bluuebunny/crossref_metadata_2025_split huggingface dataset:
SCREENING FOR RESPIRATORY FAILURE IN ALS USING CLINICAL QUESTIONING, RESPIRATORY FUNCTION TESTS AND TRANSCUTANEOUS CARBON DIOXIDE: WHICH IS THE BETTER TOOL?
Authors: Rafiq Muhammad , Proctor Alison , McDermott Christopher , Shaw Pamela 
Published: October 2015

BackgroundScreening patients regularly for evidence of respiratory failure is an important facet in the management of ALS.ObjectivesTo evaluated the relative value of symptom history, forced vital capacity (FVC) and day time transcutaneous carbon dioxide (Ptc CO2) monitoring in early detection of respiratory failure in ALS.MethodsThis is a prospective observational cohort study consisting of 50 consecutive patients with ALS. The participants underwent 3 monthly assessments for symptoms of respiratory failure, FVC and Ptc CO2 monitoring.ResultsSymptoms of respiratory failure were the most powerful tool, alerting the physician to the possibility of respiratory failure. All the patients where respiratory failure was confirmed on overnight capnometry had symptoms of respiratory failure. Shortness of breath was the most common symptom reported by the patients, present in 74% of the patients with confirmed respiratory failure. 37% of these patients had FVC of >50% predicted and only 15% had day time hypercapnia (Ptc CO2>6.0 kPa). None of the patients had day time hypercapnia without any other marker of respiratory failure. There was statistically significant difference between the day time Ptc CO2 and median overnight Ptc CO2 (p=0.0002).DiscussionThis study has emphasised the importance of symptom history in detection of respiratory failure in ALS.
PERIPHERAL NERVE BING-NEEL SYNDROME
Authors: Carr Aisling , D'Sa Shirley , Arasaretnam A , Boyd K , Johnston R , Jaunmuktane Zane , Brandner Sebastian , Marafioti T , Koltzenburg Martin , Lunn Mike 
Published: October 2015

BackgroundBing-Neel syndrome (BNS) is a rare manifestation of Waldenstrom's Macroglobulinaemia (WM) due to direct infiltration of the central nervous system. Isolated peripheral nerve infiltration has not been described but as the blood-nerve-barrier has similar characteristics to the blood-brain-barrier, CNS-penetrating chemotherapy would be required.CaseA 43 year-old man was diagnosed with WM after presenting with constitutional symptoms; IgMk paraprotein (34.6 g/L) and lymphoplasmacytoid cells in the bone marrow. He received R-CVP (Rituximab, Cyclophosphamide, Vincristine, and Prednisolone) with good partial haematological response and minimal distal sensory vincristine-related neuropathy. Ten months later he developed a rapidly progressive, symmetric sensorimotor deficit in all four limbs with impairment of walking and hand clumsiness, MRC sum score=61/70. Electrophysiology showed a non-length dependant axonal neuropathy with patchy slowing. Sural nerve biopsy revealed a patchy infiltrate WM cells. He tolerated 4 cycles of blood-nerve-barrier penetrating multi-agent chemotherapy, IDARAM (idarubicin, dexamethasone, cytosine arabinoside, methotrexate, cytosine arabinoside plus intrathecal methotrexate). A marked improvement was seen during treatment and by 3 months later MRC score was 70/70. No electrophysiological difference was detectable at this stage.DiscussionPeripheral BNS is not a recognised neurological manifestation of WM but with appropriate treatment a good outcome may be achieved.
THE NEUROPATHY SPECTRUM IN WALDENSTRöM'S MACROGLOBULINAEMIA
Authors: Carr Aisling , Jaunmuktane Zane , Brandner Sebastian , Kothari J , Blake Julian , D'Sa Shirley , Lunn Mike 
Published: October 2015

