bluuebunny/crossref_metadata_2025_split huggingface dataset:
DOES UNDERSTANDING BASIC NEUROSCIENCE CURE NEUROPHOBIA?
Authors: Charalambous Nicholas , Goh Adrian , Los Ferenc , Sharma Kanch 
Published: October 2015

IntroductionNeurophobia – ‘a fear of the neural sciences and clinical neurology’1 is an established phenomenon amongst medical students and physicians.1–3 Conclusions from previous studies indicate a need for more neurology teaching,2 3 with basic neuroscience felt to be extremely useful.2 This study examines whether a concise, focused tutorial integrating basic neuroscience with clinical neurology helps overcome neurophobia.MethodsStudents from the University of Bristol (n=56) were surveyed using quantitative and qualitative questions pre/post/1 month after a 15 minute tutorial on neurological examination, integrating the relevant anatomy and physiology with clinical findings.ResultsAnalysis was performed using Wilcoxon signed ranks and Freidman testing. Comparing pre and post surveys there was a statistically significant improvement (p<0.05) in knowledge, confidence and understanding of clinical signs. Importantly this increased confidence persisted when students were re-surveyed 1 month later. Responses to difficulties faced when learning neurology include the following themes (1) complex/overwhelming subject, (2) lack of teaching, (3) inability to correlate neuroanatomy with clinical presentation.ConclusionWhen teaching students (or even clinicians) with “neurophobia”, always commence with a review of basic neuroscience as a foundation on which to build an understanding of neurological function in health and disease.
SAFETY AND EFFICACY OF ESLICARBAZEPINE ACETATE IN ELDERLY PATIENTS
Authors: Costa R , Lopes N , Gama H , Sousa R , Nunes T , Soares-da-Silva P 
Published: October 2015

PurposeTo evaluate the safety/tolerability and efficacy of eslicarbazepine acetate (ESL) as adjunctive therapy in elderly patients with partial-onset seizures (POS).MethodPhase III, multicentre, open-label, non-controlled study in patients aged ≥65 years with ≥2 POS during an 8-week baseline and treated with 1–2 antiepileptic drugs. Following baseline, patients entered a 26-week maintenance period. ESL was initiated at 400 mg once-daily and adjusted (400–1200 mg/day) based on individual response. Safety/tolerability evaluations included treatment-emergent adverse events (TEAEs), vital signs, 12-lead electrocardiogram and physical/neurological examinations. Efficacy evaluations included change in standardised seizure frequency (SSF; seizure frequency/4 weeks).ResultsOf the 72 patients included, 47 (65.3%) experienced 152 TEAEs; most commonly, dizziness (12.5%), somnolence (9.7%), fatigue (8.3%), convulsion (8.3%) and hyponatraemia (8.3%). Three patients died of cardiac failure, glioblastoma multiforme and ischaemic stroke (relationship unlikely/not related). Overall, 16 (22.2%) patients discontinued due to TEAEs. Incidence of clinically significant findings was low for vital signs, electrocardiogram and physical/neurological examinations. SSF decreased from 2.9 at baseline to 1.2 during the maintenance period (median relative change: –54.1%).ConclusionOnce-daily ESL (400–1200 mg) as adjunctive therapy in elderly subjects with POS did not raise major safety concerns and was efficacious. Supported by Bial.
EPILEPSY IN THE ELDERLY: GETTING TO THE HEART OF THE MATTER, A CASE REPORT
Authors: Jones Lliwen , Johnston Ann 
Published: October 2015

A 77-year old man presented following two episodes of altered awareness, with facial and hand automatisms. He had been seizure-free for 23 years, taking phenytoin following excision of a meningioma.A diagnosis of symptomatic temporal lobe focal seizures was made and phenytoin substituted with carbamazepine. A 12-lead electrocardiogram (ECG) revealed prolonged PR interval of 288 milliseconds. MR imaging revealed a residual right temporal lobe cystic lesion with focal enhancement. Following discussion at the epilepsy-cardiology multidisciplinary meeting (MDT) and anticipating a neurosurgical/anaesthetic work-up, a 24-hour ECG was performed, which demonstrated high grade atrioventricular block with 6.5 seconds of ventricular standstill.He was admitted as an emergency, during which he collapsed with further ventricular standstill. He underwent pacemaker insertion, remaining seizure-free.This case illustrates the challenges of epilepsy consultations and management in the elderly with dual pathology. The urgency here was not the potential re-occurrence of the brain lesion, but the conduction abnormality which unmasked serious cardiac pathology. Careful evaluation of the ECG is vital in all patients presenting with episodes of altered consciousness however the pursuit of a recognised benign cardiac conduction abnormality may in the elderly just prove life-saving. The value of a dedicated epilepsy-cardiology MDT therefore cannot be underestimated.
SCREENING FOR DEMENTIA: IS 1 SIMPLE QUESTION THE ANSWER?
Authors: Aji Baba , Larner Andrew 
Published: October 2015

