bluuebunny/crossref_metadata_2025_split huggingface dataset:
115  SMART: A rare syndrome with potentially devastating consequences
Authors: Baheerathan Aravindhan , Lynch David , Pednekar Nichil , Blindheim Christer , Smyth Ducan , Stockford Lindsey , Rees Jeremy 
Published: May 2022

Stroke like migraine attacks after radiation therapy (SMART) is an uncommon delayed complication after cranial irradiation. Whilst classically reported as a reversible syndrome, SMART can have serious neurologi- cal sequelae. We report three cases with differing neurological presentations and outcomes.A 57 year old female with previous cranial radiotherapy for a frontal astrocytoma treated 9 years earlier presented with a two week history of headache, aphasia and right sided hemiparesis. MRI Brain demon- strated new extensive left hemispheric enhancement with extensive effacement of the ipsilateral cerebral sulci. 40 days following her presentation she started to improve and is now in rehabilitation.A 33 year old female with a background of medulloblastoma treated with cranio-spinal radiotherapy 19 years earlier presented with worsening confusion. Her GCS continued to deteriorate and she was intubated and ventilated. MRI Brain showed extensive symmetrical signal anomalies with cortical enhancement along the occipital, temporal and parietal lobe borders. 2 months later she remains in a minimally conscious state.A 65 year old male with a background of acute lymphoblastic leukaemia treated 30 years earlier who was treated prophylactic cranial irradiation presented with headache and unintelligible speech. His symptoms settled after 7 days and he made an almost complete recovery.*Aravindhan Baheerathan and David Lynch are joint first authors c.c.blindheim@gmail.com
120  Impaired phonemic discrimination in logopenic variant primary progressive aphasia
Authors: Johnson Jeremy , Jiang Jessica , Benhamou Elia , Requena-Komuro Mai-Carmen , lan Harri Sivasathiasee- , Marshall Charles , Volkmer Anna , Rohrer Jonathan , Warren Jason , Hardy Chris 
Published: May 2022

BackgroundWhilst current diagnostic criteria for primary progressive aphasia syndromes (PPA) emphasise impaired language output and linguistic processing, deficits of auditory analysis are increasingly rec- ognised in PPA syndromes. We assessed phonemic discrimination and its neuroanatomical correlates in patients representing all major PPA variants, compared with typical Alzheimer’s disease (tAD) and healthy age-matched individuals.Methods81 patients with PPA or typical Alzheimer’s disease, and 73 controls performed a phonemic minimal pair discrimination task. Neuroanatomical associations of phonemic discrimination performance across the PPA cohort were assessed using voxel-based morphometry.ResultsPatients with logopoenic variant primary progressive aphasia (lvPPA) as a group performed sig- nificantly worse than both the healthy control group and other PPA variants, after adjusting for auditory verbal working memory. Patients with typical Alzheimer’s disease showed a similar pattern of performance to the lvPPA group. Voxel-based morphometry revealed a significant association of regional grey matter in left angular gyrus with phonemic discrimination performance.ConclusionsImpaired phonemic discrimination is a component of lvPPA and may help differentiate this syndrome from other forms of PPA. These findings speak to the emerging theme of auditory perceptual dysfunction in the progressive aphasias, with implications both for refining diagnostic criteria and developing new clinical biomarkers.jeremy.johnson@doctors.org.uk
122  Head Injury discharge advice in an emergency department
Authors: Elkommos Samia , Hardy Jean , Elliot Katherine , Jenkins Peter , Dilley Michael , Griffin Colette 
Published: May 2022

