Bone health, intellectual disability and epilepsy: An observational community-based study.

OBJECTIVES: Intellectual disability (ID) and epilepsy are independent risk factors for osteoporosis. Diverse predisposing factors influence this, for example in ID, genetics and poor nutrition and in epilepsy, anti-seizure medication (ASM). Around 25% people with ID have epilepsy, majority treatment resistant. ASMs polypharmacy is common. However, little is known about the bone-related characteristics of this vulnerable group. A prospective observational cohort study of bone profile across a community ID Epilepsy service was undertaken to understand this. MATERIALS & METHODS: Participants were on minimum 2 years of ASMs. Baseline demographics, epilepsy data, bone metabolism biomarkers, bone mineral density (BMD) and vitamin D levels were collected. Doses needed to correct vitamin D insufficiency/deficiency were calculated. RESULTS: At baseline, of 104 participants, 92 (90.2%) were vitamin D insufficient/deficient. Seventy-six (73.1%) had a DEXA scan, 50 of whom-in the osteopaenic/osteoporotic range. DEXA scores between ambulant and non-ambulant patients were significantly different (p = .05) but not for ID severity. A high alkaline phosphatase (ALP) predicted lower vitamin D levels. Borderline significance (p = .06) in calcium levels between normal and high ALP was identified. There were no significant associations between parathyroid hormone, inorganic phosphate and magnesium levels, with vitamin D status or DEXA hip T-scores. Normalizing vitamin D levels (mean 101.4 nmol/L) required an average of 1951IU cholecalciferol daily. CONCLUSIONS: Vitamin D deficiency is highly prevalent in people with ID and epilepsy treated with ASMs impacting likely on their bone health. Screening with vitamin D levels, ALP and DEXA in this group should be pro-actively and routinely considered.

UK framework for basic epilepsy training and oromucosal midazolam administration.

BACKGROUND: UK wide Oromucosal Midazolam is used as an emergency treatment in community for seizures administered by family/carers with the right training. The Joint Epilepsy Council (JEC) UK which produced the training guidelines disbanded in 2016. PURPOSE: Provide standards for basic epilepsy education and rescue medication (Midazolam) administration. METHODS: The Epilepsy Nurses Association (ESNA), The International League against Epilepsy, British Chapter (ILAE) and the Royal college of Psychiatrists (RCPsych), used the Delphi process to update guidelines for the administration of oromucosal midazolam including developing a voluntary on-line test for carers. During 2017-2019 a facilitator worked with two ESNA committees to update the existing guidance and another to develop a question-bank. Both committee outputs were circulated to the ESNA membership, then ILAE and RCPsych for review. Patient-facing organizations and charities' opinions were solicited. All feedback was assimilated. A private provider was contracted to deliver the test. RESULTS: A consensus process involving two task and finish groups of 19 people each compared, reflected, debated, and engaged with stakeholders across three stages. The updated ratified guidelines were circulated nationally. The Delphi process highlighted many regions and individuals had local assessment tools and procedures in place, while others (around 50%) had no assessment provision. 278 carers with a 95% pass-rate and 100% positive feedback have undertaken the online test (10/2020). CONCLUSION: The UK-wide care provision gap in basic epilepsy-training and safe rescue medication administration is now addressed. A two-yearly update to the guidelines and test is planned.

An evaluation of the effectiveness of perampanel in people with epilepsy who have previously undergone resective surgery and/or implantation of a vagal nerve stimulator.

About 30% of people with epilepsy (PWE) are drug-resistant. Those with focal seizures may be suitable for epilepsy surgery. Those not amenable to resective surgery can be considered for vagus nerve stimulation (VNS). However, after operative procedures, around 50% of patients continue to experience seizures. A multi-center retrospective study assessing perampanel effectiveness and tolerability for PWE who have undergone surgical resection and/or VNS implantation was performed. The primary outcome was ≥50% reduction in seizure frequency while secondary outcomes included side effects (SEs), dose-related effectiveness, and toxicity. The median perampanel dose was 6 mg. Only one PWE became seizure free. A ≥50% decrease in seizure frequency was observed in 52.8% of the post-resection group and 16.9% of the VNS group (p < 0.001), while SEs were seen in 44.8% and 41.1%, respectively. Perampanel doses greater than 8 mg led to better response in both groups, especially in the post-VNS cohort. SEs were not dose-related and the safety profile was similar to previous observational studies. Perampanel can be beneficial in these two super-refractory epilepsy groups, particularly in PWE with seizures after surgical resection. Doses of more than 8 mg appear to be well tolerated and may be more effective than lower doses in PWE after surgical interventions.

