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Organization of the Epilepsies

Request for Comments

The ILAE Commission on Classification and Terminology has prepared a new manuscript on the organization of the epilepsies, based on the 2010 proposal, and revised to incorporate many comments that the Commission has received. The proposal and a quick overview are linked below, followed by comments received from November 2013 to January 2014. A task force appointed by the Executive Committe is reviewing the comments, and preparing a final proposal.

Organization of the Epilepsies full document | Quick overview

Comments

12 February 2014

To Drs Scheffer, Berkovic and colleagues,

I appreciate the recent paper as a brief but broad review of our current state of knowledge.

Apart from the input that results from very recent research findings I note the challenge to change clinicians’ use of the terms simple and complex (focal) seizures. A suggestion is made that they be recognised as focal seizures without or with impaired consciousness or awareness or responsiveness.

This sadly is an exercise pre-destined to fail because it is too wordy and too clumsy. All that is needed is ‘focal seizure without/with impairment of awareness’, a succinct definition in good English. Impairment of consciousness accompanies any impairment of awareness. Dyscognitive has the same meaning in good (but ancient) Greek.

On the other hand impairment of responsiveness CAN occur without impairment of awareness if the (e.g.) adversive, motor or even sensory experience is strong enough. That we would have called a simple partial seizure.

Sincerely, Andrew Black


28 January 2014

My hearty congratulations for the efforts made in classification of epilepsy. My comments are:

1) Regarding Status epilepticus, should be made as separate category with its etiologies.

2) According to me, one more category should be added related to trauma causing epilepsy.

3) 'Self-limited' term should be changed. thank you for the great effort the commission has made in updating and explaining the terms.

With regds.
K.K.Therisa


25 January 2014

Many thanks for this opportunity to comment on Organization of epilepsies. We are very grateful to ILAE for this information. We had a discussion on ILAE revised Terminology for organization of Seizures and Epilepsies 2011-2013 in our Neuro-psycho-pathology Center of the University of Kinshasa. We came in agreement on the points below:

(1) We accepted the new terminology for the classification of seizures;

(2) We also approved the classification of epileptic syndromes arranged by typical age at onset but we suggested adding Epilepsy with Autism in childhood group;

(3) About etiology of epilepsy, we proposed that structural etiology might not be placed in the same category with other etiologies like genetic, metabolic, immune, infectious and unknown because brain lesions can occur in these etiological forms;

(4) We considered the possibility for a provisional organization of epileptic syndromes for practitioners who work in conditions without equipments for etiological diagnosis in rural areas:
i) Epilepsy without clinical neurological deficits;
ii) Epilepsy associated with clinical neurological deficits;
(iii) Epilepsy due to demonstrated brain damage (by electro-clinical or cerebral imaging findings).

With our best regards
Prof Dr Daniel Okitundu Luwa E-Andjafono


17 January 2014

Epilepsy Action welcomes the proposed organisation of the epilepsies. We believe that this could be a positive step forward (once agreed) in diagnosing and, therefore, treating epilepsy.

Although this proposed organization is primarily for clinical use, we really would urge you to develop and agree a simplified version for patients. Our stakeholders have told us that it is extremely confusing when their health professionals describe their epilepsy in terms that are unfamiliar to them [patients and professionals using different classification and terminology]. People with epilepsy have also expressed frustration in situations when their health professionals haven’t clearly explained their epilepsy classification in a way that they can understand – or when their epilepsy specialist uses a different term to describe their epilepsy to that used by their general practitioner (GP).

Epilepsy Action believe that a simplified version of the classification (written for patients and non-specialist) is a move to alleviate these issues.

Thank you and best wishes,
Nicole Crosby-McKenna


16 January 2014

I appreciate the hard work of the Committee. However, it is disappointing to me that the Committee consisted of what seems exclusively clinicians. As a result there are a few areas of insensitivity to the feelings of and impacts on people with epilepsy. For example:

1. A somewhat ‘default’ position of labelling a person’s epilepsy as genetic can potentially compound the stigma and social prejudice that stems from people thinking those with epilepsy may be genetically inferior or significantly more likely to have a child with epilepsy. Increasing the percentage of people with epilepsy having a genetic background would be an unfortunate assumption that is contrary to good scientific policy, the medical ethics of “First do no harm.”, and sensitivity to patients. Arbitrarily inflated statistics of genetic links to epilepsy might lead to the type of fallout that eugenics played in isolating people with epilepsy in the previous centuries. A person more likely to be perceived to be of genetic defect may seem less attractive to a potential spouse. There could be increased worry among family members with epilepsy that they to might have it. Such unwarranted inflation of the statistical probabilities of having a child with epilepsy may also lead to added stress about having children. I am a person with acquired epilepsy from a brain injury. I was single when it happened and noticed a difference in the first reaction of women when they found out afterward. What about the more likely feelings of blame following a miscarriage, when emotions can override logic?

2. Dyscognitive is a confusing and inappropriate term. Why not use a phrase like transient impairment associated with focal epilepsy.

3. Patient’s and family members will likely have problems understanding the clinically based criteria. Basing a classification based on existing diagnosis can lead to transient mislabeling during the progression of the diagnostic progress.

Modern technology has also increased the ability to understand localisation of both seizure activity and interictal discharges. That should be addressed in the classification schema.

Sincerely,
Jim Chambliss

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15 January 2014

I wish to congratulate the Task force for having worked so well on a very difficult subject, that needed to be addressed, since the last ILAE CLASSIFICATION, as many advances in understanding of  the subject have taken place in the past quarter of a century. I sincerely thank you for giving me this opportunity to make my comments which are as follows-

1. IS ORGANISATION BASED ON BIOMARKERS – DIAGNOSTIC ,PROGNOSITC OR THERAPEURTIC POSSIBLE – it would make more sense in clinical prognostic and therapeutic areas.

2. BILATERAL’ VS ‘DYSCOGNITIVE FOCAL’ AND ‘ FOCAL WITH RETAINED AWARENESS’- makes good sense even in absence of EEG/MRI (restricted resources setting) is good for clinical setting but not strictly applicable in few cases as mentioned in the document e;g TS the complex phenotype is a good concept for the indeterminate presentations . Again sometimes this may not be possible in the absence of detailed EEG studies which may not be possible in all settings. Now how do we compare this with Generalized, simple focal/partial and complex focal/partial as terms used to classify seizures types in Drug Trials, for example?

3. We should aim to diagnose seizure type(s), electro-clinical syndrome and etiology where possible (this may not always be possible with the best resources, leave alone in settings of limited resources) due to heterogeneity that necessitates further delineation of biomarkers through further research in these types to define patterns of prognosis, etiology, and treatment.

4. Use of ” self limited” is a good idea than “benign” but evaluation for cognitive disturbances are not carried out so Brief QOL assessment must be done to know if indeed they are self-limited without affecting other functions or outcome.

“Those significantly affecting QOL” as part of seizure  type or aetiology or syndrome  vs “ Those that do not affect QOL”  may be a good axis, prior to AED effect that may improve or deteriorate the situation depending on appropriateness of AED use.

5. Genetic epilepsies may not be inherited(mutation)and need to be delineated:

– “ possibly genetic” should be grouped as Primary or idiopathic  generalized needing further investigation, to prove the point, .i.e gene delineation or confirmation, for geneticists or academicians or for prognosticators
– “ Genetic generalized Epilepsies” may be a difficult term to use in settings where resources to test for the gene are limited by availability and cost – moreover labeling genetic generalized epilepsy may jeopardize the social standing and add to stigma in many cultures especially in women of marriageable age or later.

For practical purposes primary generalized is used with ease  for choice of drug and prognosis  which I belive is very practical and should be retained (but there is genetic as well as phenotypic heterogeneity within this group which we still do not know how to address and organize).

Still it forms a good class of situations that respond to broad spectrum drugs and where there may be exacerbation of seizures with other class of drugs that are best avoided.

When we group all generalized epilepsies that are genetic such as TS and CAE , JME, EME –it is evident that they are not the same . Best to keep Idiopathic generalized as a group and define subtypes based heterogeneity that have genes identified and behave differently, so that treatment and prognosis can be dictated accordingly by such sub classification.

6. Organisation of epilepsies with structural etiologies needs a very  strong organization- because many of these are likely to be surgically remediable, the definition of which is constantly changing due to advances in pre-surgical and imaging technology- dichotomy based on seizure semiology that points to focal structural abnormality that may be evident or not on initial or advanced investigational studies from CT scan , MRI SPECT, PET and MEG used depending on availability in different regions.
 It prods the evaluator to set aside cases of probable structural cases for staged and variety of investigations to determine  early  pre-surgical evaluation, operability and outcome  in the treatment algorithm.

7. Finally , Immune mediated is a good subclass of organization to pursue due to newer variants being added and needing a completely different approach from other groups,  both in acute and long term settings. Post infectious, Post inflammatory, Parasitic may be other significant subgroups relevant to certain regions.

Clearly the discussion will go on and on …

Nandan Yardi


15 January 2014

Thank you for call me for commentary and congratulation for great work of the Commission on classification and terminology.

In the last four Executive terms of the ILAE Commission on classification and terminology has been presented no new classification of neither the epileptic seizures nor the epilepsies. The 2001, 2006, 2009 and 2013 reports were named as a “diagnostic scheme”, “report of the ILAE classification core group” or a “revised terminology and concepts for organization of the epileptic seizures and epilepsies” but none was named as a new classification. Perhaps, probably because implicit, authors had recognized that these schemes are no better than previous classifications.

All four reports mix concepts of the the epileptic seizure domain, with the epilepsy domain, which are very different concepts. That confussion continues in many of the commentaries. In previous reports classifications were presented separately(1964, 1969, 1970, and 1981 for epileptic seizures and in 1970, 1985 and 1989 for epilepsies and epileptic syndroms). In this respect I don’t understand Pete Engel Jr. when he say that “each class of seizures must be a unique diagnostic entity with implications for etiology, prognosis, or treatment”. This would be more applicable for each epileptic nosologic entity or each epileptic disease or specific condition but not to an epileptic seizure, and can be used in very few if any class of epileptic seizures (may by in gelastic seizures related with hypotalamic hamartoma??)

This problem is identified in the introduction of the actual report where establish (epileptic)“seizures are a separate category to epilepsies and epilepsies are a separate category to etiologies”, page 3. However, the second part of sentence is equivocal: etiology is one of the main elements that defines a nosologic entity or a disease, that is, etiology is the mainstream for definition and classification of all diseases. Each epileptic nosologic entity must be defined in its cause, or if the cause is unknown, must be specified (like in degenerative diseases , whose cause is unknown).

The presented organization of epilepsies by etiology in this report is very incomplete and is not a systematic classification.
Epilepsy is a heterogeneous group of diseases and disorders. Each nosologic entity, each disease has one or more etiologic factors. The etiologic factors that cause diseases of the nervous system, (and epilepsy is it), are:

a) endogenous (Genetic) and
b) exogenous (Infectious agents, Quimic agents, and Physic agents [mechanical trauma, electricity, irradiation, termic trauma, etc.]);
c) Other factors are influenced both by endogenous or exogenous factors (-Metobolic/ nutritional /endocrine. -Systemic diseases affecting nervous system [cardiovascular, respiratory, gastrointestinal, renal, osteoarticular, reumathic]. -Neoplastic. -Inmune. -Developmental. -Degenerative).

One disease (or epileptic disease) may be caused by multiple etiologic factors, and genetic factor affects all others.
“Structural” vs. “non structural” must be better defined and is a very useful descriptor of other causal categories, but is a morphologic and not etiologic category.

In the group named electroclinical syndromes all except three (West, Lennox-Gastaut and Dravet Syndromes) have a common feature: imaginologic or histologic (light microscope) not demonstrable lesion. These nosologic entities can be included in the non structural category, or previous idiopathic category, some of them with known genetic factor, and other await for it.
The 2001, 2006 commission reports has invaluable and systematic work that must be complement the last two reports, but I agree with the opinion that while appear better classifications, the 1981 and 1989 schemes must continue to be used with some modifications.
In the present organization of the epilepsies we are not away from Mr. Delasiauve 160 years ago (cited by the late Fritz Dreiffus in his Pediatric Epileptology 1983 page 1):“Delasiauve (1854), who used the terms idiopathic to describe seizures in which there was no anatomical demonstrable lesion, symptomatic for seizures in which there was a demonstrable lesion in the brain, and sympathetic to describe seizures due to the effects on the brain of diseases elsewhere in the body”.

Sincerely
Santiago Acebedo

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15 January 2014

Thank you very much asking for comments. I have several comments, some minor, some major ones. I apologize for many possible typos and grammatical errors in advance because I have no time to examine those carefully to be in time with the deadline.

Minor point 1:
As the paper rightly pointed out, there is a fundamental difference between monogenic and polygenic inheritance. As you know, no one calls schizophrenia or bipolar disorder simply genetic psychiatric disorders, although these are now widely accepted as polygenic genetic diseases. I know very well that this issue has been already repeatedly discussed and well-addressed in the paper presented, but, once the appendix “genetic” becomes an integral part of the name, many laymen would predictably confuse polygenic genetic diseases with monogenic one. If you really want to add “genetic” to the disease name, it would be better to name it more properly as “polygenic generalized epilepsy” instead of “genetic generalized epilepsy” to avoid detrimental confusion of the patients, family, and surrounding people.

Minor point 2:
The location of aphasic fits and other seizures with paroxysmal neuropsychological deficits in the classification seems to be unclear. While these fits with neuropsychological deficits cannot be observed and only felt by patients without appropriate intervention, you can record them objectively with appropriate testing.

Minor point 3:
The syndrome of GTC alone with generalized spike-wave on EEG seems to be inadequate. Epileptiform discharge on EEG findings can be detected in 70% at best even among patients with typical juvenile myoclonic epilepsy. Further, those patients whose GTCs occur exclusively during sleep are more likely to have focal seizures. You could say that these so-called secondarily generalized seizures are not real GTC, but  simultaneous video-EEG monitoring of these seizures are often not practical because of the frequency of the seizures. In reality, we have to judge the nature of the seizures which apparently look like GTC exclusively based on the history of the patients and testimony of the family. Whether GTC occurs mainly on awakening or during sleep, even if it is not of decisive value, could be an important and the sole clue to clinical decision for the present.

