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Investigation of Sudden Cardiac Death
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
It has become very clear that there is an excess of sudden unexpected death in subjects who have a clinical history of epilepsy.56 The phenomenon is best certified as sudden unexpected death in epilepsy (SUDEP). Implicit in this definition is that the term status epilepticus should be confined to cases of epileptic death where there is a clear history of a grand mal seizure and lacerations of the tongue. Also implicit is the exclusion of any heart disease such as myocarditis by histology. The reason subjects with epilepsy should die suddenly is not yet well defined. They are usually male with poor control of the epilepsy and polypharmacy. Is it a primary neurological abnormality affecting the heart or is there an underlying cardiac pathology such as channelopathies which needs clarification in the future? Genetic mutations linked to cerebral ion channelopathies causing epilepsy occur in up to 6% of SADS cases and whether these are cardiac or neurological deaths is not clear, but ion channel dysfunction can present in the brain and heart in the same patient.
Water-Related Deaths by Manner
Published in Kevin L. Erskine, Erica J. Armstrong, Water-Related Death Investigation, 2021
Autopsy findings in individuals who drown during or after a seizure may include those findings supportive of drowning, which in the right historical and scene context, provides the most probable cause of death—drowning due to seizures or drowning with a contributing condition of seizures. In cases in which there are little or no supportive findings of drowning, sudden unexpected death in epilepsy (SUDEP) may provide an explanation, especially when an anatomic or toxicological cause of death cannot be found. Particularly in bathtub scenes in which the decedent’s head is found well above any potential level of water, SUDEP is a reasonable consideration.
Vagal nerve stimulation: effects on seizures
Published in Hans O Lüders, Deep Brain Stimulation and Epilepsy, 2020
Large clinical trials indicate that vagal nerve stimulation reduces partial-onset seizures in adults by -30% in the first 3 months of therapy. With treatment for 2–3 years, partial-onset seizures are reduced by -40 to -45%. Other smaller series suggest similar results in children, and in patients with generalized epilepsy. While receiving VNS, one-third of patients can take fewer antiepileptic drugs. The SUDEP (sudden unexpected death in epilepsy) rate is reduced by two-thirds. The most frequent side effects are stimulus-related hoarseness, cough and throat pain, seen in 22–55% of cases; these side effects nearly completely disappear after 1-3 years of therapy.
Current and promising therapeutic options for Dravet syndrome
Published in Expert Opinion on Pharmacotherapy, 2022
Antonella Riva, Gianluca D’Onofrio, Elisabetta Amadori, Domenico Tripodi, Ganna Balagura, Valentina Iurilli, Maria Stella Vari, Alberto Verrotti, Pasquale Striano
The median seizure-onset in DS is around 5.7 months (range 1.5 to 20.6 months) [8]. The high seizure burden negatively impacts the development and is associated with the risk of Sudden Unexpected Death in Epilepsy (SUDEP), rated as 9.32/1000-person-year (98% CI 4.46–19.45) [9]. Initially, prolonged, generalized, or unilateral clonic seizures are typically triggered by fever. Between 1 and 4 years of age, other seizure types appear together with a slowing in the psychomotor development and the picture becomes characteristic of a steady state. Patients suffering from DS have multiple seizure types during the course of the disease: convulsive seizures consisting of either generalized tonic-clonic seizures, generalized clonic seizures, or alternating unilateral clonic seizures, myoclonic seizures, atypical absences and obtundation status, focal seizures (simple focal motor seizures and complex partial seizures, with or without secondary generalization), and exceptionally tonic seizures. Initial seizures are tonic-clonic and hemiclonic; often, patients with DS present with status epilepticus (SE) [8]. EEG findings are frequently generalized spike-wave (GSW), focal or multifocal epileptiform abnormalities and, more rarely, GSW with focal or multifocal epileptiform abnormalities [8]. Brain MRI is generally normal.
A review of the pharmacotherapeutic considerations for managing epilepsy in people with autism
Published in Expert Opinion on Pharmacotherapy, 2022
Lance V Watkins, Maire O’Dwyer, Rohit Shankar
The first standard of epilepsy treatment should be to reduce risk and help keep the individual safe (Figure 1). Risk should be assessed individually for each risk factor in the context of a person’s lifestyle and environment. Reducing seizure burden through treatment with anti-seizure medication will help reduce risk of injury, hospitalization, and sudden unexpected death in epilepsy (SUDEP) [75]. The goal of treatment should be seizure freedom. If this is not possible then reducing seizure frequency is the next best option, with a focus on generalized, bilateral, tonic-clonic motor seizures. Using tools such as the SUDEP and Seizure safety Checklist could help mitigate exacerbations of seizures across a lifetime [76,77]. Treatment should be targeted at accurately diagnosed seizure type and epilepsy syndrome in accordance with the ILAE guidelines. However, there must be a balance between seizure control and the impact of treatment on quality of life, such as adverse effects of anti-seizure medications. Prescribing new anti-seizure medications or changing between anti-seizure medications should be approached cautiously. It is advised to make one change at a time so that it is easier to attribute the effect of change. Dosing should be started at low sub-therapeutic doses and titration should be slow to maximize the likelihood of achieving the optimal therapeutic window [73].
Epilepsy and Seizure-Related Hospital Admissions to an Australian Neurology Unit: A Prospective Observational Study
Published in Hospital Topics, 2020
Anthony Khoo, Joseph Frasca, Emma Whitham
Drug-resistant epilepsy is defined as failure to achieve sustained seizure freedom despite adequate trials of two tolerated and appropriately used antiepileptic drugs (whether as monotherapy or in combination) (Kwan et al. 2010). This was the case in just over 50% of our hospital admissions involving patients with a known seizure disorder. While it is possible that factors such as intercurrent infection or poor medication adherence provoked seizures even in this cohort, this result suggests further measures need to be implemented to prevent adverse outcomes including sudden unexpected death in epilepsy (SUDEP), which are strongly correlated with rates of seizure freedom (Bell et al. 2010). Long-term outcome data for epilepsy surgery in selected patients with refractory focal epilepsy indicate up to 47% are seizure free at 10 years (de Tisi et al. 2011), and further data regarding referral rates for pre-surgical investigation and assessment for suitability is required.