bluuebunny/crossref_metadata_2025_split huggingface dataset:
013 The impact of aggressive blood pressure management in the post-thrombolysis setting
Authors: Harper Bethan , McNaughton Harry , Ranta Anna 
Published: July 2019

IntroductionHigh blood pressure (BP) post-thrombolysis has been associated with an increased rate of bleeding and poorer outcome. We noted frequent BPs of >180 mmHg with a target of keeping BP <180. We tested whether a more aggressive target of SBP <160 mmHg would result in fewer BP protocol violations.MethodsPatients were prospectively captured comparing patients thrombolysed during the 12 months before and 12 months following the introduction of a new more aggressive BP protocol, allowing for a 6 month transition period. Results were adjusted for baseline function and stroke severity using regression analysis.ResultsPre-protocol change 68 and post- 100 patients were thrombolysed. Baseline characteristics were similar between groups. There was a trend for a lower rate of SBPs >180 mmHg (adjusted OR 0.49; 95% CI 0.31–1.1; p=0.097) and a significantly higher rate of SBPs <120 mmHg (adjusted OR 3.06; 95% CI 1.52–6.17; p=0.002) in the aggressive BP protocol group; although events of extreme SBPs (>200 and <100 mmHg) were similar between groups. Favourable outcomes (mRS = 0–2) at 3 months were similar between groups (adjusted OR 1.27; 95% CI 0.58–2.8; p=0.56) as was the rate of symptomatic haemorrhages (adjusted OR 1.26; 95% CI 0.28–5.7; p=0.76). Model fit was improved by adding study group to the model.ConclusionsMore aggressive post-thrombolysis BP management lowered the overall BP, but did not result in improved patient outcomes. Potential explanations include a small sample size, reduced cerebral perfusion off-setting reduced bleeding risk, or high BP being merely an epiphenomenon of worse outcome rather than causative.
025 The utility of nerve blood flow in the assessment of peripheral neuropathy
Authors: Borire Adeniyi , Issar Tushar , Kwai Natalie , Visser Leo , Poynten Ann , Simon Neil , Kiernan Matthew , Krishnan Arun 
Published: July 2019

IntroductionAnimal and human studies of diabetes have shown significant alterations in nerve blood flow (NBF), which may also play a role in the development of neuropathy. However, the non-invasive assessment of NBF in human subjects has remained elusive until the recent technological advancements in ultrasound (US) technology. We undertook sonographic assessment of NBF in 75 patients with type 2 diabetes and correlated the findings with neuropathy severity scores and electrophysiological parameters.MethodsBlinded median and tibial nerve ultrasound scans were performed at non-entrapment sites using a high-resolution linear probe. NBF was quantified using power Doppler techniques to obtain the vessel score (VSc) and maximum perfusion intensity (MPI). Routine nerve conduction studies were performed, and neuropathy severity was assessed using the total neuropathy score (TNS). Aged- and gender-matched controls were enrolled.ResultsDiabetic nerves had higher rates of NBF detection (28%) compared to the control group (p<0.0001). Significant correlations were found between NBF parameters and nerve size (p<0.001), reported sensory symptoms (p<0.05) and neuropathy severity scores (p<0.001). The cohort with diabetes had significantly larger median (8.5 ± 0.3 mm2 vs. 7.2 ± 0.1 mm2, p<0.05) and tibial (18.0 ± 0.9 mm2 vs. 12. 8 ±0.5 mm2, p<0.05) nerves compared to controls.ConclusionsPeripheral nerve hypervascularity is detectable by US in moderate to severe diabetic neuropathy with prominent sensory dysfunction. Consistent with previous studies, the sonographic detection of NBF is a pathological finding.
003 Subthalamic nucleus deep brain stimulation evoked resonant neural activity predicts clinical response to DBS
Authors: Xu San San , Sinclair Nicholas C , Bulluss Kristian J , Perera Thushara , Lee Wee-Lih , McDermott Hugh J , Thevathasan Wesley 
Published: July 2019