BackgroundHead Injury (HI) is a common presentation to the Emergency Department (ED). Most injuries are mild and patients are often discharged directly from ED. However, persistent symptoms and failure to return-to-work are common. Comprehensive advice and a clear pathway if symptoms persist are critical to prevent unnecessary morbidity.ObjectivesTo audit the discharge advice and follow-up of patients discharged from an ED with a HI.MethodologyA Data Collection Tool was used to audit the advice and follow-up guidance given to patients attending St George’s Hospital ED in December 2018 with a HI. Data was audited against the NICE Guidelines for Head Injury: Assessment and Early Management (CG176).Results149 patients were identified. 41 (27.5%) were provided with written advice; 53 (35.6%) had no documentation of any HI advice being given; 55 (36.9%) had some documentation of HI advice but no documentation that written material was provided.ConclusionsA need for improvement in the quality of HI Discharge Advice from ED and access to spe- cialist HI outpatient assessment was identified. Improvements implemented included education of ED staff, new patient information sheet, and direct referral system to a specialist HI clinic. Re-audit is planned following these improvements.Samia.Elkommos@nhs.net
123  The Incidence of neurological symptoms in patients with traumatic brain injuries and return to work
Authors: McHattie Andrew , Jenkins Peter 
Published: May 2022

ObjectivesTo investigate: 1) The incidence of neurological symptoms in a traumatic brain injury (TBI) clinic; 2) The relationship between symptoms and injury severity; 3) The cause of persistent symptoms; 4) The factors preventing return-to-workBackgroundPersistent symptoms after even a mild TBI are common. The diagnosis of post-concussion syndrome is often used in this situation with the assumption that symptoms will self-resolve. However, this is frequently not the case and neurological and neuropsychiatric diagnoses including migraine, BPPV and anxiety often underlie these persistent symptoms.Methods66 patients with a range of TBI severities were evaluated in a dedicated TBI clinic. Data was collected on the incidence of neurological symptoms, symptom persistence, and return-to-work rates.ResultsThere was a high incidence of neurological diagnoses, particularly migraine and BPPV. Both were associated with an increased odds ratio of failure to return-to-work within 30 days. Migraine, in particular, was a common cause of persistent symptoms.ConclusionsNeurological diagnoses are common causes of persistent symptoms post-TBI. Their incidence is associated with a failure to return-to-work. Post-concussion syndrome is an unhelpful diagnostic label as it incorrectly assumes self-resolution of symptoms; instead, diagnostic clarity for each individual is necessary to help guide treatment.andrewwilliam.mchattie@nhs.net
124  MEGAbIT – the role of OPM MEG in assessment and diagnosis In mTBI
Authors: Allen Christopher Martin , Rier Lukas , Halsey Lloyd , Gascoyne Lauren , Dineen Robert , DasNair Roshan , Brookes Matthew , Evangelou Nikos 
Published: May 2022

Can those with mild traumatic brain injury (mTBI) be differentiated from non-head injured orthopaedic trauma controls by measuring brain wave activity? These are the preliminary data from MEGAbIT, a MRC Confidence in Concept funded study. Clinical Trials reference:NCT03867513Whilst most individuals with mTBI recover, a significant proportion have persistent difficulties. These patients have normal routine scans, and consequently a biomarker to understand the neuropathology underly- ing these symptoms is lacking. We have combined ultrahigh field 7T MRI with magnetoencephalography (MEG), to look for structural and functional abnormalities in acute mTBI. Following a systematic review of the MEG mTBI literature, we established that the most robust finding is increased power in the delta frequency band following mTBI. So far, in our study, 10 of the 13 mTBI participants have shown this abnormality.Comparisons of spectral power between the mTBI cohort and controls in other frequency bands were less sensitive biomarkers of mTBI. Full analysis to be presented will include assessment of whole-brain con- nectivity using amplitude envelope correlation, structural damage using high-resolution susceptibility weighted imaging and structural connectivity using diffusion tensor imaging. Our results are in keeping with publications suggesting that MEG can detect mTBI abnormalities when structural imaging is normal.christopher.allen@nottingham.ac.uk
130  Does visual imagery vividness have a genetic basis? A genome-wide associa- tion study of 1019 individuals
Authors: Day Jacob , Frayling Timothy , Wood Andrew , Zeman Adam 
Published: May 2022