Going down the tubes! Impact on seizure control of antiepileptic medication given via percutaneous feeding tubes.

BACKGROUND: Long-term feeding by percutaneous endoscopic gastrostomy tube (PEG) is uncommon but can present significant issues when both nutrition and medication have to be fed down the same tube. This is especially important in people with epilepsy where the dose and bioavailability of antiepileptic drugs are critical to maintain adequate seizure control. This group with long-term PEG tubes is particularly vulnerable and dependent on their carers to provide high standard of care. Indeed, approximately half of the people with long-term feeding tubes suffer from severe intellectual disability which requires careful coordination of all members of the multidisciplinary team. It is all the more important when consideration is given that around 50% of people with severe ID have seizures, mostly treatment resistant. METHOD: A detailed literature review was conducted with the focus on the numerous factors that can affect medication delivery and absorption with the potential to destabilize seizure control in people with PEG. Issues concerning the site of drug absorption, excipients and dilution, interaction between antiepileptic drugs and the nutritional feed, bioavailability, and problems with the PEG tube are considered. RESULTS: There is limited research in this area and the studies are often based on small numbers, healthy volunteers or in vitro findings. CONCLUSION: In the absence of sound research data, seizure control in each patient with a PEG needs to be considered on an individual basis. Establishing a baseline by measuring the serum levels prior to the tube insertion may be helpful, followed by checking the levels after PEG insertion.

Epilepsy awareness and emergency rescue training: Ignorance is bliss!

Status epilepticus (SE) has a high mortality rate and is associated with complications such as neurological deficit and cognitive decline. Buccal midazolam is the recommended emergency rescue medication in the UK to reduce the duration of a seizure and SE. It should be administered by an appropriately trained person. There are agreed guidelines on training standards for its administration in the UK produced by the Joint Epilepsy Council of the United Kingdom and Ireland. Training should provide an overview of epilepsy to facilitate safe, person-centered care and appropriate administration of rescue medication to people with epilepsy (PWE). Unfortunately the current guidelines do not assure satisfactory practice. An investigation was conducted to quantify the nature and degree of the problem in Cornwall, UK (population 550,000). To address the identified inconsistencies, a web-based test was developed using a focus group of experts and stakeholders. Over 800 carers for PWE took the test at different intervals of its development. A consistent 20% failure rate was noted. Over 90% of participants felt it kept PWE safer. The test was incorporated into routine clinical practice and has contributed to reduction of primary epilepsy deaths. The e-test is a cost-effective solution to help harmonize practices across different settings and can be easily adopted by other countries.

Sudden unexpected death in epilepsy: measures to reduce risk.

This review looks at the strategies that may help to reduce the risk of sudden unexpected death in epilepsy beyond that of trying to achieve seizure cessation, which is not possible for up to 30% of patients with epilepsy. These strategies include seizure safety checklists, mobile phone technology, telehealth and various devices currently available or in development. We highlight interventions where there is evidence of benefit, and draw attention for the need both to involve patients with epilepsy in risk reduction and to improve communication with those at risk.

Improving nocturnal event monitoring in people with intellectual disability in community using an artificial intelligence camera.

There is higher prevalence of epilepsy and SUDEP in people with intellectual disability (PwID) compared to general population. Accurate seizure recording particularly at night can be challenging in PwID. Neuro Event Labs seizure monitoring (Nelli) uses high-quality video based artificial intelligence to detect and record possible nocturnal seizures. This study looks to evaluate the clinical utility and acceptability of Nelli in PwID and epilepsy. Family/carers of PwID and drug resistant epilepsy with suspicions of nocturnal seizures who had not tolerated routine or ambulatory EEGs were invited to evaluate Nelli. Relevant demographics and clinical characteristics were collected. Nelli's impact, it's facilitators, barriers and feedback quality was captured from patient and professional stakeholders. Quantitative and thematic analysis was undertaken. Fifteen PwID and epilepsy and four health professionals were involved. Nelli recorded 707 possible seizure events across the study cohort of which 247 were not heard or recognised by carers. Carers recorded 165 episodes of 'restless' or "seizure behaviour" which the Nelli did not deem to be seizures. There was 93% acceptability. Thematic analysis revealed three broad themes of device acceptability, result implementation and possible seizure recognition ability. Nelli allowed for improved communication and care planning in a hitherto difficult to investigate population.

