bluuebunny/crossref_metadata_2025 huggingface dataset:
THUR 231 Developing a new rating scale for ocular myasthenia gravis
Authors: Wong Sui H , Miranda Eduardo , Lee Helena , Eggenberger Eric , Cornblath Wayne , Barnett Carolina
Published: October 2018

Ocular Myasthenia Gravis (OMG) causes ptosis and diplopia, which can be disabling. For this study, OMG is defined as MG patients who have ocular symptoms only and no generalised involvement.

A robust way of assessing the severity of OMG symptoms is important for research, to assess treatment and outcome. The rating scales recommended for MG research have a predominant focus on generalised disease, and are insufficiently sensitive for OMG due to the limited number of ocular questions.

This study aims to create a new rating scale for OMG that is sensitive, reliable and clinically usable. We present our proposal of such a rating scale, which incorporates physician- and patient-rated components.

We report the preliminary results of this pilot observational cohort study, in 60 patients with OMG. We compare the results of this with the MG composite scale.

Future work is planned to validate this rating scale and to develop this alongside the MG Impairment Index (MGII).

For future phases of this study we plan to assess the usability of this rating scale by Neurologists without specialist Neuro-ophthalmology.

We invite feedback from Neurologists at the ABN.
WED 236 Skeletal muscle channelopathies and sudden infant death syndrome
Authors: Emma Matthews , Roope Mannikko , Leonie Wong , Dimitri Kullmann , Sanjay Sisodiya , Peter Fleming , Elijah Behr , Michael Hanna
Published: October 2018

Background

Skeletal muscle channelopathies are rare genetic disorders causing periodic paralysis and myotonia. They are generally considered debilitating but not life-threatening. However cases of life-threatening respiratory muscle crises have recently been reported in infants requiring ITU support. We hypothesised muscle channelopathy gene variants may contribute to the risk of sudden infant death.

Methods

We analysed the SCN4A gene for rare variants in 278 cases of sudden infant death (SIDS) and 729 ethnically matched controls. Biophysical characterisation was performed using a heterologous expression system.

Results

Four of the 278 SIDS cases (1.4%) had an ultra-rare, functionally disruptive SCN4A variant compared to 0/729 ethnically matched controls (p=0.0057). The degree of channel perturbation associated with these four variants was qualitatively similar to the SCN4A variants previously implicated in infants with life-threatening apnoeic events.

Conclusions

Rare SCN4A variants that directly alter channel function occur in sudden infant death cases. These variants are predicted to significantly alter muscle membrane excitability compromising respiratory and laryngeal function. Data on pregnancy/post-natal complications is now being collected from parents with genetically confirmed sodium channelopathies via clinic and on-line registry. Laryngospasm has recently been implicated in SUDEP - analysis of SCN4A variants in SUDEP cases is also underway.
WED 237 Phosphorodiamidate morpholino oligomers for treatment of duchenne muscular dystrophy
Authors: Kate Maresh , May Tiet , Michela Guglieri , Joana Domingos , Volker Straub , Thomas Voit , Francesco Muntoni
Published: October 2018

Exon skipping is a novel, mutation-specific approach to treating patients with Duchenne muscular dystrophy (DMD). Phosphorodiamidate morpholino oligomers are nucleic acid analogues that selectively redirect pre-mRNA splicing to enable production of internally truncated dystrophin.

In exon 51 skipping (eteplirsen; n=36) and exon 53 skipping (golodirsen; n=25) clinical studies, internally shortened dystrophin mRNA was observed in all treated patients (per reverse transcription polymerase chain reaction). Eteplirsen increased dystrophin expression 15.5-fold, 11.6-fold, and 2.4-fold vs untreated controls (percent dystrophin-positive fibres, Western blot, and immunohistochemistry intensity, respectively; all, p≤0.007) in a 180 week study, and 2.8-fold (Western blot; p=0.008) in a 48 week study. Golodirsen increased dystrophin expression 10.7-fold (Western blot) over baseline following 48 weeks of treatment. Over 4 years, versus comparable external controls, eteplirsen slowed ambulatory decline (6 min walk test difference, 165 m; p=0.001) and cumulative risk of losing ambulation (83% vs 17%). In 2 clinical studies that included non-ambulatory patients, eteplirsen slowed pulmonary decline versus natural history data (assessed by spirometry).

Eteplirsen and golodirsen demonstrated clinical and biochemical effects in patients with DMD; ongoing studies of these compounds are further characterising their effects in various patient populations.
WED 238 Do we follow the abn myaesthenia guidelines?
Authors: Jacob Roelofs , Rose Bosnell , Geraint Fuller
Published: October 2018

Background

The ABN treatment guidelines1 for myasthenia Gravis (MG) have recently been published.

Aim

We reviewed the records of patients with MG to assess how well the ABN guidelines are being followed in a district general hospital based neurology centre.

Methods

All patients who had positive anti-AchR antibody tests from 2014–2017 and clinicians were asked to identify further patients. Retrospective review of electronic patient record, clinic letters and laboratory data reviewed.

Results

59 patients; 24 ocular and 35 generalised (by type - II: 17, III:15, IV:1, V:1, unclear:1). 63% male, average age 64 (range 18–91). 22/35 (62.8%) generalised MG patients were in remission off pyridostigmine. 13 on steroids, 17 azathioprine, 5 mycophenolate; 3 methotrexate. 2 patients were admitted to ITU, 16 patients had inpatient admissions, 11 patients had IVIG.

14/24 (58.3%) with ocular MG were in remission off pyridostigmine; 5 on steroids, 6 azathioprine. 2 patients had been admitted. 7 of 23 patients on AZT (30.4%) did not have a TMPT level recorded, with only 19 (79%) having sufficiently regular bloods monitoring.