ObjectiveTo examine the diagnostic utility of the dementia screening question from the DoH Dementia CQUIN document (2012) in consecutive patients in a dedicated epilepsy clinic, individuals in whom memory complaints are common.Results100 consecutive outpatients (M:F=61:39, median age 44.5 years) were asked ‘Have you been more forgetful in the past 12 months to the extent that it has significantly affected your life?’, as advocated in the Dementia CQUIN document. There was a 48% yes response. No patient was adjudged to have dementia. Comparing the yes/no groups, there was no difference in sex ratio, age, seizure type, or use of antiepileptic drugs (monotherapy versus polytherapy). Those answering yes were more likely to be follow-up than new patients. Intergroup difference in epilepsy duration showed a trend to longer duration in the yes group. The most common examples of memory problems volunteered were forgetting to attend appointments, take medications, or switch off appliances, suggestive of attentional rather than mnestic problems.ConclusionsThese data suggest that the Dementia CQUIN screening question has very low specificity, and hence will identify many false positives, with risk of overdiagnosis of dementia in individuals with purely subjective memory impairment.
A NOVEL LGI1 VARIANT IN LATERAL TEMPORAL LOBE EPILEPSY
Authors: Pickrell WO , Hope CHD , Higgins AT , Mullins JGL , Smith PEM , Rees MI , Chung SK 
Published: October 2015

BackgroundWe identified a family with autosomal dominant lateral temporal lobe epilepsy (ADLTLE). Given that LGI1 mutations account for around 50% of families with ADLTLE, we screened family members for LGI1 variants.MethodWe sequenced all exonic regions of LGI1 and used in-silico analysis tools to assess the potential affect of the novel variant. We screened 106 control samples for the variant and assessed the structural effect of the variant using a protein modelling platform.ResultsThe proband's seizures consist of an unilateral ‘buzzing’ sensation which progresses to unilateral limb numbness and secondarily generalised seizures. Some noises can provoke seizures. Her mother also has epilepsy with identical seizure semiology. We identified a novel heterozygous missense LGI1 variant in the proband and her mother which was not present in other family members or control samples. This variant is close to the splice site region of LGI1 exon 4 and is predicted to be deleterious. Protein modelling suggests that the variant causes conformational structural changes.ConclusionWe present a family with ADLTLE caused by a novel variant in LGI1. This variant is predicted to be deleterious, alters protein function and adds additional evidence for the role of LGI1 in ADLTLE.
THE INSULA: ISLAND OF REIL
Authors: Wynford-Thomas Ray , Powell Rob 
Published: October 2015

Just as ‘no man is an island’, despite its misleading name, the insula is not an island. Sitting deeply within the cerebrum, the insular cortex and its connections play an important role in both normal brain function and seizure generation. Stimulating specific areas of the insula can produce somatosensory, viscerosensory, somatomotor and visceroautonomic symptoms, as well as effects on speech processing and pain. Insular onset seizures are rare, but may mimic both temporal and extra-temporal epilepsy and if not recognised, may lead to failure of epilepsy surgery. We therefore highlight the semiology of insular epilepsy by discussing three cases with different auras. Insular onset seizures can broadly be divided into three main types both anatomically and according to seizure semiology:1. Seizures originating in the antero-inferior insula present with laryngeal constriction, along with visceral and gustatory auras (similar to those originating in medial temporal structures).2. Antero-superior onset seizures can have a silent onset, but tend to propagate rapidly to motor areas causing focal motor or hypermotor seizures.3. Seizures originating in the posterior insula present with contralateral sensory symptoms.
FRONT DOOR NEUROLOGY: THE GP EXPERIENCE
Authors: Binks Sophie , Green Liz 
Published: October 2015