Major point
The overwhelming majority of patients who come to us as adult epilepsy patients have age-independent focal epilepsies. However, the most of this group of patients cannot have the legitimate name in 2010 system except for Rasmussen syndrome, hypothalamic hamartoma, and medial temporal epilepsy with hippocamal sclerosis. Indeed, based on etiology classification, some patients with age-independent focal epilepsies have some names, but as the paper presented rightly pointed out, the relation between etiology and epileptic seizures are very complex. Etiology cannot and should not be identified with epilepsy. I have tentatively classified my 3,848 adult patients with epilepsy using both 1989 and 2010 classifications based on the data gained at the first visit. While, based on 1989 classification, 73% of patients could be classified, only 28% of patients could have legitimate names in 2010 classification. For specialists who are familiar with epileptic syndromes as well as phenomenology of epilepsy, this complex relationship between classification of epilepsy and that of etiology will not do a major damage in diagnosing process, but, I am afraid that this double diagnostic system leads to a serious confusion to many medical staff who are involved in epilepsy treatment and who are not specialists in epilepsy. In educational and enlightening point of view, some revisions should be made to make it more easily understandable. (See schematic comparison between 1989 and 2010 classifications).

Kousuke Kanemoto


15 January 2014

There are lots of good things about the proposals but also some very bad things!! To save space, I will make only negative comments and express disagreements!

DYSCOGNITIVE. It seems that the Commission is trying to please everyone. On the one hand, they say that focal seizures should not be classified but then, in order to please those clinicians who say that the distinction between seizures with and without impairment of consciousness is vital, it tells us that all we have to do is replace "complex partial" with "focal dyscognitive" – a simple exchange of adjectives. I cannot think of a more unsuitable term than the old "complex" and it appears that the Commission could not either, so they manufactured one! "Dyscognitive" comes from the 2001 Glossary (Blume) but that glossary has a cumbersome and impractical definition of impaired cognition and in fact, offers 2 different definitions, as follows:

["2.3 DYSCOGNITIVE
The term describes events in which (1) disturbance of cognition is the predominant or most apparent feature, and (2a) two or more of the following components are involved, or (2b) involvement of such components remains undetermined. Otherwise, use the more specific term (e.g., “mnemonic experiential seizure” or “hallucinatory experiential seizure”). Components of cognition:
• perception: symbolic conception of sensory information
• attention: appropriate selection of a principal perception or task
• emotion: appropriate affective significance of a perception
• memory: ability to store and retrieve percepts or concepts
• executive function: anticipation, selection, monitoring of consequences, and initiation of motor activity including praxis, speech]

[9.2.1 IMPAIRED COGNITION
Decreased cognitive performance involving one or more of perception, attention, emotion, memory, execution, praxis, speech (cf., Dyscognitive, 2.3)."]

This is not the same as complex partial seizures. The 1981 version had a practical operational definition: “Impaired consciousness is defined as the inability to respond normally to exogenous stimuli by virtue of altered awareness and/or responsiveness”.

The 2013 version needs to define what it means by “dyscognitive”: Is it Section 2.3 of the 2001 Glossary or Section 9.2.1 or is it the 1981 definition or is it something else? The glossary definitions are cumbersome and impractical for clinical use. The problem is that consciousness is a very complex and poorly understood area that overlaps with philosophy. We don’t need to get into those arguments – we just need a practical definition that tells us what we need to know – i.e. is the patient responsive or not? The 1981 operational definition does that. Some patients with “dyscognitive” seizures by either of the glossary definitions do not have complex partial seizures (e.g. aphasia without impaired responsiveness or awareness). “Shorthand” is not a justification for imprecision. This objection is not trivial – it has important ramifications for things such as driver licensing. If the term “dyscognitive” is to be retained, please at least change its definition to the 1981 definition.

So what term to describe focal non-convulsive seizures with impaired consciousness? We already have a term for generalised non-convulsive seizures with impaired consciousness (viz. absence). Why not use “absence” to describe any non-convulsive seizure with impaired consciousness and use the adjective focal or generalized? Many non-experts call complex partial seizures “absences” already and clinical absences may occur in frontal lobe epilepsy with secondary bilateral synchrony. This would introduce some consistency and it would be easier for students and non-experts to understand. It would be clear what a “focal absence” referred to and that it was different to a “generalized absence”. The objection is that absence is a well-understood and entrenched term that refers only to generalized seizures. I would argue that it is not well understood by many doctors (including those who refer to CPS as absences). Sure, it’s not a perfect term but it has to better than “dyscognitive”! The alternative suggestion of “focal seizure with impaired consciousness or awareness or responsiveness” is a mouthful that no-one will use in clinical practice. And if we were going to be consistent, we would change “absence” to “generalized dyscognitive seizure”.

Instead of simple partial seizures, I can live with “focal seizure with retained/impaired consciousness or awareness”, provided it is modified. “Consciousness” invites philosophical debate and is difficult to test reliably. It has to be defined in a manner that can be used clinically. What matters is responsiveness. So the term should be “focal seizure with retained/impaired responsiveness”, using the operational definition of “responsiveness” in the 1981 version, which is practical and has stood the test of time.

There seems to have been an error in the change from the 2010 “without impairment of consciousness or awareness” to the 2013 “with retained consciousness or awareness”. The 2010 term means neither is impaired but the 2013 means one can be impaired. Was this intentional? If not, the 2013 version needs the “or” changed to “and”.

GENERALIZED : “Generalized” has been redefined to reflect the fact that the onset is not really generalized but the seizure becomes rapidly bilateral. So why retain the term “generalized”, especially when the Commission endorses “cessation of using old terminology”? The problem is that the definition refers to seizures arising “at some point within” i.e. focal! The attempt to circumvent this inconsistency by saying that the focus varies from seizure to seizure is unconvincing. Similarly, defining focal as originating "within networks limited to one hemisphere" would be a good definition if it reflected what really happens. Do we really think that networks involved in the origin of focal seizures do not involve the other hemisphere? Many limbic seizures would meet the proposed definition of generalised. Are we defining these networks anatomically or using circular reasoning (it was a focal seizure, so the network must have involved only one hemisphere; it was generalised, so the network must have been bilateral)? The problem here is that the 2012 document attempts to define a seizures according to pathophysiology and anatomy that are poorly understood. The 1981 classification instead defined generalized seizures in the way that clinical neurologists do: “…in which the first clinical changes indicate initial involvement of both hemispheres”. Why not accept that the current state of knowledge does not allow a clear and simple pathophysiological or anatomical distinction such as that proposed between most focal seizures and most generalised seizures and stick with the 1981 definition until something better can be proposed. Sure, there are some focal seizures, for example arising in the frontal lobe, that will be misclassified as generalised but the proposed definition will do nothing to stop that happening. It is a change that neither clarifies nor reduces misclassification and is difficult to teach.

FOCAL v PARTIAL:. I agree that "partial" is a misleading term and should be abandoned. However, substitution by "focal" is odd. The Commission recommends "the cessation of using old terminology" but then proposes an old term. It is interesting to read Gastaut's comments in his textbook (Gastaut & Broughton 1972): He argued that focal was an inappropriate term because the seizures it described arose in a network rather than at a focal point. Unfortunately, the term he proposed (partial), which had been coined in the previous century, was even worse, because of its usual meaning in English (= incomplete). Gastaut’s objections to focal remain valid. Can we not come up with a better term than focal? If the proposed “bilateral convulsive seizures” is accepted and the key component of the proposed definition of generalised is that the network is bilateral, then why not use the term “bilateral” for generalised and “unilateral” for focal? These are corresponding terms - if you understand one, then you understand both. To emphasise that the definition depends on the site of onset rather than propagation, the term “unilateral onset” or “bilateral onset” could be used (many people already use the unofficial term “focal/partial onset seizures”). It would be immediately apparent to students and non-experts what we meant without the need for circuitous definitions. “Focal seizure evolving to bilateral convulsive seizure” could become “Unilateral (onset) seizure evolving to bilateral convulsive seizure”. It says what it is!

ELECTROCLINICAL SYNDROMES: The 2010 document stated that this term was preferable to just “syndrome” because it added precision. However, why use a term that includes only EEG and clinical features? The 2013 document defines electroclinical syndrome as including more than just EEG and clinical (viz. imaging).  What about genetics and immunology? Are these excluded because they are aetiologies? If so, then imaging should likewise be excluded. Calling a syndrome an “electroclinical syndrome” does not add precision but calls something by a name that implies less than it is. The term may be of historical interest but do we need it?

ASSOCIATIONS: It’s great that we got rid of the 2010 “constellations” but now we have “associations”. This term is not defined but examples include MTL seizures with HS and gelastic seizures with hypothalamic hamartoma. Does this differ somehow from syndromes? If so, how? If they are syndromes, then let’s call them that.

SYMPTOMATIC: The list of aetiologies is great but we need a collective term for those that were previously lumped together as symptomatic. "Structural/metabolic" was perhaps not too much of a mouthful but "structural/metabolic/immune/infectious" is not practical as a group term. retaining "symptomatic" as a collective term for "structural/metabolic/immune/infectious" will do no harm and improve communication.

“The focus of the new classification is primarily on clinical practice…” Let’s not forget that these terms need to be understood and usable not just by epileptologists and neurologists but by all doctors (at least).

Ernest Somerville

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15 January 2014

The ILAE standardized classification and terminology for ‘‘epileptic seizures’’ of 1981 and ‘‘epilepsies and epileptic syndromes’’ of 1989 provided a fundamental framework for organizing and differentiating the epilepsies. However, a revision of these classifications was mandated by subsequent major technologic and scientific advances. Since 1997, the ILAE Task Force on Classification has made significant efforts to achieve better and internationally uniform classifications as reflected in the reports of 2001 (Engel, Jr. 2001) and 2006 (Engel, Jr. 2006). Contrary to this, the 2010 report of the ILAE Commission on Classification and Terminology, 2005–2009 (Berg et al 2010), was mainly confined to ‘‘new terminology and concepts’’ that met with considerable concerns by several epilepsy authorities (Commentaries 2010) and it was not approved by the ILAE executive. Unfortunately, these concerns were not considered by the new ILAE Commission on Classification and Terminology, 2010–2013 in the report of 2014 (Scheffer et al 2014) which is rather a superficial attempt to mend the previous report and avoid some of its striking errors (see for example abandoning “constellations”!) rather than making a new and useful proposal. This failure is apparent by reading the thoughtful current comments of expert epileptologists, including eminent ILAE officials, which convincingly debate against most of the changes proposed in the report.   In addition to these, I would briefly remind on matters that I have previously detailed (Panayiotopoulos 2011; 2012) which the task force appointed by the Executive Committee would wish to review.

Epileptic seizures are given by name only without defining them though a proper revised classification should incorporate advanced knowledge of clinical, interictal, ictal manifestations and pathophysiology when known. Reflex epileptic seizures and status epilepticus are conspicuously ignored (see details in Panayiotopoulos 2011) According to Pete Wolf ‘‘a classification of seizures is presented that is unrelated to the new concepts and is even more primitive than what was proposed in 1964. This is of no use’’ (Wolf, 2011).

Epileptic syndromes are also given by name only and thus we remain dependent on previous ILAE definitions, which are often broad and obsolete.  Using “age at onset’’ as a primary dimension for organizing the epilepsies is undesirable:  1. a considerable number of epileptic syndromes have a wide range of age at onset and 2.  syndromes that are likely to be linked together on the basis of electroclinical and etiologic evidence are now separated and intermixed with a number of heterogeneous epilepsies (Panayiotopoulos 2012). The result is avoidable confusion. Compare for example table 3 of the two reports.  Though it is said that “syndromes are unchanged except for minor changes in terminology” there are significant diversities and contradictions between these 2 tables and changes with controversial terminology are unjustifiable as pointed out by many contributors.

In addition, the proposed etiological grouping is also contentious as discussed in the commentaries (see also Shorvon 2011).

Conclusion and proposal
In conclusion, it is apparent from the current and previous detailed commentaries  that this report like its predecessor does not fulfil its intent to improve the previous classifications and should not be approved by the ILAE executive.  In our modern era of harmonization, this would cause significant disarmonization. The reports consist of mainly problematic and confusing terminological and conceptual changes, often with contradictions and little if any of the recent tremendous advances in the neurosciences has been considered.   We are in desperate need of an impartial new and objective classification of the epileptic seizures and the epilepsies.   The ILAE executive could benefit by asking experts in basic and clinical science to provide a concise statement in their field of expertise and define a. each epileptic seizure in regard to clinical and EEG manifestations, and their possible pathophysiology and b. each epileptic syndrome in regard to clinical manifestations, results of relevant testing, etiology, prognosis and management.  Areas of certainties and uncertainties, agreements and disagreements should be identified and stated clearly, with documentation of the reasons for it.  Probably this is the only way forward for a truly scientific, sound, and clinically meaningful classification/organizational system for the epileptic seizures and the epilepsies.  

Chrysostomos P. Panayiotopoulos

  • References
    Berg AT, Berkovic SF, Brodie MJ et al. Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia 2010; 51: 676-85.
  • Commentaries on the Report of the ILAE Commission on Classification and Terminology. Epilepsia 2010; 51:713–24
  • Engel J, Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy:  Report of the ILAE Task Force on Classification and Terminology. Epilepsia 2001; 42: 796-803.
  • Engel J, Jr. Report of the ILAE classification core group. Epilepsia 2006; 47: 1558-68.
  • Panayiotopoulos CP. The new ILAE report on terminology and concepts for organization of epileptic seizures: A clinician's critical view and contribution. Epilepsia 2011; 52: 2155-60.
  • Panayiotopoulos CP. The new ILAE report on terminology and concepts for the organization of epilepsies: critical review and contribution. Epilepsia 2012; 53: 399-404.
  • Scheffer IE, Berkovic SF, Capovilla P et al. The Organisation of the Epilepsies: Report of the ILAE Commission on Classification and Terminology. ILAE website 2014.
  • Shorvon SD. The etiologic classification of epilepsy. Epilepsia 2011; 52: 1052-7.
  • Wolf P.  Networks and systems, conceptualizations, and research. Epilepsia 2011; 52:1198–200

15 January 2014

Home greet all members of the ILAE and thank the many emails received to discuss the special report on classification and terminología.y strongly agree simplify this and make it universal.

