bluuebunny/crossref_metadata_2025 huggingface dataset:
021 Fludarabine-induced subacute, often fatal leukoencephalopathy: a case series
Authors: Awad Amine , Solis Jamie , Albjerg Lydia , Albert Julie , Bartrum Elizabeth , Childs Richard , Nath Avindra , Khan Omar I
Published: March 2023

Fludarabine, a purine agonist, is associated with a rare dose-dependent risk of severe and often fatal neurotoxicity. Referencing the clinical course of seven such patients, we report the clinical, radiological, and pathological findings in one patient who developed a fatal leukoencephalopathy to highlight potential mechanisms of fludarabine-induced neurotoxicity. Of the seven cases, one presented with paraparesis, two with cognitive impairment, and four with blurred vision; five cases were fatal, with a variable time course of symptom onset and progression. All patients had unexplained progressive, symmetric leukoencephalopathy on serial brain MRIs. One patient underwent post-mortem neuropathological examination; she was a 49-year-old female with severe aplastic anaemia and myelodysplastic syndrome who had received fludarabine at 25 mg/m2 for five days prior to stem cell transplantation. Thirteen days later, she developed rapidly-progressive cognitive deterioration and motor weakness. Serial brain MRIs showed extensive leukoencephalopathy extending into the midbrain and pons bilaterally. She passed away at day 39 post-transplant. Histopathology revealed diffuse, gross softening of the white matter and microscopic evidence of a demyelinating process with diffuse myelin loss, axonal injury, and spheroid formation. Electron microscopy revealed abnormally proliferative, reticulated, densely packed endoplasmic reticulum, suggesting a toxic, progressive demyelinating process.
025 The safety of etriage to provide advice rather than face-to-face neurology appointment
Authors: Bennett Karina , Boisanger Louis de , Moreton Fiona , Davenport Richard , Stone Jon
Published: March 2023

The number of neurology referrals in Scotland has increased inexorably. Active triaging of primary care referrals is a method of controlling this demand and it is apparent that not all primary care referrals need to be seen in an outpatient clinic, some can reasonably be dealt with ‘advice only’. The aim was to review the safety and cost-effectiveness of offering ‘advice only’ rather than outpatient appointments for neurology patients in NHS Lothian. GP referrals triaged by a neurology consultant as ‘advice only’ were identified over a 6-month period. Data was collected on reason for referral, opinion of triaging neurologist and whether the patient re-presented to secondary services within a 12-month period with the same symptoms. 236 patients were given advice only. 70% (n=166) patients had no further secondary care presentations in 12-months. The most common presentation was headache (n=57, 13%). 1 patient had a major diagnostic change following delayed review. Offering advice prevented unnecessary appointments which would not have changed management, was safe and likely to be cost-effective. Use of ‘advice only’ response allows resources to be redirected to seeing more medically appropriate consultations.
027 Promoting atraumatic needle usage for lumbar puncture in neurology: the power of the default
Authors: Holland Christian , Moore Catherine , Tobert Vanessa , Allen James , Moore Thomas , Durley Kirsty , Edmond Evan
Published: March 2023

Lumbar puncture (LP) is a key diagnostic investigation in neurology. Post-dural puncture headache (PDPH) is a known complication of lumbar puncture, resulting in significant patient morbidity. Fine-gauge and atraumatic-tip needles have been shown to reduce significantly the risk of PDPH. While uptake of such needles has been universal in anaesthetics, neurology has lagged behind. Here we investigate a multimodal intervention to increase the usage of atraumatic needles in neurology. Over a 15 month period, LPs were reviewed at the neurology day case unit of the John Radcliffe Hospital, a large tertiary centre. We present results from 218 LPs performed, and outcome data including documentation score and atraumatic needle usage. Simulation training interventions were associated with moderate improvement in practice, with a gradual reduction over time. This may be associated with staff turnover. Changing the default needle to an atraumatic (Sprotte 22G) needle was associated with a marked and sustained improvement in practice. There was no difference in success rate with the new needle and proforma usage remained high. Default options have previously been shown to be a powerful determinant of human behaviour in clinical medicine. Our results suggest that this may be an important tool to improve clinical practice in LP in Neurology, though staff engagement and monitoring for unintended consequences of changes in default equipment are essential.
032 Clinical audit research and evaluation for motor neurone disease (CARE-MND): alignment with clinical care standards
Authors: Beswick Emily , Newton Judith , Pal Suvankar , Colville Shuna , Davenport Richard , Morrison Ian , Swingler Robert , Gorrie George , Bateman Antony , Chandran Siddharthan
Published: March 2023

Aims

To establish 14 Key Performance Indicators (KPIs) of clinical care to provide a prospective audit framework to review routinely collected data fields on the Care Audit Research Evaluation (CARE-MND). Based upon Scottish and UK motor neuron disease (MND) standards of clinical care outlined in HIS/QIS Neurological Standards (2009) and NICE MND Guidelines (2016).

Methods

14 KPI categories of clinical care were reviewed. The completion percentage of 53 fields from CARE-MND were analysed for deceased people with MND (pwMND) diagnosed between 01/01/2015 and 31/12/2018.

Results

There was a 73% average completion in data capture for deceased patients diagnosed in the specified range for the 14 KPI areas of clinical care.