IntroductionNeurological symptoms are common in primary care and may represent 10% of consultations. With fewer neurologists in the UK compared to other European nations, the RCGP encourages GP engagement with neurological disease. This project sought to delineate neurological presentations to a large Brighton GP practice.MethodDuring a four-month GP placement, an FY2 doctor recorded details of patients presenting with neurological symptoms. In tandem, six months of neurology referrals by the permanent GPs were analysed.ResultsCommon symptoms among 29 FY2 neurologically-oriented appointments included headache (n=8), paraesthesiae (n=4) and funny turns (n=3). Significant new diagnoses included right LACS, post-concussion syndrome and cervical radiculopathy. Most (65%) of patients were managed in GP. Those referred included two to TIA clinic, one to A&E and one to headache service. Of 12 GP referrals reviewed, no neurological examination was documented in 25%, and six provisional diagnoses were in agreement with neurological opinion. After neurology outpatient review, two patients were assessed as having no neurological problem.ConclusionsHeadache forms a large component of primary care neurology, but a variety of cases is seen. Acute presentations may be referred to TIA clinic or A&E. Where stated, GP opinion was frequently in line with neurology review.
CEREBRAL FAT EMBOLISM: SUSCEPTIBILITY-WEIGHTED IMAGING IS USEFUL AND PROGNOSIS CAN BE GOOD
Authors: Datta Sayan , McIntosh Catriona , Craven Ian , Macmullen-Price Jeremy , Jung Agam , Lily Oliver 
Published: October 2015

We present two cases of cerebral fat embolism. Both patients were 21-years old, male and involved in high impact road traffic collisions with no immediate neurological deficits.Patient A underwent intra-medullary nailing of right tibia/fibular fractures within 12 hours, and had external fixation for bilateral distal femoral fractures. Post-operatively, GCS was 6. Subsequent intracranial MR imaging showed widespread cerebral hypointense punctate foci on SWI (susceptibility-weighted imaging), some with associated restriction on DWI (diffusion-weighted imaging). Despite initial period of coma lasting weeks and inpatient admission over 5 months, he has been discharged to a neuro-rehabilitation unit and continues to show physical and cognitive improvement (MRS score of 3).Patient B underwent intra-medullary nailing for a femoral shaft fracture within 24 hours. Post-operatively, GCS was 9, he developed a petechial rash and became hypoxic. MR brain showed bilateral centrum semiovale DWI restriction, and again, widespread SWI punctate abnormalities. He was discharged within weeks and currently lives at home with his parents (MRS of 2).These cases show the spectrum of radiological abnormalities, the utility of SWI, and highlight that although neurological injury may be severe, there is potential for significant recovery.
WHAT IS THE NIN TELLING US?
Authors: Morrish Paul 
Published: October 2015

Data collected by the Health and Social Care Information Centre and Public Health England is throwing light on the way neurology services are needed and delivered in England. GP recorded epilepsy prevalence varied between CCG's, from 456 to 1115 people with epilepsy per 100,000 population. Community spending on anticonvulsant drugs varied between £210,000 and £690,000 per 100,000 population. Percentage of epilepsy patients with 12 months seizure freedom varied between 45% and 74%. Admissions due to epilepsy ranged between 2 and 17 admissions per 100,000 residents. Community spending on headaches and migraine varied between £69,000 and £12,000 per 100,000 residents whilst admissions due to headache and migraine varied between 8 and 134 admissions in 2012–3. Admissions with Parkinson's disease varied between 2 and 21 per 100,000 residents whilst community spending on this illness varied between £183,000 and £36,000 per 100,000. In 2012–3 there was a sevenfold difference between CCG's in neurology appointments provided, from 809 to 5,633 appointments per 100,000 population. 46 CCG's provided no new neurology appointments in their own area, whilst 14 CCG's provided over 90% of new appointments in their own area. 4.86% of all neurology appointments in England were provided by one hospital trust.These sample headlines show how data are being collected and can be analysed to prompt questions and research, to inform commissioners and providers and to improve the quality of care for patients with neurological illness in England.
MITOCHONDRIAL CYTOPATHY PRESENTING WITH CEREBELLAR ATAXIA
Authors: Pegg Emily , Dodd Katherine , Shaunak Sandip 
Published: October 2015

