Ehlers-Danlos syndromes (EDS) is an umbrella term describing 14 types, of which 13 are rare and monogenic, with overlapping features of joint hypermobility, skin, and vascular fragility, and ...generalised connective tissue friability. Hypermobile EDS currently has no identified genetic cause. Most of the rare monogenic EDS types can have neurological features, which are often part of major or minor diagnostic criteria for each type. This review aims to highlight the neurological features and other key characteristics of these EDS types. This should improve recognition of these features, enabling more timely consideration and confirmation or exclusion through genetic testing. In practice, many healthcare professionals still refer to patients as having 'EDS'. However, the different EDS types have distinct clinical features as well as different underlying genetic causes and pathogenic mechanisms, and each requires bespoke management and surveillance. Defining the EDS type is therefore crucial, as EDS is not in itself a diagnosis.
Correspondence to Dr Oliver Jonathan Ziff, University College London Hospitals NHS Foundation Trust, National Hospital for Neurology and Neurosurgery, London WC1N 3BG, UK; o.ziff@ucl.ac.uk We would ...like to commend Rahman et al for their work highlighting the reasons for delays in inpatient consultations.1 The authors found that miscommunication between referring and receiving teams was responsible for 34% of all delays each with an average delay of 1.6 days in length of stay (combining communication and power dynamic categories). Incoming calls were erratic with 33% of all calls being received on Fridays and almost half of calls coming between the hours of 9:00 and 10:00 (25%) or 16:00 and 17:00 (22%).Table 1 Comparison of phone and email referral systems Phone Email Incoming messages Total 99 100 Unique referrals 50 (51%) 65 (65%) Messages missed 62 (63%) 0 (0%) Outgoing messages Total 54 23 Messages missed 20 (44%) 0 (0%) Busiest period Weekday Friday Thursday Hour slot 9:00–10:00 12:00–13:00 Conversely, with our email system, all emails (both incoming and outgoing) were acknowledged (none were missed) within 24 hours of being sent. A post hoc survey of users revealed that e-referrals were associated with far-reaching benefits including: (1) more secure method of receiving referrals, (2) easier referral prioritisation, (3) minimises disruption to ongoing consultations, mitigating errors causes by interruptions,2 (4) documentation provides an audit trail enabling retrospective interrogation of case management, (5) instant automatic replies acknowledges referral receipt and provides key contact details, for example, specialist nurse emails and answers to frequently asked questions.
IntroductionAtrial fibrillation (AF) in patients with ischaemic ocular events is often under-investigated. We aimed to determine the prevalence of AF in these patients.SettingUniversity College ...Hospital London daily TIA clinic, main referral centre for North-Central London. Consecutive records for all patients with transient or permanent ischaemic visual loss were reviewed, January 2014-September 2016. Electrocardiograms (ECG), cardiac monitoring reports and previous AF were recorded.ResultsOf 395 patients, 220 (55.4%) male, mean age 64 years (SD=15.1), 261 (66%) had transient and 134 (34%) permanent events. ECG was performed in 360 (91%) but only 236 (60%) had cardiac monitoring; 386 (91.1%) had carotid imaging. 8% (31) had AF and 8% (31) symptomatic carotid disease (CAS). Median ABCD2 in AF was 3, similar to non-AF patients. Only 51.6% with known AF were anticoagulated. Hypertension (p=0.012), previous TIA (p<0.001) and stroke (p=0.014) were more common in AF patients; diabetes (p=0.793), smoking (p=0.653) and hypercholesterolaemia (p=0.144) were not. 90 day recurrence of stroke/TIA/ocular ischaemia was 10.1%. This was higher in AF patients (16.1%, mean 12.3 days), comparable to CAS patients (18.9%). AF patients were more likely to represent with stroke (HR=5.74, 95%CI=0.54,61.6, p=0.239).Conclusion8% of patients with ischaemic ocular events had AF, same as CAS; this is probably an underestimate, as these patients are often under-investigated with only 60% undergoing cardiac monitoring.
IntroductionOcular events are considered lower risk than other transient ischaemic attacks (TIA). We aimed to determine recurrence risk, prevalence and management of vascular risk ...factors.SettingUniversity College Hospital London daily TIA-clinic, main referral centre for North-Central London. Consecutive records for all patients with transient/permanent ischaemic visual loss reviewed, January 2014-September 2016.ResultsOf 395 patients, mean age 64 years (SD=15.1), 261 (66%) had transient and 134 (34%) permanent events. 51.1% had hypertension, 34.4% hypercholesterolaemia, 14.7% diabetes, 10.9% ocular events, 10.1% ischaemic heart disease (IHD), 7.1% atrial fibrillation (AF), 6.3% TIA, 5.1% stroke, and 12.4% were smokers. Median risk factors was 1 (range 1–6), but 88 (22.3%) had ≥3. Permanent visual loss was more common in patients with previous IHD (p<0.001), TIA (p<0.001), ocular events (p<0.001), diabetes (p=0.032), hypertension (p<0.001) and smokers (p=0.077). 90 day recurrence was 10.1%, higher (14.8%) in patients with ≥3 risk factors (HR=1.67, 95%CI=0.89–3.09, p=0.120). Secondary prevention was better in patients with past TIA than those with ocular events; 60% vs 45% received antiplatelets and 92% vs 51% statins. Only 16 (51.6%) with known AF were anticoagulated, despite all having CHADSVASC≥1.ConclusionOne-fifth of patients with ocular events had ≥3 risk factors; these had higher recurrences. Only half of patients with previous ocular events were on antiplatelets/statins. These patients should be investigated and treated as aggressively as other TIA/strokes.