Conclusions

The introduction of CARE-MND as a research register and tool to support clinical care has resulted in more complete routine data capture of information which can be used to audit and improve delivery of care. The KPI audit framework will be repeated in the future to assess variation in data capture.
033 Audit on the diagnostic pathway of patients with glioma mimics
Authors: Joe Dorothy , Lavrador Jose , Vergani Francesco , Gullan Richard , Bhangoo Ranjeev , Ashkan Keyoumars , Finnerty Gerald
Published: March 2023

Background

Conditions such as primary CNS lymphoma (PCNSL) or demyelinating/inflammatory diseases may present with similar clinical and neuroradiological features to gliomas. These ‘glioma mimics’ are commonly diagnosed with brain biopsy, but this is not always possible. We aim to optimize the contribution of less invasive investigations to the diagnostic pathway.

Method

Retrospective review of the diagnostic process of glioma mimics between September to November 2018 in a tertiary Neuro–oncology unit.

Results

The Neuro-oncology MDT gave 558 opinions excluding post –operative reviews. Twelve cases were identified as glioma mimics. 6/12 were new cases. Further investigations in five new cases took 2.5 – 11 weeks to complete. Two patients deteriorated during diagnostic workup and had expedited brain biopsies which confirmed PCNSL. One proceeded with chemotherapy and one was referred to palliative care.

Conclusion

Diagnostic tests prior to brain biopsy were not performed in all glioma mimic cases. This was because test results excluding brain biopsy were not available to start treatment before the patient deteriorated clinically. We anticipate that the frequency of glioma mimics will increase due to the greater use of immunomodulatory therapies. A dedicated protocol to achieve rapid diagnosis of glioma mimics is imperative to facilitate early, appropriate treatment.
035 RAID referral patterns in neurology in-patients
Authors: See Stephanie Mei Ling , Cahill Varduhi , Khan Geoffrey , Jacob Saiju
Published: March 2023

Background

Studies have shown that the psychiatric burden of progressive and debilitating spectrum of neurological diseases increases the general prevalence of psychiatric illnesses in neurology wards to up to 64%.

Aim

This service evaluation is to analyse the interface and referral patterns to liaison psychiatry (RAID) in neurology inpatients compared to general medicine inpatients.

Methods

Our retrospective data collection and analysis studied 100 neurology inpatients and 100 general medicine inpatients randomly selected within 1 year.

Results

The RAID referral rate from neurology inpatients was three-fold to that of general medicine inpatients. In this group, the neurological inpatients had twice the length of hospital stay, higher re-referral rates and required highly specialised psychiatry input.

Clinical Implications

This highlights the growing overlap between psychiatry and neurology as well as its vulnerable sustainability as separate entities. We discuss the possibility of altering the formal training curriculum to accommodate the cross-overs between specialties and introduce better screening processes for psychiatric illnesses within neurology wards, hence providing a more cost-effective and holistic service.
038 MACE: optimal cut-offs for dementia and MCI
Authors: Larner AJ
Published: March 2023

Objective

To determine optimal test cut-offs for diagnosis of dementia and MCI using the mini-Addenbrooke’s Cognitive Examination (MACE) and compare these with index study cut-offs (Dement Geriatr Cogn Disord 2015;39:1–11), and to calculate MACE predictive values across a range of disease prevalences.

Results

Of 755 patients (F:M = 352:403, 47% female; median age 60 years), 114 received criterion diagnosis of dementia and 222 MCI. For diagnosis of dementia, optimal MACE cut-off determined by maximal Youden index was ≤20/30 (sensitivity 0.91, specificity 0.71) and by maximal correct classification accuracy was ≤14/30 (sensitivity 0.59, specificity 0.92). For MCI diagnosis, optimal MACE cut-off determined by maximal Youden index was ≤24/30 (sensitivity 0.90, specificity 0.57) and ≤19/30 (sensitivity 0.47, specificity 0.88) by maximal correct classification accuracy. These MACE cut-offs differed from the index study (≤25/30 high sensitivity, ≤21/30 high specificity). NPV was high (≥0.9) at all prevalences of dementia and MCI examined (range 0.05–0.4) suggesting normal MACE score effectively rules out dementia and MCI.

Conclusions

In this large dataset, optimal MACE cut-offs for dementia and MCI differed from the index study reflecting the different casemix of the studies. Revision of MACE cut-offs in may be required in clinical practice to maximise test discriminative utility.
040 AD8: Likelihood to diagnose or misdiagnose
Authors: Ziso B , Larner AJ
Published: March 2023

Objective

To analyse data from two previously published individual studies and two meta-analyses of the Ascertain Dementia 8 (AD8) cognitive screening instrument to calculate the recently described ‘likelihood to be diagnosed or misdiagnosed’ (LDM) metric, the ratio of ‘number needed to misdiagnose’ (NNM = 1/Inaccuracy) to either ‘number needed to diagnose’ (NND = 1/Youden index) or ‘number needed to predict’ (NNP = 1/predictive summary index).

Results

Raw data (true positives and negatives, false positives and negatives) were extracted and LDM values for the diagnosis of dementia were calculated. For the individual studies (n=212, 67 respectively) LDM values were all <1, favouring misdiagnosis, whereas for the meta-analyses (n=3278, 3694 respectively) all LDM values were >1, favouring diagnosis. This discrepancy may be explained by the poor specificity of AD8 in the individual studies, lower than in any other study included in the meta-analyses, giving very high values for NND and NNP and lower values of NNM than in the meta-analytic data. This may relate to differences in casemix between the various studies.