Discussion

Treatment for most patients followed the ABN guidelines. The most common difficulties related to testing for TMPT and blood monitoring.

Reference

1. Sussman J, Farrugia ME, Maddison P, et al. Myasthenia gravis: Association of British neurologists’ management guidelines. Practical Neurology2015;15:199–206.
WED 241 Clinical relevance of regular blood monitoring in IG treatment
Authors: Khalil A , Compton L , Kapoor M , Groves J , Nihoyannopoulos L , Gosal D , Rossor A , Reilly MM , Carr AS , Lavin T
Published: October 2018

Background

ABN immunoglobulin (Ig) guidelines advise routine FBC and U and E monitoring with every treatment episode and screening for IgA deficiency.

Aims

We audited compliance in inflammatory neuropathy patients on longterm treatment in two UK Neurology departments. We looked for evidence of clinically relevant haematological or AKI Ig-related events.

Methods

Data was collected from Nov 2015 to Nov 2017. Accepted definitions for clinically and/or biochemically significant haemolysis, neutropenia, thrombocytopenia and AKI were used.

Results

1919 treatment episodes in 90 patients were analysed. Mean age (SD)=57.6 (14.4)years, 69.1% male, 74% CIDP (26% MMN), 94% IVIg (6% SCIg). Mean dose=1.57 (0.74) g/kg/month or 97.1 (37.3) g/infusion. No clinically significant episodes of haemolysis, neutropenia, thrombocytopenia or AKI occurred in relation to Ig treatment. An asymptomatic drop of >10 g/L Hb occurred in 68/1919 episodes in 38 individuals (3.5%); mean reduction 17.7 g/L, lowest Hb 99 g/L. Two patients with CRF (stage 3) received 28 (IV) and 104 (SC) infusions respectively without impact on eGFR. Two individuals with relative IgA deficiency (0.38 g/L, 0.4 g/L) received 16 infusions over 1.5 years without complications.

Conclusions

No clinically significant Ig-related events were identified in this representative cohort. We suggest annual screening or clinically indicated testing as safe and more appropriate in longterm IVIg use.
WED 242 A challenging case of periorbital swelling
Authors: Aginor Spanoulis , Papastergiou Yalatia , Mitropoulou Panagiota , Burke Georgina
Published: October 2018

A 54 year old lady was referred with a eighteen month history of slowly progressive, asymmetric, periorbital and facial oedema. She was thought to have inflammatory orbital pseudotumour.

During this time, she had also developed a dry mouth, joint pains and enlarged salivary glands. A salivary gland ultrasound scan was suggestive of Sjogren’s disease although antinuclear antibody and rheumatoid factor were negative. She had recently been prescribed omeprazole for mild dysphagia and hoarse voice from vocal cord oedema.

Past medical history included Hashimoto thyroiditis for which she was taking levothyroxine.

Clinical examination revealed peri-orbital and facial oedema causing proptosis of the right globe and complete lid closure. Visual acuity, eye movements and visual fields of the left eye were normal. Her voice was hoarse and she had mouth ulcers. She had a widespread erythematous rash that was thought to be a drug reaction to omeprazole.

Apart from mild lymphopenia and mildly deranged liver function, blood tests, including inflammatory markers and thyroid function, were unremarkable.

MRI of the brain and orbits revealed diffuse oedema of facial structures, including the orbital muscles. A CT body scan was unremarkable.

A temporalis muscle biopsy confirmed a high grade NK/T cell lymphoma.
WED 258 Stroke mimic diagnoses on a centralised hyperacute stroke pathway
Authors: Stefaniak James D , Heartshorne Rosie , Punter Martin , Molloy Jane
Published: October 2018

Introduction

Stroke is highly prevalent, with an estimated 25.7 million occurring worldwide in 20131. In an effort to reduce mortality and length of hospital stay2, hyperacute stroke services have been fully centralised in Greater Manchester since 2015 such that all possible strokes within 48 hours of onset are transferred for assessment at one of three stroke centres. However, there have been concerns regarding the transfer of stroke mimic diagnoses along such pathways.

Methods

A retrospective analysis was performed of patients assessed by the hyperacute stroke team in the Emergency Department of the Comprehensive Stroke Centre (Salford Hospital) in December 2015.

Results

In December 2015, 309 patients with queried stroke were assessed by the Comprehensive Stroke Centre hyperacute stroke team. Of these, 82% had been redirected or transferred from another hospital. 47% had a non-stroke diagnosis at discharge or repatriation, resulting in a combined 331 days of stay at Salford hospital by patients without a stroke. The five most common non-stroke diagnoses were TIA, migraine, infection, seizure and functional neurological disorder.

Conclusions

The optimal clinical care of non-stroke patients should be considered when planning centralisation of hyperacute stroke services.

References

1. Benjamin EJ, Blaha MJ, Chiuve SE, et al. (2017). Heart disease and stroke statistics-2017 update: A report from the American Heart Association. Circulation 135:0. doi:10.1161/CIR.0000000000000485

2. Morris S, Hunter RM, Ramsay AIG, et al. Impact of centralising acute stroke services in English metropolitan areas on mortality and length of study: Difference-in-differences analysis. BMJ 2014;349:4757.
THUR 021 Transforming acute neurology: a 4 year study
Authors: Arani Nitkunan , Bridget Macdonald , Ajay Bhoodoo , Medina Southam , Caitlyn Smyth , Andrew Tomkins , Fred Schon
Published: October 2018

We present a novel approach to acute neurological care. The key is an acute neurology triage nurse, based in the medical admission unit as well as an epilepsy specialist nurse seeing every patient referred with fits on the day of admission, a designated acute neurology consultant and acute neurophysiology and neuroradiology links. We have designated this group, a hyperacute neurology team (HANT).