BackgroundUK WM Guidelines recognise 2 mechanisms of nervous system involvement either direct tumour invasion of the CNS or antibody mediated damage to peripheral nerves. We present 5 cases illustrating an extended diversity of PNS involvement in WM with implications for treatment and outcome.CasesA 73 year-old man (IgMκ WM) developed progressively disabling tremor with stable WM. Neurophysiology was demyelinating with disproportionate DMLs; anti-MAG antibodies were strongly positive. He was treated with DRC with symptom stabilisation.A 64 year-old woman (IgMκ) with typical anti-MAG neuropathy developed severe episodic foot pain and ulceration. Skin biopsy confirmed leucocytoclastic vasculitis. She was treated with DRC but died from sepsis.A 43 year-old man (IgMκ WM) with rapidly progressive sensorimotor neuropathy had lymphplasmacytoid cellular infiltrate in sural nerve. He was successfully treated with IDARAM.A 59 year-old man (IgMλ WM) developed small then large fibre axonal neuropathy with autonomic symptoms within 2 years. A SAP scan showed moderate kidney uptake. MRI neurography directed biopsy and confirmed amyloid. Palliative treatment was offered.A 70 year-old woman (IgMλ WM) developed severe burning pains in her feet and became ataxic 2 months later. Sural nerve biopsy confirmed endoneurial amyloid. DRC was given.DiscussionThese cases highlight the importance of accurate diagnosis in WM-associated neuropathy. We propose a novel diagnostic algorithm.
AUDIT OF CSF CYTOLOGY AT THE WEST MIDLANDS NEUROSCIENCE CENTRE
Authors: Evans William , Carey Martyn , Dunne Fiona , Nagaraju Santhosh , Hayton Tom 
Published: October 2015

Cerebrospinal Fluid (CSF) cytology is thought to be the gold standard for diagnosing neoplastic meningitis. The accuracy of this test is important since it may determine whether or not a patient is given intra-thecal chemotherapy. Previous studies indicate that >10.5ml of CSF should to be collected in order to achieve a false negative rate of ≤3% and European Guidelines on CSF analysis recommend that this be the minimum volume collected.275 consecutive CSF cytology samples from the West Midlands regional clinical neuroscience and haemo-oncology centre were audited to see how many met this standard.The median volume of CSF sent for cytology was 2 ml (IQR1 to 5 ml). In fact only 2 (<1%) of the samples met the standard of >10.5 ml, though a further 22 (8%) of samples were close at 10 to 10.5 ml.A re-evaluation is needed of the way in which neoplastic meningitis is diagnosed in the West Midlands. If newer techniques are sufficiently sensitive to allow an acceptable false positive rate with a lower volume of CSF, local guidelines should be drawn up which take this into account, otherwise the doctors collecting the samples need to be educated and monitored to ensure they meet the established standard.
DOES HIPPOCAMPAL DAMAGE EXPLAIN MEMORY LOSS IN VASCULAR COGNITIVE IMPAIRMENT?
Authors: Hosseini Akram , Meng Dewen , Simpson Richard , Auer Dorothee 
Published: October 2015

IntroductionCognitive impairment (CoI) is common in old age and following cerebrovascular disease. The contribution of acute and chronic cerebral infarcts and hippocampal integrity to various domains of CoI remains unclear.Methods100 patients with recent cerebrovascular ischaemic events and >30% carotid artery stenosis (age: 75.4±9.2, 40% female) received brain MRI. Addenbrooke's cognitive examination was used for assessment. Acute and chronic ischaemic volume was identified on diffusion tensor and FLAIR images. Total ischaemic lesion load (TILL), and mean diffusivity (MD) of bi-hippocampi were calculated. A statistical cognitive prediction model was build using age and vascular risk factors between cognitively impaired subgroup against the normal cognition. Next, TILL and then hippocampal MD were added to the relevant risk factors. AUC for each model was compared.Results51% were cognitively impaired, particularly the eldest (P=0.002). Age and TILL were independently associated with CoI (P=0.03), specifically executive dysfunction. Hippocampal MD was significant predictor of overall cognition, particularly memory, after adjusting for age and infarction volume (P=0.001). AUC of model prediction confirmed superior predictability of hippocampal MD for cognition.ConclusionIn vascular cognitive impairment, hippocampal integrity independently contributes to anterograde, long recall and recognition memory, whilst volume of brain infarcts correlates with executive dysfunction.
INVESTIGATION OF MOTOR LEARNING IN PARKINSON'S DISEASE USING AN ACTION ACQUISITION TASK
Authors: Fearon Conor , Newman Louise , Quinlivan Brendan , Butler John , Lynch Tim , Reilly Richard 
Published: October 2015

