bluuebunny/crossref_metadata_2025 huggingface dataset:
Delivering acute neurology care via the Same Day Emergency Care (SDEC) model
Authors: Balaratnam Michelle , Alim-Marvasti Ali-Jesus , Lane Charlie , Bierrum William , See Isaiah , Schlattl Anne , Chandratheva Arvind , Baruah Timothy , Simister Robert , Haider Salman
Published: August 2022

SDEC is the provision of same day care for emergency patients who would otherwise be admitted to hospital. Embedded within UCLH Emergency Department (ED), the SDEC cases are referred for consultant review. We aimed to determine whether a Neurology SDEC (N-SDEC) service would have a beneficial impact at UCLH.

The service has run a weekday consultant neurologist-led service since May 2021. The service is supported by a nurse practitioner. Investigations are arranged via dedicated SDEC slots. The patient then has a nurse-led telephone follow-up. The service sees approximately 100 neurology referrals a month.

The major impact of the service is reduced time to diagnosis and treatment and reduced onward referral. Only 5% of referrals to N-SDEC required onward General Neurology out-patient services. For the new diagnoses that require sub-specialist out-patients (9%), N-SDEC has improved the patient pathway (e.g. for neurovascular, multiple sclerosis, brain tumour, and neuro-vestibular presentations). We have also established new acute pathways (e.g. for status migrainosis and acute functional neurological disorders). Feedback on the new service from ED staff shows that the N-SDEC is also popular.

Experience so far with the N-SDEC model indicates that it may provide an effective model for Acute Neurology delivery in the ED.
086 Long-term efficacy of fremanezumab in migraine patients with inadequate response to prior preventive medication classes
Authors: Naegel Steffen , Cohen Joshua , Campos Verena Ramirez , Barash Steve , Ning Xiaoping , Dhiraj Dalbir , Kudrow David
Published: August 2022

The FOCUS phase 3b study evaluated the long-term efficacy of fremanezumab in patients with chronic or episodic migraine (CM/EM) and prior documented inadequate response to 2-4 preventive treatment classes during 12-week double-blind period (DBP) and open-label extension (OLE).

Following 28-day baseline period, patients were randomised (1:1:1) to quarterly or monthly fremanezumab or placebo for DBP. Patients completing DBP entered OLE, where all patients received treatment with monthly fremanezumab. Outcomes are summarised by DBP group.

During DBP, least squares mean changes in monthly migraine days (MMD) in placebo, quarterly, and monthly fremanezumab groups, respectively, were –0.8, –3.9, and –4.5 for CM patients (n=509) and –0.6,

–3.7, and –3.8 for EM patients (n=328; P<0.0001 vs placebo); during OLE, mean MMD changes were –5.3,

–5.1, and –5.8 for CM patients (n=493) and –3.9, –5.1, and –5.1 for EM patients (n=313). Reductions were also observed for CM and EM patients in monthly headache days of at least moderate severity during DBP and increased during OLE. In placebo, quarterly, and monthly fremanezumab groups, respectively, for DBP, ≥50% reduction in MMD were 8%, 27%, and 29% for CM patients and 10%, 47%, and 43% for EM patients (P<0.0001 vs placebo) and for OLE, were 31%, 35%, and 40% for CM patients and 49%, 63%, and 56% for EM patients.

Fremanezumab demonstrated long-term efficacy in CM and EM patients with prior inadequate response to multiple preventive classes.
089 Re-admission rate for patients with headache in Southwest London – can this be addressed?
Authors: Kashif Maryam , Nitkunan Arani , Harral Josie , Mansour Nassif
Published: August 2022

Getting It Right First Time Neurology recommendations were published recently – including reviewing readmission rates for patients with headache with a local audit of any high rates. The aim of this study was to do just that.

Data were extracted retrospectively of all patients seen in Emergency Department (ED) or admitted with a primary diagnosis of headache from April 2018 to March 2019 at the four acute trusts in Southwest London. A questionnaire was completed at each hospital to understand current practice.

11,649/705,634 (1%) of ED attendances were due to headache; 356/3,652 (9.7% – separated by trust to 2.4%, 9.2%, 10.3% and 13.2%) headache admissions were re-admitted within 30 days. The trust with a specialist nurse whose phone number was given to patients as a safety net and/or who called patients 2 weeks post discharge had the least number of re-admissions. There was no clear correlation between length of stay and re-admission rate.

Limitations of this study are: uncertain accuracy of diagnostic coding and the inability to calculate re- admission to other acute trusts.

To improve quality of care, data reliability is needed. A nurse specialist who acts as a single point of access could impact on headache readmission rate.
090 Frequency of migraine and the use of preventive medication in a UK primary care population
Authors: Pain Andrew , Mount Julie , Appel Camilla , Ranopa Michael , Gulati Tania , Kernick David
Published: August 2022

Objectives

Calcitonin gene-related peptide (CGRP) antagonists are recommended by NICE as 4th line migraine preventive treatment. We report the frequency and preventive treatment patterns of migraine in the UK, focusing on adult patients who cease ≥3 oral preventive medications.

Methods

Study populations were retrieved from the Clinical Practice Research Datalink GOLD database (study period: 19/09/2012−01/01/2020; inclusion criteria: ≥12 months follow-up, current-in-dataset, adult on 01/01/2020). Patients who used ≥1 oral preventive medication with ≥3-year follow-up after first pre- scription were considered preventive users; medication/class cessation was defined as cessation with no evidence of restart within 6 months from end-of-supply date.

