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Classification and Terminology Report Comments

Comments were posted in the order they were received with the name and chapter affiliation of the submitter. The comment period has closed. Thank you for your contributions.

 

I have to admit that I was very sceptical prior to reading the proofs. However, after careful reading I want to congratulate to the meaningful step forward this manuscript really is. The only very minor remark is that I still doubt that one can claim that focal seizures may arise from subcortical structures. It may be true that hypothalamic hamartomas are the crucial trigger of epileptic seizures and that their removal may help to achieve seizure freedom. This does not necessarily mean that those seizures definitely originate from the subcortical tissue.
BJ Steinhoff, German League
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The Revised Terminology Commission Report is an excellent advance and represents much important work.  Comments below are offered recognizing the huge amount of thought that went into the current draft, and in great appreciation of everyone's contributions and expertise.

1. Although I'm sure this was deliberated on at length, it seems to me that the term "focal" is not any better than "partial."  Networks are involved in all cases so these are not truly "focal" events. Conversely, as the commission is clearly aware, "generalized" seizures certainly have focal features, so using the term "focal" for seizures that are not generalized is a bit confusing.  In sum, "focal" does not seem like an improvement to me, so why not stay with the admittedly imperfect but at least widely familiar "partial" nomenclature?

2.  Another point which I'm sure went through much discussion is the decision to lump all formerly "partial" seizures into one category = "focal" (Table 1), and then use "descriptors" (Table 2) to subclassify the different clinical types of "focal" seizures.  The problem is that the descriptors are very cumbersome and are unlikely to catch on in general usage, so we will inevitably be "stuck" using the current terms (e.g. aura, simple partial, complex partial) in any case.  Obviously these subcategories are very clinically useful.  My suggestion would be to consider some more easy-to-use concise terms for these types of partial (or "focal") seizures, and to avoid introducing new lengthy descriptors that may simply create confusion.

3.  As long as we are discussing terminology and nomenclature, here is another thought to seriously consider:
Why not change the name of the disease "Epilepsy" itself? One possible alternative would be the simple term "Seizure Disorder." The term "epilepsy" although historically rich, carries much negative baggage and stigmatization which we have not succeeded in shaking in the modern era. Popular perceptions and funding of research in our field continue to suffer, and patients newly diagnosed often still ask in fear "does this mean that I have 'epilepsy' or just seizures?" The term "seizure disorder" or another similar term, would carry none of this baggage and negative connotation. This would clearly be a major change, but it has been done in other fields, and could have very significant benefits both for patients and for those working in the field.

Again, I cannot overstate how grateful I am to the Commission for undertaking this monumental task, and I appreciate that my comments were probably already thought of and considered in detail---but offer these "gut reactions" nonetheless in case they are helpful in future refinements.
Hal Blumenfeld, American Epilepsy Society
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The term "benign" continues to be controversial. As it is described in the draft " ... it may lead to false hopes and unrealistic expectations." Since atypical forms with worse than expected evolution among "benign" epilepsy syndromes have been described, it is not wise to keep using this term. Those epilepsy syndromes that include the word "benign" in their name could then be divided into "benign" and "not-so-benign" forms, which will lead to more confusion. The use of the term "self-limited" could have the same practical difficulties of the "benign" term, since it is not possible to accurately "predict" the evolution with complete certainty in all of our patients, all of the time. This new proposal could be with us for the next 5, 10 or even 20 years, and this will mean that we will keep calling "benign" to some patients with unexpected worse evolution. The term "benign" could be used in the description of the actually so-called "benign" epilepsy syndromes, but, I would like to see that word removed from "the name" of those epilepsy syndromes that actually have it in Table 3 of the draft.
Edgar Avalos Herrera, MD, Guatemala Chapter and American Epilepsy Society
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The authors are to be congratulated in at last beginning to create a rational classification. Of course seizures start in one place and then spread or don't spread, become generalized or remain focal. Thanks for at last beginning to make sense out of descriptive nonsense.
John Freeman, American Epilepsy Society
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With respect to item IV-A, age at onset, has the commission considered the category of “older adult” for patients who develop epilepsy after the age of 60 (or possibly 65)?
Anne C. Van Cott, MD, FAAN, American Epilepsy Society
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Annotations and commments from Roger Porter, American Epilepsy Society - PDF
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The Commission should be commended for their thoughtful and comprehensive report on revised terminology and organizational concepts of the epilepsies. However, there is one glaring ommission in Table 3, under "Electro-clinical syndromes arranged by age at onset, Adolescence - Adult," and that is the group who present after the age of 20, described by Sam Berkovic's group (C. Marini et al., "Idiopathic generalized epilepsy of adult onset: clinical syndromes and genetics," J Neurol Neurosurg Psychiat 2003; 74:192-196). Under the "Adolescence - Adult" category, "Epilepsy with generalized tonic-clonic seizures alone" is listed, but epilepsy with generalized tonic-clonic seizures and absence seizures and/or  myoclonic seizures, the IGE group of adult onset with more than convulsive seizures alone, is not listed.