Conclusion

LDM is an easily calculated and potentially useful test metric. LDM values derived from meta-analytic data may differ from those of individual test accuracy studies.
041 Psychosis: a rare manifestation of moyamoya disease in an adult
Authors: Gupta Srishti , Jaiswal Amit , Sarkar Srishti , Hosseini Akram
Published: March 2023

Introduction

Moyamoya disease is a chronic progressive cerebrovascular occlusive disease with prominent basal collateral vessels which appear hazy like a puff of smoke on angiography. Neuropsychiatric disorders have been reported in 20–60% cases and the most common manifestations include depression, anxiety and other cognitive disorders. However, only a few case reports of onset of a psychotic disorder following a cerebrovascular accident. We present a rare presentation of Moyamoya disease in adulthood.

Case

A 44-year-old man from Bangladesh presented with acute psychotic episodes of visual, auditory and tactile hallucinations, paranoia, somatoform disorders and non-epileptic attacks. He had a 3-year history of mental health disorders and was later admitted to the hospital with a headache and hemiparesis secondary to an intraventricular haemorrhage. The neuroimaging showed characteristic appearances of Moyamoya disease. Neurosurgical multidisciplinary meeting discussion opted for conservative management and stroke rehabilitation. Despite the improvement of his neurological deficits, he continued to show apathy, depression, psychogenic attacks with features of executive dysfunction. Due to family history of juvenile death in the family, the patient had genetic testing and was found to have the mutation in the gene Ring finger protein 213 (RNF213).

Conclusion

This case highlights the awareness of neuropsychiatric manifestation of Moyamoya disease that could be related to the interruption of the striato-thalamo-cortical circuits. The susceptibility to Moyamoya disease-2 (MYMY2) may be conferred by variation in the RNF213 gene on chromosome 17q25.
045 Food for thought: an unusual case of frontotemporal dementia
Authors: O’Malley Ronan , Romanovsky Charles , Venneri Annalena , Blackburn Daniel
Published: March 2023

Case

A 58 year old man was referred to the memory clinic with a 2 year history of deteriorating memory and altered behaviour. His wife reported that he has become obsessed with pornography and appeared apathetic and devoid of emotion. Reportedly, he has not been well for 10 years since developing a severe postural headache. He has reported intermittent incontinence, unsteadiness, fatigue and dysphagia. Though investigated extensively, no cause had been found. There was no family history of neurological or psychiatric illness.

Physical examination was unremarkable. He scored 91/100 on ACE-R testing, losing points for memory and fluency.

The history suggested behavioural variant frontotemporal dementia (bvFTD). Frontal deficits were confirmed on neuropsychological testing.

On review, a previous MRI demonstrated signs suggestive of intrancranial hypotension with distortion of the brainstem. A diagnosis of Frontotemporal brain sagging syndrome (FBSS) was proposed. An MRI myelogram failed to demonstrate a source of CSF leakage. Despite this, the patient is currently undergoing treatment with an epidural blood patch.

Discussion

Intracranial hypotension with brain sagging has been reported in the context of Frontotemporal patterns of cognitive impairment. Attempts at treating FBSS have yielded varying results. We present our experiences in the diagnosis and management of this rare, but potentially reversible cause of dementia, alongside a review of the available literature.
11.39 Association of british neurologists sustainability special interest group (ABN sustainability SIG): formation, objectives and invitation
Authors: Cheesman Ann , Blackburn Daniel , Carroll Camille , Cohen Anna , Coebergh Jan , Sokhi Dilraj , Wong Sui H , Woolmore John
Published: March 2023

We introduce the ABN Sustainability SIG. We present our aims & objectives, and practical ways of implementing sustainability strategies in Neurology.

At the 2016 ABN annual meeting, a keynote speech by Dr. David Pencheon, then director of the National Health Service Sustainability Development Unit, highlighted the relevance and importance of Sustainability in Neurology. This planted the seed for our SIG’s formation. Initial interest was gathered from an ABN newsletter notice, via word-of-mouth and informal discussions at ABN annual meetings (2017, 2018). A series of teleconferences & email discussions enabled the formation of SIG byelaws and application to the ABN council.

Our aims and objectives

To be a positive force within the ABN to highlight issues surrounding global environmental sustainability.

To provide a forum to consider the impact of choices made in neurology practice on global environmental sustainability. This will cover all aspects of neurology including, but not limited to, clinical practice, service provision, technological and digital developments, meetings and ABN resources and investments.

To identify areas where the choice made could impact positively on global environmental sustainability and disseminate this information to the ABN membership to inform their decisions.
053 Altered phobic responses in frontotemporal dementia
Authors: Jimenez Daniel , Bond Rebecca L , Sivasathiaseelan Harri , Russell Lucy L , Greaves Caroline , Moore Katrina , Woollacott Ione , Shafei Rachelle , Marshall Charles R , Rohrer Jonathan D , Warren Jason D
Published: March 2023

Abnormal reactivity to emotional stimuli is a hallmark of frontotemporal dementia (FTD). In everyday life, we rarely experience intense fear but this can occur in the context of phobias. Some patients with dementia, particularly the behavioural and semantic variants of FTD, experience altered phobic responses. However, these have not been systematically studied. Here we collected caregiver reports about phobic responses following onset of disease in a cohort of patients representing different dementia syndromes, relative to healthy older controls. A number of patients with sporadic and genetically mediated FTD showed changes in phobic responses following onset of the disease, whereas this was rare in Alzheimer’s disease and healthy older controls. A spectrum of phobic alterations was reported, including new phobias, loss of longstanding phobias, or a mixture of these. Most phobic alterations involved perception of the patient’s own body in relation to the surrounding environment or objects approaching the body surface (e.g., claustrophobia, acrophobia, arachnophobia). Our survey reveals that altered phobic responses are phenomenologically complex and not uncommon in FTD, with a relatively narrow repertoire focused on self – nonself boundaries. This study has implications for our understanding of the pathophysiology of FTD and potentially, the brain basis of phobias.
056 Exploring the feasibility of automating verbal fluency tasks for cognitive assessment: data collection and analysis
Authors: O’Malley Ronan , Brook Eric , Lewis Alice , Sonksen Isobel , Mirhedari Bahman , Venneri Annalena , Christensen Heidi , Blackburn Daniel
Published: March 2023