For 30 years I work in epilepsy and three days a week with 32 patients a day specifically for this entity.

I want to know if they have considered focal seizures without impairment of consciousness, those that are initiated not only observable motor or autonomic symptoms but also with sensory Crisis: olfactory, gustatory, visual, auditory, abdominal (colic); psychic: panic, or versivas, dizzy, severe headache followed by 30 seconds to one minute motor, headache gradually improving in 30 minutes up to another paroxysmal presentation (2-4 times per day). These patients have EEG wave tip (pin) and / or focal or generalized paroxysms. Have a history of risk (TEC, Brain Obstetric trauma and others). No structural damage. 80% of them are teenagers.

It is important to include them in the standings because most of these patients are symptomatic and never improve, their quality of life is poor and unproductive.

Most are treated as migraine headaches or no improvement. 50% evolves generalized seizures, 25% do different focal seizures and the remaining 25% remains the same.

Thanking you in advance

Atte
Fidela D. Soto


15 January 2014

“ILAE commission on Classification and Terminology of Seizures and Epilepsies” deserves full credit on exemplary work done to make seizure, epilepsy and epilepsy syndrome terminology usable by clinicians in day to day practice. Though I have some comments and suggestions on same:

  1. The word organization has replaced classification and seems more apt at present as it denotes flexibility and possibility of future reforms and modifications.
  2. Dropping of terminologies like benign and catastrophic is justified as, many so called benign epilepsies are actually a spectrum and caregivers may get misled by term benign. Minor learning and behavioral issues were ignored under “benign” umbrella. 
  3. In etiology category term “Genetic” will be used for presumed genetic epilepsies and syndromes as well. To call them “idiopathic” seems more appropriate as without identified genetic defect it will be difficult explaining parents and family about genetic origin and providing them antenatal genetic counseling. Term idiopathic to categorize presumed genetic etiology and “Idiopathic Generalized Epilepsy” instead of “Genetic Generalized Epilepsy” is more valid in present scenario as many mutations are still unknown.
  4. Addition of “infectious” category in etiology is of particular importance to countries with high prevalence of tuberculosis, malaria and neurocysticercosis. Although “Infection” will be a more appropriate word.
  5. Keeping the option of assigning multiple etiologies is complicated and should be dealt in future with more clarity. Giving options as Genetic- structural (Tuberous sclerosis), Metabolic-genetic (Biotinidase deficiency), Infection- structural (Neurocysticercosis, tuberculomas) should be predefined for research purpose and documentation. In-fact on one aspect it is beneficial for patient management point to recognize multiple etiologies but glossary should be created to maintain uniformity.
  6. It is satisfying to see the way generalized is explained ie “rapid bilateral distribution”, “may not include entire cortex” and “asymmetry”. These phrases will remind clinicians not to get misled by pseudo-electroclinical-focality.
  7. The term dyscognitive is very useful in describing sequential semiology in focal seizures and terms unresponsiveness / altered consciousness / behavioral arrest can be replaced by term “Dyscognitive”. In classifying seizure people may still use the long term “Focal seizure with impairment of consciousness or awareness”.
  8. “Childhood Epilepsy with Centro-Temporal Spikes” is a welcome change replacing “Benign Epilepsy with Centro-temporal spikes” or “BenignRolandic Epilepsy”. It is well known now that learning and behavioral issues are not uncommon.
  9. Reflex epilepsy should be included in syndromes as it is difficult to place them any -where else. Other provoked and situational seizures also deserve a place.
  10. “Febrile seizure” is a unique condition, it may not be appropriate to keep it along epilepsy. Management and prognosis is different than epilepsies in general, so should be given a separate category.

I appreciate the task force for opening the discussion and valuing the opinion of its members. Hope my comments are useful and any further clarifications I would be happy to address.

Sheffali Gulati

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14 January 2014

Thank you for submitting this new document to our appreciation. Certainly, it represents a progress as regard to the previous one (2010) and the commission members must be congratulated for their important work. However, as no new proposal can be accepted by everybody as it is, I have the following questions and suggestions.

In general, I agree with most comments about the usefulness of changing terms that are largely used and not always less appropriate than the new ones. For example, it seems that “idiopathic” is preferred to “genetic” for generalized epilepsies such as Childhood absence E. and Juvenile Myoclonic E.  because of the psychological and social impact of the latter. Also “self-limited” may be understood by patients as not needing a treatment. “Pharmacoresponsive” would be better but difficult in practice.

Differences between “unknown” and “unclassified”? If I understand well, “unknown” is applicable only for etiology whereas “unclassified” is applicable not only for etiology but also for epilepsy type. For example, epilepsy with myoclonic absences is classified among the syndromes but its etiology is generally unknown; conversely, a patient with Tuberous sclerosis can have an unclassified epilepsy because it is not determined as focal, generalized or corresponding to a syndrome, but its etiology is known.

Table 2.
I completely agree for “focal motor” and “autonomic “seizures.
I am perplexed about “aura” to describe a “focal seizure involving subjective sensory or psychic phenomena only”. I wonder if it has been really used in the literature in this sense since 2001, date of the proposal in the glossary by Warren Blume.
As many others, I am more perplexed about “dyscognitive” for “focal seizures with impairment of consciousness or awareness”. Is cognition synonym of consciousness or awareness? A dyscognitive seizure is a very specific type of focal seizure and not only an event with disturbance of consciousness or awareness or responsivity (see the Warren Blume glossary).  Is it absolutely necessary to propose a short expression with the risk of making confusion? Why not the old “complex” term or the “focal impaired consciousness seizure” proposed in the comments? I thank Ed Bertram who returns to the Henri Gastaut concept which was the basis of the separation between the seizures with elementary semiology vs those with complex (elaborated) semiology, the former interesting only one system, and the latter involving different systems, mainly associative areas.
OK for “convulsive “ seizure that is very convenient.

Table 3.
Epilepsy of infancy with migrating focal seizures”.
Is it justified to eliminate the term malignant? Migrating focal seizures seems too imprecise because migrating focal seizures may be observed in other multifocal epilepsies, particularly in epileptic encephalopathies of various etiologies. I believe the term malignant allowed to immediately consider a specific syndrome.
The same remark for “Myoclonic epilepsy in infancy” that is too loose. I propose “Pharmacoresponsive myoclonic epilepsy in infancy” because myoclonic seizures constantly disappear although the cognitive development can be impaired in some patients. (see Guerrini et al, 2012).
For the same reason, I don’t understand what are “Self-limited infantile” and “Self-limited familial infantile.”epilepsies. Obviously they correspond to the epilepsies previously reported by Vigevano and by Watanabe, where the seizures are focal, with or without bilateralization. So, it would be preferable to change for “Self-limited focal infantile epilepsy” and “Self-limited, familial, focal epilepsy”.
As H. Oguni, I think that “Epilepsy with myoclonic astatic seizures” was more appropriate than “with myoclonic atonic seizures” given that it is almost impossible to differentiate these two sz types without a polygraphic video-EEG . Astatic does not imply a specific mechanism.

At the end it is amusing to read the title of the first reference, by Arsov et al, in press 2012, who report on GLUT1 –deficiency in the idiopathic generalised epilepsies!

Reference. Guerrini R, Mari F, Dravet C. Idiopathic myoclonic epilepsies in infancy and early childhood. In: Epileptic syndromes in infancy, childhood and adolescence (5th ed). M.Bureau, P.Genton, C.Dravet, A.Delgado-Escueta, CA Tassinari, P.Thomas, & P.Wolf, eds. 2012 John Libbey Eurotext Ltd, pp 157-173

Thank you again.

Charlotte Dravet


14 January 2014

I submit some comments to the Report of the ILAE Commission on classification and terminology about The organization of the epilepsies. The document is a commendable attempt to make order in a complex reality. A few comments:

  • For clarity and completeness, prior to the classification of the seizures, I would appreciate to state the definition of “seizure” (both self-limited and status);
  • the term “self-limited” replaces “benign”, but it can be confusing when applied to seizures;
  • the classification of seizures should include not only the self-limited seizures, but also the various types of status epilepticus;
  • the term “focal seizure with/without impaired consciousness or awareness or responsiveness”, unfortunately rather long for the current use, (text, p.4) loses “or responsiveness” in table 2; purposely?

With best regards,
Paolo Benn


14 January 2014

Many congratulations to the working group on agreeing this new organisation – quite a task! I think it is a sensible approach and will be useful in clinical practice. A few comments:

  1. Genetic versus idiopathic – I’m not sure how certain we can be about all the ‘idiopathic’ epilepsies being clearly genetic and wonder whether we should restrict the term ‘genetic’ to epilepsies where there is an identified gene
  2. Dyscognitive – in line with other comments I am unsure about this term – might be more appropriate to use terminology ‘focal seizures with/without impairment of consciousness’
  3. Febrile seizures – should this be included as a syndrome?
  4. Panayiotopoulos – is this an opportunity to remove the occipital basis – could it just be referred to as Panayiotopoulos Syndrome (without need for further qualification)
  5. Is there any useful way of capturing co-morbidities within this (or future) organisations?

Ailsa McLellan


14 January 2014

Thank you for the opportunity given to comment on the new proposals of organization of epilepsies. The followings are my comments:

1. I don't think there is general seizure. Each seizure has its biological reasons, it is difficult to image that all of the neuron or most of the neurons burst of abnormal activity at the same time without any cause. There must have been one or more genetic points, and then it rapiddly wide spread or activity at a local area. Different types of seizure stem from the different propagation.

1. I agree with the hypothesis of “network or mesh on seizure”. each type of seizure has its own particular network or mesh.

3. I am not appreciate the concept of "nonlesional epilepsy", every epilepsy patient must have been its abnormal structure. "nonlesional" – that is just because we can't find it at present. So I think "invisible lesion epilepsy" is suitable.

Thank you for your excellent work!
Duanyu Ni

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14 January 2014

I would like to congratulate and thank the commission on classification and terminology for the very hard and excellent work on such a complex and demanding matter. Thank you also for giving us the opportunity for comments in this forum. Here are few observations:

There have been a lot of discussions about the advantages and disadvantages of replacing the term IGE by the term “genetic generalized epilepsies (GGE)”, which the Committee members are surely aware of and take into account; I will only focus on a particular aspect with potential specific clinical repercussions.

As it currently stands, the term GGA does not exactly “replace” the term IGE in terms of absolute patient numbers. It practically includes only the “big four” (CAE, JAE, JME and GTCSa), leaving out a number of conditions (and patients) that cannot be diagnosed as (having) one of these 4 syndromes. Examples include eyelid myoclonia ± absences (the term as used in the new document only describes a type of absence, not a syndrome), absences before the age of 3, epilepsy with phantom absences and absence status epilepsy and a number of other patients with unclassified IGE. These conditions share the generalized-spike-wave discharge (the genetic EEG marker par excellence), while a genetic background is either demonstrated (Sadleir LG, Vears D, Regan B, Redshaw N, Bleasel A, Scheffer IE. Family studies of individuals with eyelid myoclonia with absences. Epilepsia 2012;53(12):2141-8), or seriously suspected. If these conditions remain unaccounted for (and practically outside GGE), their electroclinical characterization (for instance in a formal EEG report) would only be that of a “generalized epilepsy of unknown etiology”, omitting important clinical probabilities (i.e. absence of a structural brain lesion, favorable outcome and good response to Valproate amongst other) that are important for the management of these patients and would have been automatically implied by the term “idiopathic”, or if these conditions had been accepted syndromes of GGE/IGE (which is not the case). Another concern is that such electroclinical presentations may be squeezed by some into one of the big 4 accepted syndromes with obvious damaging effects on clinical and genetic research; a taxonomy place within the GGE (for example as “possible” GGEs) could be a solution.

Reflex epilepsies (of mixed aetiology even for homogenous syndromes such as Reading epilepsy) have been overlooked, and this has been pointed out by others. Some of these conditions (Reading or Language induce epilepsy, photosensitive generalized epilepsy or fixation off sensitivity) heavily overlap with IGE and may also be considered as possibly genetic.

Finally, Panayiotopoulos syndrome (which is also “self-limited” as the ECTS, table 3) has been reverted to the old incorrect term “early onset occipital epilepsy”, leaving out a significant number of children with this condition but without electroclinical evidence of occipital epileptogenesis. If (despite such good evidence for the contrary) we finally revert to the old term all good clinical work will be gradually undone and such children will be once more misdiagnosed. For these reasons the terms Panayiotopoulos syndrome and occipital childhood epilepsy of Gastaut should not be changed.

Once more, many thanks for the excellent work
Happy New Year to all,
Michalis Koutroumanidis


14 January 2014

I thank you for inviting my comments on this important ongoing effort. I congratulate the working group for the commendable attempt. I would like to present my views here:

1. I think the term “classification” is more appropriate. Even if it is imperfect, it is still a classification. One can draw analogy of classification of ataxias, which underwent refinements from pathological to clinical classification and then, to genetics based classification over time

2.     The term “organization of epilepsies” overlaps with “organization of epilepsy services” and this confusion may be avoided

3.   To develop a common language for communication among professionals, it is necessary to create a more rigid framework. In the present system, one is free to “organize” the epilepsies  as per his/ her preference.  This may actually impede the development of a common language

4. The present etiological categories are not all-inclusive. For example, post traumatic epilepsies without demonstrable structural changes on MRI cannot be included in the available categories. It is also unclear if endocrine conditions like hypothyroidism have to be categorised as “metabolic”. Reflex epilepsies are also not clearly organized.