There is also a need for a similar approach to status epilepticus (SE). Of particular interest to me is the group with subclinical seizures or SE with no behavioral signs of seizures (part of the continuum of adults with absence SE, at the other end of which are those who are stuporous and obviously affected), and comatose patients with electrographic SE (e.g., bilaterally synchronous periodic epileptiform discharges, or BPEDs), who can respond to treatment (DG Fujikawa, Chapter 9, "The two faces of electrographic status epilepticus: the walking wounded and the ictally comatose," in CG Wasterlain and DM Treiman, eds. Status Epilepticus: Mechanisms and Management (Cambridge, MA: The MIT Press, 2006), pp. 109-112). These individuals have epileptic encephalopathies, a term that is usually reserved for childhood epilepsies--acute in the latter case and often chronic but potentially reversible in the former case (E Licht et al., "Chronically impaired frontal lobe function from subclinical epileptiform discharges," Epilepsy Behav 2002; 3:96-100).

Again, an enthusiastic "thank you" to the Commission. I look forward to reading the comments of others regarding the Commission's Report.
Denson G. Fujikawa, M.D., American Epilepsy Society
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I have looked through attached publications and did not find those that seriously consider classification of seizures based on the electrographic pattern of seizure onset. In combination to all existing criteria for classification electrographic pattern of seizure onset could give useful information regarding mechanisms of seizure generation and help to choose AED specifically select 1st AED in the given patient.
A. Bragin, American Epilepsy Society
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Thank you for sending the manuscript which I think is very thoughtfully prepared and am sure will be a useful document for clininicians and researchers. I have just a few minor suggestions:   Table 1. The seizure type "epilepsia partialis continua" has diagnostic and therapeutic implications - please consider including it somehow in seizure classification.   Table 3. I think it is somewhat confusing to name syndromes as "seizures". Please consider renaming them  in a consistent manner, eg. "Migrating partial seizures of infancy" = "Infantile epilepsy with migrating partial seizures" "Benign infantile seizures" = "Benign infantile epilepsy with focal seizures" "Benign familial infantile seizures" = "Benign familial infantile epilepsy with focal seizures"   Table 3. presents the term CSWS but not ESES. There has been a lot of discussion on which term to choose and both are still commonly used in the literature. Please make a statement in the classification acknowledging the synonyms (to avoid confusion) and maybe clearly recommending the use of one and not the other in order to achieve a more consistent terminology in clinical research.    
Eija Gaily, Finland Chapter
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It seems like some of the new proposals are “change for change’s sake”. It took me a while to get used to the old classification of “idiopathic”, “cryptogenic” and “symptomatic” (I don’t think any non-epileptologists ever learnt it) but it worked. It certainly was not perfect but actually the concept of a “benign” idiopathic (genetic) epilepsy “syndrome” (wow, I am in trouble already with the new terminology!) was very important. It seems arbitrary to divide absence epilepsy and benign rolandic epilepsy (formerly what I would consider the main example of an idiopathic, localization-related epilepsy) into different categories, when we do not know where the genetic defect is in most absence epilepsy patients and certainly do not request genetic testing for them. Where does juvenile myoclonic epilepsy fit? Hopefully still as a “genetic” epilepsy. How about a non-lesional patient with Down syndrome? What happens in a severely retarded Dravet syndrome patient with markedly abnormal brain MRI scan – will that then qualify the patient to have “a separate disorder that appears to be interposed between the genetic defect and the epilepsy” and shift them into the “structural/metabolic” group?!