Head first - Review of epilepsy head injury risk and protection.

INTRODUCTION: The causes of falls and resultant head injuries in people with epilepsy are complex and represent important clinical issues significantly impacting on safety, quality of life and long-term prognosis. A general solution for many years has been to provide helmets for head protection. There is poor understanding of the influencing risk factors and solutions to give person-centred clinical advice. METHODS: A focused narrative review was conducted to investigate the risk factors for seizure-related head injuries and evidence of current helmets to offer protection. Four databases were searched (Embase, Medline, PsycInfo and Cochrane Library) using combinations of text words and thesaurus terms for the retrieval of articles relating to seizure-related head injury. Articles for full analysis were selected by a ten item pre-defined inclusion criteria. RESULTS: Of 104 relevant studies longlisted 21 studies met four or more of the predefined criteria and included in this review. A further nine papers were included because they added additional relevant information. CONCLUSIONS: Seizure-related head injury, although rare, may have serious consequences. High doses of antiepileptic/antiseizure drugs (AEDs/ASDs) and a prescription for three or more AEDs/ASDs are significant, modifiable, risk factors for head injury. Differentiating confusion due to post-ictal changes or concussion can be challenging. Seizure-related cardiac causes leading to injury due to ictal bradycardia or asystole is under investigated. More research is required to validate the protective benefit of various helmet designs in seizure-related head injury. It is striking that there are no International Standards for protective helmets in seizures.

Safe and sound? A systematic literature review of seizure detection methods for personal use.

PURPOSE: The study aims to review systematically the quality of evidence supporting seizure detection devices. The unpredictable nature of seizures is distressing and disabling for sufferers and carers. If a seizure can be reliably detected then the patient or carer could be alerted. It could help prevent injury and death. METHODS: A literature search was completed. Forty three of 120 studies found using relevant search terms were suitable for systematic review which was done applying pre-agreed criteria using PRISMA guidelines. The papers identified and reviewed were those that could have potential for everyday use of patients in a domestic setting. Studies involving long term use of scalp electrodes to record EEG were excluded on the grounds of unacceptable restriction of daily activities. RESULTS: Most of the devices focused on changes in movement and/or physiological signs and were dependent on an algorithm to determine cut off points. No device was able to detect all seizures and there was an issue with both false positives and missed seizures. Many of the studies involved relatively small numbers of cases or report on only a few seizures. Reports of seizure alert dogs are also considered. CONCLUSION: Seizure detection devices are at a relatively early stage of development and as yet there are no large scale studies or studies that compare the effectiveness of one device against others. The issue of false positive detection rates is important as they are disruptive for both the patient and the carer. Nevertheless, the development of seizure detection devices offers great potential in the management of epilepsy.

Developing an Evidence-Based Epilepsy Risk Assessment eHealth Solution: From Concept to Market.

INTRODUCTION: Sudden unexpected death in epilepsy (SUDEP) is possibly the most common cause of death as a result of complications from epilepsy. The need to educate and regularly review risk for all patients with epilepsy is paramount, but rarely delivered in actual clinical practice. Evidence suggests that education around SUDEP and modifiable risk variables translate into better self-management of epilepsy. OBJECTIVE: We aimed to develop and implement an eHealth solution to support education and self-management of risks, in epilepsy. METHODS: We undertook an innovation pathways approach, including problem identification, feasibility assessment, design, implementation, and marketing. People with epilepsy were provided a smartphone-based app (Epilepsy Self-Monitor, EpSMon), which translates the clinical risk assessment tool into an educational and self-monitoring platform, for the self-management of epilepsy. RESULTS: Results include the success of the marketing campaign, and in what areas, with an estimated reach of approximately 38 million people. EpSMon has proved a success in academic and clinical circles, attracting awards and nominations for awards. The number of users of EpSMon, after 3 months, turned out to be lower than expected (N=221). A 4-month trial of the app in use in the United Kingdom, and the success of the marketing strategy, point to necessary changes to the model of delivery and marketing, summarized in this paper. These include the marketing message, user cost model, and need for the availability of an Android version. CONCLUSIONS: EpSMon has proven a success in respect to its reception by academics, clinicians, stakeholder groups, and the patients who use it. There is work needed to promote the model and increase its acceptability/attractiveness, including broadening the marketing message, increasing its availability, and reducing its cost. Future development and promotion of the tool will hopefully inform iterative design of its core features for a receptive audience and lead to increased uptake as it is launched worldwide in 2016.