Results

On 01/01/2020, 30.5/1,000 (n=81,190/2,664,306, 95% CI 30.3–30.7) of study population had ≥1 migraine codes recorded over the study period. 42% of patients were preventive users. Of these, 10% had ≥3 preventive medication cessations, and 8% had ≥3 preventive medication class cessations. Patients with ≥3 preventive medication cessations were mostly female (83%), mean age was 46 years and 22% had a referral to a neurologist during the study period.

Conclusions

A considerable proportion of patients with migraine cease ≥3 lines of oral preventive medi- cation, pointing to the need for additional therapeutic options.
091 The East London Parkinson’s disease project – engaging a diverse population in research
Authors: Zirra Alexandra , Ben-Joseph Aaron , Haque Tahrina , Gallagher David , Budu Caroline , Frances Brooke , Simonet Cristina , Noyce Alastair
Published: August 2022

Background

Parkinson disease (PD) is the second most common neurodegenerative disease and the burden appears to be growing fastest in middle-low and low income countries. In the majority of obser- vational studies of PD, White, well-educated and affluent participants are over-represented.

Aims

Our aim was to engage a diverse group of people with Parkinson disease (PwP) from East London and build a research platform for previously under-represented groups.

Methods

We created a register of PwP from the Royal London Hospital which includes approximately 400 patients. In parallel, we have recruited participants to the East London Parkinson’s disease (ELPD) project; a case-control study of phenotype, genotype and biomarker characteristics. Clinical manifes- tations, UPDRS scores, data on non-motor symptoms, as well as biospecimens (buccal and skin swabs, serum samples) have been obtained.

Results

145 patients and 80 controls have been recruited so far. The mean age of PwP was 67.81 (SD 10.4); 62% were male and 59% identified as being from South Asian or Black ethnicity. The most common presenting symptom was tremor (55.9%), followed by gait impairment (16.6%).

Conclusion

The ELPD project is a platform study for under-represented patients with PD which provides important opportunities for collaboration and research to improve health inequalities.
092 Brain amylin accumulation in Parkinson’s disease is not driven by type 2 diabetes mellitus
Authors: Cullinane Patrick , Pablo-Fernández Eduardo de , Strand Kate , Bandopadhyay Rina , Silva Rohan de , Jaunmuktane Zane , Warner Thomas
Published: August 2022

Introduction

Type 2 diabetes (T2D) is increasingly recognised as being a risk factor for Parkinson’s disease (PD). One proposed mechanism is an interaction between amylin, a highly amyloidogenic pancre- atic hormone, and α-synuclein. Amylin accelerates α-synuclein aggregation in vitro and interacts with α-synuclein in the locus coeruleus, and neuronal, extracellular and vessel-associated amylin deposits have been reported in Alzheimer’s disease and control brains. However, the molecular link between amylin and PD and the role of T2D remains unclear.

Methods

The burden of amylin accumulation in post mortem anterior cingulate and frontal gyrus tissue from PD and non-neurodegenerative control cases, with and without T2D, was investigated using immu- nohistochemistry and western immunoblotting.

Results

Immunohistochemistry revealed very infrequent amylin labelling of senile plaques and neurofibril- lary tangles along with rare perivascular amylin deposits in PD and control brains, irrespective of T2D status. Although western immunoblotting revealed significantly increased amylin levels in PD cases compared to controls, there was no difference between diabetic and non-diabetic cases.

Conclusion

Our results do not support the hypothesis that brain amylin accumulation is increased by T2D. Higher amylin levels in PD may result from blood-brain barrier dysfunction but further studies are needed to clarify the relevance to PD pathogenesis.
102 Levodopa and melanoma what do clinicians think?
Authors: Symonds Alexander , Davenport Richard , Breen David
Published: August 2022

Parkinson’s disease (PD) is associated with an increased risk of melanoma, but the link between levodopa therapy and melanoma is less clear. The Electronic Medicines Compendium (EMC) explicitly contraindi- cates prescribing levodopa in patients with current, or previous, melanoma despite the lack of convinc- ing evidence.

To gauge clinical opinions on the perceived link between levodopa and melanoma, we disseminated a short online survey to neurology and geriatrics colleagues locally, as well as through the Association of British Neurologists and British Geriatrics Society.

The majority of the 91 respondents were neurologists (84%), mostly consultants (69%). Most were not aware of guidance stating that levodopa should be contraindicated in patients with a history of melanoma (56%), and responded that they did not counsel for the risk of melanoma when initiating levodopa therapy (93%). Conversely, almost one quarter responded that they would not prescribe levodopa to PD patients with a history of melanoma (24%), the majority being non-movement disorder specialists. Six clinicians reported stopping levodopa therapy after the emergence of a suspicious skin lesion or melanoma (7%).

In summary, clinicians are generally aware of the increased risk of melanoma in PD, but there is a lack of consensus on levodopa prescribing and counselling practices.
109 Embedding patient voice in trial design: the EJS ACT-PD experience
Authors: Zeissler Marie-Louise , McFarthing Kevin , Gonzalez-Robles Cristina , Chapman Rebecca , Mills Georgia , Foltynie Tom , Carroll Camille
Published: August 2022

Background

Patient-centred trials address patient relevant questions, have protocols that maximise recruitment and retention, as well as effective communication strategies. Involving patients and carers in trial design and oversight is essential for patient-centred research. In the Edmond J Safra ACT-PD ini- tiative we are developing the first multi-arm multi-stage (MAMS) trial for disease modifying Parkinson’s treatments. Here we present our patient-centred approach.

