Automated detection of focal cortical dysplasia based on magnetic resonance imaging and positron emission tomography

Focal cortical dysplasia (FCD) is a malformation of cortical development characterized by disruption of the normal cortical cytoarchitecture, which is highly epileptogenic and one of the leading causes of drug-resistant epilepsy [1]. Surgical resection is the most effective approach to control epilepsy caused by FCD. The success of surgery depends on accurately detecting the epileptogenic lesions during the presurgical evaluations. However, FCD often hides at the bottom of cortical sulci, making visual detection on MRI time-consuming and highly dependent on ...

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Drug resistant epilepsies: a multicentre case series of steroid therapy

According to the International League Against Epilepsy (ILAE), drug-resistant epilepsies (DRE) are defined as epilepsies resistant to two or more antiseizure medicines (ASM), appropriately chosen and used whether as monotherapies or in combination [1]. DRE is a challenging condition that affects approximately 10-20% of all children with epilepsy [2-6.]. According to other studies this percentage even rises to 30-40% [7–10].

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US Generic Antiseizure Medication Supply Chain: Observations from Analysis of US Government Databases

In 2015, the Center for Disease Control (CDC) estimated that in the U.S., 1.2% of the population have active epilepsy (including 3 million adults and approximately 500,000 children).[1] Over 25 antiseizure medications (ASM) are currently approved by the United States Food and Drug Administration (FDA).[2] During a critical shortage of an ASM, the feasibility of a rapid transition to an alternate ASM is often difficult.[3] The FDA drug shortage database in July 2023 indicated a national shortage of ASMs often ...

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Efficacy of Pharmacological Treatments for Dravet Syndrome: Systematic Review and Network Meta-Analysis

Dravet syndrome (DS), described as severe myoclonic epilepsy in infancy in 1978 by Dravet, is a rare genetic severe developmental and epileptic encephalopathy with a reported incidence of 1/16000 to 1/40000 [1,2,3]. DS is mainly caused by de novo mutations in the SCN1A gene (approximately 80%); however, several other genes have also been reported to cause DS or DS-like phenotypes [4].

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Epileptiform electroencephalogram discharges increase seizure recurrence risk in patients with acute symptomatic seizure due to a structural brain lesion

Acute symptomatic seizure has been defined as a seizure that occurs in close temporal association with an acute central nervous system (CNS) insult. This may be metabolic, toxic, structural, infectious, or inflammatory [1]. Acute symptomatic seizures are not considered in the definition of epilepsy and differ from unprovoked seizures in a number of ways. First, they have a clearly identifiable, acute cause that occurs close in time to the seizure, and second, they do not usually recur once the cause ...

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Clinical Analysis of PAFAH1B1 Gene Variants in Pediatric Patients with Epilepsy

Early brain development is characterized by both symmetric and asymmetric division of progenitor cells, along with the outward migration of neurons along radial glial cell fibers to form the cortex. Disruption of this complex process can lead to neuronal heterotopia [1]. The hemizygous deletions of 17p13.3 can result in isolated lissencephaly sequence (ILS) or Miller-Dieker syndrome (MDS). The 17p13.3 region encompasses several genes, including Platelet-Activating Factor Acetyl Hydrolase 1B1 (PAFAH1B1), YWHAE, CRK, and others.

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Efficacy of Vagus Nerve Stimulation in Managing Drug-Resistant Absence Epilepsy Syndromes

Absence epilepsy is the most common form of pediatric epilepsy, manifesting during both childhood and adolescence. Childhood absence epilepsy (CAE) is far more common than juvenile (JAE) and is also more likely to resolve by adulthood[1,2]. Ethosuximide is first line for management for both[3]. However if a patient doesn’t adequately respond to the first line therapy, the best next step in management is typically valproic acid[4] in males or non-reproductive age females or lamotrigine[5], either as a mono therapy or ...

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Early discontinuation of ambulatory vEEG among individuals with intellectual disabilities: a retrospective chart review

Epilepsy occurs in approximately one in four people with intellectual disabilities (IDs) (Kerr et al., 2014). Although prolonged EEG is considered useful in the diagnosis of an epilepsy in people with ID, it is recognised that undertaking an EEG presents unique challenges in this population across both patient-specific and systemic factors [2,5].

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Long-term outcome of treatment-naïve patients with mesial temporal lobe epilepsy with hippocampal sclerosis: A retrospective study in a single center

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is one of the most prevalent and well-defined epilepsy syndromes. In general, MTLE-HS is recognized as a representative form of medically refractory epilepsy with a poor prognosis compared to other focal epilepsies [1–3]. On the other hand, for drug-resistant MTLE-HS, epilepsy surgery is the most efficient with a favorable prognosis, resulting in a seizure remission rate of 60–80% [4–8]. Therefore, the overall prognosis of MTLE-HS may be disproportionately biased toward drug resistance ...

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Ketamine in the treatment of refractory and super-refractory status epilepticus: experience from two centres.

Status epilepticus (SE) is a clinical situation resulting from the failure of mechanisms that terminate seizures and/or the onset of mechanisms that abnormally prolong seizures [1]. It is referred to as refractory SE (RSE) if it continues despite parenteral administration of an adequate dose of a benzodiazepine and an antiseizure drug (ASD), and super-RSE (SRSE) if it continues 24 hours after the start of treatment with anaesthetics [2]. SE progresses to RSE in 23%-48% of patients, and to SRSE in ...

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