In 2017, the International League Against Epilepsy (ILAE) Classification of Epilepsies described the "genetic generalized epilepsies" (GGEs), which contained the "idiopathic generalized epilepsies" (IGEs). The goal of this paper is to delineate the four syndromes comprising the IGEs, namely childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic-clonic seizures alone. We provide updated diagnostic criteria for these IGE syndromes determined by the expert consensus opinion of the ILAE's Task Force on Nosology and Definitions (2017-2021) and international external experts outside our Task Force. We incorporate current knowledge from recent advances in genetic, imaging, and electroencephalographic studies, together with current terminology and classification of seizures and epilepsies. Patients that do not fulfill criteria for one of these syndromes, but that have one, or a combination, of the following generalized seizure types: absence, myoclonic, tonic-clonic and myoclonictonic-clonic seizures, with 2.5-5.5 Hz generalized spike-wave should be classified as having GGE. Recognizing these four IGE syndromes as a special grouping among the GGEs is helpful, as they carry prognostic and therapeutic implications.

Lafora disease is a rare and fatal disease characterized by seizures, progressive cognitive and behavioral deterioration, as well as cerebellar dysfunction. Currently, there is no efficacious treatment that will control the seizures and improve the cognitive decline in this disease. We report a patient with Lafora disease who experienced a dramatic amelioration in her seizure frequency as well as the associated neurological and cognitive dysfunction following initiation of treatment with perampanel administered as monotherapy. Perampanel is the first potentially efficacious treatment for Lafora disease. We discuss a potential mechanism for the efficacy of perampanel in this disease.

Dementia is a neurodegenerative condition that is considered a major factor contributing to cognitive decline that reduces independent function. Pathophysiological pathways are not well defined for neurodegenerative diseases such as dementia; however, published evidence has shown the role of numerous inflammatory processes in the brain contributing toward their pathology. Microglia of the central nervous system (CNS) are the principal components of the brain’s immune defence system and can detect harmful or external pathogens. When stimulated, the cells trigger neuroinflammatory responses by releasing proinflammatory chemokines, cytokines, reactive oxygen species, and nitrogen species in order to preserve the cell’s microenvironment. These proinflammatory markers include cytokines such as IL-1, IL-6, and TNFα chemokines such as CCR3 and CCL2 and CCR5. Microglial cells may produce a prolonged inflammatory response that, in some circumstances, is indicated in the promotion of neurodegenerative diseases. The present review is focused on the involvement of microglial cell activation throughout neurodegenerative conditions and the link between neuroinflammatory processes and dementia.

Metacognition is the conscious knowledge individuals have about their own cognitive capacities and the regulation of these activities through self-monitoring. The aim of this review was to identify the definitions and assessment tools used to examine metacognition in relation to stroke studies. A computer database search was conducted using MEDLINE, CINAHL, PsycINFO, Cochrane Reviews, Scopus and Web of Science. A total of 1412 publications were retrieved from the initial database search. Following the removal of unrelated articles, 34 articles remained eligible. 5 studies examined metacognition in relation to cognitive and/or emotional functioning, 4 examined the concept in relation to memory, while others investigated its relationship to driving, employment or restrictions in daily living. 12 studies examined metacognitive function exclusively in stroke. Only 1 study examined metacognition in the acute phase of stroke. 7 studies adhered to the standard definition of metacognition in line with the neuropsychological literature. The main assessment tools utilised included the Self-Regulation and Skills Interview (SRSI), the Self-Awareness of Deficits Interview (SADI), the Awareness Questionnaire (AQ) and the Patient Competency Rating Scale (PCRS). Assessment of metacognition has tended to focus on traumatic and other acquired brain injury in comparison to stroke. The majority of the studies that examined metacognition in stroke did not assess patients in the acute phase. The heterogeneity of assessment tools was in keeping with the variation in the definition of metacognition. The emergence of a standard metacognitive assessment tool may have important implications for future rehabilitative programmes.

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