I totally agree that continuous efforts are needed to refine the exisiting classification.

with many regards,
G.R.K. Sarma


14 January 2014

Comments about the treatment of West Syndrome:

  1. West syndrome is defined as the triad of infantile spasms, a pathognomonic EEG pattern (called hypsarrhythmia) and developmental regression. In our experience the presence of the clinical triad is uncommon. It is not possible to make a generalized prognosis for development in this patients due to the variability of causes, the differing types of symptoms and etiology.
  2. In our experience, the larger group of patients with West syndrome are those that present epileptic spasms combined with other patterns of epileptic attacks, specially, focal and tonic seizures. Great number of these patients presents developmental regression, while in the EEG recording, the presence of hypsarrhythmia is not that common. Etiology can be genetic, cryptogenic or symptomatic. Prognosis is poor and usually ends in epileptic encephalopathy.
  3. We have seen a specific group of patients presenting epileptic spasms, with scant developmental regression. The EEG (wake and sleep recording) shows paroxistic activity in posterior areas of the brain. Etiology is unknown. Prognosis is usually positive when treated with vigabatrin (good crisis control and development).   

Questions:
Should all these three group of patients (presented above) be considered in the same West syndrome classification?
Or patients in groups number 2 and 3 may be considered in a different sub classification inside the West syndrome?

Comments:
In our opinion the terms “pseudohypsarrhytmia”, “atypical hypsarrhytmia” and “unilateral hypsarrhytmia” should not be used because create confusion.

We believe that the idea to create additional groups inside the West syndrome classification will help to better diagnose and treat these patients.

Finally, we would like to propose a study including: clinical seniology, critical and intercritical sleep and wake video-eeg recording with a thorough etiologic study; to better diagnose and treat these children. We believe that with this “splitting approach” better results can be achieved.

Best regards,
Francesc X. Sanmartí

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14 January 2014

We thank the commission for the excellent work on the new organizational system. We applaud several elements-We are particularly pleased that epilepsy of unknown cause are designated as such, and not lumped into a “cryptogenic” category that suggests an underlying structural etiology that may not exist.

We are concerned, however, that the new organization does not allow categories that will guide therapeutic intervention. Strangely, in epilepsy biology often is less important than phenomenology for treatment selection. Genetic focal epilepsy (eg rolandic epilepsy) is still treated with drugs typically used for all focal epilepsies. Lennox-Gastaut, whether genetically based or structurally based, is treated in the same way (although of course this may change as we get smarter).

This revision states that “While the terms “generalized epilepsy” and “focal epilepsy” remain useful descriptors for many cases, there are, however, clinical settings in which a dichotomous approach is not supported by the electroclinical data. Therefore such an approach cannot form the basis of a biological classification.” This may be true, but it may indeed form the basis of a therapeutic classification, which is also necessary and must be acknowledged.

We are also very concerned with the change of “idiopathic generalized epilepsy to“genetic generalized epilepsy”. According to the document, these terms could be used interchangeably. This is confusing for two reasons. The first is that many patients with “IGE” do not have a known genetic cause. The second is that it will be very difficult for the non specialist (or even the specialist) to differentiate “genetic generalized epilepsies” (eg JME) from “generalized epilepsies that are genetic” such as Dravet. For non-epilepsy trained neurologists who cannot learn the intricacies of epilepsy classification, a simple understanding of the concept of IGE was essential, as treatment with broad spectrum agents is indicated, and treatment with narrow spectrum agents such as carbamazepine is typically avoided. Moreover, it is easier to say “no MRI is needed in a patient with a clear diagnosis of IGE responsive to AEDs”, than to say “no imaging is needed in a patient with genetic generalized epilepsy of the sort that does not produce structural abnormalities”.  We would argue that IGE is no more or less a category than Lennox-Gastaut Syndrome, which has been left in the classification despite a lack of even a common etiology. This does not mean that we insist on retaining the word “idiopathic”, which may also be confusing. We just suggest that more consideration be given to the name and character of this group.

The new classification will also be very problematic when considering clinical trial inclusion criteria. For example, a current clinical trial is enrolling patients with GTCC associated with IGE. In the future, the inclusion would be very complex. They would have to indicate " the presence of presumed genetic generalized epilepsy associated with generalized spike-wave, (even if spike-wave is not present on the specific patient’s EEG), and the absence of a genetic etiology associated with slow spike-wave, or developmental delay”.  
We ask that the proper consideration be taken before this classification is finalized.

Thank you for your consideration,

  Jacqueline French
Andy Kanner
Cynthia Harden
Greg Bergey
Shlomo Shinnar
Ed Faught
Ruben Kuzniecky
Greg Krauss
Martin Brodie
Dan Friedman
Mike Privitera
Mike Sperling
Steve Schachter
Antonio Gil-Nagel
Scott MIntzer
Michele Baulac
Christian Elger
Alexis Arzimanoglou

14 January 2014

Thank you for this opportunity to comment on the new proposals of organization of epilepsies.

Why is it so difficult to create a new classification of epilepsy, which would satisfy the majority of neurologists, psychiatrists, neurosurgeons, etc.?

In the 25 years that have passed since the last approval of classification of epilepsy, revolutionary changes in methods of brain research occurred; we have got a lot of new information about the etiology, genetics, and pathogenesis of epilepsy. New medications and surgical treatments were introduced into the clinical practice, as well as new social factors, changes in the environment have modified the course of the disease and this process will go and go on, causing new changes in classification.

From my point of view, replacing the terms of simple and complex focal seizures with longer definitions may complicate the formulation of diagnosis for both doctor and statistical services, if this new classification (organization) will enter the ICD-XI  ( is this process anticipated?).

Infectious etiology of epilepsy likely to be called post-infectious, as in acute stage of infectious diseases we use the term acute symptomatic epileptic seizure.  I prefer to leave the term symptomatic or to use new definition “structural-metabolic” with subsequent explanation (in the brackets) the exact etiology (e.g. structural post-infective epilepsy or structural vascular (post-stroke) epilepsy).And I like the term “cryptogenic” for a separate patient with newly diagnosed epilepsy, since this sounds more preferable than “unknown etiology” which may lead to patient’s confusion and thoughts about hopelessness for being cured.

In general, it will be difficult for me as a lecturer at the University to retrain our students, interns and young (not saying about older) neurologists to use new definitions and classification.

Lidiya Mar'yenko


14 January 2014

My comments to the EDITOR-IN-CHIEF  CNS Drugs to invited paper on "Recent advances of pharmacotherapy ofinfantile spasms" (accepted one week ago) have been following:

"The main proposal done by you is the Classification of IS. I am still hesitating to transfer the traditional classification directly to new one. However, I can do it if you sincerely wish the changes.

In this review I have used the traditional classification because they were used in the clinic studies discussed. In my opinion, the new classification cannot be directly compared with the former classification. Genetic group (although increasing) is a still a minimal group. “Unknown” etiology /Berg) seems not be similar with cryptogenic/idiopathic etiology. In many papers patients with “cryptogenic” aetiology are considered to be normal before onset of IS. Unknown aetiology would  also include some patients with symptomatic spasms when no etiology has been found. To transfer cryptogenic=”unknown” would bring confusion and mistakes in the data interpretation. I have also asked the opinion of the Epilepsy Department of HUS Children`s Hospital, Helsinki.

Some examples:
“cryptogenic etiology is characterized by normal neurological development before spasm onset and by absence of an identifiable cause, and symptomatic group associated with underlying abnormalities” Granström et al Epilepsia1999.

“infants were classified as to whether or not an underlying etiology for their spasms was known (Osborne et al 2010). “Where cranial imaging was not undertaken and history and examinations did not explain underlying etiology, the infants were classified as not fully investigated (a third Classication Group). Those in whom an underlying neurological etiology was identified were classified as proven etiology (O´Callaghan Epilepsia 2011)

“Aetiology ; non- identified” or “symptomatic” are the groups throughout the study by Lux et al  2004 and 2005 and ICISS study going-on in UK. Also Abstract from AES 2012 by well-known IS authors from US have used cryptogenic and symptomatic groups"

Raili Riikonen

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14 January 2014

I must acknowledge that this is very hard work. However, a number of points need to be considered:

Why call "generalized" seizures that are not generalized (how could there be several types of generalized seizures apart from several types of focal seizures secondarily generalized)?
“Focal seizures could originate from subcortical structures”: which structures? They need to be mainly glutamatergic: dysplasia or hamartoma? This should be mentioned.

Idiopathic epilepsies are not necessarily responsive to therapy although they will burn out. Before ethosuximide, we only had diones to control absences and many resisted to this therapy until they disappeared. Still to date, there are cases of absences who fail to respond to therapy. This remains true for “benign” rolandic epilepsy that may go unresponsive to therapy although it disappears many months later.

The concept of genetic cause should be restricted to those cases in which the gene is identified – or there is evidence from the pedigree findings or twin studies. Otherwise an alternative cause may be missed, as a consequence of considering the problem solved (“you just need to identify the gene”). We had this experience with focal seizures that were considered as symptomatic of a focal lesion until idiopathic rolandic epilepsy was identified and then frontal nocturnal epilepsy opened the genetic avenue. When a cause is unknown or only suspected, the best is to keep the category “cryptogenic” (in the pediatric understanding: it is not the lesion but the cause that is hidden).

Page 7-8, Dravet syndrome should not be considered as an epileptic encephalopathy since it was recently shown that epilepsy is not the major determining factor of cognitive delay or motor troubles (Nabbout et al, 2013; Nabbout et al, submitted).

Page 8 last paragraph, “inflammation” should be added since this is the basis of 2 causes mentioned earlier in the text: Rasmussen and the HH syndrome.

Page 9 first paragraph: pyridoxine dependent seizures should be replaced by pyridoxine dependent epilepsy. Furthermore, porphyria, uremia, aminoacidopathies are not very good examples because occasional seizures are more frequent than epilepsy. Creatine or GLUT1 deficiency, mitochondrial or peroxisomial diseases would be more appropriate. However, mechanisms of epilepsy in the case of glycogen storage disease are distinct from that of creatine or GLUT1 deficiencies, and they should not be included in the same category.

NMDAR encephalitis is not a good example (or with clear restrictions) since it was recently shown that epilepsy is strongly overdiagnosed in this context. Rasmussen and VGKC limbic encephalitis would be a better example. For HH syndrome and FIRES, inflammation is likely but still needs being established.

Infectious: like for inborn errors of metabolism, it seems odd to mix in one category stable sequelae of bacterial meningitis and SSPE that is a progressive disease. Stable sequelae, be they the consequence of ischemia, acute hypoglycemia (including from an inborn error of metabolism – b-oxidation defect or hyperinsulinism), bacterial meningitis, trauma, should be included into a “non-progressive brain lesion” category (although slow reorganization following brain trauma, herpetic encephalitis, a relapse of an inborn error of metabolism or a new ischemia is possible). This is distinct from a progressive disease like SSPE – the mechanism of epilepsy is distinct, and treatment also.

Unknown could not be as it is: it is not the same to be “unknown” because you live in a country with no imaging or genetic facilities, or to remain unknown although the work-up was done in an expert centre with all facilities. The first in my view is indeed unknown, for the second the cause remains “hidden”, thus cryptogenic, which is a different concept than idiopathic, the disease “by itself”.

Page 10: “does not enable the clinician to predict how the patient will present in terms of electroclinical semiology”. This is not very clear, since the type of metabolic disease determines a restricted range of phenotypic possibilities (Bahi-Buisson et al., 2013), and therefore it is roughly predictable, better than for genetic diseases (SCN1A, ARX for instance).

The term “unclassified” should be omitted since the authors wisely mention in the introduction that this is not a classification but an organization, and because the reality is that the etiology is cryptogenic, unknown (see earlier).

Congratulations.
Olivier Dulac


14 January 2014

Thanks for all the efforts provided. Difficult to comment on all issues discussed but I am sure the discussion will go on!

Two comments:

1) Unavoidably, every new report from the ILAE classification commission is perceived by the medical community as a "new" or "revised" classification, independently of our introductory comments.
From the point of view of a general neurology, or child neurology, treating physician what do you think would be the "clinically meaningful" difference that he/she will see between Axis 4 (Etiology) of the 2001 Report (Engel) and "Second Domain" of the proposed organization?

2) It took us nearly 15 years to get though the message that "idiopathic generalized epilepsies" are age-dependent, have a genetic predisposition and are investigated as well as treated differently from other forms of epilepsy, of a different etiology.
By pooling almost everything under the term "genetic generalized epilepsies" what are your arguments suggesting that this will work as a "clinical tool" for the treating physician ?

Do you consider "genetic predisposition" (which is a hypothesis) and "genetic etiology" as having the same meaning ?
For a given child with one or more types of seizures entering within the spectrum of the (old ?) "idiopathic" epilepsies, NOT sufficiently characteristic to allow a syndromic diagnosis and with full ignorance on the genes involved would you recommend to the treating neurologist to classify it under "Genetic Generalized epilepsy" or under "Unknown Generalized epilepsy" ?

In terms of further diagnostic investigations and/or treatment choices will you advice the junior neurologist or child neurologist to have a similar approach ?

Just for the sake of the discussion…

Best regards,
Alexis Arzimanoglou


13 January 2014

- Kudos for being inclusive in this massive overhaul which is overdue.

- I would suggest retaining cryogenic instead of unknown category of epilepsy.

- If possible retain simple and complex descriptive terms for focal seizures rather than dyscognitive  focal seizures.

Regards,
Suresh Gurbani


13 January 2014

It is indeed a great job at the ILAE for trying to bring out yet another classification/organisation in an attempt to give it better clarity, accuracy and user friendliness.

I greatly appreciate this great oppurtunity given to comment on the venture.

As commented by many of the senior Epileptologists, the attempt should be honest and pragmatic as it is since many decades and not a mere splitting the hair task, keeping in mind that not only researchers or Epileptologists but also medical students, general practitioners and even patients would need to know and understand about their disease and its implications in their life.