Methods

Seven people with Parkinson’s (PwP) and three care partners (CP) (3 male, 7 female) were recruited nationally and two allocated to each of 5 working groups (WG) developing the trial. Together they form a Patient and Public Involvement and Engagement (PPIE) WG chaired by PwP (KMcF). The group iteratively developed processes for supporting their input into WG decisions with an impact evalu- ation plan involving semi-structured interviews and a revised Patient Engagement in Research Scale).

Results

The PPIE team meets every six weeks online. Standard Reporting Forms facilitate communication between WGs and PPIE members, supporting group input into issues arising. PPIE feedback is an agenda item at all WG meetings. Post-WG PPIE debriefs with the WG chairs ensure a shared understanding of discussions. Monthly PPIE forums provide opportunity for informal education and themed discussions with PwP/CP. A PwP/CP WhatsApp group creates a safe space for sharing experiences and ideas.

Conclusion

We have developed a process by which PwP/CP can meaningfully co-design our MAMS trial, ensuring it is truly patient-centred.
117 Who is the culprit – the virus or the anti-virus?
Authors: Elkider Mohammed , Abraham Roby
Published: August 2022

49 years old gentleman. Had his AstraZeneca vaccine. 2 weeks later he became unwell. He developed fever, headache, and lethargy. Then his symptoms got worse. He became drowsy and quadriplegic. He was subsequently admitted to ITU.

His CSF showed high protein of 1.6 g/l and WBCS >400 (100% Lymphocytes). All other CSF constituent were normal. His MRI showed scattered lesions involving the brain and cord suggestive of inflammatory aetiology.

Patient was given diagnosis of Post vaccine -Acute disseminated encephalomyelitis (ADEM). He received

I.V MP followed by plasma exchange (PLEX). He showed remarkable recovery. His encephalopathy com- pletely resolved, and he regain full power in his upper limbs. But unfortunately, he remains paraplegic. A second course of I.V MP and PLEX was given with some recovery of power in his lower limb.

We noticed his LFT was consistently abnormal. Hence, we have done full hepatic screen. To our surprised his Hepatitis E IgM and IgG came back positive. Both Microbiology and Hepatology were consulted, and he was given the diagnosis of recent Hepatitis E infection.

Now we have 2 possible aetiologies to explain why he developed ADEM. But we feel that AstraZeneca Vaccine is the most likely cause.
118 Acute disseminated encephalomyelitis associated with SARS-CoV-2 vaccine in multiple sclerosis: a case report
Authors: Evans Matthew , Hanbury Georgina , Lim Emma , Dixon Luke , Singh-Curry Victoria , Nicholas Richard
Published: August 2022

We present a case of acute disseminated encephalomyelitis (ADEM) occurring 3 weeks following the AstraZeneca SARS-CoV-2 vaccine, in a patient previously diagnosed with multiple sclerosis.

Case: This 55-year-old lady had a background of multiple sclerosis (MS), but never required disease-modi- fying therapies owing to a mild disease course. She received the first dose of the AstraZeneca SARS-CoV-2 vaccine, and then was admitted with word-finding difficulties and collapse 25 days later. She deteriorated rapidly and became obtunded on day 2, requiring intubation and intensive care admission. CSF analysis revealed mildly high protein (0.76g/L) with 5 white cells/mm3, and magnetic resonance imaging (MRI) showed multifocal demyelination in keeping with acute disseminated encephalomyelitis (ADEM). She received high-dose intravenous methylprednisolone, followed by plasma exchange and tocilizumab. Despite a mild radiological and clinical improvement the patient remained fully dependent for care needs, unable to speak or mobilise.

Conclusions

A number of SARS-CoV-2 vaccines have now been associated with ADEM, but this risk still appears very low in comparison with risks of SARS-CoV-2 infection. This is the first reported case of ADEM in a patient with multiple sclerosis after SARS-CoV-2 vaccination. Literature suggests increased risk of multiple sclerosis exacerbations commonly after SARS-CoV-2 infection.
122 A case of posterior reversible encephalopathy syndrome: a rheumatologic conundrum
Authors: Mahmud Zanaib , Samaranayake Manoj , Haq Rabea , Teh Lee-Suan
Published: August 2022

Posterior reversible encephalopathy syndrome (PRES) is a syndrome characterised by reduced GCS/confusion, seizures and visual disturbance along with characteristic MRI findings. Autoimmune disorders have been identified in one third of patients with PRES.

Here we discuss a 33-year-old female with SLE, who presented with reduced GCS and arm weakness. She had an AKI and metabolic acidosis and was admitted to ICU. Although the patients GCS improved, ongoing upper limb weakness remained. MRI imaging to rule out cerebral lupus, showed high T2 and FLAIR signal within the deep white matter of the occipital, frontal and parietal lobes. This was suggestive of PRES. Whilst in ICU the patient developed hypertension and was started on amlodipine. The patient also had a long history of immunosuppression with mycophenolate, hydroxychloroquine and rituximab. A neurology referral was sent for further advice.

Although the name suggests, PRES is often not completely reversible and is not limited to the posterior cerebral cortex, as seen here. It still remains unclear whether it is the autoimmune condition or treatment of these conditions with immunosuppressant’s that is the primary association. Case reports linking PRES with immunosuppressant therapy have shown an early onset of symptoms following treatment, rather than years.
129 Multiple sclerosis relapse patterns in a population-based cohort
Authors: Wynford-Thomas Ray , Uzochukwu Emeka , Harding Katharine , Anderson Valerie , Cauchi Marija , Willis Mark , Joseph Fady , Pickersgill Trevor , Robertson Neil , Tallantyre Emma
Published: August 2022

Rationale

Relapses remain a key outcome measure in multiple sclerosis (MS), are targeted by disease- modifying therapies (DMT), and guide treatment. As DMT prescribing continues to increase, it remains unclear what effect this has had on relapse frequency and phenotype over time.