I am sorry, but I like the old classification terminology and the concept that some epilepsies are, in fact more “benign” than others.
Yu-tze Ng, MD, FRACP, American Epilepsy Society
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Page 3, item 2:
Agreeing that the 1989 report was too loose with the term "syndrome", the use of this relative to epilepsy should be made more precise. I
would propose that "syndrome" be reserved to those types of epilepsy where there is another associated exam or imaging finding, but which
is neither directly causative of, nor caused by, the epilepsy, to the extent presently known. An example would be Lennox-Gastaut syndrome,
where the mental retardation and the electroclinical constellation (syndrome?!) are associated, but not known to be _directly_ causal
one of the other, since other mental retardations exist without epilepsy, and without the given electroclinical syndrome.

- Page 11 para 6 (IV-B, 2, b)  "seizure disorder" is euphemistic and should not be used.

- Page 16 Table 1  Perhaps a slash "/" should be used henceforth, viz. "tonic/clonic", to indicate that these may occur singly, or in combination, or in
any order.

- Page 17 Table 2  The descriptions provided, while useful, are unwieldy due to their length, making it likely that the new terminology will be ignored in
favor of the present terminology. Given the importance of impairment of consciousness/awareness/responsiveness in descriptions of seizures,
the seizure classification may benefit from the coining of a new, single, and preferably short word for this concept, based on Latin or Greek.
Mark D. Bej, American Epilepsy Society
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The new classification discussion shows a lot of thought.  I have a few brief comments.
1.      There should be a simple paper with the classification and another (this one) with explanations.
2.      There should be examples of how to apply the classifications in some exemplary patients.
3.      Overall, the paper is pitched at a level of a sophisticated epileptologist, and will need subsequent translations for non-epileptologists.
4.      The committee may not agree, but I will miss simple vs. complex partial seizures.  It creates a useful dichotomy for such things as driver’s licenses, safety precautions, etc.  I agree with eliminating the semi-mystical term, “consciousness.”  I would propose that complex partial seizures remain those with objectively discernible global decrease of awareness or memory.  Simple and complex partial seizures are on a continuum, but some objective distinction is usually possible with a good history.
Robert Fisher, M.D.,Ph.D.,  American Epilepsy Society
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The classification scheme is well-thought out and a significant improvement over the prior scheme. The committee have done a superb job. Two issues related to focal might still be addressed:

The secondarily generalized seizure category is gone, but there is still a descriptor for seizures that lead to bilateral convulsive activity (tonic, clonic, or both). However, a significant number of patients develop unilateral clonic or tonic movements. As long as a new classification scheme is being implemented with descriptive wording for focal seizures, unilateral convulsive activity should be mentioned. Like bilateral convulsive activity, unilateral convulsive can produce falling and injury and these seizures are part of the spectrum of focal seizures (indeed, many seizures thought to be bilateral convlusive are really unilateral on video review of behavior). The recognition of this common behavior was lacking in the prior scheme - since there is really a spectrum of ictal behavior - and it would be useful to mention unilateral tonic, clonic, or tonic-clonic seizures in the descriptive portion of the focal seizure section.
I understand that the complex partial seizure term is thorny and was subject to much debate. However, for purposes of driving, work activities, etc, some categorization of seizures as potentially disabling or non-disabling (or activity-limiting) would be valuable because of these social aspects. When people apply for disability or accomodation on the job, it is helpful to have a generally recognized term for seizure type that everyone understands means that the seizures require accomodation or are disabling. Seizure are just a symptom after all, and it is worth building something into the system that implicates handicap or lack thereof. I am concerned that having "focal seizures" as the sole term may make it more difficult for patients to obtain services that are required, that doctors (especially non-neurologists) may not understand the seriousness of many of seizures, and that the disabling and potentially life-threatening consequences of certain seizures may not be properly appreciated by those who do not work in the field. Therefore, I suggest that focal seizures be categorized as either potentially disabling/injurious or not potentially disabling/injurious, with loss of awareness, loss of motor control, or possibility of injury being the primary criteria used to separate these categories.
Michael Sperling, M.D., American Epilepsy Society
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In India and other developing country practicing general physicians do not follow the complex epilepsy classification at the primary and secondary care levels. only few Tirtiary care centers follows the international classification. so there is a need of two tier classifications for the developing contries like India. please see the attachment.
Dr. Deepak Goel, Himalayan Institue, Dehradun, India
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Thanks for inviting me to make some comments about the revised terminology and concepts for organization of the epilepsies. I attach those I consider of some importance.
Prof. Pedro Ortiz C., University of San Marcos, Peru
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As a Neurobiologist and someone new to the terminology for classifying the epilepsies, I found the report useful and clear.  My main point of confusion was why the structural and metabolic causes were combined as one etiology.  The natural mechanisms underlying a structural versus metabolic disorder will be very different.   Thanks for all your hard work.  
Tracy Dixon-Salazar, American Epilepsy Society
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I write in regards to the ‘Revised Terminology and Concepts for Organization of the Epilepsies: Report of the Commission on Classification and Terminology’.

