre.ResultsBetween June 2014 and November 2014, 14 patients were admitted; compared to 6 in 2012 and 6 in 2013.The wait for urgent beds did not vary compared to equivalent period in 2012 and 2013; median wait 1d. The wait for non-urgent beds increased slightly between 2013 and 2014 from 15.9d to 16.7d. Both were worse compared to 2012 (9.8d), likely reflecting overall pressure on acute services.There was no increase in the average LOS for the acute neurology wards. Average LOS was 15.3d between June and Nov 2014 compared to 22.3d in the equivalent period in 2013.ConclusionDespite a large change in practice for management of CVT there was a small increase in number of patients and no significant effect on admission waiting times, average LOS or critical care bed days was noted.
A COMPARISON OF ASSESSMENT, DIAGNOSIS AND TREATMENT OF PATIENTS WITH CEREBRAL VENOUS THROMBOSIS BEFORE AND AFTER INTRODUCTION OF A NEW POLICY AND GUIDELINE ACROSS GREATER MANCHESTER
Authors: Keitley James , Lavin Timothy , Punter Martin , McMinn Jason , Kellett Mark 
Published: October 2015

BackgroundFrom June 2014, regional guidelines to transfer within 24 hours all radiologically confirmed cases of cerebral venous thrombosis (CVT) were implemented across Greater Manchester. This service review assesses the impact on assessment, diagnosis and treatment.MethodsWe prospectively recorded all CVT cases transferred into our unit between June 1st and November 30th 2014 (14 patients), and retrospectively reviewed the casenotes of patients admitted in 2012, 2013 and early 2014 (13 patients).ResultsMean number of days from diagnosis to transfer after guideline introduction was 0.4. More patients underwent fundoscopy (86% vs 77%), visual acuity (50% vs 27%), visual field (79% vs 69%) and otoscopy (29% vs 8%). Patients admitted to SRFT previously had shorter symptom to diagnosis, imaging, transfer, and treatment times.ConclusionsAfter guideline introduction we found a high level of compliance with time to transfer, with improvements in several aspects of clinical assessments. We expect that patients previously admitted to our centre were more unwell or already admitted for other reasons (e.g. neurosurgical) and therefore time from symptoms to diagnosis appears longer. We aim to extend the current evaluation to all patients in the region who weren't transferred to our centre to allow a more valid comparison.
THE IMPACT OF AN EPILEPSY-CARE BUNDLE ON THE MANAGEMENT OF ACUTE SEIZURES IN A LARGE LONDON-BASED DISTRICT GENERAL HOSPITAL
Authors: Baheerathan Aravindhan , Gananandan Kohilan , Swayne Orlando 
Published: October 2015

IntroductionSeizures account for 2–3% of presentations to the Accident & Emergency department. The National Audit of Seizure management in Hospitals (NASH) has demonstrated significant variances with regards to initial assessment and subsequent management. Standardised management protocols are crucial in optimising the care of this commonly encountered medical emergency.MethodsThe care of 30 patients admitted with seizures over a 1–month period was retrospectively reviewed. Following this a trust-wide seizure bundle was implemented and another 30 patients were reviewed. Data collection specifically assessed:
▸ Demographics of patients admitted,
▸ Immediate assessment & initial investigations,
▸ Consequent neuro-imaging & specialist investigations,
▸ Referral pathway.ResultsThe results showed poor uptake of the bundle and consequently management was broadly unchanged. The most significant results were:▸ 24% of cases did not have a senior review▸ 70% of cases were not discussed with neurology▸ 36% of cases did not have any follow up arrangedConclusionThe implementation of a seizure bundle can be challenging in the context of a unit that has a rapid staff turnover and is heavily staffed by locum physicians. Repeated staff education is required. This evidence is being used in a business case to employ an epilepsy nurse at Northwick Park. (Aravindhan Baheerathan and Kohilan Gananandan will both be presenting authors and contributed equally to this abstract).
A SENSE OF DIRECTION: BRAIN STIMULATION IN LATERALISED BRAIN FUNCTION
Authors: Li Lucia , Leech Robert , Seemungal Barry , Malhotra Paresh , Sharp David 
Published: October 2015