The vividness of visual imagery varies widely between individuals and its extremes are associated with prosopagnosia and synaesthesia. A complete lack of visual imagery, recently termed aphantasia, is typically present from birth and is more common in those with affected family members. It is therefore postulated that there is a genetic component to the variation in visual imagery vividness. To try and identify genetic variants associated with visual imagery vividness we performed a genome-wide association study of 1019 individuals in the Exeter 10000 cohort. Participants who had previously been genotyped were then asked to complete the Vividness of Visual Imagery Questionnaire (VVIQ). We did not identify any variants that reached the genome-wide significance p<5x10–8 level. However, 2 variants in linkage disequilibrium were below the p<5x10–7 level and lie within an intron of the SYT1 gene which encodes a protein important in neuronal synaptic transmission. There were no significant genetic correlations with VVIQ and other publicly available traits. Further work should focus on using larger cohorts which will have greater power to identify variants which account for smaller amounts of VIVQ variance. This would also determine whether the SYT1 variant is truly significant.jacobday@doctors.org.uk
131  Concurrent presentation of cutaneous vasculitis and Creutzfeldt-Jakob disease
Authors: Nortey Donald , Ah-Chuen Jonathan , Yoganathan Katie , Ananthavarathan Piriyankan , McAuley John 
Published: May 2022

A previously well and independent 84-year-old man presented with subacute behavioural disturbance and cognitive decline, progressing over one month to a bedbound encephalopathic state with myoclonus. Examination at that time revealed global cognitive dysfunction, dysarthria, dysphasia, myoclonus and motor pyramidal signs. MRI study revealed widespread high signal on T2-weighted imaging in the hemi- spheric deep white matter, as well as in the right caudate nucleus. EEG revealed frequent periodic triphasic complexes. CSF examination was positive for 14–3–3 and RTQuIC, with S100b 1.2mg/ml, confirm- ing a diagnosis of sporadic Creutzfeldt-Jacob Disease (CJD).During his admission, we noted the emergence of a vasculitic skin rash over his torso spreading bilater- ally over three days. Punch biopsy confirmed the presence of an acute leucocytoclastic vasculitis not suggestive of a drug-associated rash. Further immunological staining did not detect any abnormal prion protein. The rash persisted until he sadly died 4 weeks after admission.In the literature, there are only 3 reported cases of cutaneous manifestations of CJD. The emergence of an acute vasculitic rash in the context of confirmed CJD in our case may suggest immune dysregulation associated with the latter’s underlying pathophysiology, a direct manifestation of prion protein (despite negative staining) or an unrelated concurrent presentation.katieyoganathan@gmail.com
132  Evaluation of the normal pressure hydrocephalus (NPH) service at Wessex Neurological Centre
Authors: Hopkins Molly , Jones Charis , Anwar Durria-Rubat , Kipps Christopher , Pengas George 
Published: May 2022

BackgroundNPH is a condition in which ventriculomegaly occurs due to accumulation of cerebrospinal fluid (CSF). The hallmark symptoms are a triad of abnormal gait, cognitive impairment and urinary inconti- nence. A lumbar puncture (LP) is the main diagnostic test and treatment is via a ventriculoperitoneal shunt.AimTo evaluate the current service delivered and to identify factors that may improve the identification of patients most likely to benefit from treatment.MethodThis is a retrospective study including 126 patients referred between January 2015 and September 2017. From clinical notes, variables were recorded including demographics, imaging, presence of dispro- portionally enlarged subarachnoid spaces (DESH), LP results, CSF Alzheimer’s disease (AD) biomarkers, surgery outcome and complications.ResultsThe shunt success rate was 78.6% and the complication rate was 9.5%. The positive predictive value of an LP and DESH in predicting good shunt outcome was 82.5% and 77.3% respectively. There was no statistical significance between variables assessed and a positive or negative NPH diagnosis.ConclusionOur service had higher surgical success rates and lower complication rates than stated in literature. The most diagnostically accurate test is a LP, this should be done regardless of DESH. Demo- graphics and CSF biomarker analysis were not diagnostically helpful.mrkh1g17@soton.ac.uk
133  Normal pressure hydrocephalus – factors affecting surgical outcomes
Authors: Carey Charles , Inam Masna , Tsermoulas Georgios , Flint Graham , Jacob Saiju 
Published: May 2022