I agree with the comments made by Simon Shorvon,and Athansios Gaitalzis that the previously well established terminologies may be left untouched unless there found to have glaring mistakes/misunderstanding after enough research and evidence to prove the contrary.

I also join with the comments made by Peter Halasz that the facilities as well as the level and limitaions of knowledge in different scientific communities will influence the classification as is well known. Provision for unclassified groups in categories especially epileptic encephalopathies and genetic syndromes do help.

Dual pathologies need to be addressed somewhere as in the case of BECTS along with symptomatic epilepsy due to perinatal asphyxia where the "self limited" nature of BECTS is defeated by the refractoriness of the perinatal lesion as is the case of neuro developmental disorders/chromosomal disorders causing seizures with co-existing evidence of perinatal sequels in children.

Wishing you all success.

Manju George Elenjickal

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13 January 2014

Thank you for well prepared manuscript about organization of the epilepsies.

In my opinion the terms ”simple partial, complex partial, idiopathic generalized epilepsy  and secondary generalized epilepsy” are well defined and clear and no need to be replaced. There is no one word for “counsciousness and awareness” in any languages and diagnosis will be rather lengthy and complicated. The same is valid for “discognitive”.

The etiological categories have to be clarified and generalized. For example: page 9, (5)Infectious. Malaria, neurocysticercosis and cerebral toxoplasmosis are not infectious. How will be organized epilepsy with motor aphatic seizures in a patient with multiple small brain infarcts (structural lesions) due to systemic lupus erythematosus (autoimmune desease).

Best regards.
Panne Popov


13 January 2014

Thank you to the Commission for the opportunity to comment on the “Organization of the Epilepsies” proposal.  I agree with most of the concepts illustrated in the new manuscript. I only have some concerns that are shared by other previous comments.

The term “genetic” versus idiopathic could be confusing considering the existence of both idiopathic generalized epilepsies without a known or presumed genetic defect, and no idiopathic genetic  generalized epilepsies.

The use of the term “dyscognitive” replacing “complex” is confusing: patients with cognitive ictal signs and symptoms may retain normal consciousness; as others previously suggested, “focal seizures with/without consciousness impairment” could represent a good alternative.

Panayiotopoulos Syndrome should remain (extra-occipital focus included in the diagnostic criteria).

Including “Febrile seizures” among Electroclinical Syndromes appears to be confusing; it needs at least some explications.

“Self-limited” replacing “benign” appears to be ambiguous and easy to misunderstand; I agree with other previous comments that it could be simplified using only “Centro-Temporal Spikes Epilepsy” or “Rolandic Epilepsy”.

Best Regards,
Maria Matricardi


13 January 2014

My comments are on the attached pdf document.

Kenou van Rijckevorsel


13 January 2014

I'm a pediatric neurologist and member of Iraqi chapter agents epilepsy.

I read and follow the organization many time, rely I do not have such experience at the time being to say my comments and I highly appreciate your consideration to share all of us in that greet subject .

Hula Raoof Shareef

13 January 2014

Comments on "Organization of the Epilepsies" by CAAE:

1, We agree with the committee on most of the suggestions, such as replacing “benign” by “self-limited and pharmaco-responsive”, “genetic” being not the same as “inherited”. Particularly, we consent that the etiology of epilepsy can be classified into six categories and that the diagnosis of epilepsy consists of three steps, i.e., attack type, syndrome and possible causes. This classification is very practical and in agreement with current practice.

2, The committee’s proposal to develop a broad classification instead of being restricted in clinical applications is quite progressive and prospective. We like it.

3, We don’t agree on setting the “classification” as the caption of the topic, since diagnostic methods and substances are included in the committee’s proposal, other than only “classification”. Besides, we don’t think “Organization” is a good caption either, since it is rarely used and many clinicians may not be familiar with it. We would recommend “Approaches to epilepsy diagnosis”, it is essentially a language to consolidate both physicians and researchers, which makes it easier for communication.

4, “Electro-clinical syndromes” is not very intelligible and not covering new substances. We think “Epileptic syndromes” may be a better option.

5, An old method was adopted in the classification of epileptic seizure. Nothing is new except the name. Meanwhile, replacing “impaired consciousness or responsiveness or awareness” by “dyscognitive” makes it less accurate .

6, The committee didn’t classify the status epilepticus, which was in an individual category in 2001. We insist in the previous classification.

Those are for your reference.
Shichuo Li, M.D. on behalf of The Expert team of the CAAE Board


13 January 2014

First of all, I appreciate for the opportunity to comment on the “organization of the epilepsies” proposal. I think that you have done an excellent job in reorganizing seizure classification , and giving examples of electroclinical syndromes and other epilepsies.

Second, I just a few comment:

1. I think it is better that the term “focal dyscognitive seizure” replaces “complex partial seizure”). In my opinion the ‘focal seizures with impaired consciousness or awareness or responsiveness’ is too long and inaccurate.

2. I agree with the comments of Charles Raybaud (7 January 2014). And about "non-lesional temporal lobe epilepsy" , I would like "invisible - lesional temporal lobe epilepsy". I recommend "invisible - lesional " replaces "non-lesional".

Best regards,
Ping Zhang

   

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13 January 2014

Please find some comments on the new organization of the epilepsies.

I agree with some colleagues that the term Genetic is stigmatising and a bit misleading because in many cases in clinical practive we do cannot prove the genetic etiology  (at least in certain countries, this may be a long way to go). The term“Idiopathic” allows more freedom in explanation to families and is less “painful”. Besides, often Metabolic also means Genetic (in metabolically determined epilepsies). To use both terms in one patient? Like the suggestes Structural/Genetic in tuberous sclerosis? Which is true, but then – let us think also about the coding system according to ICD-10 or rather ICD-11? The new classification should fit well into the International Classification od Diseases.

As a child neurologist, I am not happy with febrile seizures attributed to epilepsy syndromes. I can understand the genetic-based scientific idea behind this, but what are acute symptomatic seizures in general then? Should we all of them call epilepsy, too? And how about the benign (or self-limiting) nature of febrile seizures, the most of which we do not treat with AEDs? Now, all pediatriacians and family doctors, also many families know that Fs is not the same as epilepsy in most cases, and many of them know when to be concerned and suspect epilepsy. I think this termination is not good from the view of clinical practice (although it may be true from the scientific point of view). I agree that a classification is expected to satisfy the needs of clinicians and scientists. But what is the priority? The patient is, I believe, thus the practitioners that are closest to the patient should not be confused, and the scientists will able to find and differentiate the FS, anyway...

I do not see much difference in terms “benign” and “self-limited” because they both mean the tendency to stop (and both have limitations, because not all the process stops along with the seizures). According to my experience, parents like the term “benign” because it gives hope. Unfortunately, I cannot find a good translation for self-limited to my Lithuanian language; nothing sounds as brief and understandable as “benign”. Is there a point just to change the wording if this does not change the meaning?
Regarding translation of the subclassification of focal seizures (I mean the level of consciousness impairement): the new proposal sounds long in all three seizre types while the old terms are brief and quite understandable. I like the term “focal” against “partial” (because partial seizure for me is “not complete” seizure, so how about when it becomes generalized?). Dyscognitive also sounds for me better that the very long new wording.

The purpose of seizure and epilepsy classification is to reach consensus in describing and understanding seizures and grouping different epilepsies in order to draw the best management plan, to avoid unnecessary investigations or potentially harmful medication, to provide advise reagrding follow-up, etc – the practical issues. For years, medical and non-medical community has used the terms that we are going to change. Does the background behind all these changes needs udate so badly that we may risk to cause new confusions?

Anyway, thank you for the big job the commission has done in trying to update and explain the terms. It is easier to criticize than to create. .. The idea to organize both seizures and epilepsies under one document seems very good, I wish the Commisssion find the best way to incorporate the new system (with some modifications, hopefully) into the new ICD.

Milda Endzinienė


12 January 2014

Thanks for the opportunity to comment. Thanks also for the efforts of the team in their thoughts, design and drafting, and to the many for their considered comments.

My impressions are not unique, and echo those of others:

- I am concerned that significant terminology changes will cause (further) confusion beyond the specialised community.
- I am concerned that invented terms such as 'dyscognitive' do not simplify understanding but rather confuse, and have the potential to be pejorative.
-Similarly, I am concerned with the use of the term 'genetic' when the risk of genetic transfer for the individual in many such syndromes is negligible, again leading to confusion, unneccesary anxiety and possible amplification of stigma.

The aims of re-organisation need to include clarity and harm-minimisation.

Thanks again for the efforts of the team.

Regards,
Ross Carne


12 January 2014

I congratulate the commission for the improved classification, which organizes etiologies into genetic, structural, metabolic, immune, infectious and unknown. The identification of electroclinical syndromes also helps to shape clinical pictures of common epilepsy disorders.

A few comments:
I liked the term “idiopathic generalized epilepsy” and I cant see the advantage over “GGE”, since in most cases the “genetic” cause cannot be proven either.

In my opinion, “dyscognitive” is too vague as a concept. The explanation: “ It is understood that dyscognitive may not always mean altered awareness but it is used here to denote altered consciousness or awareness which may be response tested” is also not very practical. We should be able to separate focal seizures with and without loss of consciousness, given our obligation to decide on a patient’s ability to drive or for other questions of eligibility. In that sense, complex partial vs simple partial seizures was not that bad.

Non-lesional needs to be defined: on the basis of CT? MRI? High-resolution MRI with specific protocol?

A section for reflex epilepsies should be opened. They can be generalized or focal, genetic or not, and it remains to be discussed if they are distributed across the different categories, or if they present their own category.

Margitta Seeck


12 January 2014

Thank you for the chance to comment on the new proposal for new organization of the epilepsies and congratulations to the task force for this achievement.

I support the idea that several etiological classes may be combined in the the case of one patient, eg structural-genetic for TS.

The term non-lesional is problematic (page 10, paragraph unclassified epilepsies). Is it not just another and less precise way of saying that the etiology is unknown? In the context of this paragraph, I suggest replacing ”non-lesional” with ”no identified structural etiology”.

It is a bit confusing that generalized seizures are classified to much detail and focal seizures lack all subcategories in Table 1, although they are described later in Table 2. Would it be reasonable to include the subcategories of ”with and without impairment of of consciuousness or awareness” in Table 1?

Eija Gaily

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12 January 2014

Thank you for the opportunity to share the ILAE Commission on Classification and Terminology in this interesting proposal about the Organization of epilepsies.

The replacement of “classification by organization” is well supported and I agree with this new approach. I have the following comments:

  1. The terminology “Benign” is not ideal to describe some types of what we categorize “mild types of epilepsy”, but the proposed term “self-limited” is not better and confusing regarding the term of describing underlying etiology, clinical course, responsiveness to AED, and prognosis.
    I prefer to maintain the used “Benign” terminology, till suggest a “better objective terminology”.
  2. Under Generalized and focal epilepsies: not always a dichotomy: I prefer for the changing of nomenclature denoting focal seizures, to use more “homogeneous nomenclature” as follow:
    1. Focal seizure with impaired consciousness or awareness or responsiveness (to replace Complex partial seizure)
    2. Focal seizure with retained consciousness or awareness (to replace Simple partial seizure)

OR

    1. Focal dyscognitive seizure (to replace Complex partial seizure)
    2. Focal seizure without cognitive impairment (to replace Simple partial seizure)

  Also I agree with Ghaieb Bashar Aljandeel> in his proposal to consider “Complex Focal seizure” and “Simple Focal seizure” as an alternative to “Complex partial seizure” and “Simple partial seizure”

  1. I agree to include febrile seizure in the new organization of epilepsies.
  2. In the new proposal suggesting etiological categories I think it’s proper to include under “Structural” : Primary structural and Secondary structural, where Primary structural include TSC, Polymicrogiria …etc and Secondary structural include Infections, Hypoxic Ischemic Encephalopathy, Stroke …etc. 

Issam Alkhawaja


12 January 2014

Only one comment: Replace West syndrome with "epileptic spasms of infancy"

70 percent present with IS without hypsarytmia and a large percentage have not stopped development , consequently dó not fulfil the criteria of West. syndrome.

The treatment and management is however completely identical as well as the prognoses (almost!) A pity to leave out these children from the syndrome

Best wishes,
Peter Uldall


12 January 2014

I'd like to congratulate the committee for this amazing continued process. Classification of epileptic seizures types, syndromes and etiologies is an important work and remains challenging.

- I'm not sure that febrile seizures should be listed in the epileptic syndromes, as it belongs to occasional seizures which are not described here. An explanation about the differences between epilepsy and occasional seizures may avoid over-medication of the latest.

- A word about consequences of early seizures in infancy on cortical maturation could be added, 1/ to emphasize the need of an "urgent" treatment and 2/ as it could explain the change in epileptic syndromes when children grow.

- I'm not confortable with the word "self-limited", as a treatment can be needed. Maybe the idea that it is transient more than self-limited could be used. The patients could hear that the disease will cure by itself and it could lower the treatment compliance.

- Infantile spasms should be included in both generalized and focal seizures, regarding  the heterogeneity of the etiologies and therefore, of the treatment with drugs and/ or surgery.

Best regards,
Mathilde Chipaux


10 January 2014

Dear Colleagues,

My main doubt on the current document regards the etiological categories. In order to be concise, I submit  to you the following etiological categories with few and brief notes:

- GENETIC EPILEPSY
Seizures are a direct expression/consequence of a genetic disorder and are the only or the predominant manifestation; epilepsy is, in all respects, a disease.

- EPILEPSY SECONDARY / ASSOCIATED TO A BRAIN PATHOLOGY / DISORDER
Brain pathology/disorder, with or without structural lesions, can be of any kind: metabolic, immune, infectious, traumatic, vascular, atrophic, tumoural… and genetic of course. In the latter case, the primary disorder has a demonstrated genetic cause, but seizures are the direct manifestation not of the genetic disorder but of the consequences of it; examples of this kind: seizures due to malformations of cortical development, seizures associated to various genetically determined metabolic disorders. In this way, the dichotomies structural vs genetic, metabolic vs genetic, which can easily generate misinterpretations, can be avoided.

