Rohit Shankar

Sudden unexpected death in epilepsy (SUDEP): Development of a safety checklist

PURPOSE The incidence of sudden death appears to be 20 times higher in patients with epilepsy compared with the general population. Epilepsy-related death, particularly sudden unexpected death in epilepsy (SUDEP), is still underestimated by healthcare professionals and this may reflect the mistaken belief that epilepsy is a benign condition. The risk of death associated with epilepsy appeared rarely to have been discussed with patients or their families. It appears the decision to discuss SUDEP and also to peg SUDEP risk is arbitrary and clinical. Unfortunately there is no structured evidenced mechanism at present to represent person centered risk of SUDEP and there is currently no easy manner or template to have this discussion with the family and the patient. METHODS We conducted a detailed literature review in Medline, Embase and Psychinfo databases to extract the common risk factors as evidenced from literature till date. Research into risk factors has identified a number of risk factors for SUDEP, some of which are potentially modifiable. RESULTS Based on the literature review, we believe that the ascertained risk factors could be employed in clinical practice as a checklist to reduce an individual patients risk of SUDEP. The SUDEP safety checklist may be of practical use in reducing risks in some individuals and is definitely of use in helping communication. CONCLUSIONS An evidence based checklist identifying the major risk factors can help both clinicians and patients to focus on minimizing certain risk factors and promote safety by focusing on the modifiable factors and guide treatment. It can be a tool to open a person centered discussion with patients and to outline how individual behaviors could impact on risk.

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.

Attitudes Toward Psychiatry Among Final-Year Medical Students in Kumasi, Ghana

ObjectiveMost sub-Saharan African countries have fewer psychiatrists than one per one million people. One possible reason could be that medical students have a negative attitude toward the specialty. The authors evaluated the attitudes toward a career in psychiatry of final-year medical students in Kumasi, Ghana, and compare these with attitudes of medical students in Spain and the United States.MethodsMedical students were given a 28-item questionnaire on attitudes toward psychiatry, which was used in previous studies in Spain and the United States.ResultsGhanaian students (N = 94) had a fairly positive view of psychiatry, similar to those in Spain, although less positive than U.S. students. About 15% were considering psychiatry as a career option. There was evidence of significant stigmatization of patients with mental illness and psychiatrists and concern about the use of coercive detention of patients.ConclusionThe difficulty recruiting physicians into psychiatry in Ghana, and perhaps other African countries, is unlikely to be due to negative attitudes and may be due to a lack of opportunity to train in psychiatry.

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.

Does intellectual disability increase sudden unexpected death in epilepsy (SUDEP) risk

PURPOSE An estimated 1.4 million people in the United Kingdom (UK) have intellectual disability (ID) with 210,000 having severe or profound ID. Of these, approximately 125,000 have epilepsy, representing one quarter of all patients with epilepsy in the UK. For those with full scale intellectual quotients (FSIQs) of less than 50, half have epilepsy, with half of these having treatment resistant epilepsy. One of the two major causes of mortality within this population is sudden unexpected death in epilepsy (SUDEP). METHODS We performed a literature review exploring the extent to which ID was considered as a risk factor for SUDEP. We also considered whether there was any relationship between the types of health care system in which the studies were conducted and whether ID was considered in studies of SUDEP. RESULTS We identified 49 studies which had explored risk factors for SUDEP, of which, approximately 50% (n=23) considered ID in the planning stages. Of these studies 60% (n=14) found ID was a risk factor for SUDEP. 60% of all the studies were conducted in countries where the health care system was publicly funded. CONCLUSIONS Overall we found ID definitions and specified standardized mortality rates and impact of institutionalization to be quite poorly presented.

Chromosomal microarray testing in adults with intellectual disability presenting with comorbid psychiatric disorders

Chromosomal copy-number variations (CNVs) are a class of genetic variants highly implicated in the aetiology of neurodevelopmental disorders, including intellectual disabilities (ID), schizophrenia and autism spectrum disorders (ASD). Yet the majority of adults with idiopathic ID presenting to psychiatric services have not been tested for CNVs. We undertook genome-wide chromosomal microarray analysis (CMA) of 202 adults with idiopathic ID recruited from community and in-patient ID psychiatry services across England. CNV pathogenicity was assessed using standard clinical diagnostic methods and participants underwent comprehensive medical and psychiatric phenotyping. We found an 11% yield of likely pathogenic CNVs (22/202). CNVs at recurrent loci, including the 15q11-q13 and 16p11.2-p13.11 regions were most frequently observed. We observed an increased frequency of 16p11.2 duplications compared with those reported in single-disorder cohorts. CNVs were also identified in genes known to effect neurodevelopment, namely NRXN1 and GRIN2B. Furthermore deletions at 2q13, 12q21.2-21.31 and 19q13.32, and duplications at 4p16.3, 13q32.3-33.3 and Xq24-25 were observed. Routine CMA in ID psychiatry could uncover ~11% new genetic diagnoses with potential implications for patient management. We advocate greater consideration of CMA in the assessment of adults with idiopathic ID presenting to psychiatry services.

Can technology help reduce risk of harm in patients with epilepsy

Sudden unexpected death in epilepsy (SUDEP) is possibly the most common cause of death as a result of complications from epilepsy, accounting for between 7.5% to 17% of all epilepsy-related deaths1 and 50% of all deaths in refractory epilepsy.2 The UK has 600 000 people with epilepsy (PWE), 30% being treatment resistant. Sudden death is 20-fold higher in PWE than the general population. Epilepsy is the fifth highest cause of life-years lost in men and eighth in women in the UK. The public health burden of SUDEP alone is estimated as second only to stroke among neurological conditions.3 Forty-two per cent of all deaths are considered avoidable.4 Consequently, the National Institute for Health and Care Excellence (NICE) epilepsy guidelines in 2004 and 20125 recommend discussion of SUDEP with newly-diagnosed PWE. This is rarely delivered and until recently only 4% of PWE had a recorded SUDEP discussion.6 In their current publication of the NHS Outcomes Framework, the government prioritises the prevention of amenable mortality, making it a core focus for NHS services. Epilepsy mortality features in new NICE Standards as well as NICE Clinical Guidelines.5 However, the dilemma remains of when, where, how, and what to discuss about epilepsy risk, especially SUDEP. Further to the discussion there is a lack of a structured monitoring of risk especially in primary care. Meaningful management of SUDEP risk in particular, and epilepsy risk in general, is arbitrary, non-person centred and with no evidenced mechanism. In clinical practice, especially in primary care, the lack of any tools to support risk management is of concern. Risk management has been highlighted as vitally important to reducing avoidable epilepsy-related deaths, both in research and reporting but also via Prevention of Future Death reports …

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.

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.

Managing anti‐epileptic drug treatment in adult patients with intellectual disability: a serious conundrum

About a quarter of people with epilepsy have intellectual disability (ID). This group has communication issues, premature mortality, more treatment resistance, difficulties in making informed choices and greater risks of physical and mental health comorbidities. There is no specific prescribing guidance for this large and vulnerable group. The literature on prescribing for epilepsy in this group was reviewed, in particular examining how antiepileptic drugs (AEDs) work regarding their side effect profiles, effects on specific epilepsy syndromes associated with ID and their individual strengths and weaknesses based on the nature and degree of ID.