Movement learning is complex, involving multiple structures including cortex, cerebellum and the basal ganglia. In idiopathic Parkinson's disease (PD) there is initial loss of dopaminergic innervation to the caudal putamen, which governs habitual movement. With disease progression, however, this spreads to involve more anterior regions of the basal ganglia involved in goal-directed behaviour.Given the loss of phasic dopamine signaling in these areas we expect motor learning to be impaired in PD. The goal of this study is to investigate movement learning in PD and healthy controls using a computer-based action acquisition task.A cohort of PD patients and age-matched healthy controls were asked to repeatedly manipulate a joystick in order to move an unseen cursor on a computer screen, initially to a seen target (task 1) and then to an unseen target (task 2). By examining how these movements are refined in order to locate the target from task 1 to task 2 (which requires action selection by the basal ganglia) we quantify the rate at which movements are learned.The results show differences in parameters associated with the execution of the action acquisition task for the PD cohort when compared to healthy controls, supporting impaired motor learning in PD.
THE PREDICTIVE VALUE OF DIFFERENT MOTOR FEATURES IN PARKINSON'S
Authors: Macleod Angus , Counsell Carl 
Published: October 2015

BackgroundOther than tremor dominance (TD) or the postural instability-gait disorder phenotype (PIGD), little is known about what baseline motor characteristics influence prognosis in Parkinson's disease (PD).MethodsWe analysed data from the PINE study, a prospective, community-based incident cohort of PD in Aberdeen, Scotland, recruited between 2002–09, with long term follow-up. Baseline motor features derived from the UPDRS part 3 scale were assessed as independent predictors of four outcomes: death, dependence (Schwab & England <80), “death or dependence”, and progression to Hoehn & Yahr stage 3 (H&Y3) using Cox regression adjusting for age.Results198 patients were analysed (median 6.1 years follow-up). In univariable analyses, bradykinesia score, axial score, limb score, axial/limb ratio, dopa-responsive signs, and dopa-non-reponsive signs were significant predictors of all outcomes. In univariable analyses, asymmetry predicted dependency and death or dependency and TD/PIGD only predicted H&Y3. In multivariable analyses bradykinesia and axial/limb ratio were independent predictors of death and death or dependency and axial score and dopa non-responsive symptoms were predictors for dependency and H&Y3.DiscussionDifferent motor characteristics were independent predictors of different outcomes. However, a variable relating to severity of axial signs was a common theme across three of these outcomes. Thus, at diagnosis, axial motor features appear to be important predictors of prognosis, perhaps stronger than the TD/PIGD classification and deserve further study.
SUSCEPTIBILITY WEIGHTED IMAGING TO DETECT NIGRAL IRON ACCUMULATION IN PARKINSON'S DISEASE
Authors: Martin-Bastida Antonio , Politis Marios , Loane Clare , Lao-Kaim Nicholas , Valle-Guzman Natalie , Kefalopoulou Zenovia , Gesine Paul , Foltynie Thomas , Barker Roger , Piccini Paola 
Published: October 2015

IntroductionDopaminergic neuron loss in substantia nigra (SN) in patients with PD is associated with deposits of iron. Susceptibility weighted imaging (SWI) is a high-resolution MR-based imaging technique for quantifying iron depositions in vivo. SWI may be a robust biomarker for clinical characterization of PD.MethodsForty-two patients with PD were studied with SWI imaging. Average phase shift (radians) and percentage iron deposition of the SN were analysed using SPIN software. SWI data were also compared between PD patients with early (4.0±0.5 PD duration) vs. established PD (7.7±1.9), and according to low (21.2±4.5) vs. high (41.3±8.7) UPDRS-III-assessed motor symptoms severity. Data were interrogated using analysis of covariance (age), relative to healthy controls, and using post hoc univariate tests.ResultsPD patients had higher phase shift values (p<0.01) and iron deposition percentage (p<0.001) in the SN bilaterally, compared to healthy controls. Phase shift values were significantly higher in patients with established PD compared to those with early PD (p<0.05) and higher UPDRS-III motor scores (p<0.05).ConclusionsPD patients show higher levels of deposits of iron in SN compared to healthy controls, and increased iron levels in SN are associated with prolonged disease and increased motor disability
A CASE OF ATRAUMATIC CONVEXAL SUBARACHNOID HAEMORRHAGE DUE TO UNUSUAL CAUSE
Authors: Zaidi SAH , Agrawal Amit , Davey Richard 
Published: October 2015

