bluuebunny/crossref_metadata_2025_split huggingface dataset:
072 Signal recognition particle antibody associated necrotizing myositis with ‘burnt-out’ paravertebral muscle atrophy
Authors: Harinesan Nimalan , Reddel Stephen W 
Published: July 2019

IntroductionNecrotizing autoimmune myositis (NAM) is an increasingly recognised myositis.1 While diagnosis is primarily from muscle pathology, antibodies to signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl–coenzyme A reductase (HMGCR) are also associated.CaseA 67 year old woman presented with proximal weakness and elevated creatine kinase (CK) levels following a complicated AMI, CABG and commencement of atorvastatin. Muscle biopsy confirmed necrotizing myositis and SRP (not HMGCoAR) antibodies were positive. Recovery and rehabilitation was slow and her CK did not normalise for 12 months. She was treated with immunotherapy including intravenous immunoglobulin, oral and intravenous corticosteroids, azathioprine, and mycophenolate.Residual hip, abdominal and truncal weakness remained, affecting her gait and posture. CK levels during this period remained normal. MRI demonstrated extensive paravertebral muscle loss with fatty replacement without oedema. Small myopathic units without active features were present on EMG.ConclusionThis case illustrates several important aspects of NAM, which remains a rare disorder. Recovery can be incomplete in up to 40%, especially if control is delayed.2 In our opinion, normalisation of CK is an important target and may be necessary for full recovery, and rising CK may predict relapse.3 We suggest MRI and EMG of weak muscles may assist in distinguishing persisting myositis from ‘burnt-out’ disease, clarifying whether immunotherapy should be increased or not. However relapse on weaning immunosuppression remains frequent. While SRP antibodies in patients who had also taken statins has been previously reported, the quick sequential onset in this case suggests possible causality.ReferencesMammen AL. Autoimmune Myopathies. Contin Lifelong Learn Neurol. 2016;22(6):1852–1870.Watanabe Y, Uruha A, Suzuki S, et al. Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy. J Neurol Neurosurg Psychiatry 2016;87(10):1038–1044.Ramanathan S, Langguth D, Hardy TA, et al. Clinical course and treatment of anti-HMGCR antibody–associated necrotizing autoimmune myopathy. Neurol Neuroimmunol Neuroinflammation 2015;2:e96.
081 Lymphoma: a great imitator in neurology and its many faces
Authors: Yeh Wei Z , Muthusamy Subramanian , McKelvie Penny , Collins Steven , French Ann , Filshie Robin , Reardon Katrina 
Published: July 2019

IntroductionThe label ‘great imitator’ refers to conditions which can cause varied manifestations and mimic diseases. Lymphoma is worthy of this title. We present three cases.Cases1: 66-year-old-man with progressive vertical diplopia and unsteady gait over four weeks. MRI brain and spine demonstrated a supratentorial para-falcine soft tissue lesion, mid-thoracic cord enhancement and right axillary mass. Serum ACE was elevated. Serum HIV serology was positive. Right axillary mass core biopsy diagnosed Burkitt lymphoma.2: 50-year-old man with a 4-week history of constitutional symptoms on a background of ITP and splenomegaly. During admission he developed urinary retention, bilateral lower limb weakness and numbness and confusion. Infectious and vasculitic screens were unremarkable. CT chest, abdomen and pelvis demonstrated splenomegaly. CSF and bone marrow analyses were non-diagnostic. A random skin biopsy diagnosed intravascular lymphoma.3: 65-year-old man with two weeks of headache and diplopia on a background of previously treated Burkitt lymphoma. CSF analysis showed 45×106/L white cells and glucose < 0.6 mmol/L. Cytologic analysis was negative for malignancy. Bacterial culture and Cryptococcal antigen were negative. FDG-PET dramatically showed disseminated spinal and cranial leptomeningeal disease. MRI brain showed thin dural thickening correlating to area of increased uptake on PET. He was diagnosed with Burkitt lymphoma relapse and treated with chemotherapy and autologous stem cell transplant.ConclusionThe varied manifestations in our cases demonstrate the ability for lymphoma to mimic infective, inflammatory, granulomatous (including sarcoidosis) and neoplastic aetiologies. When the diagnosis is uncertain, the possibility of this great imitator should be considered.
086 Clinical, investigational and treatment factors do not determine prognosis of patients with inflammatory neuropathies
Authors: Kapoor Mahima , Carr Aisling , Lunn Michael P , Reilly Mary M 
Published: July 2019