Introduction

Fluency tests are widely employed in the assessment of cognition. We assessed the feasibility of integrating these tasks into an automated cognitive assessment tool; the ‘Digital Doctor’.

Methods

15 each of healthy controls (HC), Alzheimer’s disease (AD) and mild cognitive impairment (MCI) were recruited. Participants named as many words beginning with P (phonemic fluency) and as many animals (semantic fluency) as they could within one minute.

Results

Manual Analysis: HC named the same number of words for both tasks compared to normative data. Patients with AD and MCI were able to complete the task and Z-scores showed significant decreases in category fluency between the AD and HC groups, -2.25.

Automated Analysis: Automatic Speech recognition (ASR) was 77.9% accurate for semantic testing and 64.8% for phonemic testing, ASR overestimated the mean scores in the AD and MCI groups. Comparison of phonemic fluency in AD compared to HC yielded a significant Z score, -2.17.

Conclusions

Automated collection of fluency data is feasible and comparable to manually collected data. The over-estimation of the number of correct responses provided by the ASR will be improved with more data along with training of the Machine Learning algorithms to recognise certain, expected words.
057 Proteomic analysis of TDP-43 pathology in frontotemporal dementia
Authors: Shafei Rachelle , Toomey Christina , Foiani Martha , Mills Kevin , Lashley Tammaryn , Rohrer Jonathan
Published: March 2023

Introduction

Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder presenting with behavioural or speech problems. Identification of the underlying molecular abnormality during life remains elusive, and there are no reliable biomarkers of TDP-43 proteinopathies. Proteomic analysis of the TDP-43 proteinopathies provides a unique opportunity to better understand the disease-associated pathways and determine surrogate biomarkers of TDP-43 pathology.

Materials and methods

We set out to investigate the proteomic profile of TDP-43 proteinopathies using label-free quantitative mass spectrometry. 15 cases were selected: 9 had histologically confirmed TDP-43 inclusions as their primary pathology, 3 had Alzheimer’s disease (AD) pathology (without TDP-43 disease) and 3 were healthy controls. Three regions from each case were examined: frontal lobe, temporal lobe and hippocampus. Samples were pooled according to pathological group and brain region before mass spectrometric analysis (Synapt G2-Si nanoAcquity LC system).

Results

21 proteins were significantly upregulated in TDP-43 proteinopathies compared with AD and 30 proteins were downregulated. The majority of proteins identified are implicated in ribosomal function, RNA transportation and mitochondrial matrix enzymatic function. Whilst, several proteins identified have already been shown to be implicated pathophysiologically in FTD/ALS the majority have not, providing a new insight into disease pathogenesis.

Discussion

Preliminary proteomic analyses of brain tissue from TDP-43 proteinopathies has identified a number of differentially expressed proteins, providing a starting point for new biomarker development to distinguish different pathological subtypes of FTD during life.
11.51 Introducing atraumatic needles to the neurology ambulatory day case unit – a quality improvement project
Authors: White Daniel , Scotton Will , Shah Zohra , Nakornchai Tagore , Critchlow Tamsin , Mitchell James , Sinclair Alex , Woolmore John
Published: March 2023

Introduction

Atraumatic needles are associated with a decreased incidence of postdural-puncture headache. They also reduce the need for additional treatment and have similar efficacy to conventional needles. The aim of this Quality Improvement Project (QIP) was to encourage the use of atraumatic needles in Neurology ambulatory care by developing a sustainable Lumbar Puncture (LP) training method.

Methods

A specialised atraumatic needle training video was guru.kumar@nhs.net created for junior doctors starting in Neurology. This accompanied further teaching and opportunities to practice LPs on a simulation mannequin under supervision. Atraumatic needles were added to standard stock and supply was ensured.

Two audit cycles recorded the number of LPs performed using an atraumatic needle. Patient age, body mass index, length of stay, pain experienced and any need for image guidance were also recoded. Junior doctor confidence was measured before and after training.

Results

81 LPs were performed in the first cycle, 83 in the second. Atraumatic needle use increased from 26% to 50% between cycles. Junior doctor confidence increased with training from 2/10 to 8/10 (p=0.02).

Conclusions

Dedicated induction teaching and observed simulation practice increased junior doctors’ confidence in, and frequency of, the use of atraumatic needles.
063 ‘Less contented as a person’: an update from the LGI1-QoL study
Authors: Binks Sophie , Veldsman Michele , Jacob Saiju , Maddison Paul , Coebergh Jan , Isabel Leite M , Husain Masud , Irani Sarosh R
Published: March 2023

Objective

To establish a detailed phenotype of LGI1-antibody patients long-term through a cognitive, neuropsychiatric and quality of life (QoL) battery, and relate outcomes to biochemical, clinical and genetic factors.