Background and aimStudies have demonstrated the role of magnesium on acute migraine attacks and prophylaxis via voltage-dependent blockade of N-methyl-D-aspartate (NMDA)-coupled channels. Our aim was ...to study the tolerability of intravenous (IV) magnesium in the treatment of migraine in the Emergency Department (ED) and the Same Day Emergency Care (SDEC) unit of University College Hospital, London.MethodsWe performed a non-randomised open-label trial to establish the safety of IV magnesium sulfate. Patients were recruited from the ED or the SDEC unit of the hospital after being reviewed by consultant Neurologists from Aug 2021 – Jan 2022. Magnesium levels were measured prior to administration of the drug and complications were closely monitored.Results35 patients with status migrainosus were recruited; 30 were females and their mean age was 39 years (SD: 13.5 in years). All had normal serum magnesium levels prior to drug administration. No ECG changes were identified. None developed serious adverse symptoms during or immediately after infusion. A beneficial effect was noted in 15 patients, no effect in 10, and missing data in 10.ConclusionAdministration of IV Magnesium is an effective and tolerated adjunct for the management of migraine. Efficacy studies are currently underway.
Background and aim5-10% of emergency department (ED) presentations are primarily neurological. We investigated the impact of the introduction of an acute neurology service to the ED, using the same ...day emergency care (SDEC) model.MethodsWe performed a retrospective review of consecutive referrals to a consultant-led service at University College London Hospital during weekday afternoons from 5th May 2021 to 20th Jan 2022.ResultsOf 664 Neurology referrals, female sex was more common than male (60% vs 35.8%, p<0.0001, Fig. 1).Most referrals were from ED Majors (30%, Fig. 2). The most common presenting complaints were headache(n=187), weakness(n=34), dizziness(n=28), and numbness(n=26). Referrers’ working diagnoses included no diagnosis (n=69), unspecified headaches(n=62), migraines(n=42), and stroke(n=23) (Fig. 3). The most common diagnoses after Neurology review were migraines(n=160), vascular events(n=21), functional(n=16), and seizures(n=12) (Fig 4).Following review, working diagnosis changed in 307(46.2%), and the following planned actions were cancelled: hospital admission in 204(30.7%); stroke referral in 190(28.6%); imaging in 45 and lumbar puncture in 33. 170(25%) cases were fully managed in SDEC which would otherwise have followed the urgent 2-week-wait pathway.ConclusionsAcute Neurology input in the ED had major impacts on working diagnoses, hospital admis- sions, urgent outpatient referrals, and emergency investigations.
IntroductionFunctional Neurological Disorder (FND) is common and can lead to multiple emergency department (ED) presentations and unnecessary investigation. Early identification and intervention have ...been shown to reduce health care costs and re-presentation to acute services by more than 50%1.We created a pilot model to provide early access to a multi-disciplinary FND team for patients assessed by the ED-embedded neurology team (Stroke or Neurology SDEC pathway) to have a new diagnosis of FND. The MDT provided patients with individualised information about FND, pathway navigation, and shown self-management tools.MethodsWe reviewed data for all patients seen to date by the pilot service.ResultsSince commencement in November 2021, nine patients have been seen by the MDT. All patients were seen within two weeks by a neurophysiotherapist and neuropsychiatrist with neurology support.6 were female and median age was 41 years. All presented with limb weakness and/or sensory distur- bance. 7 reported prior anxiety or depression. 6 had psychiatric factors, including Emotionally Unstable Personality Disorder and health anxiety. Follow up, 2 of the 9 patients so far demonstrated reduced health anxiety and increased confidence.ConclusionAn acute FND pathway can provide timely intervention and reduce symptoms for new presentations with FND.1. Stepped care for functional neurological disorder, A new approach to improving outcomes for a common neurological problem in Scotland. Report and Recommendations, NHS Improvement, February 2012
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.
Ten major risk factors account for 90% of the population attributable stroke risk. Smartphone applications may be used for education and self-managing risk factors. We aimed to explore use and ...attitudes towards smartphones in a TIA clinic.MethodsFrom September 2015 consecutive patients attending University College Hospitals, London TIA clinic completed a questionnaire, relating to, stroke risk factors and use of smartphones. A likert scale (strongly disagree=1, strongly agree=10) evaluated attitudes towards Smartphone applications in stroke education and management.Results118 patients completed the questionnaire. Mean age 62.7 (±15.8) years, 57 (48.3%) male. Twenty (17%) were ≥80 years. Internet access was available for 98 (83.1%). Smartphones were used by 78 (66.1%), including 7 (35.0%) ≥80 yrs. Applications used included internet access in 61 (78.2%), calendar 55 (70.5%), social media 39 (50.0%) and games 20 (25.6%) games. Only 16 (20.5%) used lifestyle applications. Few, 3 (3.8%), recorded medical information on their smartphone despite 25 (21.2%) presenting without knowing their medications.Patients reported that they were in favour of accessing stroke information on Smartphones, median response 8 (interquartile range (IQR) 4.75), and that they would use an application showing progress; e.g updates on weight, exercise and blood pressure, median 8 (IQR 4) and to record medications and results, median 8 (IQR 5).Patients attending hospital TIA services commonly have access to Smartphones and would support using Applications for stroke education and risk factor management. Further studies of actual usage of pilot prevention Smartphone applications are required.