Epilepsy emergency rescue training.

The NICE audit of epilepsy related deaths revealed that 1200 epilepsy deaths occur every year in the UK, with 42% potentially avoidable.[1] Convulsive status epilepticus (SE) is a life-threatening condition with over 20% mortality rate, especially if early treatment is not initiated.[2] Ten percent of all UK emergency department (ED) admissions are due to epilepsy, usually over represented by cases of SE.[3] Six out of seven epilepsy cases seen in the ED are admitted into medical care.[4] Patients with chronic and/or treatment resistant epilepsy carry a higher risk of premature death. When a seizure lasts for five minutes or more then the patient is at high risk of continuing to SE and this may result in causing brain damage or death.[2] Buccal midazolam is an emergency rescue medication prescribed on a special named patient license to reduce the duration of an epileptic seizure and prevent SE.[2,5] It should be administered by a trained person and is widely used due to its effectiveness and social acceptability. In the UK, epilepsy education and training courses are expected to be conducted by epilepsy professionals in line with the agreed training guidelines of Joint Epilepsy Council (JEC) backed up by evidence from NICE.[6,7] Training should provide an overview of epilepsy to facilitate safe care and appropriate administration of rescue medication for people with epilepsy (PWE) when experiencing a prolonged seizure. The medication is prescribed on specialist advice by the GP or specialists directly. Unfortunately the JEC guidelines are not robust enough to provide assurances of safe care. This problem had a myriad of complexities and an appropriate solution using web based resource was piloted, tested, and applied successfully using quality improvement methodology.

Keeping patients with epilepsy safe: a surmountable challenge?

This quality improvement project was inspired as an answer to a problem that intellectual disability teams have been struggling to manage whilst caring for people with epilepsy (PWE). The issue was that despite guidance to discuss the possibility of sudden unexpected death in epilepsy (SUDEP) be discussed with a newly diagnosed PWE this is rarely done. Additionally when, how, and what to discuss about SUDEP and reduce its risk is arbitrary, non-person centred, and with no structured evidence. Prior to initiating changes a discussion of SUDEP was recorded in just 10% of PWE. We introduced a check-list to help identify risk factors for SUDEP. We then modified the check-list, and then used it via telehealth, a way of contacting patients and their carers over the phone using the check-list approach. Following interventions, discussions of SUDEP are now recorded in 80% of PWE. Feedback from patients, carers and primary and secondary care professionals has been positive. We are now developing an app so that patients and carers can monitor their own risk factors, thus empowering them and increasing their knowledge and awareness of SUDEP.

A community study in Cornwall UK of sudden unexpected death in epilepsy (SUDEP) in a 9-year population sample.

PURPOSE: Epilepsy-related death, particularly sudden unexpected death in epilepsy (SUDEP), is underestimated by healthcare professionals. One argument that physicians use to justify the failure to discuss SUDEP with patients and their families is that there is a lack of evidence for any protective interventions. However, there is growing evidence of potentially modifiable risk factors for SUDEP; although large-scale trials of interventions are still lacking. We determined the main risk factors associated with SUDEP in a comprehensive community sample of epilepsy deaths in Cornwall UK from 2004 to 2012. METHODS: We systemically inspected 93 cases of all epilepsy and epilepsy associated deaths which occurred in Cornwall between 2004 and 2012 made available to us by the HM Cornwall coroner. These are the deaths where epilepsy was a primary or a secondary cause. RESULTS: 48 cases met the criteria for SUDEP and we elicited associated relevant risk factors. Many findings from our study are comparable to what has been reported previously. New points such as most of the population had increase in either or both seizure frequency/intensity within six months of death and majority did not have an epilepsy specialist review in the last one year to demise were noted. CONCLUSION: This study is the first epidemiological study in England occurring in a whole population identifying systemically all deaths and the first large scale review in UK of SUDEP deaths since 2005. Being a community based study a key issue which was highlighted was that in the SUDEPs examined many might have been potentially preventable.