Methods

60 patients have now undergone detailed evaluation for the LGI1-QoL study, extending the previously-reported preliminary cohort. Selected assessments included domains clinically observed as impaired such as emotionality, fatigue and QoL.

Results

Patients (40 male) aged 44–92 were assessed at a median of three years post-onset (range 4 months-15 years). Compared to published thresholds in age-appropriate healthy controls, 32% were impaired on the Addenbrooke’s Cognitive Examination-Revised, 39% on the Frontal Assessment Battery, 39–52% on two different fatigue scales, 43–51% on the two different measures of the carer-reported Neuropsychiatric Inventory Questionnaire, and 40% on the Life Satisfaction Questionnaire (LSQ) – Life as a Whole. LGI1-antibody patients were significantly (p≤0.05) more impaired on five LSQ sub-domains compared to an available dataset for healthy elders.

Analysis of retrospective pre- and post-illness analyses of LSQ, the EQ-5D-5L, another patient-related QoL measure, and the Modified Rankin Scale showed significant (p≤0.01) decreases in most domains. Further clinical and paraclinical correlations will be presented.

Conclusions

Many LGI1-antibody patients demonstrate sustained deficits across cognitive, neuropsychiatric, carer-reported and QoL domains.
064 Seizure patterns and trajectory in patients with epilepsy secondary to NMDA vs LGI1 auto-immune encephalitis
Authors: Seddigh Arshia , Mostafa Omar Ezzat , Karim Abid , Wysota Barbara , Samarasekera Shanika , Jacob Saiju
Published: March 2023

Background

The longer-term trajectory of seizures associated with autoimmune encephalitis (AIE) is not clear. Recent data in NMDA AIE suggests seizures manifest early and are generally short lived, but in LGI1 AIE they occur later and continue longer. We characterised seizure semiology and associated prognosis in two cohorts of AIE patients.

Methods

Case note review of 14 patients with a ‘definite’ diagnosis of anti-NMDAR AIE and 14 patients with LGI1 AIE showed the latter to occur in older adults. Patients were categorized as having seizures within one month of presentation or no seizures and the semiology was classified as focal or generalised onset.

Results

In the NMDA group A, 3/7 patients (23%) had focal onset compared to 6/11 patients (55%) in the LGI1 group, with predominantly faciobrachial dystonic seizures. NMDA patients were more likely to be seizure-free at 6 months (9/12, 75%) compared to LGI1 patients (6/14, 43%). Similarly, much fewer NMDAR patients remained on ant-epileptic medications at 1 year compared to the LGI1 patients. Neither seizure semiology at presentation nor presence of inflammation on MRI correlated with longer term seizure trajectory in both groups.

Conclusion

Seizures are common presenting symptoms in these AIE groups. More NMDA patients were seizure-free at 6 months and did not require AEDs after 1 year compared to the LGI1 patients. LGI1 patients represented a more susceptible group of AIE patients to epilepsy.
075 Meningitis management audit at a single centre: further support for culture-independent techniques in diagnosing meningitis
Authors: Williams Simon , Hassan-Smith Ghaniah
Published: March 2023

Objective

Rapid identification of meningitis and initiation of rational treatment is critically important. We reviewed whether our management was consistent with recent recommendations, for patients treated August 2015-July 2018.

Methods

Patients aged ≥16 coded as having meningitis/meningoencephalitis/specific meningitides (total codes=27) at QEHB over this period (n=497) were identified by informatics and data analysed using electronic records. Specific patient groups were analysed including post-neurosurgical and community-acquired meningitis.

Results

183 patients met the inclusion criteria, for community-acquired meningitis (n=118) or post-neurosurgery (n=65). Within the A&E subgroup (n=92), 80% received neuroimaging pre-LP despite 78% not having contraindication to LP. Median time to LP was 19hours (IQR:11.0–31.0); none performed within 1hour. Antibiotics were administered prior to LP in 72% cases. No neurosurgical patients had meningococcal/pneumococcal serum or CSF PCR sent. Serum PCR was sent in 11% of community-acquired meningitis patients - 23% were positive. CSF PCR was sent in 28% - 22% were positive. None of these cases had positive CSF culture.

Conclusions

Despite BIA recommendations, time-to-LP was delayed and culture-independent techniques such as serum/CSF PCR for bacterial pathogens were under-utilised, despite out-performing CSF culture. The emergence of novel culture-independent methods, including targeted point-of-care and unbiased-metagenomic sequencing, are likely to become increasingly relevant.
079 Genetic testing in epilepsy: a new imperative for precision
Authors: Scott Nicholas , Hamilton Leigh , Symonds Joe , Heath Craig , Abbasi Hina , Goldberg Danielle , Hewitt Russell , Marshall Victoria , Zuberi Sameer , Leach John Paul
Published: March 2023

We reviewed the results of testing in patients from the West of Scotland attending adult epilepsy clinics with MRI-negative drug-resistant epilepsies or epilepsies with co-morbid intellectual disability,

Methods

45 eligible (>18y) cases were referred for testing. Clinicians completed a structured electronic clinical referral proforma to aid variant interpretation. Cases were tested on a custom-designed 104-gene panel focusing on disorders for which MRI would not provide a diagnosis. Variants were classified using UK Association of Clinical Genetic Science Guidelines. Variants of uncertain significance (VUS), likely pathogenic, and definitely pathogenic variants were discussed by a multidisciplinary team. Diagnostic variants were confirmed by Sanger sequencing.

