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The goal of this paper is to describe epilepsy syndromes that begin in childhood (age 2-12 years). Additional syndromes that have a variable age at onset, including in childhood, are described in the paper on epilepsy syndromes with onset at a variable age. 1 The childhood onset syndromes can be broadly divided into three main groups: (1) self-limited focal epilepsies (SeLFEs); (2) generalized epilepsy syndromes, which are thought to have a genetic basis; and (3) developmental and/or epileptic encephalopathies (DEEs), which often have both focal and generalized seizures, including Lennox-Gastaut syndrome (LGS), developmental epileptic encephalopathy with spike-and-wave activation in sleep (DEE-SWAS), and epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS), or may have generalized seizures alone, such as epilepsy with myoclonic atonic seizures (EMAtS), or focal/multifocal seizures alone, such as hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) and febrile infection-related epilepsy syndrome (FIRES).Childhood is also the typical age of onset of childhood absence epilepsy (CAE); this syndrome is covered in a separate paper on the idiopathic generalized epilepsy (IGE) syndromes. 2
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.
Epilepsy syndromes have been recognized for >50 years, as distinct electroclinical phenotypes with therapeutic and prognostic implications. Nonetheless, no formally accepted International League Against Epilepsy (ILAE) classification of epilepsy syndromes has existed. The ILAE Task Force on Nosology and Definitions was established to reach consensus regarding which entities fulfilled criteria for an epilepsy syndrome and to provide definitions for each syndrome. We defined an epilepsy syndrome as "a characteristic cluster of clinical and electroencephalographic features, often supported by specific etiological findings (structural, genetic, metabolic, immune, and infectious)." The diagnosis of a syndrome in an individual with epilepsy frequently carries prognostic and treatment implications. Syndromes often have age-dependent presentations and a range of specific comorbidities. This paper describes the guiding principles and process for syndrome identification in both children and adults, and the template of clinical data included for each syndrome. We divided syndromes into typical age at onset, and further characterized them based on seizure and epilepsy types and association with developmental and/ or epileptic encephalopathy or progressive neurological deterioration. Definitions for each specific syndrome are contained within the corresponding position papers.
Cerebral palsy is a common neurologic problem in children and is reported as occurring in approximately 2-2.5 of 1000 live births globally. As is the case with many pediatric neurologic conditions, very little has been reported on this condition in the African context. Resource-limited settings such as those found across the continent are likely to result in a different spectrum of etiologies, prevalence, severity as well as management approaches. This review aims to establish what has been reported on this condition from the African continent so as to better define key clinical and research questions.
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