This study compares all admissions in 2014, the year before the team was established with 2015–2017. The total number of referrals has increased from 720 in 2014 to 1248 in 2017. The percent of patients seen on the day of referral has risen from 59% in 2014 to 92% in 2017.

Average length of stay for patients with a primary diagnosis of epilepsy has gone down from 4.1 days in 2014 to 3.4 in 2017. Multiple admissions for epilepsy has reduced from 28 in 2014 to 21 in 2017. Patients suitable for early discharge are seen in consultant or nurse «outpatient hot clinics» or nurse telephone clinics.

The cost of establishing this service has been relatively small (£106,000) and the service benefits enormous. We feel this model is worthy of wider debate.
284 Acute neurology in aneurin bevan university health board
Authors: Anderson Joe
Published: October 2018

Aneurin Bevan University Health Board (ABUHB) provides for a population of 6 40 000 in South East Wales, in addition to South Powys. The large geographical area contains 3 sites with acute unselected medical intake as well as 3 other hospitals. The neurology department, comprising 6 consultants (1 stroke/neurology), 1 associate specialist and 1 trainee, is based in Newport. Service evaluation (2013) revealed that 1 in 6 neurology clinic appointments were used to see patients discharged from acute medicine, with a median waiting time of 14 weeks. In 2015 the ‘Neurologist of the Week’ service was launched; 5 consultants participate on a weekly rota. Routine work is cancelled and replaced with a 10 DCC (~40 hours) acute neurology week. This includes 3 acute clinics (each 3 patients), daily MAU round for the largest site, and a triage and advice service for primary and secondary care. Visits to other hospital sites are made when needed. The service has led to significant improvements in quality of care, neurology training and undergraduate teaching and is highly valued by colleagues. Repeated evaluations show ~55% of acute clinic patients are discharged, with ~40% of appointments preventing or shortening an admission. Diagnosis is significantly changed in ~40% of consultations.
285 Piloting and implementing an acute neurology service
Authors: Peukert Thomas
Published: October 2018

Over the past decade, the number of patients attending the emergency department (ED) at the Royal Victoria Hospital Belfast, with neurological symptoms, has doubled. Typically, over 50% of these patients would subsequently be admitted to hospital. In 2013, a pilot project was conducted with the aim of evaluating the effectiveness of a rapid access neurology clinic on reducing such admissions.

A dedicated neurology clinic was set up offering 12 slots per week. Patients were seen within 10 days of ED staff booking them into the clinics. Early results indicated that within the first month 28 admissions were avoided. As a result rapid access neurology clinics were rolled out. Two acute neurologists were appointed and since 2015, 3 rapid access clinics run per week (15 slots). In addition to the rapid access clinics, the acute neurology team also offer two additional services:

Reviewing all patients who have been admitted under the medical take with neurological symptoms

Patients who attend ED overnight but require urgent evaluation/tests can be sent home and will be seen the next morning by the acute neurology team

Analysis indicates approximately 1250 admissions are avoided each year with an estimated cost saving of over £2 million.
290 CSF chitinases as novel biomarkers for MND
Authors: G Thompson Alexander , Elizabeth Gray , Marie-Laetitia Thézénas , D Charles Philip , Samuel Evetts , T Hu Michele , Kevin Talbot , Roman Fischer , M Kessler Benedikt , R Turner Martin
Published: October 2018

The development of biomarkers for amyotrophic lateral sclerosis (ALS, the commonest phenotype of MND) is a priority to reduce diagnostic delay, allow earlier assessment of therapeutic activity, and provide pathogenic insight.

Liquid chromatography tandem mass spectrometry with label-free quantification was used to quantify CSF proteins in individual participant samples. A longitudinal cohort comprised patients with ALS (n=43) and primary lateral sclerosis (PLS, n=6). A cross-sectional cohort comprised healthy (n=20) and disease control patient CSF samples (Parkinson’s disease n=20, MND mimic disorders n=12).

Among 773 identified proteins, significantly elevated levels of three macrophage-derived chitinase proteins were detected in the ALS group, specifically chitotriosidase (CHIT1), chitinase-3-like protein 1 (CHI3L1) and chitinase-3-like protein 2 (CHI3L2). Levels correlated with rate of disease progression (CHIT1 r=0.56, p<0.001; CHI3L1 r=0.31, p=0.028; CHI3L2 r=0.29, p=0.044), and levels of the axonal degeneration marker phosphorylated neurofilament heavy chain (r=0.62, p<0.001; r=0.49, p<0.001; r=0.41, p<0.001). Levels of CHI3L1, but not CHIT1 or CHI3L2, increased over time in those with low initial values (gradient=0.005 log abundance units/month, p=0.001).

Microglial activation has been implicated in the pathogenesis of MND and neuroinflammatory pathways are a major target of therapeutic interest. CSF chitinases may be potential pharmacodynamic as well as diagnostic biomarkers.
298 Neurosarcoidosis – review of 91 patients from three UK neuroscience centres
Authors: Reem Farwana , Alex Murley , Lauren Ankrah , Dan Whittam , Tom Hayton , Anu Jacob , Cris Constantnescu , Saiju Jacob
Published: October 2018

Background

Neurosarcoidosis is a rare neuro-inflammatory disease with a wide variety of clinical presentations.

Methods

Retrospective audit was conducted independently at Birmingham, Liverpool and Nottingham to identify patients with neurosarcoidosis and pooled data was analysed.