IntroductionIdentifying patients who need long-term immunoglobulin (IVIg) treatment in patient with inflammatory neuropathies is essential as recent treatment trials show a remission rate of up to 40%.AimsCompare retrospective data on clinical, investigational and treatment factors in patients who have ceased IVIg with patients who have failed a cessation trial.Methods15 patients who successfully suspended IVIg infusions were compared with 15 in whom decreasing or stopping IVIg was unsuccessful.Results30 patients (12 with CIDP and 3 with MMN in both groups) were diagnosed 39.5 months from onset of symptoms in the successful group vs. 40.7 months in the unsuccessful group (p=0.953). There was a significant difference in the summed upper limb sensory amplitudes on electrophysiology prior to starting IVIg between the patients with CIDP (17.4 mV vs. 9.8mV p=0.007). There was no difference in the average doses between the groups. A successful cessation trial was attempted at a mean of 60.5 months post starting treatment, compared with 60 months in the unsuccessful patients.ConclusionThere is a need for objective biomarker to measure disease activity because other than one neurophysiology marker, other factors did not help predict a successful cessation trial of IVIg.
099 Do clinical nurse specialist led stroke follow-up clinics reduce post-stroke hospital readmissions and recurrent vascular events?
Authors: Kao Alex , Lanford Jeremy , Wong Lai-Kin , Ranta Anna 
Published: July 2019

IntroductionPost-discharge stroke follow-up clinics have been associated with improved outpatient care and reduced readmission. Pre-2014 there was no consistent follow-up care offered at Wellington Hospital. Our aim was to determine whether the establishment of a clinical nurse specialist (CNS) follow-up clinic reduced the readmission 12-monthrate.MethodsThis is a sequential comparison of patient admitted with stroke one year prior and one year after the clinic was established in 2013. The primary outcome was hospital 12-month hospital readmission rate; main secondary outcome was recurrent vascular event. Patients were identified from the hospital discharge records and underwent detailed electronic chart review. Results were adjusted for differences in baseline characteristics.ResultsWe identified 874 patients; 439 pre- and 435 post-nurse clinic implementation. There was no significant difference between the one-year readmission rate after the establishment of the stroke follow up clinic (adjusted OR=1.06; 95% CI, 0.85–1.64; p=0.804) and no difference in recurrent composite vascular events at one-year (adjusted OR=1.20; 95% CI, 0.68–2.11; p=0.528). The median (IQR) time to follow-up to clinic after discharge was 85 (63–98.5) days. There was a trend towards a reduction in vascular events when limiting the analysis to patients who actually attended clinic, but this trend disappeared when adjusting for baseline inter-group differences.ConclusionsThere was no reduction in the one-year hospital readmission or vascular event recurrence rate for patients with stroke following the establishment of a nurse specialist led stroke follow up clinic. Earlier timed follow-up and the psychosocial value offered by these clinics requires further evaluation.
109 Severe dysautonomia in NMDAR encephalitis
Authors: Donaldson Alana , Venkat Abhay , Zhai Shaun , Patel Ronak , Gawarikar Yash 
Published: July 2019

IntroductionAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the more common forms of autoimmune encephalitis, predominantly affecting children and women of the child-bearing age. It is characterised by memory deficit, behavioural disturbance and seizures. Dysautonomia is recognised as a feature but rarely the first symptom of the condition.1 Here we present a case of severe dysautonomia preceding the diagnosis of NMDAR encephalitis.MethodsWe conducted a retrospective review of the admissions to the neurology ward at the Calvary Hospital, Canberra in 2018 to identify patients diagnosed with NMDAR encephalitis.ResultsOne patient was identified from the registry data. A 37-year-old woman presented with a week-long history of symptomatic orthostatic hypotension. Her supine systolic blood pressure was 110mmHg with a 46mmHg postural drop. Over the first week of hospitalisation, she became increasingly disoriented and erratic in behaviour with fluctuating levels of consciousness requiring intensive unit care. Her CSF demonstrated lymphocytic pleocytosis and NMDAR antibodies were detected in both CSF and serum. She was treated with IVIG, IV steroids and subsequently Rituximab. A pelvic teratoma was found and removed. Her symptomatology including dysautonomia improved substantially by the end of her six-week hospital admission. Her modified Rankin Scale was zero at three months.ConclusionAutonomic dysfunction is not a common feature of autoimmune encephalitides. Our case highlighted the possibility that dysautonomia can be the initiating symptom of this disease entity. Physician awareness is important in the early recognition and treatment of this condition.ReferenceTitulaer MJ, McCracken L, Gabilondo I, Armangue T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013;12(2):157–165.
110 The benefits of a clinical triage tool in acute stroke
Authors: Alanati Khaled , Byrnes Sean , Evans James , Campbell David , Wall Ellen 
Published: July 2019