My comments are as follows:

  1. It has been very correctly pointed out that the Commission’s main goal has been to develop a methodologically and conceptually sound and clinically meaningful revision to the classification of the epilepsies and seizures.
  2. Till now, there have been two main classifications – the classification for seizures (1981) and the epilepsies (1989).
  3. While attempting any revised terminology and concepts for organization of seizures and the epilepsies, it is imperative that seizures and the epilepsies should continue to be classified separately. The main reason for doing so is that majority of patients with epilepsy who receive any treatment are still treated at the primary health care level. The ground realities of health care providers working at the primary level are such that what ever we provide to them needs to be very simple and practical. Moreover, almost 80% of those with epilepsy today live in developing countries where the situation is in stark contrast to that in the developed world. It is only a small percentage of epilepsy patients that receive treatment at the secondary and tertiary care centers. The needs of the clinicians working in such centers (both in the developed and developing world) are totally different and must be addressed separately.
  4. The provisional report of the Commission on Classification and Terminology has indeed made a very good attempt to address this very important issue. The modifications suggested in the ‘mode of seizure onset and classification of seizures’ (Table 1) and ‘description of focal seizures according to degree of impairment during seizure’ (Table 2), basically still revolve around the 1981 Classification of seizures. Any improvements in the 1981 Classification (some have been suggested by the Commission) will certainly make it even more useful for physicians working at the primary care level.
  5. The concept of describing epilepsies as ‘electro-clinical syndromes’ is a welcome step in the right direction. The 1989 Classification of epilepsies certainly needs to be modified and revised in view of the immense amount of new information that we have today and will continue to have in the future. While re-organizing the ‘electro-clinical epilepsy syndromes’, a few important aspects of evolution of one epilepsy syndrome into another and the clinical overlap between certain epilepsies need to be considered. Just to give a few examples, a young girl presenting with absences at age 6-7 years with occasional GTCS will be classified at having CAE. She may go on to develop myoclonic jerks later on in life but will continue to be classified as having CAE. On the other hand, another girl presenting with myoclonic jerks and GTCS at age 14-15 years with a history of having absences at age 8 years will be classified as having JME!! Another similar example is the clinical overlap between the rare syndromes of benign familial neonatal seizures (BFNS) and benign familial infantile seizures (BFIS).
  6. In summary, the ILAE should organize the classification into 2 sets. The first set should integrate the ‘mode of seizure onset and classification of seizures’ with the ‘description of focal seizures according to degree of impairment during seizure’. This will be for use mainly by the average clinicians treating epilepsy at the primary care level. The second set should be a comprehensive description of epilepsies as ‘electro-clinical syndromes’ based on our current knowledge with provisions for taking into account the overlap and evolution of one syndrome into another and modifications in the suggested organization as and when needed. The second set should be for use mainly by the clinicians working at the secondary and tertiary care, teaching, researchers and possibly clinical trials with new AEDs.
  7. Finally, the Task Force on Classification and Terminology should have adequate representation from the developing world where majority of the people with epilepsy live today and where the impact of the revised terminology and concepts will perhaps have the maximum impact in the near future!!

I hope these comments are useful.
SATISH JAIN, MD; DM; FRCP, Indian Epilepsy Society
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My objection is within Section III, Etiologic Designation. This is a concept which makes a great deal of sense, and in fact, it is high time we started examining epilepsy more in this manner than by where seizure onset is located (which may be just an accident of pathology).

But lumping "structural" and "metabolic" together is not just strange, but downright couterintuitive. These are two distinct etiologic designations that are vastly different in terms of pathophysiology, clinical manifestations, and treatment (especially with location- specific modalities such as surgery or stimulation). It is absolutely nonsensical to put them together.