Aim

To examine relapse patterns using 20 years of longitudinal data and correlate with DMT use.

Method

Prospectively collected relapse data from the South Wales MS registry was analysed from 2000- 2020. Relapse rate and phenotype according to DMT use were analysed across time epochs.

Results

Of 6,165 relapses in 1,460 patients; 76% were in females and 15% occurred on DMT. Annualised relapse rates (ARR) showed a decreasing trend from 2000 (ARR=0.23) to 2020 (ARR=0.08). Using cross- sectional analysis of data from years 2005 versus 2020, rates of DMT exposure increased from 6% to 31%, and ARR was significantly associated with DMT use (p<0.01). Polysymptomatic relapses and relapses associated with full recovery, were both more likely to occur in people on DMT.

Conclusion

Increasing use of DMTs was associated with decreasing ARR in this population-based MS cohort over 20 years. We also demonstrate that relapses occurring on DMTs appear to be associated with more favourable recovery.
133 Multiple sclerosis and the risk of infection: a consensus guideline
Authors: Cauchi Marija , Willis Mark , Backx Matthijs , Brownlee Wallace , Ford Helen , Gran Bruno , Jolles Stephen , Price Sian , Tallantyre Emma
Published: August 2022

Multiple sclerosis (MS) is an inflammatory condition affecting the central nervous system. Infection is a major consideration in the MS population due to its relevance to several stages of the disease process:

(i) it has been suggested that infective processes may be ‘triggering’ or aetiological factors for MS, (ii) concurrent infection is known to exacerbate symptoms in MS, (iii) people with MS are at higher risk of infection when compared to the general population, and this risk is exaggerated in those receiving disease modifying therapies (DMTs).

This guidance document was developed by specialists in the field of MS, Immunology, Infectious Disease and Pharmacy. A modified Delphi approach was used to develop clinically relevant, evidence-based consensus guidelines to help physicians navigate the complex interaction between DMTs and infectious diseases.

We focus on specific risks predisposing people with MS to infection and how to manage these risks. We also provide recommendations on how to screen for, prevent, and manage infection in this population, in particular tuberculosis, progressive multifocal leukoencephalopathy, hepatitis B, human papillomavirus, herpetic and other opportunistic infections. We also discuss vaccination and the COVID-19 pandemic in people on DMTs.
134 Predictors of serum vitamin D level in multiple sclerosis
Authors: Kuri Ashvin , Vickaryous Nicola , Jacobs Benjamin Meir , Awad Amine , Dobson Ruth
Published: August 2022

Background

Low serum 25(OH)D3 (vD) in early disease may be associated with worse prognosis in multiple sclerosis (MS). Determinants of serum vD concentration are established in healthy individuals, but poorly studied in people with MS (pwMS). Unpicking the determinants of vD concentration in pwMS may have important preventative and therapeutic implications.

Methods

We recruited a study cohort of pwMS from the UK MS Register (n=315) and matched controls (n=232). We measured serum 25(OH)D3 and genotyped six vD-associated SNPs. We developed a weighted genetic risk score (GRS) using these SNPs and validated it in 373,357 UK Biobank participants. We then assessed predictors of vD levels in our cohort and constructed multivariable models to assess the influence of GRS.

Results

The GRS was well-validated, being strongly associated with vD status in the Biobank cohort (p<2x10-16). In our study cohort, taking vD supplementation, having MS, lower BMI, increased age and supplementation dose were associated with higher vD levels (false discovery rate, FDR<5%). In multivariable models adjusting for these confounders, GRS was strongly associated with vD level in non-supplementers (p=0.004), but not in supplementers (p=0.47).

Conclusions

Our findings suggest vD supplementation is the most important determinant of vD level in pwMS, and genetic determinants may play a relatively minor role.
139 Characterising DARC (Detecting Apoptosing Retinal Cells) spots in optic neuritis
Authors: Choi Soyoung , Maddison John , Nicholas Richard , Cordeiro Francesca
Published: August 2022

Purpose

Optic neuritis (ON) is a common feature of Multiple Sclerosis (MS). DARC is an intravenous fluo- rescent-tagged annexin V (ANX776) identifying cell stress and apoptosis with retinal imaging. We aimed to differentiate DARC spots in ON from healthy subjects using descriptive measures (DM) of DARC spots identified by a convolutional neural network-aided algorithm (CNN-AA).

Methods

DARC spots were identified from anonymised DARC images obtained from ON (n=12; mean age=46) and healthy (n=68; mean age=48) participants after ANX776 administration using the CNN-AA (ISRCTN10751859). DM characteristics: mean, standard deviation (std), skew, kurtosis, coefficient of variation (cv), Euclidean distance from the nearest blood vessel (bvdist) and the 7Hu moments, were analysed on ON (n = 357) and healthy (n = 2457) eyes using two-tailed student t-tests. Distributions of spot metrics were analysed using two-sample Kolmogorov-Smirnov test.

Results

The mean, cv and kurtosis (p £ 0.0001) and hu1 and std (p £ 0.001) were found to be significantly different between DARC spots in ON and healthy controls. Distributions of the individual DM metrics analysed, showed a significant difference (p £ 0.0001) between ON and healthy spots for the same variables (mean, cv, kurtosis, hu1 and std).

