Classification of the epilepsies: New concepts for discussion and debate—Special report of the ILAE Classification Task Force of the Commission for Classification and Terminology

Scheffer, Ingrid E.; French, Jacqueline; Hirsch, Édouard; Jain, Satish; Mathern, Gary W.; Moshé, Solomon L.; Perucca, Emilio; Tomson, Torbjörn; Wiebe, Samuel; Zhang, Yue Hua; Zuberi, Sameer M.

doi:10.1002/epi4.5

Cited by 115 publications

(103 citation statements)

References 33 publications

(60 reference statements)

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“…The EEG, starting around PN40, shows hypsarrhythmic background and decremental responses with the spasms . This is also a model of epileptic spasms due to structural lesions, according to the term proposed by the recent ILAE classifications . There is no report yet of interneuronopathy or cognitive and/or neurodevelopmental deficits in this model.…”

Section: Neurological Disorders Associated With Interneuronopathies: mentioning

confidence: 89%

Interneuronopathies and their role in early life epilepsies and neurodevelopmental disorders

2017

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GABAergic interneurons control the neural circuitry and network activity in the brain. The advances in genetics have identified genes that control the development, maturation and integration of GABAergic interneurons and implicated them in the pathogenesis of epileptic encephalopathies or neurodevelopmental disorders. For example, mutations of the Aristaless-Related homeobox X-linked gene (ARX) may result in defective GABAergic interneuronal migration in infants with epileptic encephalopathies like West syndrome (WS), Ohtahara syndrome or X-linked lissencephaly with abnormal genitalia (XLAG). The concept of “interneuronopathy”, i.e. impaired development, migration or function of interneurons, has emerged as a possible etiopathogenic mechanism for epileptic encephalopathies. Treatments that enhance GABA levels, may help seizure control but do not necessarily show disease modifying effect. On the other hand, interneuronopathies can be seen in other conditions in which epilepsy may not be the primary manifestation, such as autism. In this review, we plan to outline briefly the current state of knowledge on the origin, development, and migration and integration of GABAergic interneurons, present neurodevelopmental conditions, with or without epilepsy, that have been associated with interneuronopathies and discuss the evidence linking certain types of interneuronal dysfunction with epilepsy and/or cognitive or behavioral deficits.

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Section: Neurological Disorders Associated With Interneuronopathies: mentioning

confidence: 89%

Interneuronopathies and their role in early life epilepsies and neurodevelopmental disorders

2017

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“…The response was so extensive and the feedback on important concepts so conflicting that the panel in charge of reviewing the public comments determined that further pubic engagement was necessary to ensure the highest possible level of agreement. The roadmap followed by the panel to solicit further engagement and to respond to feedback from stakeholders is described in an article published in Epilepsia Open in 2016, which again invited feedback from the global community . Further comments and opinions were then deliberated and considered in finalizing the present position document which defines the Classification of the Epilepsies in 2017.…”

Section: Methodsmentioning

confidence: 99%

ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology

et al. 2017

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Summary The ILAE Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances which have taken place since the last ratified classification in 1989. As a critical tool for the practising clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies and communication around the world. The new classification originates from a draft document submitted for public comments in 2013 which was revised to incorporate extensive feedback from the international epilepsy community over several rounds of consultation. It presents three levels, starting with seizure type where it assumes that the patient is having epileptic seizures as defined by the new 2017 ILAE Seizure Classification. After diagnosis of the seizure type, the next step is diagnosis of epilepsy type, including focal epilepsy, generalized epilepsy, combined generalized and focal epilepsy, and also an unknown epilepsy group. The third level is that of epilepsy syndrome where a specific syndromic diagnosis can be made. The new classification incorporates etiology along each stage, emphasizing the need to consider etiology at each step of diagnosis as it often carries significant treatment implications. Etiology is broken into six subgroups, selected because of their potential therapeutic consequences. New terminology is introduced such as developmental and epileptic encephalopathy. The term benign is replaced by the terms self-limited and pharmacoresponsive, to be used where appropriate. It is hoped that this new framework will assist in improving epilepsy care and research in the 21st century.

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“…Family members underwent phenotyping by direct interviews using a seizure questionnaire, review of medical records and/or the results of clinical investigations. Epilepsy was classified according to the latest International League against epilepsy revised organization …”

Section: Methodsmentioning

confidence: 99%

DEPDC5 mutations in familial and sporadic focal epilepsy

et al. 2017

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Classification of the epilepsies: New concepts for discussion and debate—Special report of the ILAE Classification Task Force of the Commission for Classification and Terminology

Cited by 115 publications

References 33 publications

Interneuronopathies and their role in early life epilepsies and neurodevelopmental disorders

Interneuronopathies and their role in early life epilepsies and neurodevelopmental disorders

ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology

DEPDC5 mutations in familial and sporadic focal epilepsy

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