It makes far more sense to put "metabolic" together with "genetic," as any metabolic defect obviously has a direct, specific, potentially identifiable genetic cause. In fact, the report makes this point itself by describing a couple of examples which could easily be considered genetic or metabolic in classification. These have nothing to do with epilepsy that is related to tumors, hemorrhages, etc. Most of these are acquired, though even when they are not (e.g. tuberous sclerosis, dysplasia) they are completely different beasts from things like lissencephaly and Otahara syndrome.

"Structural" needs to be split out from the others in some fashion.
Scott Mintzer, MD, American Epilepsy Society
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First of all, I appreciate the great effort of the committee to develop this update.

1. I would like to recommend the updated classification be published in two separate papers.

1.1. The first paper will summarize the definition of terminology and the classification of seizures and epilepsies with criteria to identify seizure types, epilepsy syndromes and constellations, which were actually
        adopted by the committee to make this update, not something described elsewhere (e.g. textbooks and reviews) because these criteria may vary among clinicians and researchers. The description needs to
        be self-contained so that there is no need to take extra time to look back at older classifications to understand the updated one. These features are especially relevant for novices and non-epileptologists.
1.2. The second paper will provide more detailed explanation about how this update was made, e.g. how each seizure type/syndrome was decided to represent a distinct 'natural class'. This will be for experts,
        especially for ones who are interested in how the classification is developed. Unfortunately, for most of us, the decision process of the committee on distinctness of seizure types/syndromes and the degree
        of their distinctness is unclear, since no solid official data have been shown to public.

2. Seizures

2.1. Since the diagnostic scheme in 2001 proposed a change in concept of epileptic seizures, it may be helpful if this update includes the definition of seizures to explicitly show whether the conceptional change
       is still valid or not.
2.2. I disagree that eyelid myoclonia is placed under absence seizures, because it is not similar to other types of absences in the following points: 1) decreased responsiveness is not a consistent feature, 2) seizure
       duration is much briefer, 3) different ictal EEG pattern, and 4) different triggering factors. If eyelid myoclonia is to be placed under absence seizures, it needs to share some features with other types of absence
       seizures to form a common 'stem' where different 'branches' arise. I do not see many common features.
2.3. Focal seizures classified as only one 'natural class' seem too simple. Why did the committee decide there are no 'subclasses' under focal seizures?
2.4. It would be helpful to have a classification for status epilepticus. Otherwise, we have epilepsy syndromes/constellations with seizure types undefined officially, such as myoclonic status in myoclonic encephalopathy
       in nonprogressive disorders and epileptia partialis continua in Rasmussen syndrome. I am grateful that epileptic spasms and myoclonic absences are now recognized as new seizure types to partially solve this issue.

3. Epilepsies

3.1. It would be helpful to have the etiology of each epilepsy syndrome described somewhere (either genetic or structural/metabolic, or both).
        It is also important to avoid confusion, if some 'idiopathic' epilepsies are to be categorized under 'epilepsy of unknown cause', not under 'genetic epilepsy', as described in the page 10.
3.2. The reason of addition and the criteria of new syndromes, which were not present in 1989 classification, should be addressed as mentioned in the comment 1.2.
3.3. LKS is often associated with CSWS-like EEG, but not always. Since the ILAE report in 2006, LKS has been placed under ECSWS. This suggests the committee decided LKS is a subclass
       of ECSWS in which presence of CSWS is required. Has the presence of CSWS become a requisite to make a diagnosis of LKS?
3.4. Three samples of constellations are presented. The reason why these are recognized as constellations, not syndromes, needs some more specific explanation.

Tomoyuki Akiyama, MD, PhD, Japan Epilepsy Society
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I have read carefully the dosssier about new ideas in classification and terminology, and my suggestions are in the attached file.
Prof. Dr. Jorge A. Ure, Argentinian Chapter
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I have reviewed the report “revised terminology and concepts for organization of the epilepsies”. My first words should go to congratulate the Commission members for the thoroughness and clarity of their manuscript.  
Detailed Comments
Antonio Gil-Nagel, MD, Spanish League Against Epilepsy
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The Commission is to be thanked for what must have been a huge amount of work. Detailed Comments
Colin Ferrie, UK League Against Epilepsy
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It is a great advance done by the Commission. Reading the paper on terminology and concepts I have two remarks:

1.       I really do not see clearly the border or limit between constellation and epilepsies secondary to specific structural or metabolic lesions or conditions. The definition of constellation is “a group of configuration of ideas, characteristics, objects etc, that are related in some way.” So where is the limit or the definition of some way of relation between epilepsies in the category of constellation and epilepsies secondary to…I think both are related in some way, where is the limit that one is more related to epilepsy than the other?