BackgroundNormal pressure hydrocephalus (NPH) is a disease that causes gait disturbance, urinary incontinence and dementia and is treated through ventriculoperitoneal shunting (VPS). Although CSF infusion studies are currently used to predict the likelihood of improvement following VPS, many see no benefit or deteriorate after initial improvement. It is currently unknown whether preoperative characteristics could predict the likelihood of postoperative improvement.AimsTo investigate associations between postoperative symptomatic improvement and patients’ mean baseline and plateau CSF pressures, the time between NPH diagnoses and performing VPS, and patients’ age at diagnosis.MethodsA retrospective audit was performed using data collected between 14/05/2018 and 18/06/2018 from 65 NPH patients treated with VPS at University Hospital Birmingham. Percentages of patients whose symptoms improved and did not improve postoperatively were calculated. Two-tailed unpaired t-tests were performed to analyse associations between the pre-operative factors studied and symptomatic improvement. The level of significance used was p = 0.05.Results69.0% of patients with gait disturbance, 52.9% with urinary incontinence and 56.4% with cognitive impairment at baseline showed improvement at their first postoperative follow up. Just 31.0% of patients with gait disturbance, 47.1% with urinary incontinence and 43.6% with cognitive impairment showed improvement at their last postoperative follow up. None of the pre-operative factors studied were associated with significant differences in the rates of symptomatic improvement at patients’ first or last recorded follow ups.ConclusionsThe majority of patients showed improved gait, incontinence, and cognition postoperatively at their first recorded follow up. The mean rates of improvement in all 3 symptoms studied at patients’ last recorded follow up were lower than at their first recorded follow up. There were no associations with symptomatic improvement with the number of weeks between diagnosis and VPS, age at diagnosis, baseline CSF pressure and plateau CSF pressure. saiju.jacob@uhb.nhs.uk
134  Apathy in cerebrovascular small vessel disease is characterised by distinct white matter tract changes
Authors: Saleh Youssuf , Heron Campbell le , Veldsman Michele , Drew Daniel , Plant Olivia , Schulz Ursula , Sen Arjune , Manohar Sanjay , Rothwell Peter , Husain Masud 
Published: May 2022

IntroductionClinical apathy is a poorly understood neuropsychiatric syndrome characterised by a sig- nificant decrease in goal-directed, motivated behaviour. It occurs in ~30% of patients with cerebrovas- cular small vessel disease (SVD). With the aim of improving our mechanistic understanding of apathy, we conducted a multimodal investigation combining validated behavioural paradigms and magnetic resonance imaging (MRI) techniques.Methods83 patients with MRI evidence of SVD were recruited from the Oxford Vascular Study (OXVASC) and Oxford neurology clinics. They were investigated using a novel effort-based decision making task and the Apathy Evaluation Scale (AES). Structural and diffusion weighted MRI was conducted to measure white matter lesion load (WMLL) and tract integrity, indexed by Fractional anisotropy (FA).ResultsPatients with apathy demonstrated a significant reduction in motivated behaviour and were sig- nificantly less incentivised by low levels of reward. Diffusion weighted imaging demonstrated that apathy was characterised by focal changes to limbic association tracts, including the uncinate fasciculus and cingulum bundle, as well as fronto-striatal white matter tracts. Importantly, global measures of disease severity did not independently associate with apathy.DiscussionReduced incentivisation by low reward characterised apathy in SVD, as previously reported in Parkinson’s disease, suggesting a common mechanism underlying apathy across neurological diseases. The association of apathy with focal white matter tract changes is consistent with disruption to key frontal and fronto-striatal circuits which have been previously implicated in effort-based decision-making for rewards.youssuf.saleh@ndcn.ox.ac.uk
135  A national audit of service provision for patients with atypical parkinsonian syndrome
Authors: Allen Claire , Ghosh Boyd 
Published: May 2022