Conclusion

DM of DARC spots distinguish ON and healthy subjects. Further studies with larger ON groups are needed to replicate this finding.
160 Ophthalmological involvement in m.3243A>G-related mitochondrial disease
Authors: Devine Helen , Ng Yi , McFarland Robert , Gorman Grainne , Browning Andrew
Published: August 2022

Background

The m.3243A>G mtDNA is the most common pathogenic mtDNA variant and has a very varied clinical phenotype. Vision has a high metabolic demand and there are large numbers of mito- chondria in the retina, particularly in the photoreceptors and the retinal pigmented epithelial layer. Little is known about the heteroplasmic/phenotypic correlations of retinopathy in patients with m.3243A>G- related mitochondrial disease.

Objective

The overall aim of the study was to explore retinal involvement in patients with m.3243A>G- related mitochondrial disease. Specifically, to describe the retinal and optic nerve changes and to determine whether the presence of maculopathy and/or optic nerve changes predict the development of CNS involvement in patients with the m.3243A>G mutation

Methods

Patients with genetically confirmed m.3243A>G-related mitochondrial disease were identified from the UK Mitochondrial Disease Patient Cohort; a registry of patients under active follow up at one of the three UK Mitochondrial Centres (Newcastle, London and Oxford). Participants underwent detailed clinical and ophthalmological examination. Ophthalmological phenotype was correlated with a validated clinical rating scale score (NMDAS) and m.3243A>G heteroplasmy levels.

Results

Retinal defects were common in m.3243A>G-related mitochondrial disease with subgroups of patients demonstrating maculopathy or optic nerve involvement.

Conclusion

Characteristic retinal defects are common in m.3243A>G-related mitochondrial disease.
163 Immunosuppression guidelines ongoing implementation project
Authors: Japzon Nicole , Gurney Rowan , Basgaran Amedra , Compton Laura , Lunn Michael , Carr Aisling
Published: August 2022

In 2016, a hospital approved guideline on first line immunosuppression was introduced. This included evidence-based guidance on consent, prescription, dosing, safety and efficacy monitoring of corticos- teroid, azathioprine, methotrexate, mycophenolate, IVIg, SCIg, rituximab and cyclophosphamide use in common inflammatory neuromuscular diseases. It was accompanied by drug-specific patient information booklets and disease-specific clinical outcome measurement tools. The centre for neuromuscular diseases has not recorded any serious adverse events associated with these medications since its introduction.

With junior doctors rotating in 3-6 monthly cycles, we identified the need for recurrent implementation efforts to maintain guideline impact. With an iterative process of four sequential cycles we:

1) performed a survey of adequacy of admission/discharge documentation,

2) created electronic patient record (EPR) proformas for admission/discharge,

3) included a training session at changeover hospital induction,

4) added a training session to mid-rotation peer-led teaching rota.

This process resulted in optimal admission/discharge documentation (as per guidelines) of: Cycle 2) 61%/96%; Cycle 3) 100%/70%; Cycle 4) 86%/100%.

Here we highlight the potential for loss of compliance with guideline use by junior medical staff over multiple changeovers, demonstrate the need for ongoing implementation efforts and underline the benefit of integrated electronic proformas (content in poster).
169 An audit into the management of functional neurological disorder in Salford
Authors: Houston James , Cooper Paul
Published: August 2022

Background

Functional neurological disorder (FND) is a debilitating condition impacting all aspects of life. Poor understanding of FND, and lack of clinical service pathways, may result in poor management, compared to recent NICE (2021) Quality Standards. We audited patients diagnosed with FND after admission to Salford Royal, including the care pathways used.

Methods

Salford Royal electronic patient records were accessed to populate an existing data collection tool used for analysis of neurological services. Patients admitted over a 4 month period were identified using ICD-10 coding (F44.5), with appropriate inclusion and exclusion criteria. We excluded patients with non-epileptic seizures as their prime diagnosis.

Results

We identified 64 admitted patients, with 10 excluded. Of 54 patients analysed, 91% had a motor deficit, 53% had a sensory deficit, many therefore presenting with functional motor disorders, often with impaired mobility, and likely to benefit from a multidisciplinary team approach. 44% of patients had no documented management plan for their FND at discharge, and in 80% of patients follow-up plans were unclear. (It is acknowledged that limited access to outpatient letters from community services may have impacted results.)

Conclusion

FND patients are being failed by our services, which are not meeting NICE Quality Standards. The unit is currently developing a multidisciplinary service for FND patients, which has proved challenging in the present climate. We plan to repeat the audit once our proposed service is established.
170 Commencing novel therapies for adult SMA patients: experience from a hybrid clinic
Authors: Cader Zahra , Nevin Katie , Maidment Lindsay , Packwood Shirley , Chilimina Nyaose , Monks Kimberley , Hewamadduma Channa
Published: August 2022

Spinal muscular atrophy (SMA) is a progressive neuromuscular disorder that affect both adults and children. Two novel therapies were approved for patients in England by NICE (Nusinersen via Management Access Agreement (MAA) and Risdiplam via Early Access to Medicine scheme (EAMS)). Setting up baseline assessments, designing new pathways, acquiring personnel and resources have been challenging. We present a pathway analysis of the new clinic set-up, process of patient choice, risk minimisation in intro- ducing the two novel therapies, and the impact therapies have had on adult cohort of SMA patients.