A previously fit and well 59 year old man presented with a 3 year history of slowly progressive decline in mobility with increasing unsteadiness and falls. He also reported clumsiness of both hands and his wife noted poor memory. There was no family history of note, including deafness and diabetes. He drank alcohol occasionally. On examination he had signs consistent with a cerebellar and pyramidal syndrome with subcortical cognitive impairment.An MRI scan of the brain and spine showed significant generalised cerebral atrophy. CSF protein was 0.80g/L, with normal cell count and no oligoclonal bands. Blood tests were normal or negative for antineuronal antibodies, vitamin E, coeliac disease, autoimmune screen. Genetic tests for spinocerebellar ataxias, Freidreich's, fragile X syndrome and DRPLA were also negative. Muscle biopsy revealed mitochondrial aggregation with COX negative fibres, in keeping with a mitochondrial disorder. Genetic testing found a novel mtDNA variant (p.GLY46Asp) at low levels within the MT-CO3 gene.He has subsequently developed myoclonus and generalised tonic clonic seizures but there is no evidence of other system involvement.Mitochondrial disorders should be considered in the differential diagnosis of cerebellar ataxia, even in the absence of multisystem involvement or a significant family history.
URINARY, BOWEL AND SEXUAL FUNCTION IN PATIENTS WITH FRIEDREICH'S ATAXIA
Authors: Lad Meher , Parkinson Michael , Rai Myriam , Pandolfo Massimo , Murphy Sinead , Emmanuel Anton , Panicker Jalesh , Giunti Paola 
Published: October 2015

BackgroundFriedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disorder leading to ataxia, weakness, peripheral neuropathy, diabetes and cardiomyopathy. Although patients also report urinary, bowel and sexual symptoms these have barely been described in the literature.Methods59 Patients seen in a research clinic in a year were included. Questionnaire scores measuring urinary, bowel and sexual symptoms were compared with validated measures of disease severity.ResultsUrinary symptom scores correlated significantly (p=0.021) with duration of disease symptoms and spasticity scores (p=0.045). Quality of life measures of patients with urinary symptoms correlated with ataxia rating scales (SARA) (p=0.036) and non-ataxic symptoms (INAS) (p=0.003). Neurogenic Bowel Disease scores correlated with severity of ataxic symptoms (SARA) (p=0.024) and activities of daily living (ADLs) (p=0.001). Sexual symptom scores did not correlate with any established measures of disease severity and disease duration.ConclusionsUrinary and lower gastro-intestinal symptoms can have a severe impact on patients with FRDA although they are under-recognised. They impact on quality of life and correlate with established measures of disease severity.
DURAL AVM AS A CAUSE OF RAISED PRESSURE SYMPTOMS IN TREATED CVST
Authors: Abdel-Aziz Khaled , Mirza Nasir , Mbizvo Gashirai , Putharan Mani , Krishnan Anita 
Published: October 2015

Case:A 56 year old man presented with new onset headaches with visual symptoms. Physical examination was normal apart from asymmetrical papilledema and reduced visual acuity 6/9 bilaterally. CSF opening pressure was 26 cm H2O. CTV showed partially recanalised thrombosis in the superior sagittal sinus and the right sigmoid and transverse sinuses which appeared chronic. His headaches initially improved with propranolol but he continued to complain of visual symptoms; repeat CSF opening pressure was 31 cm H2O and repeat CTV was reported as, ‘more occlusive’. He was therefore anti-coagulated and treated with acetazolamide.Six weeks after starting treatment, ophthalmological assessment found worsening visual fields despite reduction in disc swelling on OCT. CSF pressure remained elevated at 30 cm H2O. Repeat CTV and 4D CTA identified a type IIB occipital dural AV fistula around the thrombosed venous sinuses, causing occipital lobe congestion; the likely cause of the visual field defects.The patient underwent cerebral angiogram with embolisation of the dural fistula. Following embolization his headaches and papilledema resolved, and visual acuities and fields normalised.ConclusionNeurologists should be aware of dural AV fistula as a complication of cerebral sinus thrombosis which may cause persistent raised pressure symptoms in treated patients.
FAMILIAL MIGRAINE LIMB PAIN SYNDROME (MLPS): THREE GENERATIONS AND SEVEN YEAR FOLLOW-UP
Authors: Angus-Leppan Heather , Guiloff Roberto Jaime 
Published: October 2015

We describe a family with migraine-limb pain syndrome (MLPS) in children and adults over three generations, in an autosomal dominant pattern.MLPS is intermittent pain occurring in any limb, temporally related to a migraine episode, cluster headache or cluster migraine. It is usually ipsilateral to the headache, but can alternate and may spread with a migrainous march over 20 minutes or more. Body or chest pain (corpalgia) is also described. Limb or body pain may not coincide with headache at presentation. The association may be misdiagnosed as radiculopathy, thoracic outlet syndrome or arthritis.There is one previous report of a family with childhood limb pain, followed by migraine in adulthood, in a pattern suggesting autosomal dominant inheritance.We report a second family with three generations of MLPS starting in childhood or adulthood, with variable duration, commencing in any limb. All the patients with limb pain had concurrent or associated migraine with aura. Pain intensity in the previous family was mild, but poorly responsive to treatment. In our family the symptom was treatment responsive with variable intensity.Convergence of nociceptive input from the trigeminovascular system in the cervical spinal cord, brainstem, thalamus and cortex is a possible physiological basis for MLPS.
CHARACTERISTICS OF FUNCTIONAL AND ORGANIC STROKE MIMICS
Authors: Gargalas Sergios , David Anthony , Khan-Bourne Najma , Shotbolt Paul , Weeks Robert 
Published: October 2015