IntroductionDBS can improve motor deficit in Parkinson’s disease (PD) patients. Existing devices have limitations due to electrode positioning errors, fallible manual programming and delivery of continuous ‘open-loop’ stimulation despite fluctuating patient state. This results in partial efficacy, adverse effects and increased cost. One solution is to use an electrical feedback signal or ‘biomarker’ recorded from DBS electrodes. The most widely studied signal has been spontaneous local field potentials (LFPs), particularly beta band (13–30 Hz) and high frequency oscillations (HFO) (200–400 Hz). Here, we report a novel biomarker in the form of a large amplitude, evoked potential, with a characteristic oscillatory decay, termed evoked resonant neural activity (ERNA).1MethodsLFPs and ERNA were recorded in 14 patients with PD (28 hemispheres) undergoing STN DBS surgery. The four contacts in each electrode array were ranked according to ERNA amplitude, beta power, HFO power and proximity to the anatomically ideal stimulation location. At least 3 months after surgery, motor scores (UPDRS III, reaction time) were evaluated off-DBS and during stimulation delivered through each electrode contact in a randomised order.ResultsERNA amplitude, beta power and contact proximity to the anatomically ideal stimulation location predicted magnitude of therapeutic response to DBS. However, after exclusion of covariance, ERNA amplitude remained the only significant predictor of DBS response.ConclusionERNA is a readily recordable, large amplitude signal that accurately correlates with motor response to DBS. It holds significant potential as a biomarker for guiding electrode implantation, ideal contact selection, automated parameter fitting and delivery of closed-loop DBS.ReferenceSinclair NC, McDermott HJ, Bulluss KJ, Fallon JB, Perera T, Xu SS, et al. Subthalamic nucleus deep brain stimulation evokes resonant neural activity. Annals of neurology 2018;83(5).
034 Prospective study of 24-hour levodopa-carbidopa intestinal gel therapy for unresponsive freezing of gait
Authors: Nagaratnam Sai , Chang Florence Ching-Fen , Tsui David S , Mahant Neil , Wolfe Nigel , Kim Samuel D , Ha Ainhi D , Griffith Jane M , Fung Victor SC 
Published: July 2019

IntroductionWe report a prospective, open-label study of 24-hour levodopa-carbidopa intestinal gel (LCIG) as treatment for levodopa ‘unresponsive’ freezing of gait (FOG) associated with Parkinson’s disease.Method14 patients with disabling FOG, documented as being levodopa ‘unresponsive’ following dose cycle assessment, were commenced on continuous 24-hour infusion LCIG therapy with the night-time rate at 50–80% of the daytime infusion rate. Patients underwent baseline and 6 months gait assessments, documentation of their falls frequency and completed FOG questionnaires. The statistical analysis was performed with SPSS 25 software (IBM), using Wilcoxon Signed Rank test and p < 0.05 was set as statistically significant.ResultThe fall frequency score reduced from 3 to 1 at 6 months (p=0.011), The FOG questionnaire was 19.3±1.5 at baseline and reduced to 16.0±1.7, at 6 months, respectively (p=0.039). The mean 360 degree turn time was not significantly changed (p=0.77). The daytime LCIG infusion rate was not significantly different before and after 24-hour LCIG therapy (p=0.315). 7 subjects developed visual hallucinations, one required temporary withdrawal from 24-hour LCIG, but as FOG worsened on 16-hour LCIG, he elected to recommence 24-hour LCIG infusion with improvement in FOG with a reduction in falls. 1 subject had postural hypotension which was ameliorated after adjustment of LCIG infusion rate.ConclusionWe provide further evidence that 24-hour LCIG therapy can reduce levodopa ‘unresponsive’ FOG and associated falls.
037 Frontotemporal dementia or frontal variant alzheimer’s disease? A case series
Authors: Powell Alice , Foxe David , Halliday Glenda M , Piguet Olivier , Hodges John R , Burrell James R 
Published: July 2019

IntroductionAccurate prediction of the underlying neuropathology in behavioural variant frontotemporal dementia (bvFTD) is essential for future targeted therapy trials and prognostication. Alzheimer’s disease (AD) pathology has been reported in a significant proportion of patients with clinical bvFTD. We sought to determine whether detailed clinical and neuroradiological assessment was sufficient to distinguish bvFTD with AD pathology from bvFTD with frontotemporal lobar degeneration (FTLD).MethodsTwo patients with clinically diagnosed probable bvFTD but AD pathology at autopsy, were identified. The clinical, neuropsychological and imaging features of these patients were compared with those of ten patients with clinically probable bvFTD and proven FTLD pathology (tau, TDP-43, FUS).ResultsBoth patients with AD pathology presented with behavioural symptoms typical of bvFTD as well as memory impairment. Executive function, memory and visuospatial skills were impaired in both pathologic groups. Language skills were relatively spared in those with AD pathology. Neuropsychiatric symptoms were frequent in both groups but significant depression and anxiety were seen only in those with FTLD pathology. Dementia severity and caregiver burden were similar. The degree or topographical distribution of atrophy on MRI did not differ.ConclusionsAlzheimer’s pathology may cause bvFTD symptoms which are otherwise indistinguishable to those caused by FTLD pathology. While there may be subtle differences in patterns of cognitive deficits, standard neuropsychological testing is insufficient to discern the underlying pathology. Similarly, structural imaging cannot be used to reliably identify AD pathology. Better access to amyloid biomarkers may be needed to more accurately define bvFTD caused by AD pathology.
038 Tremor: a clinical and neurophysiological study
Authors: Fois Alessandro , Mahant Neil , Vucic Steve , Fung Victor SC 
Published: July 2019