Results

Among 45 cases, likely pathogenic and pathogenic (diagnostic) variants were identified in 8 (18%), with VUS in 10 (22%). Pathogenic variants were identified in the following genes; GAMT, CHRNB2, SCN2A, PCDH19, PRRT2, SLC2A1, SCARB2, and SBTX1B.

All cases with abnormal results or VUS were offered referral to a Genetic Epilepsy Clinic for counselling. Genetic findings altered therapy in several cases: some with VUS became seizure-free with targeted changes in treatment. Determination of clinical significance was sometimes helped by further familial testing.

Conclusion

Genetic testing is an essential tool in the management of adult patients with drug-resistant epilepsy, particularly those with childhood onset and/or accompanying learning difficulties. Abnormalities can be expected in approximately 40% using these criteria, which can aid both management and family counselling.
080 A quality improvement project on the management of convulsive status epilepticus at ICHT
Authors: Wade Charles , Evans Matthew , Osugo Martin , Quinn Taryn , Dorsey Rachel , Everett Alex , O’Dwyer John , Johnson Mike , Gorgoraptis Nikos
Published: March 2023

Convulsive status epilepticus (CSE) is a common neurological emergency with significant morbidity and mortality. We developed a CSE management protocol for a tertiary neurosciences centre with the aim of standardizing practice and increasing the likelihood of favourable outcome. To do this we undertook a questionnaire-based audit of current management principles in A&E staff (n=24), as well as a retrospective case-based audit of 3-months of acute seizure presentations in the same department (n=-103). We identified variable understanding of optimal management, lack of awareness/availability of informative resources and subsequent suboptimal management. Following this we developed and implemented a trust-wide CSE management protocol based on national guidance, literature review and the local expert opinion of neurology consultants and neuroscience pharmacists. The protocol covers early, established and refractory CSE and includes pharmaceutical management, important investigations/observations and who to involve and when. The protocol was published on the trust Intranet and summary flowchart posters were displayed in A&E. We organised teaching sessions for A&E staff and those involved in acute medicine. Though not complete at time of abstract submission, we intend to re-audit CSE management in the near future, assessing protocol adherence and will also present the results of this.
090 Anti-epileptic medications: causes of adherence failure
Authors: Thaller Mark , Maxwell Maxine , Moor Carl-Christian
Published: March 2023

Introduction

This project’s primary aim was to assess the reasons behind the recurrent adherence failure to anti-epileptic medication (AEDs) and its effects, in the Staffordshire area. This was done via a questionnaire administered by the Royal Stoke University Hospital Clinical Epilepsy Nurse Specialists over 3 months in 2018. Analysis occurred subsequently to avoid result bias.

Results

57 neurology patients completed this questionnaire with 81% (47) reporting a planned change to AEDs. However, there were also unexpected medication changes (35%), wrong doses (4%) and wrong drugs (9%) dispensed. Impacts of the medication changes included side effects (55%), seizures (5%) and medication-related confusion (35%) with pharmacy cause reported in 65%. This was statistically significantly more likely to occur if the dispensing pharmacy was a chain. Pharmacy chains in our region were responsible for 77% (44) of the dispensing. Running out of medications also was common (19, 33%) with 63% of these being for non-patient reasons.

Conclusion

In a population who already frequently have memory problems, there is a high non-patient related cause for poor AED compliance. We recommend informing patient of changes (including to cheaper generics) and move from a micro-regulated profit-driven system to a more flexible patient-centred approach.
092 The rare case of praxis and thinking reflex epilepsy
Authors: Zirra Alexandra , Fauzi Nor Amelia Mohd , Sidhu Meneka
Published: March 2023

Background

We report a rare case of reflex epilepsy in a 36-year-old female who was initially diagnosed with juvenile myoclonic epilepsy (JME).

Case Presentation

Aged 15, spontaneous left sided myoclonic jerks and rare generalised tonic clonic seizures (GTCS) began. JME was diagnosed then, based on generalised spike wave discharges.

As GTCS occurred once a year, she opted against treatment. Aged 26, due to seizure exacerbation, levetiracetam was commenced with complete seizure cessation.

Aged 34, ascending electric-shock-like sensations from her feet with myoclonic jerks occurred exclusively whilst performing or even thinking about specific tasks such as hair brushing and complex arithmetic. No spontaneous seizures occurred. She was diagnosed with anxiety and referred to the epilepsy clinic at NHNN.

Borderline hypothyroidism was diagnosed 6 months prior to symptom onset and treated with levothyroxine. There was otherwise no change in behaviour or cognitive function.

On video-telemetry habitual reflex events were preceded by brief bursts of predominantly left fronto-central spike-and-slow wave complexes. Inter-ictally there were independent left central spike-wave-complexes suggesting a left fronto-central focal reflex epilepsy syndrome.

Levetiracetam was optimised and lamotrigine commenced with complete cessation of seizures.

Conclusion

It is important to recognise reflex epilepsy syndromes, rare entities, as they are frequently misdiagnosed.
098 The adult profile of SUDEP using the epilepsy deaths register
Authors: Craig Donald , Hanna Jane , Osland Karen , Kerr Mike , Thomas Rhys H
Published: March 2023

Introduction

There is no ‘standard’ sudden unexpected death in epilepsy (SUDEP). Attention can be focussed on prevention in those thought to be most at risk of SUDEP but every person with epilepsy is at risk. We sought to identify the median profile of the people with a definite SUDEP death.