2.       In the same philosophical idea of “labeling these syndromes as pharmaco-responsive is likely more meaningful to clinicians and families” than the term of benign or idiopathic, so the term “catastrophic epilepsies” should be discouraged or abolished. I know catastrophic is not a term of the Classification or Commission, but it is highly used in the literature without any opinion of the Commission. This term discourages physicians and patients relatives, and gives the meaning “there is nothing to do”, where really there is a lot to do, surely more than in pharmaco-responsive epilepsies.
Jaime Carrizosa Moog, Colombian League Against Epilepsy
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Sorry if these are too late to be of any use, but here are a few comments on the July 28 2009 commission report on Revised terminology and concepts for organization of the epilepsies.  Thank you for soliciting input from the masses.   

In general, I really like the main concepts, and obviously extensive time, effort, debate, and deep thought went into this. Kudos!  Now for my hopefully constructive comments:   The main problem with attempting to use this widely is its complexity. More specific comments:  

Constellations:  This is a bit vague and probably too subtle/complex a differentiation.  If it's from a lesion such as a hamartoma (which is listed as an example), why wouldn't that be in the next category (due to structural lesion)?  The differentiation from electroclinical syndrome seems to be subtle as well, and mainly relates to the former having to have age-specific manifestations.  If that is the intent, then perhaps "electroclinical syndrome" should be termed "age-specific electroclinical syndrome" or "electro-clinico-developmental syndrome".  Or just call them all syndromes and do away with the requirement of being age-specific (I'd vote for that approach on my first read through).   Give an example of metabolic cause of epilepsy.   As we are discussing epilepsy, not acute symptomatic seizures, should make that clear (this is not referring to hyponatremia or renal failure, right?).  I assume this is more congenital metabolic.  

Genetic vs structural metabolic: what if a patient with a known genetic syndrome is found to have subtle cortical dysplasia?  Or that whole syndrome is found to on histology or advanced MRI techniques?   Does that then become structural, and not genetic in etiology?  Ultimately there is some "cause" or interposed factor between the gene and the epilepsy in all genetic syndromes, though often unknown at this point.   Clarify via quantification how familial something has to be to count as "genetic".   

Comments arising from reading the Tables:

Table 1: Many terms will need definitions, which won't be so straight-forward: typical vs atypical absence, myoclonic absence, epileptic spasms. Why are myoclonic-atonic and myoclonic-tonic considered separate seizure types, but not some fairly well-described ones such as tonic-absence (disclosure: I wrote up a series on that one) or clonic-tonic-clonic? What should one do w/ drop attacks that are not fully characterized? Spasms: As defined here, seems to me these qualify as generalized.  Doesn't matter if they have focal signature, right?  Involves widespread bilateral network.  

Table 2: These probably need labels.  Maybe just keep aura, SPS w/ objective features, CPS and 2nd GTC??  That would make many people happy… What if unclear if alteration in awareness?  Can you just say "focal seizure, NOS"  

Table 3:  Should probably decide whether to include all eponymous and non-eponymous names, or just do non-eponymous ones. (EIEE for Ohtathara, SMEI for Dravet, etc). Is ADNFLE really age-specific to childhood? There are many that present in adolescence.  Prob need to define how age-specific is req'd to be a "syndrome" and not a "constellation" as defined.  I'm ok w/ calling them all electroclinical syndromes, incl the ones listed as constellations.  Some are age specific, some are not, but not sure why a syndrome has to be age specific if it meets all the other criteria.   Thanks again for the efforts of the commission, and the chance to comment.  
Lawrence J. Hirsch, MD, American Epilepsy Society
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Comments on the ILAE revised clssification and terminology. Link to document.
Professor LIAO Weiping, China Association Against Epilepsy (CAAE)
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I would appreciate if you could consider the attached  in response to your invitation for comments  for  “The Report of the Commission on Classification and Terminology”.    Link to document.
CP Panayiotopoulos MD PhD FRCP, UK Chapter of ILAE
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It was a pleasure to review the commission report from July 28, 2009. The report focuses on the organization of epilepsies and resolved many of the inconsistencies seen with prior classifications. Although not the main emphasis of the report, a few clarifications regarding the proposed way to classify seizures would be helpful:  

