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Sudden unexpected death in epilepsy
Published in Helen Whitwell, Christopher Milroy, Daniel du Plessis, Forensic Neuropathology, 2021
Christopher Milroy, Daniel du Plessis
Epilepsy has been defined as a disorder of the brain characterised by an enduring predisposition to generate epileptic seizures and by neurobiological, cognitive, psychological and social consequences of this condition. The International League Against Epilepsy (IALE) produced a practical clinical definition of epilepsy in 2014 (Fisher et al. 2014). This states that epilepsy is a disease of the brain defined by the following conditions:At least two unprovoked (or reflex) seizures occurring more than 24 hours apart.One unprovoked seizure (or reflex seizure) and a probability of further seizures similar to the general recurrence risk (at least 60 per cent) after two unprovoked seizures, occurring over the next 10 years.Diagnosis of an epileptic syndrome.
Epilepsy
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Donald C. Barr, Andres M. Kanner
A diagnosis of epilepsy can be considered under the following conditions:2At least two unprovoked (or reflex) seizures occurring >24 hours apart.One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years. The presence of a prior brain insult such as a stroke or trauma, an electroencephalography (EEG) with epileptiform abnormalities, a significant brain-imaging abnormality, or a nocturnal seizure are the variables suggestive of an increased risk of seizure recurrence following an initial unprovoked seizure.Diagnosis of an epilepsy syndrome.
Factors That Can Exacerbate Seizures
Published in Stanley R. Resor, Henn Kutt, The Medical Treatment of Epilepsy, 2020
David E. Burdette, Robert G. Feldman
A subset of seizure disorders exists in which a seizure is the direct sequela of a specific and often well-defined stimulus. These sensory induced or reflex seizures are believed to occur in at least 5 to 6% of epileptics (35). They may result from any number of inciting events, most of which may be identified by the sensory type, i.e., visual, auditory, somesthetic, gustatory, or olfactory. Some of the reflex epilepsies appear to be triggered by more complex, associated thought processes, but for simplicity’s sake, they will be considered under the sensory stimulus that is primarily involved by that process.
Therapeutic approach to neurological manifestations of Angelman syndrome
Published in Expert Review of Clinical Pharmacology, 2022
Michele Ascoli, Maurizio Elia, Sara Gasparini, Paolo Bonanni, Giovanni Mastroianni, Vittoria Cianci, Sabrina Neri, Angelo Pascarella, Domenico Santangelo, Umberto Aguglia, Edoardo Ferlazzo
Epilepsy occurs in about 80% of AS patients [5]. Seizure onset is most prevalent between 1 and 3 years of age [5,7,8,10]. AS due to gene deletion is associated with the most severe epilepsy phenotype, followed by the isolated UBE3A mutation. Instead, the uniparental disomy subtype is associated with the least severe epilepsy phenotype [11,12]. Non-deletion patients, in general, seem to have a better prognosis [11,12]. Multiple seizure types are usually present simultaneously; seizures are polymorphic and manifest as atypical absences, myoclonic, bilateral tonic-clonic, focal motor, or atonic seizures [5,7,8,11]. Reflex seizures have also been described [13,14]. The first epileptic manifestation may be precipitated by fever [15]. More than 95% of AS subjects with epilepsy have daily seizures, at least during childhood. Generally, during adolescence and adulthood, seizure frequency tends to decrease [5]. However, recurrence of seizures may occur in third or fourth decades and many patients also have persistence of subtle absence and myoclonic seizures throughout adulthood, with an increase of seizure frequency and severity in individuals over 25 years. AS subjects with 15q11-q13 deletion appear to have a worse seizure outcome [5].
Precipitation and inhibition of seizures in focal epilepsies
Published in Expert Review of Neurotherapeutics, 2018
Two types of seizure-inducing factors [9] can be distinguished. The first are factors, most of them nonspecific, that temporarily increase the risk of having a seizure. Sleep deprivation, fever, prolonged stress, and menstruation belong here [9]. The second type are triggering stimuli that directly precipitate seizures in certain patients. These seizures are called reflex seizures. Some precipitating factors are sensory and others, cognitive. They are all syndrome-specific and sometimes even case-specific [2].