Results

88% of the 91 patients identified presented over 40 years of age. From the data available, 70% were White Caucasians, 21% Afro-Caribbeans and 8% were Asians. Optic neuritis (31%), limb weakness (30%), headaches (20%) and facial palsy (19%) were the most common clinical presentations with nearly a third of patients (27/91) having moderate or severe disability (modified Rankin scale ≥3) at onset. 27/58 (46.6%) of patients had raised serum ACE levels and 37/44 (84%) patients had raised CSF protein with 28/41 (68.3%) demonstrating CSF lymphocytosis (mean – 54, range 5 to 380 cells). 35/91 patients had radiological changes affecting the brain parenchyma with 23% showing leptomeningeal enhancement. 75% of the 36 patients with data available for CT chest showed evidence of pulmonary sarcoidosis. Steroids were administered in 84.9% (73/86) patients with 21 receiving steroid sparing drugs, mainly Azathioprine and Mycophenolate.

Conclusion

We discuss the varied clinical presentation of neurosarcoidosis across three major UK neuroscience centres and attempt to risk stratify patients prior to immunomodulatory therapy.
THUR 037 Identification of biofluid markers of TDP-43 pathology
Authors: Rachelle Shafei , Martha Foiani , Carolin Heller , Amanda Heslegrave , Ione Woollacott , Katrina Dick , Jason Warren , Jonathan Schott , Henrik Zetterberg , Jonathan Rohrer
Published: October 2018

Introduction

Frontotemporal dementia (FTD) is usually caused pathologically by either tau or TDP-43. Previous biofluid assays of TDP-43 have not so far proved to be sensitive or specific for identifying those cases with TDP-43 pathology.

Material and methods

We set out to investigate the novel TDP-43 Simoa assay (Quanterix) assay in both plasma and CSF in a cohort of patients recruited from the University College London FTD observational studies with known or likely TDP-43 pathology (17), non-TDP-43 pathology (13), and healthy controls (10).

Results

The mean [standard deviation] plasma TDP-43 concentration was higher in those with likely TDP-43 pathology (155.1 [223.4] pg/ml) than those with non-TDP pathology (112.39 [252.9] pg/ml), and healthy controls (50.0 [23.1] pg/ml), but the differences between groups was non-significant, with substantial overlap in concentrations between all three groups. The mean CSF TDP-43 concentration was 2.9 [0.3] pg/ml in those with likely TDP-43 pathology, 2.8 [0.4] pg/ml in those with non-TDP pathology, and 3.1 [0.5] pg/ml in healthy controls.

Discussion

The assay tested in this study does not accurately distinguish between those with likely TDP-43 pathology and either disease controls or healthy individuals. There remains an urgent need to develop a better biofluid assay for pathological TDP-43.
THUR 052 Epilepsy in the land of ice and fire
Authors: L Wojtowicz Alex , H Thomas Rhys
Published: October 2018

Background

Epilepsy is often explained through allegory; from magical thinking to misfiring neurons. It is important to appreciate pervasive media portrayals which influence lay attitudes toward epilepsy. George R. R. Martin’s series of fantasy novels A Song of Ice and Fire, and the HBO television adaptation A Game of Thrones, introduce characters with ‘mundane’ and ‘mystical’ epilepsy to millions. Cases are presented to highlight these varying portrayals.

Cases

RA, 8 years old, has ‘shaking sickness’. Triggered by stress, his hands ‘shake’ with subsequent involvement of all limbs. He loses consciousness, is incontinent of urine, and demonstrates post-ictal confusion. He is enmeshed with his mother and his condition is viewed as a manifestation of his unsuitability to rule.BS fell at 9 years old, resulting in a coma. Subsequently he remains paraplegic with dialeptic episodes. During absences, he can ‘possess’ animals or people, and experiences impossible hallucinations. These abilities are portrayed as empowering for a boy who suffers heavy stigma against physical disability.

Conclusion

Fans will probably not adopt ‘magical’ views but may internalise stigma weighed against characters with both ‘mundane’ and ‘magical’ epilepsy. Due to the large audience, clinicians working with epilepsy patients might benefit from awareness of these portrayals.
WED 061 ‘real life’ experience with brivaracetam as add-on treatment for epilepsy
Authors: Rhiannon Hughes , Melissa Maguire , Jo Geldard , Markus Reuber
Published: October 2018

Background

Brivaracetam received its UK licence as an adjuvant drug for focal epilepsy in adults in 2016. There is little data on its efficacy and tolerability in ‘real life’ settings. We present an interim analysis of a multicentre service evaluation of brivaracetam.

Methods

Data was retrospectively collected from clinical records at two UK centres of consecutive patients treated with brivaracetam and at least one follow up. Data were also extracted on previous levetiracetam use. Seizure frequency was categorised at baseline and follow-up (daily/weekly/monthly/yearly/none).Results: Of the 44 patients identified (17 male, mean age 39, range 19–66), 75% had a history of levetiracetam exposure (LE+) and 25% did not (LE−). Mean brivaracetam exposure was 10.3 months (2–21), the mean daily dose was 200 mg (50–400). Retention was 91% vs 100% in LE+ versus LE− groups at 3 months and 82% in both groups at 6 months. Seizure category improvements were seen in 20%/36.4% in the LE+ vs LE− groups, seizure category deteriorations in 0% vs 9%. There were no serious adverse events.

Conclusion

Brivaracetam emerges as a potentially useful adjuvant medication for focal epilepsy. It may be better tolerated by some patients than Levetiracetam and more effective than this drug.
WED 066 Web-based media as a self-management tool for people with epilepsy
Authors: Alison McKinlay , Leone Ridsdale
Published: October 2018

Background

There is little evidence on how people with epilepsy (PWE) use web-based media in self-management of their condition. This study focused on the benefits/risks for PWE using social networking sites and web-based media.