IntroductionThe advances in management of ischemic stroke and the extended treatment window have greatly increased the demand for acute stroke assessment. There is a need for fast and accurate triage both to identify candidates for acute stroke assessment and to minimize cost and time expended on unnecessary assessments and investigations.We examined the potential benefit of a web-based triage tool designed to be used in the emergency department to identify patients suitable for hyperacute treatment based on the current standard of care.MethodsWe performed a retrospective analysis of Electronic medical records of 235 patients, reviewed by the neurology team at Gosford Hospital for the consideration of hyperacute treatment over six months in 2017. Each patient’s time of onset of symptoms, premorbid function and presenting deficit, as well as the type of treatment received, were collected. By entering those data to our new triage system, we estimated the number of clinical reviews and advanced imaging that could have been avoided.ResultsOut of the 235 patients that were reviewed, 71 patients either received thrombolysis and/or were sent for endovascular treatment, and 164 patients were not suitable for hyperacute treatment. Using the triage tool, we identified that 26% (n=61) of the rapid clinical assessment and 32% (n=42) of CT perfusion scans performed could have been avoided.ConclusionUse of a web-based triage tool is not only effective to identify patients suitable for hyperacute management but also to avoid over-investigation and prioritize rapid neurological clinical assessments.
111 Recurrent headaches with psychosis, CSF lymphocytosis, vessel beading and papilloedema- autoimmune/viral encephalitis with vasculopathy or unusual presentation of reversible cerebral vasoconstriction syndrome (RCVS)?
Authors: Coppin Christopher J , Barnett Michael , Masters Lynette , Aziz Amjed , Narasimhan Manisha 
Published: July 2019

IntroductionHeadache is a common Neurology presentation in both outpatient and ED settings. We present a challenging headache in a previously asymptomatic young female patient.CaseA 27 year old female patient presented to ED with severe headache, vomiting and photophobia, after multiple presentations elsewhere, diagnosed as migraine, with normal neurological examination and MRI. Episodes of excruciating headache were associated with writhing and vomiting but spontaneously resolved with residual background pain. Indomethacin helped but she re-presented with headache and psychosis requiring intubation and ICU admission.Investigations included CSF - protein 0.85, leucocytosis 58 cells (lymphocytic) and normal cerebral venography. Acyclovir was commenced but viral PCRs were negative and she was extubated. MRI was suspicious for subtle posterior sulcal hyperintensity and beading in occipital and posterior cerebral arteries.Another episode occurred ten days later requiring intubation, examination showed papilloedema. CSF examination showed raised pressure, normal protein and 48 lymphocytes; flow cytometry and cytology were unrevealing. NMDA receptor antibody returned positive in serum but not CSF. Subtle beading in right PCA branches remained. IVIG and steroids were used with dramatic response. Further investigation revealed positive EBV serology only and papilloedema resolved. Steroids were weaned and she remains in remission.ConclusionWe present an interesting case of recurrent acute headaches with intracranial hypertension, psychosis and CSF lymphocytosis. The recurrent headaches and vessel beading suggest RCVS, perhaps triggered by viral or autoimmune encephalitis. The NMDA receptor antibody result should be interpreted with caution given the absence of antibody in CSF and dramatic recovery.
112 Axonal polyneuropathy with onset in young adulthood due to TUBB3 mutation
Authors: Yang Po Sheng , Forrest Kimberley K , Wilson Ian B 
Published: July 2019