IntroductionTremor is a common clinical problem seen in a number of diseases. Robust classification and diagnosis of tremor remains controversial due to overlap in clinical features and lack of established biomarkers. This hampers effective research including therapeutic trials. We present our research protocol for a cross-sectional study which aims to find more robust methods of tremor classification and diagnosis.MethodsAdults with upper limb tremor of varying aetiologies, diagnosed using current clinical criteria (including essential tremor, Parkinsonian tremor, and dystonic tremor), and age-matched controls are eligible for recruitment. Participants undergo a clinical and neurophysiological assessment, including accelerometry, surface electromyography, long-latency stretch reflexes, temporal discrimination, and tonic vibration reflexes. Data will be analysed using a cluster analysis to identify robust tremor syndromes and biomarkers associated with them. We aim to recruit 100 participants prior to analysis.ResultsAt time of writing, 13 participants with upper limb tremor have been studied (6 with essential tremor, 5 with dystonic tremor, and 2 with indeterminate tremor; mean age 66 years, range 18–85). Participants tolerated the clinical and neurophysiological studies well with 100% completion rate after recruitment. With current rates of recruitment we anticipate completion of recruitment and commencement of data analysis in October 2019.ConclusionsOur protocol aims to identify robust tremor phenotypes and biomarkers for them. This will allow patients with tremor to be classified into more biologically homogeneous diagnostic categories, aiding future research into the mechanism of tremor and more rational clinical trial design.
042 Respiratory function and cognitive profile in motor neuron disease
Authors: Huynh William , Sharplin Lara E , Caga Jashelle , Highton-Williamson Elizabeth , Kiernan Matthew C 
Published: July 2019

IntroductionMotor neuron disease (MND) is increasingly recognised as a multisystems disorder with 30–50% of patients having mild to moderate cognitive impairment. Mechanisms of cognitive dysfunction in MND are multifactorial but chronic hypoxia secondary to respiratory dysfunction may contribute to cognitive decline in patients.ObjectivesThe current study aimed to identify the relationship between respiratory function in MND patients and the presence and degree of cognitive impairment.MethodsMND patients were prospectively recruited from a multidisciplinary MND clinic. Patients meeting the criteria for frontotemporal dementia were excluded. Baseline clinical assessments including respiratory function as assessed by spirometry were recorded with FVC ≤ 75% considered to have reduced respiratory function. Cognitive testing was performed utilising the Addenbrooke’s Cognitive Examination (ACE).ResultsFrom a cohort of 100 MND patients 48% were categorised as having impaired respiratory function whilst 52% had normal function. Compared to the group with normal respiratory function (ACE: 86.83±1.5), patients with respiratory dysfunction had significantly reduced cognitive function (ACE: 90.68±0.89, P=0.025). Subscores demonstrated significant differences between the groups with respect to domains in memory, attention with a trend observed in fluency. There was a significant correlation between FVC and ACE scores as well as between FVC and memory and fluency subscores (P<0.01).ConclusionMND patients with respiratory compromise were more likely to develop reduced cognitive function. In addition to improving physical function, it remains plausible that non-invasive ventilation may alter the progression of cognitive impairment in MND patients, thereby potentially improving their overall quality of life and carer burden.
051 Overlapping syndrome of giant cell arteritis and ANCA-associated vasculitis complicated by severe axonal neuropathy: a case report
Authors: Perju-Dumbrava Laura , Islam Fariha , Makonahalli Rohitha , McLean Catriona , Griffiths Hedley , Malhotra Abhishek 
Published: July 2019