Method

The voluntary Epilepsy Death Register collects information on the circumstances surrounding the death of a person with epilepsy to identify and improve risk factors that contribute to these deaths. 349 post-mortem confirmed individual entries allowed the production of a profile of the composite, median adult patient at risk of SUDEP.

Results

The median death would be male (58.5%) aged 19–30 (48.7%) who lives in the family home (73.3%). and currently in work (45.6%). They were diagnosed more than 10 years ago (53.5%), taking antiepileptic medication (90.1%) and under the care of a specialist in epilepsy (76.2%). They were not known to forget medication (56.4%), had not had a recent AED change (58.7%), Their death was unwitnessed (81.0%) and they were found on their back (57.4%). 55.5% of families did not know that epilepsy could kill.

Discussion

SUDEP can occur in otherwise well people with epilepsy. SUDEP should be discussed and safety checks embedded for everyone rather than just ‘at risk’ subgroups.
Revisiting $$B\rightarrow K^{(*)} \nu {\bar{\nu }}$$ decays in the Standard Model and beyond
Authors: Bečirević Damir , Piazza Gioacchino , Sumensari Olcyr
Published: March 2023

In this letter we revisit the Standard Model predictions for $${\mathcal {B}}(B\rightarrow K^{(*)}\nu {\bar{\nu }})$$

B
(
B
→

K

(

∗
)

ν

ν

¯

)

and discuss the opportunities that open up when combining its partial decay rate with that of $$B\rightarrow K^{(*)}\ell \ell $$

B
→

K

(

∗
)

ℓ
ℓ

. In the Standard Model a suitable ratio of these two modes can be used to extract $$C_9^\textrm{eff}$$

C
9
eff

, which is essential for a reliable phenomenological analysis of the $$B\rightarrow K^{(*)}\ell \ell $$

B
→

K

(

∗
)

ℓ
ℓ

angular observables. The same ratio also proves to be more sensitive to the presence of New Physics in many plausible extensions of the Standard Model. We also suggest that the separate measurement of $${\mathcal {B}}(B\rightarrow K\nu {\bar{\nu }})$$

B
(
B
→
K
ν

ν

¯

)

for high and for low $$q^2$$

q
2

’s can be helpful for testing the assumed shape of the vector form factor, because the lattice QCD data are obtained at high $$q^2$$

q
2

’s, whereas the low $$q^2$$

q
2

region is obtained through an extrapolation.
Impact of Tokenization on Language Models: An Analysis for Turkish
Authors: Toraman Cagri , Yilmaz Eyup Halit , Şahi̇nuç Furkan , Ozcelik Oguzhan
Published: March 2023

Tokenization is an important text preprocessing step to prepare input tokens for deep language models. WordPiece and BPE are de facto methods employed by important models, such as BERT and GPT. However, the impact of tokenization can be different for morphologically rich languages, such as Turkic languages, in which many words can be generated by adding prefixes and suffixes. We compare five tokenizers at different granularity levels, that is, their outputs vary from the smallest pieces of characters to the surface form of words, including a Morphological-level tokenizer. We train these tokenizers and pretrain medium-sized language models using the RoBERTa pretraining procedure on the Turkish split of the OSCAR corpus. We then fine-tune our models on six downstream tasks. Our experiments, supported by statistical tests, reveal that the morphological-level tokenizer delivers a challenging performance with de facto tokenizers. Furthermore, we find that increasing the vocabulary size improves the performance of Morphological- and Word-level tokenizers more than that of de facto tokenizers. The ratio of the number of vocabulary parameters to the total number of model parameters can be empirically chosen as 20% for de facto tokenizers and 40% for other tokenizers to obtain a reasonable trade-off between model size and performance.
A Case of Necrotizing Fasciitis Mimicking a Burn in an Elderly Patient
Authors: Khan Madiha , Bhatia Shubham , Cervellione Kelly L. , Louis Martine A.
Published: March 2023

Necrotizing fasciitis travels along the fascial plane and surrounding soft tissue, leading to ischemia and necrosis. Fournier’s gangrene is a type of necrotizing fasciitis invading the deep and superficial planes of the perineal/genital region. It is rapidly progressive in nature and may have life-threatening consequences. Fournier’s often exhibits a misleading clinical presentation and can be mistaken for other conditions, such as hematoma, phlebitis, cellulitis, or septic arthritis. Since the ramifications of delayed diagnosis can be clinically significant, recognition of potential mimics is important to prevent morbidity or mortality. We report a case of Fournier’s gangrene mimicking a second-degree burn, an exceedingly rare presentation.
Case Report: Rare Case of Staphylococcus pasteuri Endocarditis
Authors: Merrild Esben , Winther Mette , Dahl Jonathan Nørtoft , Ebsen Tine Sneibjerg , Leth Steffen , Winther Simon
Published: March 2023