Total of 58 patients included (31 had type 2 SMA and 27 had type 3[only 11/27 were ambulant]. The average age of patients with type 2 and 3 SMA was 25 and 33 respectively. 19 patients chose risdiplam (oral) and 22 are on nusinersen (intra thecal). We analysed factors that govern patients’ treatment decisions.

We report factors that helped early success in our hybrid clinic set-up. Set criteria on each scheme; but potential side effects, information availability, route of administration (mainly previous spinal surgery), speed at treatment initiation but not COVID directed many patients’ treatment decisions. A battery of outcome measures were analysed to establish treatment impact at 12 months.
175 A case series of anti-HMGCR immune mediated necrotizing myopathy: the West Midlands data
Authors: San Pyae Phyo , Davies Nicholas , Srinivasan Venkataramanan , Samra Amrit , Paluri Srinivasa , Ubben Simon , Kumar Prateek , Kiolachidis Konstantinos , Dhar Saikat , Moor Carl-Christian
Published: August 2022

Background

Among immune mediated myopathies, anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) immune mediated necrotizing myopathy (IMNM) has been increasingly rec- ognised over the last two decades.

Majority are associated with statin exposure and typically present with hovering high creatinine kinase (CK) and/or progressive proximal myopathy despite discontinuation of statin.

Method

We analysed the data of 18 cases of anti-HMGCR IMNM from West Midlands region from 2016 to 2022.

Results

16 had statin exposure. Only 1 was asymptomatic but the rest experienced progressive proximal myopathy. Strikingly, two-thirds reported dysphagia and 3 required Intensive Unit admissions.

Anti-HMGCR antibody was positive in all cases. CK levels remained high (mean=9053IU/l) despite dis- continuation of statin. A trend of persistently high ALT, lowering creatinine and weight loss reflected smouldering muscle loss.

The asymptomatic patient did not need treatment. Significant improvement/remission was achieved spontaneously in 1, with steroids or IVIG alone in 10 and with combined immunotherapies in the rest. 1 patient died despite improvement in CK level.

Conclusion

Anti-HMGCR IMNM, especially when associated with statin, responds well to immunotherapy. We suggest having a low threshold for checking anti-HMGCR antibody in patients with persistently high CK levels and/or proximal limb/pharyngeal muscle weakness, especially in the presence of statin exposure.
192 Tacrolimus-induced chronic inflammatory demyelinating polyradiculoneuropa- thy (CIDP): a case series
Authors: Evans Matthew , Dahdaleh Sam , Karunaratne Kushan , Pritchard Jane , Rinaldi Simon , Viegas Stuart
Published: August 2022

Introduction

Calcineurin inhibitors are used for immunosuppression after solid organ transplantation, and tacrolimus is the most commonly agent used. It is associated with several adverse effects including oppor- tunistic infection, nephrotoxicity, diabetes and increased cancer risk, but less well known is its association with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP). Case series: We describe 7 cases of patients developing CIDP following tacrolimus treatment post transplantation. There were 3 cardiac, 3 lung and 2 renal transplants. Time since starting tacrolimus to development of CIDP varied widely from 5 months to 14 years. All cases were clinically consistent with CIDP with demyelinating neuropathy on neurophysiology. Intravenous immunoglobulin (IVIg) was tried in 6/7 cases but only showed clinical effec- tiveness in 2 patients, who required long-term treatment 2-monthly while continuing tacrolimus. In 3 of the IVIg non-responders (and 1 patient not trailed on IVIg), immunosuppression was switched to an alternative agent (sirolimus) with long-term resolution of neuropathy. Some cases were confounded: 1 associated with EBV and another CMV viraemia.

Conclusion

Various case reports have demonstrated a possible association between tacrolimus and CIDP, but this is the largest case series to date. IVIg responsiveness alone is generally poor, but resolution is possible when switching to alternative agents.
193 Unusual presentation of hereditary neuropathy with liability to pressure palsies (HNPP)
Authors: Foo Eng Chuan , Al-Samak Ali , Lwin Khine Khine , Thura Myat , Tun Shwe Zin
Published: August 2022

Introduction

HNPP is an autosomal dominant disorder with estimated prevalence between 0.84- 16/100,000, commonly presenting as mononeuropathies involving pressure-prone areas. Presentation as both a brachial plexopathy and spinal accessory neuropathy is rare, and previously unreported.

Case Report

A 30-year old female with no known medical co-morbidities presented with a 3-month history of right shoulder pain and weakness, and intermittent numbness of the elbow and hand. Examina- tion showed wasting of right trapezius and spinati muscles, shoulder droop, and weakness involving the right sternocleidomastoid, shoulder, and median and ulnar-supplied hand muscles. Nerve conduction studies and electromyography revealed axonal-loss pattern neuropathy of right suprascapular and spinal accessory nerves. Focal demyelination was seen in bilateral median and ulnar nerves across the wrists and elbows. Needle EMG showed denervation changes in right infraspinatus and trapezius muscles. Sub- sequent genetic testing of patient and her mother revealed deletion of chromosome 17p11.2 including PMP22. She improved clinically following a period of intensive physiotherapy.

Conclusion

HNPP typically affects pressure-prone sites such as the median, ulnar or peroneal nerves. Brachial plexopathy is uncommon, and involvement of spinal accessory nerve is very rare. Recognition of symptoms, with appropriate targeted electrodiagnostic and genetic investigations is important in achieving an accurate diagnosis and management.
194 CANVAS-related sensory ganglionopathy without severe ataxia – the importance of muscle afferents in proprioception
Authors: McKenna Mary Clare , Kinsella Justin , Houlden Henry , Connolly Seán
Published: August 2022

Background

Sensory ganglionopathy is typified by selective peripheral sensory axonal loss on nerve conduction studies (NCS) and can occur with CANVAS (cerebellar ataxia with neuropathy and bilateral vestibular areflexia) syndrome.