Methods

We recruited 14 PWE who had volunteered after seeing information provided by Epilepsy Action, UK. We asked open-ended questions about online media use. Interviews lasted 60–90 min. Sessions were recorded, transcribed, and thematically analysed using Nvivo.

Results

Seven men and seven women participated, age range: 33–73, average diagnosis length was 25 years. Twelve participants used web-based media to gather information about their epilepsy. Seven used apps to manage their epilepsy by logging seizures or medication reminders. Six participants were hesitant to use web-based media due to privacy concerns. Four participants felt that epilepsy was underrepresented or misrepresented online. Three participants preferred traditional sources of epilepsy-related information.

Discussion

Results show the value of web-based media in providing information and support to PWE, with the caveat that concerns around privacy and disclosure can undermine potential benefits. Health service providers and advocacy groups can assist by ensuring online information is accurate and up-to-date. Further research may help in developing understanding and future services.
THUR 075 Evaluating a personal information pack in epilepsy
Authors: Evan Edmond , Arpan Mehta , Alison Taylor , John Jacob
Published: October 2018

A mainstay of epilepsy management is patient education and engagement. Previous educational interventions have varied greatly in number of sessions, teaching methodology and cost. This study assessed the impact of a low-cost intervention consisting of a personal information pack.

Methods

Forty-six consecutive patients with an existing epilepsy diagnosis attending clinic at MKUH NHS Trust were enrolled and pack provided. A baseline questionnaire assessed patient understanding and service utilisation (GP, A and E, specialist nurse). The questionnaire was repeated 3 months later. Patient engagement with the pack was assessed (completeness of information in pack, patient rating of usefulness).

Results

Fourteen patients were lost to follow-up - thirty-two completed the second interview. There was a trend towards reduced service utilisation. Specialist nurse visits were significantly reduced (2.75 visits/year pre-intervention vs 1.11 post-intervention, p=0.01). Patient understanding was not significantly changed. Patient rating of usefulness was positive (2.4/3 on Likert scale). Two thirds of patients had not filled out the pack or added basic details only.

Discussion

This simple, low cost intervention may reduce some types of service utilisation and be found helpful by patients. A planned redesign involves the provision of a low-effort pre-filled information card together with the larger pack.
WED 078 ‘not just a headache’: social media use in people with migraine
Authors: Leone Ridsdale , Rosanna Swindale , Peter Keighley , Carly Pearson
Published: October 2018

Introduction

‘Invisible’ conditions like migraine may leave individuals seeking support and information. We aimed to describe how people with migraines use and benefit from social media and to identify harms of social media use.

Methods

Twenty participants were recruited via migraine charities. Semi-structured interviews were conducted with questions based on a topic guide. Interviews were transcribed and analysed using thematic analysis.

Results

We found people with migraine use social media to better understand their condition and treatment options. It offers instant access to continuous information and social support from empathic others. Participants viewed social media as an outlet to vent frustrations and to validate their migraine experience. They referred to the invisible and episodic nature of migraine which contributes to misunderstanding of the impact and/or severity of the condition. Some masked their online migraine-related behaviour, using different sites or closed online groups, which sometimes changed their online behaviour in other areas. Harms of social media included inaccuracy of information, occasional negativity, and privacy issues.

Conclusions

Social media can provide people who experience migraines with instant, continuous access to social support and health information, from empathic others. This can validate their illness experience, reassure and help to reduce feelings of isolation.
WED 079 Epidural blood patch: impact of non-luer one-way butterfly needle
Authors: Asif Mahmood , Paul Sharpe
Published: October 2018

The epidural blood patch (EBP) requires a syringe that connects to the intravenous and epidural route. Thereby contravening Department of Health guidance, which recommends adoption of equipment that, prevents wrong route drug administration. To comply with these recommendations a non-Luer butterfly needle with one-way valve has been produced by GBUK. The one-way valve and length of tubing has the potential of activating the clotting cascade. This could reduce the time clinicians have to utilise the blood in the syringe. Also any alteration in clotting could affect the therapeutic value of the EBP. The primary objective of this research was to determine if phlebotomy using this new 21G needle altered blood clotting, determined by thromboelastograph analysis, compared to a standard 21G hypodermic needle.

We performed paired phlebotomy on 10 healthy volunteers; producing a study capable of revealing a 25% change in R-time (power 80%). Mean R-time was increased with the new needle, however remaining within normal range (7.3 vs 6.6 min (p=0.04)). This would not limit the time to utilise the blood before clot formation in the syringe. With no clinically relevant difference in MA or LY30, it is unlikely to have any impact on the therapeutic value of the EBP.
WED 080 Experiences of specialist referral and GP access to MRI for headache
Authors: Alison McKinlay , Raphael Underwood , Asif Mazumder , Rachael Kilner , Leone Ridsdale
Published: October 2018

Introduction

When General Practitioners (GPs) refer patients with headache to neurologists, it is often because the patient and/or doctor want imaging. Some GPs now access Magnetic Resonance Imaging (MRI) directly. We aimed to describe patients’ experience of GPs using direct access, compared to patients who saw a specialist first.

Methods

We invited participants to semi-structured Interviews about two months after imaging. Interviews were audio-recorded, transcribed, and analysed using thematic analysis in Nvivo.

Results

We interviewed 10 patients from each pathway in South London, eleven women, median age 41, range 20–72. We found more similarities than differences between groups. Ten said they received a clear scan result explanation, while six had difficulty understanding results. Eleven participants felt relief once results were received, while five still wanted an answer on the underlying cause for symptoms. Seven felt the specialist appointment wait time was long. Those using the direct-access pathway were more likely to report MRI results delay.