  1. Definition of seizures: does the term seizure refer to clinical symptoms or electrographic findings or both ?
    1. If focal seizures are an electroclinical entity, table 2 should include: * without any clinical signs and symptoms (for pure electrographic seizures). An electroclinical definition of seizures would make it unnecessary to create a separate classification for epileptic spasms –focal epileptic spasm related to a focal lesion or which resolve with surgery could be classified under focal seizures and generalized epileptic spasms related to a diffuse etiology under generalized seizures. Otherwise, to be consistent, one would have to create a separate entity for staring spells, depending if they are “focal” or “generalized”
    2. On other hand, in certain passages the report seems to refer to seizures as a purely clinical entity of signs and symptoms. P.8, 3rd paragraph states that manifestations (seizures) and pathophysiology are kept separate in this commission report. “Generalized seizures can be asymmetric” (p.5, 3rd paragraph) seems to assume there is only one category to which asymmetry can be applied (clinical asymmetry ?). However, if seizures are electroclinical phenomena, asymmetry or even the term generalized could be also applied to the EEG or the clinical symptoms. Which do not necessarily go in parallel.
    3. The manuscript should clearly state how focal vs. generalized seizures are categorized (on the basis of clinical symptoms, electroclinical findings, a construct defined by resective surgery or some other neurophysiologic concept). 
    4. This definition would also help to get an idea what the authors anticipate as a future “natural class” for focal seizures.
    5. It would be also useful to clearly distinguish the term “seizure” as the manifestations of a sudden, abnormal excessive or synchronous neuronal activity in the brain and not to be confused with non-epileptic spells (frequently also called seizures).
  2. Seizure description, Page 6, 1st paragraph:
    1. Descriptors of a seizure (and the sequence of their occurrence) is essential for the practicing physician in any patient to determine if the symptoms and signs are likely provoked by a sudden abnormal neuronal activity of the brain (aka epileptic seizure) and not something non-epileptic. I don’t think the usefulness of a systematic seizure description is limited to special circumstances like presurgical evaluation.
    2. It would be useful to clarify the role of the 2001 ILAE seizure terminology glossary in describing seizures.

Thank you for the tremendous amount of work and diligence creating this report.  
Stephan Schuele, MD, MPH, American Epilepsy Society
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It has been a great pleasure for me to read the report. I'm also happy that my ideas from my participation in the task force committee 2000-2005 have been discussed. These include the terminology "simple" and "complex", "idiopathic" and "symptomatic",  and "benign" , which are now abandoned. The report is very well written, but I have a few comments.
IV - A. Age at onset
According to the pediatric grouping: neonate (the first 4 weeks of life), infant (1-12 months), child (1-12 years), adolescence (12-18 years) adult (from18 years). The United Nations Convention on the Rights of the Child defines a child as 'every human being below the age of 18 years'. Thus, the pediatric grouping is a subdivision, and this is consistent with the purpose of grouping our syndromes.
The pediatric grouping should be used in the classification.
IV - B.1 Epileptic encephalopathy
I don't understand that we should use this terminology. A basic brain dysfunction is the cause to seizure manifestations, an epileptiform EEG, behaviour disorders, etc. As you say ' ...."epileptic encephalopathy" does not imply that all individuals with these disorders will appear encephaloapthic...'. You have partly summarized this in the last paragraph. You have also defined electro-clinical syndromes, and in the definiton of each syndrome there should be a description of different symptoms, which we also do.
Orvar Eeg-Olofsson, MD, PhD, Sweden
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As some will know, Fred Andermann, Renzo Guerrini and I are currently preparing a book on the 'Aetiology' of epilepsy, for publication next year. 
I personally feel that aetiology is not given appropriate weight in the proposed ILAE classification schemes, and as you have asked for comments on the proposal, I attach a few musings relating to the aetiological categorisation of epilepsy as we are formulating it in our book (currently in draft not final form). 
I hope this is of some interest. 
Simon Shorvon