CaseA 61-year- old woman presented with what was thought to be a refractory, temporal artery biopsy-proven giant cell arteritis (GCA). After 4 months of therapy she was unable to get the prednisolone dose below 40 mg/day, so weekly subcutaneous Tocilizumab was introduced but with minimal response after 8 weeks. She was then admitted to hospital with a severe rapidly progressive length dependent sensorimotor peripheral neuropathy. Nerve conduction studies showed predominantly an axonal neuropathy with multiple pseudo-conduction blocks.Sural nerve and gastrocnemius biopsies revealed a necrotising vasculitis with numerous giant cells and active axonal degeneration. She proved to be ANCA-PR3 positive, without other systemic manifestations. ANCA-associated vasculitic neuropathy was diagnosed and she was treated with two 1g Rituximab infusions, 2 weeks apart.On follow-up after a month, she had regained some strength but still required a wheelchair for mobility. Further Rituximab infusion after 6 months is planned; prednisolone had been successfully weaned to 10 mg/day.ConclusionPeripheral nerve involvement, which is relatively common in ANCA-associated vasculitis, has also been reported in 14% of GCA cases. But ANCA-PR3 positivity is rare in biopsy-proven GGA, with only a handful of well-documented cases.Heightened suspicion of an alternative diagnosis in the face of an unusual clinical course (lack of steroid response and appearance of small vessel vasculitic symptoms for what is accepted to be a large vessel vasculitis) is critical and our experience highlights the important fact that a diagnosis of one of these disorders does not preclude the subsequent diagnosis of the other.
054 Impact of online adult headache guideline on headache referral to neurology clinic
Authors: Huang Leon , Bourke David , Ranta Anna 
Published: July 2019

IntroductionHeadache is a common problem in primary care and is one of the main reasons for general practitioners to consult neurology service. Decision for referral may be influenced by uncertainty of diagnosis and referrer’s clinical confidence in identifying risks of secondary causes. We examined the effect of an online guideline for adult headache on referrals to neurology service, and analyse the quality of the referrals and outcome of their clinical assessment.MethodsWe examined the referrals in selected months within 2 years before and 2 years after online publication of a headache guideline developed by our neurology department. Referrals for headaches being the primary complaint were analysed. The primary outcome was the proportion of neurology referrals for headache as the primary complaint pre- versus post guideline.ResultsNine hundred neurology referrals before and 801 neurology referrals after the publication of online headache guideline were included. There was a significant reduction in proportion of neurology referrals for headaches (OR = 0.73 (0.55 – 0.96), p = 0.026). The proportion of headache referrals requiring face-to-face assessment in clinic was also reduced from 69% before and 53% after publication of the headache guideline (p=0.014). There are similar proportions of patient referred without adequate trial of preventative medication prior (33.8% vs. 27.7%), and over used analgesics (21.9% vs. 25.0%).ConclusionsThe headache guideline demonstrated effectiveness in reducing demand for assessment of headaches in neurology clinic. Headache management prior to referral is an ongoing area of concern.
005 Electroclinical characteristics of autoimmune encephalitis as outcome biomarkers
Authors: Wesselingh Robb , Broadley James , Kyndt Chris , Buzzard Katherine , O’Brien Terence , Monif Mastura 
Published: July 2019

IntroductionSeizures are a common characteristic of Autoimmune encephalitis (AIE). The use of the electroclinical characteristics to assist in the diagnosis of AIE has been explored1 however use of specific electroencephalogram (EEG) changes has not been examined with regards to outcome prediction.MethodsPatients with AIE were recruited retrospectively across 4 hospitals in Victoria. Clinical Data was collected during admission and at final follow-up. EEGs of patients were reviewed using an objective proforma. Associations between EEG biomarkers and clinical outcomes were demonstrated using logistic regression modelling.ResultsWe recruited 88 patients with AIE and available EEGs. Presence of rhythmic delta, superimposed fast activity and an abnormal background were significantly more common in N-methyl-D-aspartame receptor (NMDAR) antibody associated AIE patients (p<0.05). ICU admission was associated with rhythmic delta epileptiform activity (OR 3.25, p=0.046), sharp elements in the EEG abnormality (OR 3.55, p=0.05), and an abnormal background rhythm (OR 3.56, p=0.03). Development of drug resistant epilepsy was associated with prolonged duration of abnormality on EEG (OR 11.99, p=0.013), and sharp elements in the EEG abnormality (OR 7.29, p=0.02).ConclusionWe have identified EEG biomarkers that differentiate NMDAR AIE from other subtypes, and likely represents an objective description of extreme delta brush which has previously been described in NMDAR AIE.2 We have also demonstrated biomarkers associated with important outcomes that can be used to help guide treatment and prognosis.ReferencesLimotai C, Denlertchaikul C, Saraya AW, Jirasakuldej S. Predictive values and specificity of electroencephalographic findings in autoimmune encephalitis diagnosis. Epilepsy Behav 2018;84:29–36.Veciana M, Becerra JL, Fossas P, Muriana D, Sansa G, Santamarina E, et al. EEG extreme delta brush: An ictal pattern in patients with anti-NMDA receptor encephalitis. Epilepsy Behav 2015;49:280–5.
057 A rare presentation of a rare disease: adult onset krabbe’s disease presenting with progressive hypertrophic polyradiculoneuropathy
Authors: MacDonald Allycia , Lamont Phillipa , Leong Wai 
Published: July 2019

