Sudden Unexpected Death in Epilepsy (SUDEP) is a significant cause of death for people with chronic epilepsy. Good practice guidance in the UK and the USA expect SUDEP to be discussed with the individual. The event rarity, methodological variance and lack of robust research into the pathological mechanisms, associated risk factors, and management strategies have created a challenge on how and what to discuss. There are some significant associations which allows for risk assessment and mitigation. Areas covered: The current understanding of static and modifiable risk factors for SUDEP and how to manage these more effectively are reviewed. Longitudinal risk may be assessed using standardised risk assessment tools which help in communicating risk. Technological advancement allows measurement of physiological parameters associated with seizures and risk of SUDEP using small wearable devices. Further evidence is needed to demonstrate such technologies are efficacious and safe. Expert commentary: Risk reduction should be an important part of epilepsy management and we suggest a Gold Standard of Care which healthcare professionals and services should aim for when approaching SUDEP risk management. A Minimum Standard of Care is also proposed that is practical to implement, that all people with epilepsy should expect to receive.

A quarter of people with Intellectual Disability (ID) in the UK have epilepsy compared to 0.6% in the general population and die much younger. Epilepsy is associated with two-fifths of all deaths with related polypharmacy and multi-morbidity. Epilepsy research on this population has been poor. This study describes real-world clinical and risk characteristics of a large cohort across England and Wales. Methods:A retrospective multi-centre cohort study was conducted. Information on seizure characteristics, ID severity, relevant co-morbidities, psychotropic and antiseizure drugs (ASDs), SUDEP and other risk factors was collected across a year. Results:Of 904 adults across 10 centres (male:female,1•5:1), 320 (35%) had mild ID and 584 (65%) moderate-profound (M/P) ID. The mean age was 39.9 years (SD 15.0). Seizures were more frequent in M/P ID (p <0•001). Over 50% had physical health co-morbidities, more in mild ID (p<0•01). A third had psychiatric co-morbidity and a fifth had an underlying genetic disorder. Autism Spectrum Disorder was seen in over a third (37%). Participants were on median two ASDs and overall, five medications. Over quarter were on anti-psychotics. Over 90% had an epilepsy review in the past year but 25% did not have an epilepsy care plan, particularly those with mild ID (p<0•001). Only 61% had a documented discussion of SUDEP, again less likely with mild ID or their care stakeholders (p<0•001). Conclusions:Significant levels of multi-morbidity, polypharmacy and a lack of systemised approach to treatment and risk exist. Addressing these concerns is essential to reduce premature mortality.

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There is now a clearer understanding of the possible pathological mechanisms that contribute to SUDEP. SUDEP is the culmination of multifactorial predisposing and precipitating factors and has been linked to particular candidate genes. A number of static and modifiable risk factors for SUDEP have been consistently identified. Recent guidance has emphasised the importance of communicating SUDEP risk to individuals at the earliest appropriate time. SUDEP risk assessment should be integral to the care of individuals with epilepsy. The use of evidence-based risk assessment tools may provide an opportunity to communicate identified risks in a person-centred holistic way. There is increasing evidence to support the use of wearable seizure monitoring devices to help reduce the frequency and impact of convulsive seizures, perhaps the number one risk factor for SUDEP.

Recent publication of the Medicines and Healthcare products Regulatory Agency (MHRA) in the United Kingdom has strengthened the regulatory measures for valproate medicines. It highlights the importance of making women of childbearing age with epilepsy aware of the teratogenic risks of valproate and encourages the withdrawal of it from those currently prescribed. While a significant directive, it raises concerns of not having considered the impact on special populations such as women with Intellectual Disability (ID). While it is important that women with ID are not excluded from such safety initiatives, due caution needs to be taken on a case by case basis preferably, to ensure their best interests are central to the decision making. Many women with moderate to profound ID cannot have informed consented sexual relationships not to mention cognitive incapability to make informed choices on medication suitability. These women are at potential risk of having their epilepsy control undermined due to the MHRA directives. Around 30% of people with moderate to profound ID have seizures of which 60% are considered treatment resistant. In this vulnerable population changes to medication without clear clinical and social insights could lead to increased harm levels. This paper enumerates the challenges of application of the new directive to these special populations and proposes a pathway based on individual cognitive ability to provide informed consent to facilitate the continuation or removal of valproate. It is important not to lose sight of individual circumstances and the importance of working collaboratively toward providing person center care.