IntroductionThe TUBB3 gene encodes the protein Beta-tubulin isotype III, a component of the microtubule cytoskeleton. Mutations in this gene have been associated with axonal polyneuropathy, however usually associated with congenital fibrosis of the extraocular muscles (CFOEM) and other abnormalities of cerebral development.1 2 We report a case of isolated neuropathy associated with a TUBB3 mutation.MethodsCase report - clinical information and next generation sequencing results were obtained.ResultsA 64 year old man presented with a severe, progressive, length dependent sensorimotor polyneuropathy which commenced in his late twenties. There was no clinical involvement of the extraocular muscles and cognition was normal. Family history was limited, but there were no other members affected.The patient had previously been extensively investigated including sural nerve biopsy, which confirmed axonal neuropathy without a specific diagnosis. Intravenous immunoglobulins and steroids had been trialled without benefit.A neuromuscular gene panel utilising next generation sequencing was performed and demonstrated heterozygosity for a variant of the TUBB3 gene (D417N substitution).Case series describing TUBB3 mutations show a large heterogeneity in phenotypic expression depending on the amino acid substitution.2–4 There is also heterogeneity in patients with D417N mutations, although a small number have been reported to develop a polyneuropathy without CFOEM1.ConclusionsThis case strengthens previous reports that TUBB3 mutation can be associated with a pure, axonal, sensorimotor polyneuropathy and highlights the use of next generation sequencing in streamlining the diagnostic process.ReferenceTischfield M, et al. Human TUBB3 mutations perturb microtubule dynamics, kinesin interactions, and axon guidance. Cell 2010;140(1):pp.74–87.Ncbi.nlm.nih.gov. (2019). Congenital fibrosis of the extraocular muscles - Conditions - GTR - NCBI. [online] Available at: https://www.ncbi.nlm.nih.gov/gtr/conditions/CN043677/ [Accessed 14 Feb. 2019].Omim.org. (2019). OMIM Entry - * 602661 - TUBULIN, BETA-3; TUBB3. [online] Available at: https://www.omim.org/entry/602661 [Accessed 14 Feb. 2019].Krupa, K. and Bekiesinska-Figatowska, M. (2013). Congenital and Acquired Abnormalities of the Corpus Callosum: A Pictorial Essay. BioMed Research International, 2013, pp.1–14.
Varicella zoster virus infections increase the risk of disease flares in patients with SLE: a matched cohort study
Authors: Sun Fangfang , Chen Yi , Wu Wanlong , Guo Li , Xu Wenwen , Chen Jie , Sun Shuhui , Li Jiajie , Chen Zhiwei , Gu Liyang , Wang Xiaodong , Li Ting , Ye Shuang 
Published: July 2019

ObjectiveTo explore whether varicella zoster virus (VZV) infection could increase the risk of disease flares in patients with SLE.MethodsPatients who had VZV reactivations between January 2013 and April 2018 were included from the SLE database (n=1901) of Shanghai Ren Ji Hospital, South Campus. Matched patients with SLE were selected as background controls with a 3:1 ratio. Patients with SLE with symptomatic bacterial infections of the lower urinary tract (UTI) were identified as infection controls. Baseline period and index period were defined as 3 months before and after infection event, respectively. Control period was the following 3 months after the index period. Flare was defined by SELENA SLEDAI Flare Index. Kaplan-Meier analysis, Cox regression model and propensity score weighting were applied.ResultsPatients with VZV infections (n=47), UTI controls (n=28) and matched SLE background controls (n=141) were included. 16 flares (34%) in the VZV group within the index period were observed, as opposed to only 7.1% in UTI controls and 9.9% in background controls. Kaplan-Meier curve revealed that patients with a VZV infection had a much lower flare-free survival within the index period compared with the controls (p=0.0003). Furthermore, after adjusting for relevant confounders including baseline disease activity and intensity of immunosuppressive therapy, Cox regression analysis and propensity score weighting confirmed that VZV infection within 3 months was an independent risk factor for SLE flares (HR 3.70 and HR 4.16, respectively).ConclusionsIn patients with SLE, recent VZV infection within 3 months was associated with increased risk of disease flares.
Parental occupational exposure to low-frequency magnetic fields and risk of leukaemia in the offspring: findings from the Childhood Leukaemia International Consortium (CLIC)
Authors: Talibov Madar , Olsson Ann , Bailey Helen , Erdmann Friederike , Metayer Catherine , Magnani Corrado , Petridou Eleni , Auvinen Anssi , Spector Logan , Clavel Jacqueline , Roman Eve , Dockerty John , Nikkilä Atte , Lohi Olli , Kang Alice , Psaltopoulou Theodora , Miligi Lucia , Vila Javier , Cardis Elisabeth , Schüz Joachim 
Published: July 2019