As I have had occasion to express my though in the previous comment, in these cases, the definition of epilepsy as a disease has to be based on the clinical judgement and the terminology can be left to the physician on the basis of a global evaluation of the clinical picture in the specific patient. This can be particularly significant if we consider that the present document “has been designed primarily for clinical practice”. For example, if a patient has a calcified meningioma generating frequent epileptic seizures without any other disorder, although these seizures are a symptom of the tumoural pathology, they achieve the dignity of disease and the diagnosis of “tumoural epilepsy” can be formulated. Similarly, if a patient exhibits epileptic seizures triggered by a brain ischaemic area and these seizures are the most important/predominant manifestation, the diagnosis of “vascular epilepsy” can be made……..and so on.

By contrast, if a patient as a malignant rapidly progressive tumour with various severe neurological signs and symptoms, including epileptic seizures, it is more appropriate the definition of “epileptic seizures symptomatic of a cerebral neoplasia” rather than “tumoural epilepsy”.

Epileptic encephalopaties can be included in this category.

I am aware that the matter can trigger an endless discussion (as, we know, it is almost usual in a clinical setting and even, but to a less extent, in a scientific context !)  and that the present suggestions can be better organized and improved, but what I have suggested might be one of the various acceptable  solutions given the fact that the document (I repeat) “has been designed primarily for clinical practice”.

- EPILEPSY OF MULTIPLE ETIOLOGY (presumed or demonstrated) (multiple= two or more causes)

- EPILEPSY OF UNKNOWN (UNIDENTIFIED) ETIOLOGY

Minor point: although the reasoning leading to the term organization is correct, the term epilepsy classification completed by “on the basis of our present knowledge” could be maintained in the official document. In fact, given the practical purpose of the document, the term “organization”, although very clear in its intents, might result a little warped for the “clinical epileptologist community”.

Once again, thank you for this opportunity to comment on your very complex work and warm greeting for all of you.

Francesco Pisani

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10 January 2014

Congratulation for this interesting proposal on a new organization of epilepsies and thank you for asking for comments.

I have one comment in order to provide a complete classification of seizures. I suggest that commission should to include the Status epilepticus definition.

Kind regards,
Concepción Gómez Rives


10 January 2014

Thank you very much for the report and more importantly the valuable discussion. The discussion clarified many issues in the report.

I absolutely agree with other colleagues' opinion the guidelines are intended to ease clinical practice and to give the best quality of treating epileptic patients. No need to change everything. Reviewing the discussion and vote for the least important relevant changes help in applying the best management for patients. No need for a revolution. The discussion should be refined and added to the summary of the report of ILAE.

With best regards,
Sahar Hassanei


10 January 2014

Thank for undertaking this enormous work. I have a few concerns:

1) I would rather we used impaired consciousness or awareness instead of dyscognitive as cognitive funvtions may go beyong awareness.

2) I wonder whether we should really include febrile seizures in a scheme for the organization of the epilepsies.

3) I wonder whether "self limited" in the place of "benign" does not still suffer from the same constraints? Will a qualifier e.g. "mostly benign or self limited" be a useful addition?

thank you
Richard Idro


10 January 2014

I have already made my comments. However, I agree with the previous author's expectations for addition of two categories in the schema:

1. Stimulus mediated ( Reflex ) epilepsies 2. Status epilepticus with diffrent varieties including NCSE.

Thanks and regards,
Kavita Srivastava


10 January 2014

I like the proposed modification of the approach to seizure categorisation, no matter what it is to be called.

Specific comments:

In relation to reflex seizures, the descriptor: "with specific sensory/cognitive precipitation" [see Ritaccio], could easily be replaced with the term "triggered by ......." (arithmetic, music, etc), in much the same way as we may say "provoked by ......." (fever, syncope, medication etc).

Because "dyscognitive" sounds very specific but has acquired several meanings, it seems important to avoid it.

Experience with propofol during surgical procedures, indicates that individuals may remain fully conscious but be unable to remember the events of the procedure. Doubtless this happens with seizures, but is not often easy to evaluate. Perhaps, therefore, 'focal seizures with impairment of consciousness, awareness or memory' is what is required to categorise seizures without a clear mind.

"Aura" smacks of migraine and adds another name for a focal (sensory) seizure, so could be dispensed with.

Yours sincerely,
John Willoughby

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10 January 2014

I am grateful for the 'overhaul' exercise by ILAE and the authors. While the proposed changes are welcome and agree with their view, few additional points might require thinking:

1. In developing or resource poor countries/settings, often complete work up for etiology may not be possible especially for the genetic, immune or metabolic causes; in that case what will be situation? 'unknown' or should a 'separate category' be considered.

2. Another situation in the similar setting could be incomplete evaluation e.g. one gene or one immunological assay, which might be negative/ non-contributory and thereafter it might be clubbed under 'unknown', which might not be the correct way.

3. Under the 'infection' category- one commonly comes across acute symptomatic seizures (provoked) due to CNS infections, which could be sometimes recurrent in the acute setting. Some of the patients might then develop 'unprovoked seizures'; It is unclear here for the clinicians to categorize. Moreover, for example in Neurocysticercosis, unprovoked seizures are commonly due to unresolved speck of calcification and not due to acute infection.

4. There is no category for surgical remediable epilepsies!

5. 'Dyscognitive' term may be avoided - it may underused in future.

6. Deleting 'Genetic generalized epilepsy' might be a welcome step; we should continue to use 'Idiopathic generalized epilepsy'

Regards and Best wishes,
Sanjib Sinha


10 January 2014

Thank you for this opportunity to comment on the new proposal.

A. Since it is not really a new classification, i would propose to call this work : The comprehensive organization of the Epilepsies. It seems to me that the adjective " comprehensive"  bring subtile sense that it is not a classification but it bring to the epileptologist an emphasis on the two domains: electroclinical and etiology.

B. In the paragraph (B) 2 :  Structural
- For etiology, may be "Vascular" could be individualize (that it is more frequent that immune ad least in children): hypoxic-ischemic damage, vascular seq, sturge weber
- Moreover, I will propose to change the following sentence as: " This in turn highlights the need for consideration for epilepsy surgery should the patient - specially in children-  fail medical therapy, " and add two articles in the references:
1. Cross JH, Jayakar P, Nordli D, Delalande O, Duchowny M, Wieser HG Guerrini R, Mathern GW.Proposed criteria for referral and evaluation of children for epilepsy surgery: recommendations of the subcommission for pediatric epilepsy surgery. Epilepsia 47(6):952– 959, 2006.
2; Harvey H, Cross JH, Shinnar S, Mathern G and the ILAE Pediatric Epilepsy Surgery Survey Taskforce. Defining the spectrum of international practice in pediatric epilepsy surgery patients Epilepsia, 49(1):146–155, 2008

C. Finally, for the table 2 on the description of the focal seizure, it will great to add new considerations in order to deepen the organization of the seizure:
- with or without aura / if aura, specify: motor, autonomic sensory, psychic phenomena
- impairment of consciousness or awareness: no impairment/ altered awareness / with complete impairment

I am really very grateful for this work.I hope this comments will be fruitful

With my best regard
Christine Bulteau-Peyrie


10 January 2014

I would like to comment on the “Approaches to epilepsy diagnosis” from a practical point of view.

Efforts (and possibilities) to find both electroclinical and etiological based diagnoses depends in every country on one hand on available diagnostic capacities and on the other hand on how much the physicians are decided to reach the end with the diagnosic procedure (undrpinned by the conviction that such effort is meaningful in terms of possibilities to choose best therapies). The later aspect is strongly related to the level of knowledge in epileptology.

Therefore I would emphasize to work out our regional standards about what should be done in the course of diagnostic procedure in certain types and syndromes of epilepsies. I am aware about the diversity of sublevels in the health care system’s levels even within continents, regions and countries. However I am convinced that we should give certain guidelines in this regard (worked out by the different Leagues of ILAE), which will exercise some kind of attraction.to use up to date facilities both in electrophysiological, neuroimaging and neuropathological diagnostics.

Peter Halasz


10 January 2014

Thank you for continuing to remind us about this important business. I applaud the new approach of Organization rather than a classification and like the framework proposed by the authors. I hope that may of the comments will get serious consideration and provide us a good product at the end.

One concern I have is that a substantial number of patients suffer from conditions that mimic epileptic events and often get diagnosed with “epilepsy” but do not have true epilepsy. Depending on the study, 15 to 85% of patients who present to epilepsy centers have been found to have non-epileptic conditions that masquerade as epilepsy. In my own series that spanned a period from March 1995 to February 2006, of the 1,648 records reviewed, 465 patients (28 %) were found to have what I prefer to refer to as Non-Electrogenic Epileptiform Disorders (NEEDs). Of those patients with NEEDs, 359 (77.2%) were on 1 to 5 AEDs with an average of 1.6 per AED per patient at the time of diagnosis o NEEDs by AVEEG monitoring. 106 (22.8%) were not on AEDs. Based on the estimated daily dose per patient for their drugs at that time, the average annual cost was $2295.72 per patient for medications alone. Although most patients had psychogenic events, several were due to physiological events such as cardiac arrhythmias, sleep disorders, and aphasic, hemiplegic or confusional migraines. Considering the economic cost of ER visits, hospitalizations, and the physiologic burden of un-necessary medications, this group needs recognition by their inclusion in the group of “epielpsies”, granted that these are not truly epileptic.

These patients need to be grouped somewhere and it may be time for us to include such cases in the organization of the epilepsies, considering that the group itself is heterogeneous and “syndromic” rather than “phenpmenologic” and as such a poor fit for inclusion in the classification of seizures.

Thank you again for your persistence and the opportunity to comment on this matter.

With best regards
K.J. Oommen


10 January 2014

I thank the ILAE Commission on Classification and Terminology for giving me the opportunity to share opinion on The Organization of epilepsies.

1. Classification of seizures: If the term Dyscognitive is to be used as a “shorthand” for impaired consciousness or responsiveness or awareness, I would like to suggest the term “Eucognitive” for retained consciousness or or responsiveness or awareness to avoid long phraseology.

2. Electroclinical syndromes: The term ‘self-limited’ is proposed in the organization of epilepsies to replace the word ‘benign’ but it appears to give an impression that epilepsy remits without need for any treatment. The term ‘age-limited’ may sound more appropriate as all these electroclinical syndromes start and remit at certain ages.

3. The etiological classification has been made more elaborate by dividing into 6 categories. Some of these categories especially infectious and metabolic may actually be acute symptomatic seizures and fall out of the scope of the definition of epilepsies, and may not require anti-epileptic medication but respond to specific treatment such as pyridoxine (pyridoxine dependent seizures), biotin (biotinidase deficiency) etc. Neurocysticercosis/tuberculomas is another example where seizures begin, at least initially as acute manifestation of the neurological insult caused by infection and antiepileptic medications are used for the duration of the presence of lesion.

Sincerely,
Jatinder Singh Goraya

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10 January 2014

The ILAE Commission has done a great job trying to forward the issue of classification of epilepsy in the light of new scientific developments. I would like to add some comments:

1. Etiological classification
The new manuscript of the ILAE Commission on Classification and Terminology expands on the previous Organization proposal (Berg et al. 2010) adding Immune and Infectious causes to the list of etiological categories. In this respect, I would like to suggest the addition of “Neurodevelopmental” conditions as an important category. This would include conditions such as Intellectual Disability, Autism Spectrum Disorders and Attention-Deficit/Hyperactivity Disorder, as well as Schizophrenia and Cerebral Palsy. All these conditions share a bidirectional epidemiological association with epilepsy (i.e., the presence of one condition increases the risk of the other and vice versa) suggesting shared underlying mechanisms. The latter is further supported by the growing recognition of common genetic causes for these disorders. People with neurodevelopmental conditions may suffer from different seizure types, and they may have MRI scans and EEG abnormalities that do not allow classification under the dichotomy General vs. Focal or Idiopathic/Genetic vs. Symptomatic/Structural vs. Cryptogenic/Unknown, or under one of the electroclinical syndromes. Therefore, a separate etiological category will hopefully help the clinician to guide investigations and further management (i.e., neuropsychological & behavioural assessment, genetic testing, detection of comorbidities; educational help, treatment of comorbidities, etc).

2. Electroclinical syndromes
The classification retains the terms “Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)” and “Landau-Kleffner syndrome (LKS)”. Both are CSWS-related conditions and have recently been described within the so-called “Epilepsy-Aphasia Spectrum”. In addition, a number of other CSWS-associated idiopathic/genetic or symptomatic/structural conditions have also been described (i.e., Acquired Opercular Deficit, Frontal Deficit with/without Autistic Regression, Atypical BECTS); these are not represented anywhere in the classification. As a recent survey has clearly shown, the use of CSWS (and ESES) is a cause of major diagnostic confusion and should be abandoned in its current form. Perhaps, the Committee should try to re-classify these conditions under one name (i.e., CSWS-related encephalopathy”) or  -at least- change the acronym of Epileptic encephalopathy with continuous spike-and-wave during sleep from CSWS to ECSWS (E, standing for epileptic encephalopathy), so as to avoid diagnosis of an electroclinical syndrome based on EEG findings (as the term CSWS implies).

3. Change of terminology
I agree with the comments of many epileptologists who raised concerns about the usefulness of terms such as, “dyscognitive”, “GGE”, “Early onset occipital lobe epilepsy” (instead of Panayiotopoulos syndrome), “BECTS” (instead of Rolandic epilepsy), or the replacement of Secondarily Generalized seizures. For example -and in my experience- the use of BECTS may be misinterpreted, as some paediatric neurologists make this diagnosis in children with centrotemporal spikes on EEG irrespective of the seizure type.

The proposed terms seem in many ways nothing more than a descriptive expansion of the previous terminology. The transition to a classification system based on (A) the well-established electroclinical syndromes and; (B) etiologically based diagnoses does not advance the field, contains significant overlap (as different categories may co-exist), and -in addition- creates problems with designing and interpreting epidemiological studies. As Simon Shorvon has suggested, the “well established terminology should be preserved unless there are good reasons not to do so.”

