Epilepsy is a common condition in the population of people with intellectual disability and for many proves intractable. Over the last 20 years a host of new antiseizure medications (antiepileptics) have been introduced to the market, and the evidence base to support their use in people with intellectual disability is reviewed. This will aim to cover new treatment approaches such as cannabinoid preparations succinctly.

In 2012 the Enquiry moved to the National Perinatal Epidemiology Unit (NPEU) as part of MBRRACE-UK. CEMD Reports became annual and included near-misses. Life-threatening complications are 100 times commoner than death in pregnancy. In 2005 the NPEU had established a surveillance system through which all obstetric units notify uncommon complications. This facilitated a Confidential Enquiry into Maternal Morbidity, which identified an increase in severe haemorrhage due to abnormal placentation, and inadequate treatment of breast cancer due to withholding drugs in pregnancy. Positive trends included better long-term results of treating psychosis. The Enquiries’ work was hampered by NHS bureaucracy, the need for recommendations to be cost-neutral and a media embargo – even though the CEMD had shown that public information is vital for improving safety. Recently the CEMD revealed an increase in deaths from sudden death in epilepsy (SUDEP) due to withdrawal of medication in pregnancy, and continuing disparity in mortality rates between ethnic groups in the UK. Structural biases in the maternity care system persist, as shown by Black Lives Matter movement and the COVID emergency.

A seizure is defined as abnormally coordinated electrical activity that is clinically noted as abnormal sensations, movements, or behaviors. Epilepsy is defined as the tendency to have seizures, practically as two unprovoked seizures at least 24 hours apart or one seizure with a 60% chance of additional seizures. The two overall epilepsy types are generalized and focal. Focal epilepsy most commonly indicates a focal brain injury like traumatic brain injury or tumor. In contrast, generalized epilepsy is due to a genetic etiology, not necessarily inherited. An epilepsy diagnosis is clinically made and can be supported by typical EEG and MRI findings. Nearly 4% of people develop epilepsy, with the largest peak during later years and the second peak during childhood. Nearly 70% of people are seizure-free after five years, whether via control with medications or, less commonly, the epilepsy itself remits. Life expectancy for epilepsy patients is lower. A key contributor to epilepsy mortality is sudden unexpected death in epilepsy (SUDEP). Uncontrolled generalized tonic clonic seizures are a key risk factor for SUDEP.

The insula is known to play a central role in the regulation of autonomic function, although the precise nature and localization of such functions have not been conclusively identified. Autonomic and particularly cardiac disturbances are strongly implicated in sudden unexpected death in epilepsy (SUDEP). Herein, we discuss the contribution of the insula to seizure-related cardiac dysfunction and SUDEP. We also present two illustrative cases of intractable epilepsy patients with structural damage to the insula who succumbed to SUDEP. Both had progressive changes in heart rate variability (HRV), a standard measure of cardiac autonomic function, which noninvasively reflects sympathetic and parasympathetic balance using EKG recordings. In addition, further evidence of insular resection leading to autonomic changes is suggested in patients with intractable epilepsy and radiological evidence of insular damage after epilepsy surgery. The presence of intrinsic insular lesions or acquired insular damage in patients with refractory epilepsy may predispose to cardiac dysfunction and contribute to SUDEP pathomechanisms; however, a definitive association to SUDEP is still undetermined.