Case: A 53-year old woman had a 4-year history of some unsteadiness, slurred speech and distal sensory symptoms. She also reported a daily cough over the past 10 years. Clinically, she had dysarthria and mild sensory ataxia. Proprioception and deep tendon reflexes were preserved.

Results of Investigations: NCS demonstrated markedly reduced sensory nerve action potential (SNAP) amplitudes in upper and lower limbs with normal motor NCS. The tibial H-reflexes were strikingly retained. Genetic testing confirmed biallelic RFC1 intronic repeat expansion mutation consistent with CANVAS syndrome.

Discussion

The retained H-reflexes (and tendon reflexes) indicates relative sparing of the group Ia muscle afferents that originate from muscle spindle primary endings and are known to contribute significantly to proprioception. This accounts for the relatively mild gait ataxia in the setting of CANVAS syndrome despite marked sensory NCS abnormalities.
205 Relationship between thrombin generation and micro-embolic signals in moderate-severe asymptomatic and symptomatic carotid stenosis patients
Authors: Subramanian Arun , Murphy Stephen , Smith Deirdre , Tierney Sean , Egan Bridget , Rushe Hannah , O’Sullivan Jamie , O’Donnell James , Hamilton George , McCabe Dominick
Published: August 2022

Background

Data are limited on thrombin generation biomarker profiles in carotid stenosis patients with or without cerebral micro-embolic signals (MES).

Aims andMethods

In this prospective, multi-centre, observational study, plasma thrombin generation parameters were compared in patients with ≥50-99% asymptomatic(N=34) vs. symptomatic carotid stenosis in the early (≤4 weeks; N=39) and late phases (≥3 months; N=31) after TIA/ischaemic stroke. Nested longitudinal follow-up was performed in symptomatic patients. Transcranial Doppler ultrasound classified patients as MES+ve or MES-ve.

Results

There were no significant differences in thrombin generation parameters between early or late symptomatic vs. asymptomatic patients (P≥0.17). In symptomatic patients with matched data at each timepoint, peak thrombin production (365.1 vs. 327.2nM; P=0.003) and endogenous thrombin potential (2242.6 vs. 2078.4 nM*min; P=0.048) decreased between the early and late phases overall. The fall in peak thrombin during follow-up was significant in symptomatic subgroups who underwent revascularisation (N=26; P=0.007) and who were MES-ve (N=14; P=0.029), but not in those on optimal medical therapy alone (N=5; P=0.19).

Discussion

Thrombin generation decreases over time and following revascularisation in TIA/stroke patients with moderate-severe symptomatic carotid stenosis, including those who are MES-ve. These data improve our understanding of haemostatic/thrombotic biomarker profiles in patients with carotid stenosis.
Parallel Session 1: Muscle, nerve and quality| Wed 18 May, 1115 – 1230|04 Project NeuroSafe: safety practice in neurology services
Authors: Kipps Christopher , Mummery Catherine
Published: August 2022

Background

How do we show that neurological services and neurological practice are safe? We aimed to identify the range of safety practices in neurology, develop a means of measuring safety practice activity at individual, service and national levels, assess safety culture across UK neuroscience centres and create benchmarks to allow future comparison of safety practice. We intend that this will provide information for service improvement and individual revalidation.

Methods

Items assessing safety practice were collated from medical literature and direct experience of ABN Quality and Service Committee members. These were combined into a pilot survey for distribution to ABN Advisory Groups and ABN Committee members.

Results

18 questions on individual practice and 15 service-level questions were identified for the pilot. Results of the pilot will be presented and delegates will be asked for feedback during the meeting on the safety questions and their use in appraisal and revalidation.

Conclusion

A suite of individual metrics can be combined to evidence safety practice at a personal and service level in neurology.
Idiopathic intracranial hypertension: evaluation of births and fertility through the hospital episode statistic dataset 2002–2020
Authors: Thaller Mark , Mytton Jemma , Wakerley Benjamin , Mollan Susan , Sinclair Alexandra
Published: August 2022

Introduction

Idiopathic intracranial hypertension (IIH) predominantly affects women of reproductive age with obesity with a distinct profile of hyperandrogenism and insulin resistance. Polycystic ovary syndrome (PCOS) has an established adverse fertility phenotype that typically affects obese women. IIH may impact reproductive health.

Methods

Prospective cohort study of women with IIH aged 18-45 years from the English Hospital Episode Statistic dataset between 1st April 2002 and 31st March 2019. Comparison made to PCOS and general population controls. Main outcome measures were pregnancies, gestational complications, and method of delivery.

Results

Data was collected from 17587 IIH, 199633 PCOS, and 10947012 general population patients. The live birth rate, adjusted for age, was significantly lower amongst women with IIH (54.1%) compared to PCOS (67.9%), p<0.0001 and general population (57.7%), p<0.0001. Pre-eclampsia and gestational diabetes risks were higher following a diagnosis of IIH (5.3-fold and 2.7-fold respectively, p<0.0001) compared to general population controls. Following a diagnosis of IIH, elective caesarean section rates were more than twice that of general population (OR 2.4).