Athanasios Gaitatzis


10 January 2014

I don’t think that “focal dyscognitive seizure” captures the essence of an impaired level of consciousness that we assume for a complex partial seizure.  Perhaps this has to do with what we clinically infer clinically when consider the terms cognition vs. consciousness. Using the workforce’s understanding and use of the terminology, I don’t fully understand why a simple partial seizure becomes “focal seizure with retained consciousness or awareness” rather than “focal seizure with retained consciousness or awareness and responsiveness.”

Kevin M. Kelly


10 January 2014

Thanks for the laborious work of the commission. I have read it for seveal times and have found it excellent. I think that the proposal is a great advance for clinical diagnosis and reseach on epilepsy, and then will benefit the diagnosis and the choosing of therapies for clinician, and I think it will be further improved. As a clinician of neurology, I agree with the changes in this proposal. However, I have a few comments below.

1. the replacement of the term complex partial seizures is good, but it will be confusion that dyscognitive can also be used at focal seizure with impairment of consciousness or awareness. I think the phrase “impairment of consciousness or awareness” is distinct and enough. As impairment includes the conception of different degree, extent and content of consciousness or awareness.

2. Using the term self-limited in this proposal is welcome, because Self-limited replaces “benign” will benefit for patients to establish the confidence to overcome the disease, the term of “benign” always give some patients incorrect conception that the disease is not harmful, and this will drive them not cooperate with their doctors.

3. Simple classification or organization is not the destination, the destination is solving the problems in clinical and reseach on epilepsy. I suggest that the suggestions or guideline of epilepsies according to this proposal should be published as soon as possible, in order to judge the advantages and disadvantages of the proposal in practice.

Sincerely,
Wang Miao


10 January 2014

I appreciate the opportunity to provide my opinion and in a very special way exalt the efforts of the Commission, I leave you my comments below:

1. the definition of the title of this text is not clear, I think that we should work from the point of view of semantic and semiological aspects, to describe if the proposal is classification, organization or manual of diagnosis.

2 I disagree with the change in the terminology of complex focal seizures.

3 I disagree with the change of terminology from primary generalised Epilepsies, or idiopathic.

4. I support the change of the term benign by self-limited

5. I suggest to continue using the description system diagnosed by axis, which is more broad descriptively and also allows you to focus on the case of the patients in any field, both at the clinic, as in research and at different levels of complexity in care medical, particularly in the middle where i work (Latin America, Colombia), because the involvement and applicability of the changes in the model of diagnostic, entail changes in the legislation, education and therapies offered for people with epilepsy.

6. I suggest to check the manual designed from WHO and PAHO in conjunction with some Latin American colleagues for the diagnosis and treatment of epilepsy in primary care. (AIEPI - integral attention to prevalent disease in infancy). To be implemented or adjusted elsewhere according to the expectations of each country. View pdf.

Thanks again for the wonderful work of the team.

Carlos Ernesto Bolaños Almeida

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10 January 2014

Thank you for the opportunity to comment on the Organization of the Epilepsies. It is a major step forward and the authors really have to be congratulated.

As a pediatrician I have practical questions;

Can classification be established in levels?

1. If we included febrile seizures in Epileptic seizures, shall we do that after first attack or after second, third... and when shall we start with other diagnostic procedures according to epileptic seizures (EEG, MRI, genetic...). Answering this questions (determine it in classification) we all can spare lot of money and time wasting on expensive procedures for children with first episode of usually simple febrile seizure.

2-The term 'genetic generalized epilepsy' might give false and very 'harmful' message to parents of children having epilepsy, about the role of 'genetics' in epilepsy. If genetic epilepsy can be differentiated according to heritability quotient (probability to appear) it will be less harmful for patients giving them information, and taking decision to have another child in case prenatal diagnosing procedure can not be done.

I think we shall construct a diagnostic manual defining diagnostic procedure in few levels, according to clinical observation, EEG, MRI, and further more sophisticated methods (which are not available in great part of word). In this way we well decrease the amount with unclassified epilepsies, even using term PROBABLY ..

Many thanks
best wishes and regards
Marija Knezevic-Pogancev


9 January 2014

After much thinking about the epilepsy classification I have this other thought. If the classification is based on pathophysiology underlying the seizure, any form of disturbance of consciousness should be classified as generalized epilepsy as follows:
1. Generalized: Non- Convulsive- absence, complex partial seizures
   Convulsive- tonic clonic, clonic, tonic; Jacksonian(partial with secondary generalization), .
Myoclonic
2. Partial or Focal - sensory,motor, autonomic, visual, auditory, smell - without any alteration of sensorium

After much thinking about the epilepsy classification I have this other thought.  If the classification is based on pathophysiology underlying the seizure, any form of disturbance of consciousness should be classified as generalized epilepsy as follows:
1. Generalized: Non- Convulsive- absence, complex partial seizures
Convulsive- tonic clonic, clonic, tonic; Jacksonian(partial with secondary generalization), .
Myoclonic
2. Partial or Focal - sensory,motor, autonomic, visual, auditory, smell - without any alteration of sensorium

Thanks so much.
Sincerely yours,
Maria Lina Renales


9 January 2014

Let me quote from your paper "in 1964 by Henri Gastaut and colleagues with the first ILAE proposal for a classification of seizures "all attempts at classification of seizures are hampered by our limited knowledge of the underlying pathological processes within the brain and that any classification must of necessity be a tentative one and will be subject to change with every advance in scientific understanding of epilepsy" (Gastaut et al 1964).

If there is something we have always known but disregarded only to "rediscover" in recent times (under the term "epileptic encephalopathy") is the fact that cognitive and emotional factors are an intrinsic element within the epilepsies. We have always minimized that component (relegating it to "comorbidity" at best) but it stubornly returns once and again. Referring to Gastaut's statement cited above, that fact implies that Epilepsy is not a paroxysmal condition but a permanent one that may present some paroxysmal manifestations (such as seizures). Indeed, that is why routine "intercritical" EEGs are abnormal!! True, in some patients and in some epileptic conditions  cognitive and emotional elements are not prominent. In many cases, this only means that those elements where not looked for hard enough. But even for those cases in which they are truly absent, that argument is only as true as is the fact that in Landau-Kleffner syndrome (and no one disputes it is an epil eptic condition) seizures can be totally absent!
So in short: "our present knowledge of the underlying pathological processses..." (Gastaut 1964) clearly show that epilepsy is NOT defined by seizures. Yet we stubornly insist on anchoring the basics of epilepsy around seizures.

This is not to ignore the presence of seizures of course. But we do need to acknowledge what is of the essence and what is just one of the posible manifestations, albeit strong.

As for the rest of the proposal, my sincere congratulations for being able to depart from the passionate adherence to minute seizure manfestations  that was threatening to make the classification a second edition of Phrenology, one century later!

Thank you very much for your effort and your disposition to submit the paper for comments

Sincerely,
Jorge Eslava-Cobos


9 January 2014

Thank you for the opportunity to share opinion on The Organization of Epilepsies by the ILAE Commision. I agree with the comments of Pete Engel (15th- November) and Simon Shorvon (6th-January) about Organisation vs Classification and Changing Terminology.

The widely accapted term of IGE as well as SPS, CPS and secondarily generalized seizures  should be maintained. The term genetic in GGE may lead to misunderstandings that it is inherited and can have a great impact on the patients and their families if the primary care physicians only have very limited time for medical consultations or are not well trained in epilepsy.

Best regards,
Thuy Nguyen Thanh

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8 January 2014

The commission should be congratulated on its work. They have thoughtfully approached the epilepsies and constructed a new organization which has merit. I have some concerns, however, to mention.

I appreciate the need to replace the term "idiopathic" in the prior classification. The term "genetic" is appropriate but may be easily misinterpreted, since it can include various types of epilepsy ranging from relatively benign forms without significant neurolgoical co-morbidity, (and in which prognosis is good), other types associated with severe epilepsy and serious neurological impairment, and yet others producing separate metabolic or structural defects (e.g., pyridoxine dependent, tuberous sclerosis) that fundamentally differ from the others. Given the ambiguities that can be associated with the term "genetic," clear distinctions should be considered within this category that helps distinguish between the mild (usually relatively benign) forms and the more severe forms. This is important for reasons related to need for diagnostic testing and management. For entry in therapeutic trials, this distinction would be critical. The present classification has the advantage of drawing a line with diagnostic and therapeutic implications, and the new organization should do so as well while improving upon it. There is no reason to use the term "idiopathic" when we have good reason to believe that a genetic cause underlies the epilepsy, but we need to keep clear distinctions between childhood absence, Dravet's syndrome, and TS, for example.

I suggest that the other, non-genetic causes be grouped into an 'Acquired" category, which could include infectious, immune, traumatic, tumoral, cerebrovascular and other causes. These could be further subcategorized by the seizure types - those with focal seizures, those with generalized seizures, and those with both types. The etiology could be a characteristic of these subcategories. Given that the diagnostic evaluation and treatments are the same for most types, I do not understand the rationale for splitting them into separate groups (except for autoimmune, which would still merit a subcategory and thereby be easily distinguished). Moreover, the line between some of the proposed categories is not particularly clear. For example, some infectious and structural epilepsies are thesame. The most common infection world-wide, cysticercosis, causes structural lesions. The autoimmune epilepsies can also cause structural abnormalities on occasion. Post-traumatic epilepsy is also a common etiology/type, but not mentioned in the organization and may or may not produce an obvious structural lesion. I am skeptical as to the advantage of including some causes such as immunological ones within the organization, but excluding others. Naturally, the unclassified category must remain.

In addition, I would favor dropping the term "dyscognitive." From a practical standpoint, impairment of consciousness is a critical distinction, and "dyscognitive" seizures can include patients with aphasic seizures who could still drive a motor vehicle under the old terminology, since the seizures fit in the simple partial category. For better or worse, governmental authorities, insurance companies and others now all know the difference between complex and simple partial seizures, and while replacing them with the longer terms "with or without impairment of consciousness" is more precise and perfectly fine, I believe we should discourage terms like "dyscognitive" which will be open to misinterpretation (see Dr. Blumenfeld's comment).

Lastly, I would include the subdivision of types of focal seizures in Table 1 where the subtypes of generalized seizures are listed. I see no reason to list the generalized seizures in detail and not include the subtypes of focal seizures. I would also replace the term seizure "classification" with "organization" and would include an organizational category of "unclassified." For practical reasons (databases, validation of schemes), it is useful to have a listed category that means "we don't know.";

Sincerely,
Michael Sperling


9 January 2014

I have one additional comment to add to those that I made on 1/8/14. I echo the concerns of many regarding the elimination of the term "idiopathic generalized epilepsy." To list syndromes according to age of presentation rather than by semiology and electroclinical findings is to lump disparate syndromes with no common features together. Childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy and adult-onset generalized epilepsy have clinical and EEG features in common and should be considered together, as they were in the previous classification.

Sincerely,
Denson G. Fujikawa


8 January 2014

In general, I think that you have done an excellent job in reorganizing seizure classification, classification of focal seizures according to the degree of impairment, and giving examples of electroclinical syndromes and other epilepsies.

I have one comment regarding the last category--electroclinical syndromes, with respect to those presenting in "adolescence - adult." Dr. Berkovic has pointed out that idiopathic generalized epilepsy may present well into adulthood, and therefore should not be classified as "juvenile." The category "Epilepsy with generalized tonic-clonic seizures alone" may pertain to members of this group, except that there are cases in which myoclonic seizures and/or absence seizures as well as convulsive seizures present during adulthood. It might be better to classify those with idiopathic generalized epilepsy according to age of presentation - childhood, adolescence or adulthood, since those with IGE presenting during adulthood may show all three types of seizures - absence, myoclonic and convulsive, two of the three or only one of the three (that being convulsive seizures). What they have in common is the typical EEG signature of frontally predominant, bilaterally synchronous 2-3 Hz spike or poly-spike and slow-wave discharges.

Sincerely,
Denson G. Fujikawa


8 January 2014

There has to be some simple logical reckoning within a new classification scheme on sensory/cognitive precipitation ("reflex") phenomenology.

The category has been an inappropriate orphan too long. Reproducible precipitation of stereotyped seizures by established sensory/cognitive triggers are, by physiologic imperative, imbedded in both focal and generalized paradigms.
The most accurate solution is to include "with specific sensory/cognitive precipitation" across all seizure types.....along side other proposed qualifiers like "with dyscgnitive features".

Then, finally, the misnamed "reflex epilepsies" can finally and appropriately merge into the syndromes they embellish. Only then, may we begin to emerge from the late twentieth century silliness of "teeth-brushing epilepsy", "epilepsia arithmetices", and the like. Thank you.

Anthony L. Ritaccio

1. Reflex seizures.
Ritaccio AL. Neurol Clin. 1994 Feb;12(1):57-83. Review.

2. Reflex seizures and reflex epilepsy.
Xue LY, Ritaccio AL. Am J Electroneurodiagnostic Technol. 2006 Mar;46(1):39-48. Review.

3. Cognition-induced epilepsy.
Ritaccio AL, Singh A, Devinsky O. Epilepsy Behav. 2002 Dec;3(6):496-501.

The role of dominant premotor cortex and grapheme to phoneme transformation in reading epilepsy. A neuroanatomic, neurophysiologic, and neuropsychological study.
Ritaccio AL, Hickling EJ, Ramani V. Arch Neurol. 1992 Sep;49(9):933-9.


7 January 2014

I am not a clinical epileptologist, but a neuroradiologist who has been involved in epilepsy for almost 40 years, starting in Marseille under the patronage of Henri Gastaut. Obviously, the main evolution since the Berg paper of 2010 (the introduction of a domain of specific etiological classes) is for me a significant advance in the Organization of the Epilepsies.

I have only three comments to make.

The first comment in [B] Etiologically based diagnoses, [2] Structural, concerns the first sentence “A structural etiology refers to abnormalities visible on structural neuroimaging where the electro-clinical assessment etc.”. What about the cases in which an appropriate MR study with specific protocols and high field and considerable  experience of the neuroradiologist fails  to find anything, while neuropathology (after surgery is performed according to MEG data) discloses an obvious focal cortical dysplasia? I would think that the concept of structural epilepsy should include the MR-unremarkable, histologically characterized lesions.