Conclusion

Patient reassurance was linked closely with results receipt and worry linked with wait times. Some felt MRI results did not provide sufficient explanation for symptoms. Improvement in both pathways can be achieved, providing results are delivered in a clear, timely manner.
THUR 084 Cranial autonomic symptoms in cluster headache induced by nitroglycerin
Authors: Wei DY , Goadsby PJ
Published: October 2018

Introduction

Cluster headache (CH) is characterised by attacks of unilateral excruciating headache, ipsilateral cranial autonomic symptoms (CAS) and/or agitation. Studying of CAS can further our understanding of CH pathophysiology, but is limited by the episodic nature of the disease. Nitroglycerin (NTG) is known to induce CH. The aim of this study is to characterise CAS induced by NTG.

Methods

CH patients received intravenous NTG 0.5 mcg/kg/min over 20 min. CAS and headache phenotype were recorded. The study was approved by the NHS Research Ethics Committee.

Results

Twenty-three patients participated: 83% male and 61% episodic cluster headache. The most common spontaneous CAS reported were lacrimation, nasal congestion and conjunctival injection. Agitation was reported in 96%. Nitroglycerin induced ipsilateral CAS in 91% of the patients, with 74% with ipsilateral pain. Most commonly induced CAS were nasal congestion, lacrimation and periorbital swelling. Agitation was reported in 61%. The majority of the CAS (80%) induced by NTG presented before the onset of severe pain.

Conclusion

We demonstrate that NTG effectively triggers ipsilateral cranial autonomic symptoms in CH patients and that they often present in a phase before the onset of pain reflecting the underlying pathways during a cluster headache attack.
THUR 101 SCA-1 with unusual early dysautonomia and delayed cerebellar signs
Authors: Eleanor d’Ersu , NurAizaan Anwar , Talal Al-Mayhani , Meneka Sidhu
Published: October 2018

A 59-year-old right-handed bus driver presented with gradually worsening erectile dysfunction, urinary frequency and nocturia over 18 months. He had a past medical history of hypertension with regular medications including amlodipine, aspirin and atorvastatin. Initial examination revealed a mild bilateral intention tremor.

The patient had undergone brain imaging showing brainstem and cerebellar atrophy with a ‘hot cross bun sign’. The patient was then referred for autonomic function tests that demonstrated mild cardiovascular autonomic changes, and suggested sympathetic dysfunction. A working diagnosis of multiple system atrophy (MSA) was made.

Over the next two years, his tremor worsened with the development of slurred speech and unsteady gait. On examination, he had prominent fasciculations and wasting in the right shoulder, severe bilateral intention tremor with past-pointing. In addition he had Parkinsonism and signs of a sensorimotor neuropathy.

This atypical clinical evolution with prominent neuropathy and cerebellar signs led to investigations for spinocerebellar ataxia (SCA), particularly type 2 or 3 (given the autonomic dysfunction). However, the genetic testing showed a mutation in ATXN1, confirming a diagnosis of SCA type 1.

To our knowledge, this is the first reported case of prominent early autonomic dysfunction associated with SCA type 1.
THUR 104 Global MD registry and dystonia non-motor symptoms study
Authors: Zehra Yilmaz , Kathryn Peall
Published: October 2018

Myoclonus Dystonia (MD) is a rare hyperkinetic movement disorder with an early childhood onset. Estimates of MD prevalence among European populations places this at 1 in 5 00 000 individuals, however no worldwide estimates currently exist. MD patients experience alcohol-responsive myoclonus predominantly affecting the trunk and upper limbs, and dystonic posturing typically involving the neck and hands (writer’s cramp) but may also involve other parts of the body (e.g. lower limbs and trunk). Individuals with MD also frequently report non-motor symptoms (NMS) such as psychiatric co-morbidity, significant pain that impacts function and daily living, and disturbance to sleep.

This study aims to develop a global register of MD patients of all ages, and further characterise the NMS in 500 adult MD patients via validated online psychiatric, sleep and pain questionnaires and cognitive tests. The register will also be used to establish a research hub to direct future research into elucidating disease mechanisms, and develop novel therapeutic targets for MD. The online questionnaires will be developed using the Bristol Online Surveys platform. All data will be encrypted to ensure patient confidentiality, and then analysed using Microsoft Excel and R statistical software package. P values less than 0.05 will be deemed as statistically significant.
WED 114 Pathways to diagnosis in PSP, CBD and PD
Authors: Diane Swallow , Carl Counsell
Published: October 2018

Misdiagnosis and delayed diagnosis are common in PSP/CBD. To inform approaches to reduce diagnostic delay, it is essential to systematically evaluate patient pathways to diagnosis.

Cases with a final diagnosis of PSP (n=28), CBD (n=2), and 30 age-sex matched PD controls, were identified from the Parkinsonism Incidence in North-East Scotland study. Using general practitioner, hospital and research records, referral and diagnostic time intervals from symptom onset to death were recorded.

Comparing PSP/CBD to PD, the median (interquartile range) time intervals from index symptom to first secondary care referral [0.7 (0.01, 2.53) vs 0.02 (0.00, 0.73) years] and review [0.84 (0.18, 2.56) vs 0.13 (0.07,0.88) years], and, first neurologist referral [1.67 (0.70, 4.45) vs 0.12 (0.00, 1.56) years] and review [1.72 (0.88, 4.53) vs 0.23 (0.11, 1.65) years], were significantly longer in PSP/CBD (p=0.001 to 0.031). The average time intervals from index symptom to a parkinsonian syndrome diagnosis [2.26 (0.85, 5.41) vs 0.10 (0.00, 0.90) years], inclusion of the final diagnosis amongst differential diagnoses [3.62 (2.06, 7.21) vs 0.16 (0.00, 1.53) years], and the final diagnosis as primary diagnosis [4.22 (2.28, 7.63) years vs 0.67 (0.10, 3.04) years] were similarly longer in PSP/CBD (p≤0.001).