Conclusions

This data indicates there are lower age-adjusted total pregnancy rates, increased risk of pre-eclampsia and gestational diabetes and a doubling of elective caesarean section rates in those with a diagnosis of IIH.
West is best – a strongman with numb feet
Authors: Edwards Marc , Powell Robert
Published: August 2022

A 64-year-old post office worker presented with several years of progressive unsteadiness and tingling sensation in his feet. He was an enthusiastic bodybuilder and had been regularly taking anabolic steroids since his 20s.

On examination he had a broad based gait and Romberg’s test was positive. There was mild dysarthria, dysmetria and an intention tremor. There was wasting evident in his right hand related to a known right ulnar neuropathy. Power was full throughout all limbs but reflexes were reduced. Pin-prick sensation was reduced to the knees and proprioception and vibratory sense were reduced distally.

Neuroimaging revealed cerebellar atrophy and nerve conduction studies an axonal peripheral neuropa- thy. On further questioning the patient disclosed his dietary regime of 4 cans of blended tuna daily for the past 25 years. Mercury levels were significantly raised at 256nmol/l (normal range <25nmol/l). He was advised to stop eating tuna and was prescribed dimercaptosuccinic acid. The level subsequently dropped to 48nmol/l.

Chronic mercury toxicity can present in a variety of ways due to its deposition in different organs (kidneys, liver, brain). In this case, excessive exposure to organic mercury in tuna fish led to a peripheral neuropathy and cerebellar ataxia.
Cryptic confusion
Authors: Vonberg Frederick , Dawson Angelo , Scott Gregory
Published: August 2022

Neurotoxicity is often a late consideration, particularly if the neurotoxic agent is given with therapeutic intent, because the poisoning occurs without the diagnostic clues typical of an intentional attack. We present the case of a 76 year old woman with renal failure, who was admitted with confusion and hal- lucinations. Several days previously, she had started Aciclovir because of a vesicular rash. Her condition was attributed to varicella zoster encephalitis and she was switched from oral to intravenous Aciclovir. Two days later, and after peritoneal dialysis, she had deteriorated further, becoming completely unre- sponsive with upper limb myoclonus.

The 9-Carboxymethoxymethylguanine (CMMG) level was elevated at 21.2 mg/L (normal <2mg/L). A diagnosis of Aciclovir-induced neurotoxicity (AIN) was made. Aciclovir was stopped, the patient received haemodialysis, and she made a significant recovery.

AIN occurs almost exclusively in patients with renal failure. Reduced excretion of Aciclovir, or its prodrug Valaciclovir, results in the toxic accumulation of CMMG. Critically, peritoneal dialysis has little effect on removing Aciclovir. AIN is characterised by triad of hallucinations, involuntary movements, and delusions of death, including Cotard’s syndrome. Diagnosis is challenging as AIN can resemble viral encephalitis. AIN should be considered in encephalopathic patients with renal failure who are taking Aciclovir.
002 Neuromyelitis optica or HIV: the chameleon strikes again?
Authors: Clark William , Tanti Matthew , Vinjam Maruthi
Published: August 2022

A young male presented with a one week history of left leg weakness and sensory loss. On examination there was severe UMN weakness of the left leg and reduced sensation. An MRI head revealed a large right frontal lobe inflammatory lesion, with two further smaller lesions. He subsequently developed severe weakness in both legs, mild weakness in the left arm and urinary retention. He had brisk reflexes and a sensory level at T8. His MRI spine revealed diffuse long segment cord signal change between C6 and T4 in keeping with neuromyelitis optica so he was treated with high dose then weaning steroids. We consid- ered inflammatory, infective, and neoplastic differentials. His HIV screen was positive, he had a low CD4 count (242 cells/mm3) but also a low HIV viral load which is unusual for HIV 1; HIV 2 PCR was negative. HIV broadened the differential to vacuolar myelopathy, infections of the immunocompromised, and CNS lymphoma. Serum MOG and aquaporin 4 antibodies were negative. CSF testing for infections and hae- matological malignancies was negative and his viral load was low. He is improving with therapies, steroids and antiretrovirals. Currently the working diagnosis is seronegative neuromyelitis optica spectrum disorder.
009 Asymmetric peri-optic cerebrospinal fluid space distension in cluster headache
Authors: Farag Mena , Yeung Joshua Au , Heller Simon , Virgo Jonathan , Childs Lucy , Oliver Rupert
Published: August 2022

Distension of the optic nerve sheath is a typical neuroimaging finding seen associated with elevated intracranial pressure and a hallmark MRI feature of papilloedema. Cases of asymmetric and unilateral papilloedema have been reported though the pathophysiological mechanism remains unclear and may be multifactorial.

We present a patient in her mid-thirties with episodic cluster headaches with left peri-optic nerve cerebro- spinal fluid (CSF) sheath distension identified on MRI head and orbits. No obstructive lesion was identified at the orbital apex. Optical coherence tomography revealed bilateral mild symmetrical subclinical optic nerve thinning. Optic nerve function, assessed with acuity, Ishihara colour vision and Goldman perimetry, was normal bilaterally. CSF opening pressure measured 23.5 cm H2O and constituents were bland.

To our knowledge, this is the first description of asymmetric peri-optic CSF space distension in cluster headache with ipsilateral clinical and radiological phenomena. The findings raised the possibility of an asymmetric presentation of elevated intracranial pressure. Our patient, however, had no clinical or other radiological features suggestive of this. The radiological findings ipsilateral to our patient’s cluster headaches raises the difficult question about a causal relationship or simply a mere coincidence between the clinical and radiological picture.