IntroductionIn 2011 the PSP Association commissioned a care pathway for patients with Atypical Par- kinsonian Syndrome (APS). This pathway recommends prompt assessment by a specialist and a MDT approach to outpatient care. We sought to review whether these standards are being met.MethodQuestionnaires were produced and sent as Freedom of Information requests to 127 NHS trusts in England. 65 trusts responded.Results8 trusts saw patients in specialist APS clinics, 36 in movement disorder clinics and 21 in general neurology or elderly care clinics. In each setting a therapist or nurse was present in 88%, 53% and 10% of clinics respectively and a named point of contact was provided to patients in 86%, 100% and 62% of clinics respectively. 31/65 trusts provided information on patient numbers. Across these, 1179 patients with APS were seen over a year of which 33% were seen in specialist clinics, 51% in movement disorder clinics and 16% in general clinics.ConclusionOur audit suggests that the majority of patients with APS do have access to review by a specialist, however significantly fewer trusts provide a MDT approach to patient care as recommended in the pathway. Further work is required to meet these standards.c-allen@doctors.org.uk
137  Accuracy of using administrative data to identify epilepsy cases: a systematic review of validation studies
Authors: Mbizvo Gashirai K , Bennett Kyle , Schnier Christian , Simpson Colin R , Duncan Susan E , Chin Richard FM 
Published: May 2022

PurposeTo ascertain the accuracy of using administrative healthcare data to identify epilepsy cases.MethodWe searched MEDLINE and Embase from 01/01/1975–03/07/2018 for studies evaluating the diagnostic accuracy of administrative data in identifying epilepsy cases using any disease coding system. Two authors independently screened studies, extracted data, and quality-assessed studies. We assessed PPV, sensitivity, NPV, and specificity. The primary analysis was narrative.ResultsThirty studies were included between 1989–2018. Risks of bias were low, high, and unclear in four, 14, and 12 studies, respectively. Coding systems included ICD-9, ICD-10 and Read Codes, with or without antiepileptic drugs (AEDs). PPVs included ranges of 5.2–100% (Canada), 32.7–96.0% (US), 47.0–100% (UK), and 37.0–88.0% (Norway). Sensitivities included ranges of 22.2–99.7% (Canada), 12.2–97.3% (US), and 79.0–94.0% (UK). Nineteen studies contained ≥1 algorithm with a PPV >80%. Sixteen studies contained ≥1 algorithm with a sensitivity >80%. PPV was highest in algorithms consisting of disease codes (ICD-10 G40–41, ICD-9 345) in combination with ≥1 AED. The addition of symptom codes to this (ICD-10 R56, ICD-9 780.3, 780.39) lowered PPV. Sensitivity was highest in algorithms consisting of symptom codes with ≥1 AED. Whilst using AEDs alone achieved high sensitivities, the associated PPVs were low. Most NPVs and specificities were >90%.ConclusionIn the first global systematic review of this topic, we conclude that it is reasonable to use administrative data to identify people with epilepsy in epidemiological research.mbizvogkm@gmail.com
144  Brain tumour related epilepsy with co-existing non epileptic attacks: characteristics of a clinically challenging cohort
Authors: Samarasekera Shanika , Liang Di 
Published: May 2022

BackgroundThe co-existence of non epileptic attacks (NEAD) in patients with brain tumour related epilepsy (BTRE) is poorly described. Non epileptic attacks (NEAD) co-occur in up to 30% of patients with epilepsy (PWE) [SS1] . Adverse life events are associated with development of NEAD; their co-occurrence in those with BTRE is potentially un-surprising .MethodsClinical trajectories of patients with BTRE and co-existing NEAD were characterised. The diagnosis of NEAD was based on specialist’s observation of attacks and/or capture of attacks on video. Patients had been referred because of persisting symptoms in spite of escalating antiepileptic therapy.ResultsOf seven patients, six developed NEAD following development of BTRE. One developed NEAD de novo following tumour biopsy. Onset of NEAD was not temporally linked with the diagnosis of a brain tumour. In five, NEAD onset occurred when seizures were controlled (< 1 seizure/month). All seven nev- ertheless reported fear of developing uncontrolled seizures and identified their NEAD as more disabling than their epilepsy.Six were initially misdiagnosed with escalating seizures. Patients were eventually managed with polytherapy (two found adjunctive clobazam helpful) and behavourial strategies including mindfulness.ConclusionNEAD can co-occur with BTRE and should be considered where seizure type symptoms are escalating.[SS1]S.R. Benbadis, W.A. HauserAn estimate of the prevalence of psychogenic non-epileptic seizures.Seizure, 9 (2000), pp. 280–281 shani@doctors.org.uk