IntroductionGloboid Cell Leukodystrophy (Krabbe’s disease) is a rare autosomal recessive condition caused by defects in the lysosomal enzyme galactosylceramidase. The disease typically occurs in infants and is rapidly progressive. Fewer than 5% of cases develop symptoms in adulthood, the majority of which present with spastic paraparesis.CaseA 40-year-old Italian man presented with a 20 year history of gradually progressive asymmetrical hand paraesthesias and weakness. EMG demonstrated demyelinating features and absent sensory responses. CSF examination revealed elevated protein with no cells and negative oligoclonal bands. Treatment with intravenous immunoglobulin (IVIg) was commenced and continued for 2 years for presumed CIDP without clinical response. Upper limb wasting and weakness progressed over the following 10 years with associated loss of reflexes, prompting consideration of an underlying genetic neuropathy. DNA testing for common CMT mutations was negative. MRI cervical spine and brachial plexus demonstrated diffuse bilateral non-enhancing enlargement of cervical nerve roots. IVIg was recommenced over the next 10 years with continued clinical deterioration to the point of bilateral flail arms with relatively preserved lower limb motor function, in addition to bulbar and respiratory dysfunction by age 64. MRI brain demonstrated thickening of a number of cranial nerves without contrast enhancement, and extensive T2-hyperintensity of periventricular white matter. Next generation sequencing revealed two novel mutations in the galactosylceramidase (GALC) gene, and beta-galactocerebrosidase activity was reduced, confirming the diagnosis of Krabbe’s disease.ConclusionThis case highlights an unusual presentation of Krabbe’s disease with progressive hypertrophic polyradiculoneuropathy, associated with a novel genetic mutation.
006 Can seizure-related heart rate differentiate epileptic seizures from psychogenic non-epileptic seizures?
Authors: Yong Hue Mun Au , Minato Erica , Paul Eldho , Seneviratne Udaya 
Published: July 2019

IntroductionThis study aims to (i)evaluate the diagnostic sensitivity, specificity and predictive values of seizure-related heart rate (HR) in differentiating epileptic seizures(ES) from psychogenic non-epileptic seizures(PNES), (ii)define the most useful point of HR measurement: pre-ictal, ictal-onset, maximal-ictal or post-ictal, and (iii)define the HR cut-off points to differentiate ES from PNES.MethodsAll video EEG(VEEG) at Monash Health from May 2009 to November 2015 were retrospectively reviewed. Baseline(during wakefulness), one-minute pre-ictal, ictal-onset, maximal-ictal and one-minute post-ictal HR were measured for each ES and PNES event. Events less than ten seconds or with uninterpretable ECG due to artefacts were excluded. ROC curve analysis was performed to study the diagnostic accuracy reflected by area under the curve(AUC). The AUC was interpreted as follows; ≤0.5, differentiation of PNES from ES no better than chance; 0.80–0.89, good differentiation; and 0.9–1, excellent differentiation.ResultsVEEG of 341 ES and 265 PNES from 130 patients were analysed. The AUC for pre-ictal, ictal-onset, maximal-ictal and post-ictal HR were found to have poor differentiation between ES and PNES. Comparing PNES and bilateral tonic-clonic ES, AUC for absolute maximal-ictal HR was 0.84(CI 0.73–0.95) and for absolute post-ictal HR was 0.90(CI 0.81–1.00). Using Youden’s index, to diagnose tonic-clonic ES, the optimal cut-off point for absolute maximal-ictal HR was 114bpm (sensitivity 84%;specificity 82%;PPV 26.7%,NPV 98.5%) and for absolute post-ictal HR was 90bpm(sensitivity 91%;specificity 82%;PPV 30.3%;NPV 99.1%).ConclusionsThese findings suggest that seizure-related HR increase is useful in differentiating bilateral tonic-clonic ES from PNES. Based on the AUC, the best diagnostic measureme.