A new training seminar has been published to help neurologists recognise and manage epilepsy safely and effectively in people with neurodevelopmental disorders.

It flags up the risk of sudden unexpected death in epilepsy (SUDEP) in this population, which may be up to 20 times higher than in the general population.

The online seminar, Epilepsy in adults with neurodevelopmental disability: what every neurologist should know, was published in Epileptic Disorders, a journal of the International League Against Epilepsy.

It aims to consider the complexity of epilepsy with neurodevelopmental disorders (which include intellectual disability, autism spectrum disorder, and attention deficit hyperactivity disorder) and their collective impact, not as discrete conditions.

This requires reasonable adjustments to services and clinicians in order to provide person-centred equitable care.

Higher rates of physical and psychiatric comorbidity

The seminar was co-designed, co-produced and co-authored by an expert by experience and includes case studies and a video to highlight what can go wrong and how it can be avoided.

It highlights that people with neurodevelopmental disorders and epilepsy have higher rates of physical and psychiatric comorbidity, polypharmacy, neuropsychiatric side effects of drugs, and premature mortality including SUDEP.

Therefore assessment for people with neurodevelopmental disorders can be complex and lengthy, involving a wide range of professionals. Diagnostic investigations are useful, however, they require a high level of co-operation and tolerance. Often situations where investigations are most needed are the most challenging.

This complexity of assessment can often lead to misdiagnosis due to misattribution or misinterpretation of other physical health conditions, psychiatric/psychological presentations, and behavioural disturbances. If there is misdiagnosis, this will lead to risk of harm to the individual through untreated epilepsy, or conversely the risk of harm due to lengthy periods of over medication with anti-epileptic drugs that will be ineffective.

In regard to treatment, the seminar states that the evidence-base for seizure treatment in people with neurodevelopmental disorders remains limited and extrapolating results from large randomised controlled trials in the general population is of limited use and not directly applicable to people with neurodevelopmental disorders.

The evidence-base that exists suggests that treatments are less effective for this population when compared with the general population.

It concludes that knowledge of core neurodevelopmental disorders principles and access to wider allied healthcare professionals, to provide holistic multidisciplinary input, is essential to good outcomes in terms of seizure control and quality of life.

Epilepsy and LeDer review

Epilepsy deaths have been flagged as a significant concern in the Learning Disabilities Mortality Review (LeDer) that looks into deaths in people with learning disabilities. 

It was also the second most frequently reported potentially treatable cause of death.

Of the 348 epilepsy death reviews completed in 2019, 32 directly mentioned SUDEP. Common themes identified included observed changes in the person prior to death, mention or absence of assisted technologies, and problems with care related to the person's epilepsy.

A more recent analysis of the data demonstrates that between July 1 2016 and December 31 2019, one in five epilepsy deaths reviewed (as a 45% sample of the total) by the LeDeR programme were recorded as SUDEP.

The LeDer report made the recommendation that the SUDEP and Seizure Safety Checklist (for clinicians) and EpSMon app (for people with epilepsy) should be used as a safety measure for people with epilepsy. 

This advice was mirrored in the training seminar and the authors said that individuals, carers and families should be counselled on the risk of SUDEP at the earliest appropriate time in the diagnostic process.

It added that the NICE quality standards state that each person with epilepsy should have a comprehensive epilepsy care plan that is communicated between all healthcare professionals involved.

This care plan should include specific details regarding the epilepsy, management plans, and risk assessment. Risk assessment should be personalised and each risk should be considered individually. Where available, risk assessments should be person-centred and include standardised semi-structured, evidence-based tools such as the SUDEP and Seizure Safety Checklist.