Proposed classification: ILAE Definition of the Idiopathic Generalized Epilepsy Syndromes

Members of the Nosology and Definitions Task Force of the ILAE have developed four position papers on syndrome definitions at various ages. The ILAE guideline process requires obtaining feedback and comments from its members on the proposed paper. These comments from our international community will be reviewed by the working group before finalizing the paper.

Please see the Draft: ILAE Definition of the Idiopathic Generalized Epilepsy Syndromes

The manuscript will be open for comments until 15 July 2021. Please use this survey to enter your comments and they will be published below.

Thank you for your help in this important effort of the ILAE.

See all four Proposed papers for Nosology and Definitions

Comments

14 June 2021

I wish to raise two points in the IGE paper; both are about JAE

The first is about the role of hyperventilation in JAE: it is quite true that hyperventilation in CAE= childhood absence epilepsy may prove or disprove absences in untreated cases BUT its effects in JAE in our practice is not that much and it may not initiate absences even in untreated patients for reasons like the age factor and the original number of spells being quite lesser than in CAE.

Secondly the myoclonic jerks according to our education is not exclusionary in JAE since occasional jerks may be encountered in the course of of the disease, but they are not consistent and not prominent and are definitely not the presenting sign or symptom

Ghaieb Aljandeel


12 June 2021

Congratulations for a concise and complete summary on IGE.

Regarding JME, it would be worth mentioning that a subset of patients may present focal ictal features, particularly head version without loss of awareness. Being an understandable "red flag", they would not exclude the diagnosis if all the remaining picture and clinical-EEG correlation is typical for the syndrome. This point is relevant due to the pharmacological choice implications.

Patricia Braga


12 June 2021

Very good.

Nensi Manusheva


12 June 2021

Well defined and certainly adds useful guidance on classifying these seizures with some room for personal expert judgement.

Arun Swaminathan MD


12 June 2021

I follow one comment that title used should be "Generalized Genetic Epilepsies" in which "genetic" is on second place. Be careful with such classification - in some societies - the stigma against genetic diseases could influence not only the individuals but also their familial and social relationship.

Antonio Martins da Silva


7 June 2021

Dear colleagues of the ILAE chapter on Idiopathic Generalized Syndromes,

I am a co-worker of Pr. Hirsch in Strasbourg and we have discussed this pivotal article, which is very interesting. However, I would like to comment on the main Figure, Figure 1, which will be reproduced manifold in the years to come. The blue triangles on the right side of the figure are very confusing. They suggest a scale of intelectual disability, but they do not give a normal range (all patients classified 'encephalopathy'). Furthermore, it is not clear whether the triangles correspond to a scaling for the entities in the dashed squares or only for the entities given in green (Myoclonic-atonic epilepsy etc) or none (!). Is there really neurpsychological data that allows the placement of 'Epilepsy with Eyelid myoclonia' onto such a range of intellectual disability?

This figure is highly important and will be used to resume the work in slide shows all around the world, I would therefore recommend to revise it.

Thank you very much in advance.

Best,
Vera Dinkelacker 


29 May 2021

I’m working in a area with migrant from Middle East, North Africa, and Africa

Some patients report that the term Generalized Genetic Epilepsy could be better or more appropriate:

1) Use of "Genetic" as the second word (i.e., Generalized Genetic) could be less stigmatizing (control study?)

2) In some countries and communities, it might be difficult to get married if you have a genetic disease. In such countries could we  encourage expanding the use of Idiopathic to mean “disease by itself”?

Paul Voulleminot


29 May 2021

Should we consider an MRI negative patient -- male 25 years (onset) no developmental encephalopathy with generalized epileptiform EEG trait and only Generalized Tonic Clonic seizures occurring 80% during sustained sport activities and 20% 2 hours after awakening -- as GTCA.

1) Physical activity induced GTC should be considered as “Red Flag”.

2) If it is a GTCA, genetic diagnostic test could or should be performed: Genetic (SCN1A, K channel, Long QT...)

3) Mild Mitochondriopathy should be seek

4) Lamotrigine, Lacosamide, Carbamazepine should be avoided until there is rare SUDEP case in GGE

Serge Chassagnon


22 May 2021

From a conceptual point of view it is bizarre, if not incorrect, to want to insert a group of syndromes without etiology ("Idiopathic": adj. Denoting a disease or condition the cause of which is not known or that arises spontaneously [Oxford Medical Dictionary]) in a broader grouping defined by a specific etiology (Genetic), even if only presumed.

P.S. E. Hirsch appears twice in the disclosures (with different attributions).

Paolo Benna


18 May 2021

Generalized seizure types -- absence, myoclonic, tonic-clonic and myoclonic-tonic-clonic seizures, with 2.5-5.5 Hz generalized spike wave -- should be classified as having GGE, fully agreed and the frame work is excellent.

However, few idiopathic generalised epilepsies with normal brain imaging study using epilepsy protocol have genetic origin. Birth hypoxia and subtle hypoxic ischemic injury might be the etiopathogenesis for epilepsy in those cases.

GP Burman


13 May 2021

Comments on the prominent motor symptoms related to 3 Hz spike-waves (absences), like the perioral, or those involving head movement should also be inserted to help new beginners.

Betül Baykan