Referral and diagnostic time intervals are significantly longer in PSP/CBD when compared to PD. Identifying factors that both improve the timing and destination of referral decision-making, and the accuracy and timeliness of diagnosis is necessary.
THUR 116 Vascular prevention in patients with parkinson’s disease
Authors: Isla Kempe , A Grosset Katherine , G Grosset Donald
Published: October 2018

Background

Vascular prevention is appropriate for patients with a vascular history (secondary prevention) and increased risk (primary prevention). Cerebrovascular disease adds to gait and cognitive problems in patients with Parkinson’s disease (PD).

Methods

A convenience cross-sectional sample of consecutive PD patients attending the Neurology Movement Disorder clinic was assessed, and QRISK3 scored when appropriate (cases without vascular events, age <85 years).

Results

Of 100 cases, mean age 66.5 (SD 9.0) years, 52.0% male, with PD duration 8.3 (SD 5.5) years, 15 had a vascular history meriting statin therapy, of whom 12 (80.0%) were prescribed statins. 22 had a high vascular risk (QRISK3 >20%), mean QRISK3 28.6 (SD 7.7) of whom 2 (9.1%) were prescribed statins. We are now actively assessing QRISK3 and recommending statin therapy where appropriate.

Conclusions

Secondary vascular prevention with statins is more commonly implemented than primary prevention, in patients with PD. In patients without a vascular diagnosis, vascular risk should be assessed and statin therapy offered where appropriate, noting that around one-fifth of patients have a high vascular risk and are not on statin treatment.
THUR 122 Parkinson’s kinetigraph improves movement disorder service provision
Authors: Christopher Kobylecki , Lucy Partington-Smith , Monty Silverdale
Published: October 2018

Introduction

Objective evaluation of symptoms of Parkinson’s disease (PD) can be challenging. There is increasing interest in technological solutions to assess, monitor and manage people with PD.

Objective

To evaluate the effect of the Parkinson’s Kinetigraph (PKG) on management of patients with PD in a large tertiary movement disorder service.

Methods

We retrospectively reviewed the notes of 47 patients with PD (22 female, 25 male) who underwent PKG recording over a six month period. The indications and PKG findings, and the subsequent effect on clinical decision making and service provision were recorded.

Results

Management was significantly altered in 25 patients (53%), while in 13 patients (28%) PKG confirmed the use of advanced therapies such as deep brain stimulation. Significant effects were seen with regard to service provision. Outpatient appointments could be deferred with advice following PKG in 15 (32%), advanced therapies assessment was improved in 16 (34%), while inpatient admission was avoided in six patients (13%).

Conclusion

The use of PKG has enhanced service provision in our movement disorder service. In particular, it enhances our assessment of patients considered for high-cost advanced therapies, allows more efficient use of clinic appointments, and has the potential to reduce hospital admissions.
WED 131 Evaluating the clinical utility of the parkinson’s kinetigraph (PKG)
Authors: Hutchinson Lewis , Dominey Thea , Pearson Emma , Murphy Fiona , Bell Lucy , Carroll Camille
Published: October 2018

Objective

To evaluate the utility of the Parkinson’s Kinetigraph (PKG™) in the remote management of Parkinson’s disease (PD).

Background

There is a movement in Parkinson’s care from a clinic-based model1 to P4 medicine, meaning medicine that is predictive, preventive, personalised and participatory.2 The development of wearable technology provides an opportunity to monitor patients remotely, and deliver targeted care. The PKG™ is a wrist-worn device that objectively measures Parkinson’s symptoms.

Aim

To evaluate the utility of the PKG™ in managing PD patients remotely, and the perception of service users.

Method

PKG™ data were collated in real time. Patient acceptability data were collated via a patient questionnaire (n=61).

Results

Between July 2015 and January 2018, 216 PKGs were performed. A variety of symptoms were identified, including different types of ‘OFF’ times (wearing off (25%), delayed on (6%) no drug response (8%)) and non-motor complications (fragmented sleep (33%) and daytime somnolence (21%)), with subsequent treatment recommendations being made. Patient acceptability of the PKG™ was high, 81% of patients being satisfied not having to travel for clinic appointments.

Conclusions

The PKG™ facilitated remote treatment recommendations. Remote management was acceptable to patients. Future evaluations will evaluate patient outcome.

References

. van der Eijk M, Nijhuis FAP, Faber MJ, Bloem BR. Moving from physician-centered care towards patient-centered care for Parkinson’s disease patients. Parkinsonism Relat Disord [Internet]. Elsevier; 1 November 2013;19(11):923–7. Available from: https://www.sciencedirect.com/science/article/pii/S1353802013001697?_rdoc=1&_fmt=high&_origin=gateway&_docanchor=&md5=b8429449ccfc9c30159a5f9aeaa92ffb [cited 2018 February 6]

. P4 medicine: how systems medicine will transform the healthcare sector and society. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4204402/pdf/nihms532619.pdf [cited 2018 April 6]
WED 145 Human T-lymphotropic virus (HTLV)-associated encephalitis
Authors: Ania Crawshaw , Divya Dhasmana , Carolyn Gabriel , Brynmor Jones , Steve Sturman , Nick Davies , Graham Taylor
Published: October 2018

Clinical features of human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy (HAM) are predominantly consistent with cord pathology. Imaging and autopsy studies have also demonstrated brain inflammation in HAM though this is generally subclinical. Six cases (of whom four had HAM) have previously been reported with encephalopathy in HTLV-1 infected patients, without alternative identified aetiology. Histopathology was reported in three of these, with perivascular CD8 +lymphocytic infiltrates in the brain, similar to that seen in the spinal cord in HAM.