A 45-year-old woman was admitted with severe pain in the right leg and dyspnea. Her medical history included previous Staphylococcus aureus endocarditis, biological aortic valve replacement, and intravenous drug abuse. She was febrile but did not have any focal signs of infection. Blood tests showed raised infectious markers and troponin levels. Electrocardiogram showed sinus rhythm without signs of ischemia. Ultrasound revealed thrombosis of the right popliteal artery. The leg was not critically ischemic, and therefore, treatment with dalteparin was chosen. Transesophageal echocardiography showed an excrescence on the biological aortic valve. Empiric treatment for endocarditis was started with intravenous vancomycin, gentamicin, and oral rifampicin. Blood cultures subsequently grew Staphylococcus pasteuri. On day 2, treatment was changed to intravenous cloxacillin. Due to the comorbidity, the patient was not a candidate for the surgical treatment. On day 10, the patient developed moderate expressive aphasia and weakness in the right upper limb. Magnetic resonance imaging showed micro-embolic lesions scattered across both hemispheres of the brain. Treatment was changed from cloxacillin to cefuroxime. On day 42, infectious markers were normal, and echocardiography showed regression of the excrescence. Antibiotic treatment was stopped. Follow-up on day 52 did not show any signs of active infection. However, on day 143, the patient was readmitted with cardiogenic shock due to aortic root fistulation to the left atrium. She quickly deteriorated and died.
The Characteristic of the Minimal Ideals and the Minimal Generalized Ideals in Rings
Authors: Sema Rigena , Petro Petraq , Hila Kostaq
Published: March 2023

In this paper, we prove that the characteristic of a minimal ideal and a minimal generalized ideal, which is meant to be one of minimal left ideal, minimal right ideal, bi‐ideal, quasi‐ideal, and (m, n)‐ideal in a ring, is either zero or a prime number p. When the characteristic is zero, then the minimal ideal (minimal generalized ideal) as additive group is torsion‐free, and when the characteristic is p, then every element of its additive group has order p. Furthermore, we give some properties for minimal ideals and for generalized ideals which depend on their characteristics.
Natural Compound Isoliensinine Inhibits Stress-Induced Hair Greying by Blocking β2-Adrenoceptor
Authors: Yan Lingchen , Li Miaomiao , Zhu Meidi , Sun Ruishuang , Gao Ying , Zhao Xiaojing , Ling Yuanqiang , Xu Xuejuan , Chu Weiwei , Wang Xusheng
Published: March 2023

Chronic and acute stress caused by emotional or physical insults can affect the function of other organs via the brain-body axis. As one of the smallest organs in mammalian, hair follicles are highly susceptible to stress. Under stress, the sympathetic nerves release norepinephrine (NA), which acts directly on the β-2 adrenergic receptors on melanocyte stem cells (MeSCs) within the hair follicles, causing the MeSCs to lose quiescence and enter a rapid proliferation state followed by differentiation and migration, leading to rapid loss of MeSCs and, ultimately, grey hair. Here, we screened out β-2 blockers forming the ZINC 15 compound database, found a natural product isoliensinine, and was effective in preventing stress-induced hair greying in mice. The study sheds light on the development of products that use natural compounds to prevent stress-induced hair greying.
Quantitative Weighted Bounds for Littlewood-Paley Functions Generated by Fractional Heat Semigroups Related with Schrödinger Operators
Authors: Yang Li , Li Pengtao
Published: March 2023

Let

L
=
−
Δ
+
V

be a Schrödinger operator on

ℝ

n

, where

Δ

denotes the Laplace operator

∑

i
=
1

n

∂

2

/
∂

x

i

2

and

V

is a nonnegative potential belonging to a certain reverse Hölder class

R

H

q

ℝ

n

with

q
>
n
/
2

. In this paper, by the regularity estimate of the fractional heat kernel related with

L

, we establish the quantitative weighted boundedness of Littlewood-Paley functions generated by fractional heat semigroups related with the Schrödinger operators.
Notas sobre o uso do trabalho enquanto atividade terapêutica e suas interfaces com a Terapia Ocupacional
Authors: Lancman Selma , Barros Juliana de Oliveira
Published: March 2023

Trata-se de ensaio teórico-reflexivo sobre o desenvolvimento e consequente evolução do conceito de trabalho terapêutico e sua influência na complexificação das teorias psiquiátricas, desde o seu nascimento até meados do século XX, configurando, ao final deste período, a emergência da psicopatologia do trabalho. A partir de teóricos da época, são tecidas algumas intersecções com a terapia ocupacional, enquanto campo de conhecimento e de práticas assistenciais.
Afirmação e retomada de uma trajetória: novas perspectivas da Revista de Terapia Ocupacional da USP
Authors: Soares Carla Regina Silva , Oliver Fátima Corrêa , Barros Juliana de Oliveira , Batista Marina Picazzio Perez , Othero Marília Bense , Muramoto Melissa Tieko , Galheigo Sandra , Lancman Selma
Published: March 2023

xx
Dynamic Change Analysis of Vegetation Carbon Sinks in Agricultural Land
Authors: Lei Na , Zhang Jing , Zhang Yang
Published: March 2023

The method of ecological economics was applied to calculate the vegetation carbon sink from farmland and its dynamic change characteristics in Shaanxi Province from 2009 to 2018. Results showed that the vegetation of agricultural land increased by 3.3×1010 t over the
period studied, with an average rise of 36.5%. In Shaanxi Province, the vegetation carbon sinks of cultivated land, forest land, and garden plots all showed an increasing trend, while grassland carbon sinks showed a decreasing trend. The vegetation carbon sinks of cultivated land mainly came
from the three crops of wheat, corn and rice, and the carbon sinks were all above 10 t/hm2. The carbon sink of rice is the highest among the main crops, with an average value of 54.75 t/hm2. The change trend of vegetation carbon sink of forest land and garden land was
consistent with the area. The decline was 0.09%. From 2009 to 2010, the garden area increased by 140,000 hm2, an increase of 19.83%. Since 2010, the garden area began to decrease, with a mean annual change rate of 3.85%. Vegetation carbon sink from grassland was the highest in 2009,
at 6801475.3 t, the lowest in 2010, 4883565.8 t, with an average annual decrease of 0.64%.