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Autoimmune encephalitis (AEs) encompasses a variable spectrum of clinical manifestations pointing to a widespread brain involvement, well beyond the limbic system alone [1]. Besides mental status alterations, cognitive deficits and behavioral/psychiatric disorders, seizures are part of the clinical core of AE [2], and often represent the most alarming and impressive sign at the disease onset, leading the patients to seek medical advice. The subacute onset of seizures, especially in elderly subjects, urges the need to investigate (and eventually rule out) numerous possible etiologies or mimics, ranging from vascular accidents and toxic-metabolic encephalopathies to neurodegenerative diseases.
Status epilepticus (SE) is one of the most common life-threatening neurological emergencies with high mortality and morbidity [1]. SE has a bimodal age distribution, with elderly individuals having the second highest incidence after infancy [2].
The novel antiepileptic drug (AED) perampanel is used in monotherapy and as an adjunctive treatment for focal, focal to bilateral, and generalized tonic-clonic seizures in patients with epilepsy. Perampanel has a high protein-binding ratio (> 95 %) and long half-life (∼105 h) and is mainly metabolized by cytochrome P450 (CYP) 3A4 [1]. Thus, it is highly susceptible to interactions with CYP-inducing AEDs (inducers), such as phenytoin (PHT), carbamazepine (CBZ), and phenobarbital (PB). In contrast, stiripentol is a strong inhibitor of CYP3A4, which can elevate perampanel concentration.
Hypoxic ischemic encephalopathy (HIE) occurs in 1–8 newborns per 1000 live births and it is the most common cause of seizures in term neonates. [1] Many studies in asphyxiated neonates before the advent of therapeutic hypothermia [TH] have shown a relationship between clinical and/or electrographic seizures, especially status epilepticus, and abnormal outcome. [2] Due to the overlapping between the ischemic hypoxic damage and the injury resulting from seizures activity, it is still difficult to establish the impact of seizures in the exacerbation of brain damage and in the worsening outcome of asphyxiated newborns [3].
The recently published study “Clinical characteristics and prognosis in a large paediatric cohort with status epilepticus” provided an impressive amount of data about the real-world features of paediatric status epilepticus [1].
The Publisher regrets that due to a typesetting error the following text was included in the footnote for Table 3:
The intellectual function of patients with epilepsy has been a subject of interest for centuries [1,2]. It was long thought that all patients with epilepsy have poor cognition and progressively worsening memory. This belief started to change in 1985 with the results of a prospective, double-blind, placebo-controlled study done by Mattson et al through the Veteran’s Administration [3]. Six hundred twenty-two patients were enrolled, followed over one to six years, and given extensive neuropsychological tests before initiation of antiseizure medications and then throughout the study.
Epilepsy with eyelid myoclonias, originally depicted by Jeavons in 1977, is a reflex epilepsy characterized by jerking of the eyelids with or without absences precipitated by eye closure or by light (eyelid myoclonia, EM), eye closure-induced EEG paroxysms and photosensitivity. Childhood-onset, female predominance and a normal development are typical features, though a mild intellectual disability has been reported. Sotos syndrome is a disorder characterized by a distinctive facial appearance, learning disability and overgrowth in childhood with macrocephaly, caused by heterozygous pathogenic variants or deletions in NSD1 gene.
Lafora Disease (LD, OMIM# 254780) is a progressive myoclonic epilepsy inherited in an autosomal recessive manner, due to rare mutations in either the EPM2A or the NHLRC1 genes, encoding for the laforin glycogen phosphatase on chromosome 6q24 and the malin ubiquitin E3 ligase on chromosome 6p22.3 [1], respectively. The absence of either of the two proteins results in Lafora bodies, which contain polyglucosan, a poorly branched form of glycogen, in neurons, muscle and other tissues, and main drivers of neurodegeneration [2].

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