We describe three further cases of encephalitis in the UK HAM cohort (n=142). Clinical features included: reduced consciousness; fever/hypothermia; headache; seizures; focal neurology. Investigation showed: raised CSF protein; pleocytosis; raised CSF:peripheral blood mononuclear cell HTLV-1 proviral load ratio; MRI either normal or showing diffuse, partially reversible leucoencephalopathy. No alternative aetiology was found. Two died following 1–5 recurrent episodes over a period of 1–5 years, despite responses to immunosuppression.

We reviewed the existing literature together with our new cases. 7/9 had concurrent HAM. All seven deteriorated sub-acutely preceding encephalopathy. 7/9 were treated with IV steroids, six improved. Six died, five despite steroids.

We propose that HTLV-associated encephalopathy may be part of the spectrum of disease seen in HAM itself.
THUR 150 Treatment and outcomes in primary angiitis of the CNS (PACNS)
Authors: Xin Chua Wei , Dan Whittam , Saif Huda , Anu Jacob
Published: October 2018

Background

PACNS is a rare and heterogeneous disorder with many uncertainties regarding management and outcomes. Published adult UK series total just 20 patients.

Method

Hospital record search of an adult tertiary neurosciences centre identified 14 cases of PACNS from 1033 potential cases over 20 years.

Results

All 14 cases were ‘definite’ by Calabrese Criteria (1988), confirmed by brain biopsy in 8/14 and by angiography in 6/14.

The median age of onset was 52 years and most presented with focal deficits (86%), headache (36%) and cognitive change (29%). MRI showed multifocal deep white matter change in 86% and infarcts in 50%. Gadolinium enhancement was seen in 43%. Two patients had solitary ring-enhancing lesions and one patient had signs of cord ischaemia. CSF was abnormal in 90%.

All patients received corticosteroids, 11/14 cyclophosphamide, 3/14 Mycophenolate Mofetil, 3/14 Methotrexate and 2/14 Azathioprine. At 13.3 months median follow-up, all but the two patients with solitary lesions remained on immunosuppression. Clinical relapses occurred in 30% and radiological relapse in one case despite immunosuppression.

Only 62% had a favorable outcome (mRS 0–2). One patient died as a direct consequence of PACNS and one patient died due to co-morbidities.

Conclusion

PACNS remains a treatable neuroinflammatory disease with poor outcomes.
THUR 154 NMDAR-AB encephalitis: frequency of diagnosis and outcomes
Authors: H Lim , D Whittam , E Jackson , N Williams , D O’Connell , C Chadwick , G Keir , S Huda , A Jacob
Published: October 2018

Background

The association of N-methyl d-aspartate receptor-antibodies (NMDAR-Abs) and encephalitis is now well recognised.

Methods

Retrospective review of frequency of diagnosis and outcomes in encephalitis with NMDAR-Abs identified at the Walton Centre between 2012–2017.

Results

NMDAR-Abs were detected in 29/1131 (3%) of sera and/or CSF samples. Of 20 (69%) patients with encephalitis, 50% were identified in the last two years. Median onset age was 34(17–75) years and 60% (12/20) were female. Median symptom duration before diagnosis was 24(2–720) weeks between 2012–2015, improving to 14(1–96) weeks between 2015–2016. Five patients (25%) had an infectious prodrome (one prior HSV-1 encephalitis). Psychiatric/cognitive symptoms, seizures, and movement disorders were present in 80% (16/20), 70% (14/20), and 55% (11/20) of patients respectively. Three patients had ovarian teratomas.

Electroencephalograms were abnormal in 65% (15/23) and MRI brain in 37% (7/19) patients. Unmatched intrathecal oligoclonal bands and CSF pleocytosis were present in 31% (5/16) and 60% (15/25) of samples respectively. Immunotherapy was beneficial in 73% (11/15) of patients. Two patients died (sepsis and multi-organ failure) and two improved spontaneously. At a median follow-up of 9 (5–180) months, 69% (11/16) of patients had an mRS ≤2.

Conclusion

Although the recognition of NMDAR-Ab encephalitis has improved, there is still a significant delay to diagnosis. The majority of patients have good outcomes.
THUR 156 Safe prescribing of immunosuppression: quality improvement project
Authors: Kathryn Bush , Vivien Smith , Gemma Maxwell , Neil Archibald
Published: October 2018

Background

The use of immunosuppression therapies in Neurology has extended exponentially since they were first used in the 1970s for myasthenia gravis. However there are no NICE or neurology specific guidelines in this area for Neurologists to refer to in their clinical practice.

Method and results

This 2014 retrospective case notes audit compared medical documentation to ‘10 key statements’ pertaining to the safe use and monitoring of immunosuppression therapies. This is a framework for clinical practice that was first published by The British Association of Rheumatology and The British Association of Dermatology in 2008.

A number of key areas of poor documentation around drug safety, pregnancy and vaccinations were identified and in response generic and drug specific safety ‘check-lists’ and guidelines were produced, to be filled out with all patients, prior to starting therapies.

In 2016 following the implementation of the quality improvement project, a repeat audit of patient’s medical notes showed a clear improvement in documentation.

Conclusions

Drug specific guidelines led to an improvement in documentation and we believe that this also reflects safer prescribing and improved clinical practice. We are now in the process of developing guidelines to be used throughout the entire Northern Deanery.