The second comment, purely semantic, is about [5] Infectious. I would recommend Infectious/Parasitic rather.

The third comment refers to [C] Unclassified epilepsies. In the second paragraph mention is made of “non-lesional temporal lobe epilepsy”. I may have missed it but I found no definition of what a “non-lesional epilepsy” is in the rest of the article. And I have heard different clinicians using it diversely for epilepsies without neuro-imaging abnormalities, epilepsies with no structural abnormalities, and “cryptogenic” epilepsies. This paper is so clearly written, with so much emphasis on the importance of semantic details, that I feel the term  “non-lesional” should either be defined, or excluded.

Overall this is a very good and useful work. It really provides a better vision of the way epilepsies should be understood. I like it.

Best,
Charles Raybaud

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7 January 2014

First of all, I want to thank the members of The ILAE Commission on Classification and Terminology for this careful work. I would like to comment on a few points

1. Should the febrile seizures be included in table with EPILEPSY SYNDROMES

2. The term 'genetic generalized epilepsy' is probably misleading and not very accurate. It might give a false but also 'harmful' message to patients about the role of 'genetics' in epilepsy. The current term 'idiopathic' may be even better

3. ‘dyscognitive' is shared by a lot of comments. It is an obscure term, and also not true! the old term 'complex partial seizure' or 'simple partial seizure' are more descriptive more practical and easier to understand

4. ‘self-limited' that replace 'benign' may be give a picture to epileptic patients that it does not need 'treatment' which is not always true. This may negatively affect the 'compliance' of epileptics

5. Regarding etiological factors for epilepsies, my suggestion according age group should be added to the causes to be more informative and accurate.

Best regards:
Ayied Mottub


7 January 2014

It is indeed a major task to undertake the organisation of epilepsies encompassing all the major changes in knowledge that have happened ever since the start of the process over several decades.

The proposed new organisation is simple and useful specially the replacement of the term complex partial seizures. Using the term self-limiting here is welcome too.

One of the purposes of the organisation should be to be able to provide in a snapshot 1.The possible type of seizure the patient has single or multiple. 2. What is causing this- genetic, structural, combined etc. This has to some extent been dealt with in the new organisation. Including autoimmune epilepsies is also welcome. Making sure that the surgically remediable syndromes are listed separately will increase awareness for an early surgical referral which in a developing country like ours is still not forthcoming.
For the developing regions where stigma and discrimination is high a diagnosis of GGE on an OPD prescription slip/ diagnosis could create chaos in the marriage for a young girl and hence the term idiopathic must remain.

However at the end of the day the most important part of what we as clinicians look forward to as a promise to our patients is how a particular person with epilepsy (PWE) will do and what impact will the organisation have on the patient and the caregiver and there family. Also whenever research is required funding is needed. The last but most important issue remains unaddressed in the present organisation, unlike the one put forth by Prof Engel which had a specific axis for severity/impact/disability/comorbidity. Adding this to the current system will make it more holistic for counselling the affected and for justifying resources for research in those epilepsies which are difficult for both the person with epilepsy and the clinicians managing them. as this both for the clinician researcher and the patient.

Too frequent a change in any system can also be challenging to clinicians specially in resource poor settings as they already face a challenge in managing the huge numbers hence any change should be simple and practical for a universal use.

Manjari Tripathi and P. Sarat Chandra


7 January 2014

Congratulations for the hard amd effective job. I have only two concerns: should the febrile seizures be included in table with EPILEPSY SYNDROMES - I think it needs at least a comment. I fully agree with previous comments that we should replace the West syndrome with infantile spasm. And my personal minor concern – I like quite much the term discognitive however it is very hard to translate this term into polish language in order to make it understandable but we will try our best!

Best regards,
Maria Mazurkiewicz-Beldzinska

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6 January 2014

Comments on the Organization of the Epilepsies

My comment and the points I want to raise may seem a repetition for the opinions of others because of being late to comment on one side and also because those who participate so far raised almost all the important suggestions. I may tabulate my suggestions as follows:

1-The term 'genetic generalized epilepsy' is probably misleading and not very accurate. It might give a false but also 'harmful' message to patients about the role of 'genetics' in epilepsy. The current term 'idiopathic' may be even better!

2-A dislike to the term 'dyscognitive' is shared by a lot of comments. It is an obscure term, not understandable and also not true! I did not understand why the old term 'complex partial seizure' is hated and condemned. Then what is the problem if we say 'complex focal seizure' or 'simple focal seizure'. It is more descriptive, more practical and easier to understand!!

3-The term 'secondarily generalized' is again not bad, and not wrong and shorter than the suggested long statement of 'focal evolving to a bi-lateral convulsive seizure' which is at least not practical!

4-The world 'self-limited' which replaces 'benign' may give an impression to families of epileptic patients that it does not need 'treatment' which is not always true. This may negatively affect the 'compliance' of epileptics

5-The suggestions about the 'electro-clinical syndromes' are well done

6-The problem to be addressed here is that we want to have a 'common language' for those who deal with 'epilepsy'. The medical people who deal with 'epilepsy' are 'heterogenous', involving different specialties like 'pediatricians, neurologists, neurophysiologists, psychiatrists, internists, GPs and even 'gynecologists' and others. It is not easy at all to arrive to all these people and 'educate' them by those new terms which are not very acceptable even by neurologists!! It means that there will be a wide gap between 'ILAE' and those people. They will start their own way of 'interpreting and understanding epilepsy' because it is not easy for them to 'cope' with this tendency of rapidly changing terms from easy and clear terms into difficult, long, and obscure ones

A fact which should be known by 'ILAE' is that the 'language' used now in teaching in colleges and academies, or in hospitals and nurseries in interpreting seizures and epilepsies are the old terms like 'complex partial, simple partial, idiopathic, symptomatic, cryptogenic' in spite of the new 'terms' that were suggested in the last twenty years. The reason for that is the 'ease and expressivity' of those old terms!! I am afraid that the 'ILAE' will go further into unnecessary 'new change' of terms', a process which may increase the 'genuine gap' between the ILAE and the 'core' or the 'bulk' of physicians dealing with epilepsy worldwide. I do believe that the ILAE in order to lead the 'world society' of epilepsy should slow its 'train' to adapt with this 'bulk' of physicians. They are 'hundreds of thousands' of medical people who are the real everyday 'dealers' with epileptic patients in the world!

Ghaieb Bahar Aljandeel


6 January 2014

First of all, I want to thank the members of The ILAE Commission on Classification and Terminology for this careful work. I would like to comment on a few points.

-This report should definitely comment shortly on the status epilepticus and its classification to avoid further confusion on this important issue. As an example status epilepticus and especially NCSE in the ICU need urgent attention by the epilepsy community and could not be abandoned to the ICU staff.

-Like many other experts, I personally do not like the term “dyscognitive” which is another unfortunate term and could not easily replace the familiar “complex partial” which was also an old wrong choice and actually not a scientifically correct term. On the other hand “dyscognitive” indicates a much larger spectrum dealing with any kind of cognitive dysfunction. Focal seizures with impaired consciousness or responsiveness or awareness (FSIC/RA) could be used for being clear; it could be simply used in acronyms like the indicated acronyms FSIC/RA in time, like the well-known GTCS or MTLE-HS.

-A new genetic generalized epilepsy form should be added as “late onset GGE” or “adult onset GGE” to save this ignored and unclassifiable but still substantial group of adult GGE patients and to attract attention of the scientific community and practicing physicians as well.

-As a last point it is really a little bit awkward that The Commission on Classification proposed an “Organization.” Why not state that it is a current view of classification of epilepsies to be more “real” rather than politically correct!

Betul Baykan


6 January 2014

Thank you for the opportunity to comment on the Organization of the Epilepsies. It is a major step forward and the authors can only be congradulated.

My major concern is the category of focal onset seizures. The terminology of complex and simple partial seizures are now removed as well as secondary generalized seizures. These terms however have served us well in clinical trials and in helping patients understand their condition. It was an easy concept. Young physicians and other health care providers could use the terminology with ease as well. This does not mean they were adequate. However, I hope the authors will try to be more specific when describing focal seizures to enable us to have clear terminology. In the manuscript there are various examples of alternative terminology that can be OK to use, but this just increases confusion with too many choices.

My suggestion is to try to coin some specific terms and abbreviations  like CPS and SPS that we all can agree on that can be used as a tool for education and research. Terminology has to be structured and clear in order for it to replace a working (although long from perfect) system.

Elinor Ben-Menachem

6 January 2014

I would like to share my opinion about some classification aspects:

1. ‘ Focal seizures – originate within networks limited to one hemisphere, which may be discretely localized or more widely distributed’. The network is described a bit vague, confusing (I’d say); is there any way to ‘localised’ this network more precisely or just mention the term local or hemispherical?

2. I would also prefer the term – focal dyscognitive seizures’ instead of the first, longer one: ‘focal seizures with impaired consciousness or awareness or responsiveness’ – easier and more practical to use; or, if I’m alowed: ‘focal seizures with cognitive dysfunction’

3. In the classification/examples of electroclinical syndromes: #self-limited epilepsies – I would suggest the term of #epilepsies with self-limited seizures, because the clinical aspect that is self- limited are the seizures, cognitive disorders could remain longer after seizures cessation.

PS. I admire though all your efforts for a more aqurate classification and organization of seizures and epilepsies, respective electroclinical syndrome, new classifications reflect for sure more clear the terms.

Best regards,
Mihaela-Adela Vintan

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6 January 2014

My suggestion regarding the ILAE classification of epilepsy 2010:

1: Arrangement of epiliepsies and electro clinical syndrome does not reflect the etiology and seems to be of limited value.In this regard classification on the basis oh etiology as genetic, structural, metabolic and unknown seems to be more useful

2: A classification based on the management option should be attempted , for eg epilesy that can be managed pharmacologically forming one group and epilepsy amenable to surgical correction forming the other group

3: A classification encompassing the seizure semiology along with the underline etiology would also be a better way forward.For eg Complex partial seizure further sub-classified based on etiology as structural, metabolic and idiopathic/genetic would better help in management

4: Genetic epilesy shoud not be included in idiopathic group due to prevalent social taboo, myth, etc.

Please do consider my suggestion and reply

Thanking you,
Goutam Ganguly


6 January 2014

ILAE gives us important knowledge about epilesy diagnosis and treatment.

Hari Ram


6 January 2014

Hola a todos los expertos e integrantes de la ILAE,

Junto con poder saludarlos y desearles un próspero año 2104, les envío mis preocupaciones y sugerencias.

En cuanto a la etiología genética y hereditaria hay que hacer la distinción, si bien se quiere expresar como genético los cambios en la estructura de los genes y hereditaria la predisposición a padecer la enfermedad, e hace necesarios tener en cuenta la condicionante del medioambiente que influye en que una condición predispuesta se exprese o no (epigenética). También puede haber confusiones en relación a las etiología metabólica y separar las de causas genéticas y otros daños metabólicos no relacionados específicamente con cambios en la estructura de los genes.

En el documento se refiere solamente a la clasificación de la epilepsia, pero no se menciona la clasificación de las crisis epilépticas. Solo se refiere a la distinción entre generalizadas y focales ¿Cuál sería la propuesta entonces?

Un saludo afectuoso,
Teresa (Tess) de las Mercedes Sosa Sánchez


6 January 2014

The idea of an ‘organisation’ (or of a diagnostic manual) is an interesting and thoughtful one, and it is kind of the Commission to invite responses to this paper. In the spirit of debate, and of open consultation, I would like to bring up two issues for the consideration of the epilepsy community.

1.  Organisation .v. classification
This is a novel issue, but I agree with the comments of Pete Engel (made below – 15h November). For the reasons Dr Engel articulates, this is not a classification, and I concur with him that references to classification should be removed.

2. Changing terminologies
I remain convinced that changes in terminology are largely unhelpful. The changes have drawbacks and disadvantages which outweigh the perceived advantages. This particularly applies to the change from ‘idiopathic’ to ‘genetic’ (and thus of Idiopathic Generalised Epilepsy (IGE) to Genetic Generalised Epilepsy (GGE). The arguments against changing these terms can be summarised as follows:
i. The fact is that these epilepsies are not simply ‘genetic’. They are likely to have a strong influence also from external factors as is the case with most human behaviours; which are all, at the same time, products of genes and environment; and to label complex behaviours ‘genetic’ is misleading. In relation to idiopathic epilepsy – if a patient only has seizures on awakening following too much alcohol and lack of sleep, the alcohol and lack of sleep are as much ‘causes’ as any putative gene. In these multifactorial, setting, ‘idiopathic’ is a more truthful term.

ii. Furthermore, no ‘genes’ underlying the great majority of idiopathic epilepsies have been uncovered, so to assign them as ‘genetic’ seems unfortunate.

iii. Changing terminology causes immense confusion amongst non-specialists. It took decades for the generalists (and patients) to refer to tonic-clonic instead of grand mal, or partial instead of focal. Changing from IGE to GGE will cause the same confusion and diminish the authority of ILAE.

iv.  Confusion will arise in non-medical circles, such as in legislation, regulation, and in legal settings (I have seen the consequences of similar changes). The effects of this confusion can be particularly detrimental to people with epilepsy.

v.  To call something ‘genetic’ can be in some cultures highly stigmatizing. I fear women may find their marriage/life prospects adversely affected  in these cultures, simply because of the word genetic has been added in to their diagnosis.

Some of the same arguments apply to changing other terms such symptomatic / cryptogenic / benign etc.  

The loss of the term ‘Panayiotopoulos syndrome’ is another example.  All these are all well established and fully understood and I would argue for their retention. 

This is not to decry the value of constructing a diagnostic manual or organization  – simply to urge that in doing so, well established terminology should be preserved unless there are good reasons not to do so.

Simon Shorvon


Read comments made prior to 6 January.

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