ObjectiveTo describe a rarely described case of severe internal carotid artery stenosis presented as convexal subarachnoid haemorrhage (SAH) in the setting of acute ischemic stroke.Case reportA 52 year old female, smoker, presented with right sided numbness, slurred speech and difficulty in walking. Neurological observation revealed mild expressive dysphasia and subtle right sided weakness. Brain imaging showed left cortical sulcal SAH with ischemia in the left corona radiata. Carotids ultrasound showed 90% stenosis of left internal carotid artery that was later confirmed by magnetic resonance angiogram. She was treated conservatively for acute ischemic stroke and later underwent left carotid endarterectomy.ConclusionsThis case shows that ICA stenosis can cause convexal SAH and it should therefore be added to the differential diagnosis of atraumatic SAH. The association is under recognised and can be missed due to the unusual location of the blood and failure to image the extra cranial carotid arteries.
NETWORK DYSFUNCTION IN POST-STROKE APHASIA
Authors: Geranmayeh Fatemeh , Wise Richard 
Published: October 2015

Recovery of language function after aphasic stroke is the product of activity in domain-general and domain-specific distributed brain networks. These may include left and right fronto-temporo-parietal, cingulo-opercular, and default mode networks.This functional MRI study investigated the effects of a previous left hemisphere stroke on brain activity during speech production in fifty-three patients. The results were related to twenty-four healthy participants. The analyses investigated not only local activity, but also functional connectivity both within and between distributed networks.Although activity within individual networks was not predictive of speech production, the relative activity between networks was a predictor of both within-scanner and out-of-scanner performance, over and above that predicted from lesion volume and various demographic factors. The specific imaging predictors were the differential activity between the default mode network and both the left and right fronto-temporo-parietal networks, respectively activated and deactivated during speech. These networks also showed altered between-network functional connectivity in patients.Therefore the systems neuroscience of recovery of function after focal lesions is not adequately captured by notions of brain regions ‘taking over’ lost domain specific functions, but is best considered as the interaction between what remains of domain-specific networks and the domain-general systems that regulate behaviour.
CADASIL IN NORTHERN IRELAND: A RETROSPECTIVE CLINICAL, NEUROIMAGING AND GENETIC STUDY
Authors: McMacken Grace , McKenna Eugene , McKee Shane , McConville John 
Published: October 2015

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a familial cerebral small vessel disease caused by mutations in the NOTCH3 gene on chromosome 19. Characteristic features include migraine with aura, recurrent subcortical ischaemic events, cognitive impairment and mood disturbances. The disorder is often overlooked or misdiagnosed, and true population prevalence and disease burden is still undetermined. We identified NOTCH 3 mutation positive CADASIL patients from the Northern Ireland genetics databases. We assessed neuroimaging where available and reviewed medical records.21 gene positive patients were identified within this population. Two mutations accounted for 7/9 pedigrees (arg 182 cys and arg141cys). Mean age of diagnosis was 46 years (range 28–65 years). 10/21 patients had a known family history of CADASIL at time of genetic testing. 19/21 patients were symptomatic: migraine with aura (61%); stroke (52%); TIA (43%); dementia (29%); mood disturbance (29%); seizures (14%). MRI features were highly variable but showed striking similarities within families; some exhibiting changes considered classical of CADASIL and others demonstrating only non-specific white matter changes. One patient exhibited vasculitic type changes on MR angiogram. This study demonstrates the wide variability in the phenotype and MRI features of CADASIL.
THE IMPACT OF TRAUMATIC BRAIN INJURY ON PATIENT-REPORTED PHYSICAL AND MENTAL HEALTH
Authors: Gorgoraptis Nikos , Zaw-Linn Joanna , Feeney Claire , Jimenez Carmen Tenorio , Niemi Mari , Malik Aysa , Ham Timothy , Baxter David , Goldstone Anthony , Sharp David 
Published: October 2015

Traumatic Brain Injury (TBI) is a major cause of disability, with staggering social consequences, but little is known on what determines the impact of TBI from the patient's perspective.In an audit of 240 patients (172 males, median age: 42 years, range: 20–89 years) who attended our multidisciplinary TBI clinic, we examined the impact of cognitive function, depression, sleep, history of substance abuse, age, injury severity (172 moderate-severe, 68 possible-mild TBI) and time since injury (median: 4 months, range: 20 days–28 years) on two patient-reported measures of quality of life: SF–36 Health Survey and Nottingham Health Profile (NHP).Worse outcomes in NHP and both Mental and Physical sub-scores of SF–36 were associated with poorer cognitive performance, as measured by ACE–R. Depression, examined using BDI–II, showed a strong negative correlation with both quality of life measures. Sleep-related symptoms were also strongly associated with worse patient-reported outcomes. Patients reported poorer outcomes in both SF-36 and NHP when examined at a later time since their injury, and this effect could not be attributed to older age.Impairments in cognition, mood and sleep following TBI may have a significant impact on patients' perception of their physical and mental well-being.
ULTRASOUND ASSISTED LUMBAR PUNCTURE; THE NOT-SO-STEEP LEARNING CURVE
Authors: Weerasinghe Kalani Chisha , Sharma Anupama , Weerasinghe Asoka , Tung Patrick 
Published: October 2015