Many cognitive functions demonstrate varying degrees of hemispheric lateralisation. Lateralised pathology can lead to striking deficits, such as the dyscalculia produced by dominant parietal lesions.Transcranial direct current stimulation (tDCS) non-invasively delivers weak electrical currents to the brain, resulting in transient changes in neuronal excitability: anodal tDCS is thought to be facilitatory, whilst cathodal is inhibitory.We investigated the effect of bi-parietal tDCS on numeracy, spatial attention and, sustained attention. We hypothesised that tDCS has distinct effects because of varying lateralisation (numeracy left, spatial attention right and sustained attention uncertain). We performed a single-blinded, cross-over, sham-controlled study. Eighteen healthy right-handed subjects performed cognitive tasks during 3 sessions of bi-parietal tDCS stimulation: sham, right cathodal plus left anodal (RC/LA) and left cathodal plus right anodal (LC/RA).Inhibition of the left parietal lobe by LC/RA stimulation impaired numeracy performance, compared to sham or RA/LC stimulation (F(2,16)=3.684, p=0.048). LC/RA stimulation also resulted in significantly impaired sustained attention performance, as compared to sham or RA/LC stimulation (F(2,34)=5.3, p=0.01).We demonstrate that bilateral tDCS modulates numeracy and sustained attention in an electrode polarity-dependent manner. This method can be used to interrogate lateralised cognitive functions in future studies of healthy and diseased populations.
FIRST CASE SERIES OF VENOUS SINUS STENTING IN IIH PATIENTS IN WALES
Authors: Malik Gauhar Abbas , Joshi Yogish 
Published: October 2015

BackgroundIdiopathic Intracranial Hypertension (IIH), is defined by increased cerebral spinal fluid (CSF) pressure in the absence of other causes of intracranial hypertension. There has been recent interest in the role of intracranial venous sinus stenosis in IIH. The raised pressures in IIH are argued to worsen by the secondary appearance of the venous sinus stenosis.Objective5 patients have undergone endovascular pressure measurement in Wales and their clinical details including history, examination, initial management, neuroimaging pre- and post venous stenting, and follow-up (6–24 months) to provide the first case study of patients undergoing Venous sinus stenting in Wales.Methods5 patients with IIH refractory to first line treatments underwent venography and manometry and 4 patients underwent stenting of the venous sinuses after this procedure had shown a pressure gradient proximal to stenosis in the lateral sinuses.ResultsThree patients were rendered asymptomatic, two were improved including one patient unmasking a different headache disorder following treatment.ConclusionsStenting in venous stenosis provides a further treatment option to patients refractory to first line treatments with IIH. This case series highlights in selected cases treatment is promising with good outcomes.
AUTOLOGOUS HAEMOPOIETIC STEM CELL TRANSPLANT IN MS: SHEFFIELD COHORT
Authors: Kelsey P , Bell SM , Jessop H , Snowden JA , Sharrack B 
Published: October 2015

BackgroundAutologous haemopoietic stem cell transplantation (AHSCT) is an evolving therapy for multiple sclerosis (MS). Here we present the Sheffield experience since 2006.MethodsRetrospective review of MS cases referred for and treated with AHSCT. Toxicity, clinical and radiological outcome were assessed.Results14 patients (8 Rapidly evolving severe [REMS], 3 Relapsing Remitting [RRMS], 3 Secondary Progressive [SPMS]) were selected for AHSCT. One patient underwent AHSCT on a clinical trial (data excluded from analysis). Mean age 34.6 years (SD 8.69), median pretreatment EDSS 6.5 (IQR0.5). All had clinically and radiologically aggressive disease, non-responsive to standard treatment. Follow up range was 3 to 96 months.All patients were successfully mobilized, 2 patients progressed only to harvest (1 compliance issues, 1 leg ulcer). All patients were treated according to international guidelines. No excess toxicity or treatment related mortality was observed.Post-transplant MRI's showed no active disease. No patients with RRMS suffered relapse post-transplant, median reduction in EDSS scores was 2 at both 100 days (IQR 2) and at last follow up (IQR 2.5). EDSS scores stabilised in patients with SPMS.ConclusionAHSCT is an effective treatment with acceptable toxicity in patients with aggressive, inflammatory MS.