ObjectivesPreviously published studies on parental occupational exposure to extremely low-frequency magnetic fields (ELF-MF) and risk of acute lymphoblastic leukaemia (ALL) and acute myeloid leukaemia (AML) in their offspring were inconsistent. We therefore evaluated this question within the Childhood Leukemia International Consortium.MethodsWe pooled 11 case–control studies including 9723 childhood leukaemia cases and 17 099 controls. Parental occupational ELF-MF exposure was estimated by linking jobs to an ELF-MF job-exposure matrix (JEM). Logistic regression models were used to estimate ORs and 95% CIs in pooled analyses and meta-analyses.ResultsORs from pooled analyses for paternal ELF-MF exposure >0.2 microtesla (µT) at conception were 1.04 (95% CI 0.95 to 1.13) for ALL and 1.06 (95% CI 0.87 to 1.29) for AML, compared with ≤0.2 µT. Corresponding ORs for maternal ELF-MF exposure during pregnancy were 1.00 (95% CI 0.89 to 1.12) for ALL and 0.85 (95% CI 0.61 to 1.16) for AML. No trends of increasing ORs with increasing exposure level were evident. Furthermore, no associations were observed in the meta-analyses.ConclusionsIn this large international dataset applying a comprehensive quantitative JEM, we did not find any associations between parental occupational ELF-MF exposure and childhood leukaemia.
The invalidity of “negative mass” description of the dark sector
Authors: Stepanian A. 
Published: July 2019

It is shown that the concept of “negative mass” introduced by Farnes [Astron. Astrophys. 620, A92 (2018)] to describe the dark sector within a unifying theory with the negative cosmological constant contradicts both the essence of the General Relativity (GR) and the available observational data. A viable model with modified weak-field GR is mentioned.
Initial state radiation correction and its effect on data-taking scheme for σB(e+e−→ ZH) measurement
Authors: Han Shuang , Li Gang , Zhou Xiang , Jie Quan-Lin 
Published: July 2019

The measurement of Born cross-section of [Formula: see text] process is one of the major goals of the future Circular Electron Positron Collider, which may reach a precision of 0.5% at 240 GeV. Such unprecedented precision must be guaranteed by both theoretical and experimental sides, such as the calculations of high order corrections, the knowledge of the [Formula: see text] line shape. Uncertainty of the radiative correction factor at 240 GeV caused by the [Formula: see text] line shape is evaluated in this work. Therefore, dedicated data-taking schemes are proposed in order to precisely calculate the ISR correction factor.
Coherence of type class resolution
Authors: Bottu Gert-Jan , Xie Ningning , Marntirosian Koar , Schrijvers Tom 
Published: July 2019

Elaboration-based type class resolution, as found in languages like Haskell, Mercury and PureScript, is generally nondeterministic: there can be multiple ways to satisfy a wanted constraint in terms of global instances and locally given constraints. Coherence is the key property that keeps this sane; it guarantees that, despite the nondeterminism, programs still behave predictably. Even though elaboration-based resolution is generally assumed coherent, as far as we know, there is no formal proof of this property in the presence of sources of nondeterminism, like superclasses and flexible contexts.
This paper provides a formal proof to remedy the situation. The proof is non-trivial because the semantics elaborates resolution into a target language where different elaborations can be distinguished by contexts that do not have a source language counterpart. Inspired by the notion of full abstraction, we present a two-step strategy that first elaborates nondeterministically into an intermediate language that preserves contextual equivalence, and then deterministically elaborates from there into the target language. We use an approach based on logical relations to establish contextual equivalence and thus coherence for the first step of elaboration, while the second step’s determinism straightforwardly preserves this coherence property.