Lumbar puncture is a frequently undertaken procedure in Neurology and Acute Medical settings. It can at times be challenging requiring several attempts causing patient discomfort and anxiety.Ultrasound assisted Lumbar Puncture/Epidurals are currently undertaken by Anaesthetists trained in the technique but not by Neurologists or Medical Physicians in the United Kingdom.Our goal was to train non-Anaesthetic doctors to use ultrasound to assist lumbar punctures through a short training course. The Sonography Assisted Lumbar puncture Training (SALT) course was conducted at Mid Yorkshire Hospitals NHS trust which consisted of 80 minutes of didactic teaching and 160 minutes of supervised scanning.A total of 31 candidates of which 35.48% (n=11) were Neurology trainees and consultants undertook this course. The end of course assessment revealed that 87.09% were able to stabilize the probe and optimize the ultrasound image independently, whereas 93.54% were able to identify the relevant sonoanatomy independently. The remaining candidates demonstrated these skills with minimal prompting.Ultrasound assisted lumbar puncture is a useful technique, which improves the success rate in difficult lumbar punctures. This study shows that it can be taught in a controlled environment through a focussed short training programme.
THE SPECTRUM OF MIGRAINE IN 306 PATIENTS WITH CADASIL
Authors: Tan Rhea , Markus Hugh 
Published: October 2015

Migraine is often the first manifestation of CADASIL, the commonest monogenic cause of stroke. We aimed to determine the pattern of migraine, and its relationship with other features of CADASIL. Of 314 patients seen in a national referral clinic, 71.6% reported migraines. Migraine was the first feature of CADASIL in 67.6% of symptomatic individuals. Females (81.7%) were more likely to develop migraine than males (66.9%), and suffered migraine at an earlier age (27.0±12.7 vs 32.3±13.2 years, p=0.004).89.8% of migraneurs experienced aura. Where aura occurred it was visual in 69.7% sensory 58.7%, dysphasic 30.3%, confusional 17.9%, motor 15.9% and sensorimotor 9.95%.A self-limiting encephalopathy occurred in 33 patients (10.8%); 72.7% of these evolved from a migraine episode. Patients with confusional migraines were more likely to develop encephalopathy (OR=3.93, 95% CI=1.51–9.73, p=0.0024).Non-migraneurs had a higher cumulative risk of stroke than those migraneurs (HR 2.13, 95% CI 1.53–2.96, p=6.8×10−6).In conclusion, the spectrum of migraine in CADASIL differs from that in the general population. Acute encephalopathy is a common feature of CADASIL and usually evolves from a migraine episode. Non-migraneurs have a higher risk of developing stroke, however the reasons for this are not well understood.
PATIENT SATISFACTION WITH MEDICAL ENCOUNTERS IN NEUROLOGY OUTPATIENT CLINICS
Authors: Reuber Markus , Toerien Merran , Wiseman Hannah , Shaw Rebecca , Duncan Rod 
Published: October 2015

AimRecent public policy documents have emphasised the need for healthcare practitioners to give patients choice. As part of a larger qualitative project investigating how neurologists give patients choices, we explored whether evidence of patient choice is associated with higher patient satisfaction.MethodFourteen neurologists and 223 patients were recruited in neurology outpatient clinics in Glasgow and Sheffield. All participants completed post-appointment questionnaires assessing whether choice was offered or perceived. Clinicians also rated the extent to which symptoms were medically explained. Patients completed the Medical Interview Satisfaction Scale-21 (MISS-21).ResultsTwo dominant factors contributed to the total MISS-21 scale, ‘rapport’ and ‘distress-relief’. Regression analysis showed that the extent to which symptoms were medically explained predicted most of the variance on both subscales and the total MISS-21 score. Spearman's rho correlations showed greater patient satisfaction if symptoms were medically explained and if no choice had been offered or perceived.ConclusionThere was no evidence that giving patients choices in interaction increased patient satisfaction with clinical encounters. Our findings suggest that patient satisfaction is most strongly influenced by the extent to which neurologists perceive symptoms as medically explained.