AimTo establish whether the groups of patients with conditions that mimic Stroke display certain characteristics which differentiate them from patients with true Stroke.MethodsAll admissions to King's College Hospital's Hyper–Acute Stroke Unit (HASU) over 12 months were reviewed using SINAP data. Subgroups of functional stroke mimics (FSM) and medical mimics (MM) were identified through consensus between experts. Statistical analysis comparing these subgroups to Stroke admissions was performed using Chi2, Kruskal–Wallis and Mann–Whitney methods.ResultsFSM patients (N=98) were younger (mean age 49, p<0.001) and mostly females (63.27%, p=0.003). They had the shortest stay on HASU and were more likely to have had MRI during admission. Compared to MM (N=198), FSM were more likely to have had history of Asthma, Back Pain, Migraine or Depression (p=0.001). FSM presented later compared to Strokes (N=904) (latency FSM 4.57 hrs vs. Stroke 2.17 hrs, p<0.001) and were less likely to present with cortical features (Visual disturbances, Dysphasia or Neglect, p<0.001) or facial weakness or numbness. MM were much more likely to present with altered consciousness (MM 15.34% vs. FSM 4.08% vs. Stroke 1.99%, p<0.001).ConclusionBoth functional and medical stroke mimics appear to have distinct features that could aid the diagnostic process.
THE MANAGEMENT OF MIGRAINE IN PRIMARY CARE
Authors: Ralston Katherine 
Published: October 2015

Migraine is a common and debilitating neurological disorder. This study aimed to audit the management of adults with migraine in a Newcastle GP practice against NICE guidelines (2012) on the acute and prophylactic treatment of migraine.A search of practice records for patients with a new diagnosis of migraine between December 2013–14 identified 38 patients. Medical records were reviewed to determine if their management was consistent with NICE guidance.Acute treatment was indicated in 35 patients, with 27 (77%) receiving correct treatment. 1 patient (3%) received unrecommended treatment, while 7 (20%) received no treatment. Prophylactic treatment was given to 11/13 (85%) patients where it was indicated. Of these, 6 (55%) received first line treatment, 1 (9%) second line treatment and 4 (36%) unrecommended treatment. All patients should be followed up after treatment initiation. This occurred or was planned in 14/35 (40%) acute patients and 6/11 (55%) prophylactic patients.The majority of patients with migraine received the correct acute treatment, however over one third of patients received unconventional preventative medication and follow up was sporadic. A template prompting the correct management of migraine has been incorporated into the computer system following this audit to improve patient care.
THERAPEUTIC EFFECT OF INTRANASAL EVAPORATIVE COOLING IN PATIENTS WITH MIGRAINE. A REPORT OF THE RESULTS OF THE COOL HEAD I, PILOT STUDY
Authors: Vanderpol Jitka , Bishop Barbara , Matharu Manjit , Glencorse Mark 
Published: October 2015

BackgroundIntranasal evaporative cooling due to vascular anatomy, allows the transfer of venous blood from nasal and paranasal mucous membranes to the dura mater, providing an excellent anatomical basis for the cooling processes. The aim of this study was to ascertain whether it may be an effective intervention in an acute migraine attack.MethodsWe conducted a prospective, open-label, observational, pilot study. A total of 20 treatments were administered in 15 patients who satisfied the ICHD 2 diagnostic criteria for migraine. All patients provided pain and migraine-associated symptoms severity scores.ResultsImmediately after the treatment, 8 treatments (40%) resulted in pain and symptoms freedom, a further 10 treatments (50%) resulted in partial pain and symptoms relief. At 2 hours, 9 treatments (45%) provided full pain and symptoms relief, with a further 9 treatments (45%) resulting in partial pain and symptoms relief. In summary 13 patients (87%) had benefit from the treatment within 2 hours that was sustained at 24 hours; without significant side effects.ConclusionsIntranasal evaporative cooling was safe and gave considerable benefit to patients with migraine. An adequately powered randomised controlled study will be required to confirm the potential of this application in the treatment of migraine.