Proposed classification: Syndromes in Children

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

Please see the Draft: ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood

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

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

See all four Proposed papers for Nosology and Definitions

Comments

11 May 2021

Regarding the differential diagnosis of Self-Limited Epilepsy with Autonomic Seizures (SeLEAS), I'd like to add not only other medical disorders associated with intermittent vomiting, but also surgical conditions. Sometimes it mimics like an intestinal obstruction clinically.

Kyaw Linn


10 May 20201

I agree with the rationale behind the changes but there needs to be some improving internal consistency in the way that titles are constructed. This is harder to see in this paper compared to the other three.

Within the self-limited group, it is confusing to switch some to a title of self-limited but not all in that group. I can understand losing the terms idiopathic, but in one instance you have switched that to childhood whereas another you have given up the term childhood and replaced with self-limited. This is confusing to me. I would prefer an approach where we can see an improved internal consistency across the titles. These types of issues make the teaching and diagnostic coding around epilepsies really tricky to implement and also make it tricky for patients when understanding or describing their epilepsy type.

Given the pragmatic difficulties of ever increasing title lengths and the difficulties around how much of epilepsy syndromes characteristics to capture in the title, the retention of eponymous or acronym titles as legitimate alternative titles to me does seem sensible and where possible I would favour retaining these even if their more complicated non-eponymous title needs improving. The retention of some eponymous titles as practical alternative handles would help professional and patient communication in my view..

Given this, I would prefer an approach such as either

  • Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS)
  • Self-Limited Epilepsy with Autonomic Seizures (SeLEAS, Panayiotopoulos syndrome)
  • Self-Limited Occipital Visual Epilepsy
  • Self-Limited Photosensitive Occipital Lobe Epilepsy;

or

  • Childhood Epilepsy with Centrotemporal Spikes (CECTS)
  • Childhood Epilepsy with Autonomic Seizures (Panayiotopoulos syndrome)
  • Childhood Occipital Visual Epilepsy (COVE)
  • Photosensitive Occipital Lobe Epilepsy; (POLE)

Both of these approaches have an internal consistency in keeping with your rationale. On balance I favour the second list as it retains your rationale, avoids unnecessarily changing CECTS and I can see it working more easily in practice.

Colin Dunkley


7 May 2021

1. Regarding table 2 on Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS)

In the alert column its written usual seizure frequency more than daily..Doesn't it imply that SeLECTS has normally daily seizure frequency which we know isn't true and if a SeLECTS has a seizure frequency of daily episodes it should be considered as a major alert.

2. Regarding Developmental and/or Epileptic Encephalopathy with spike-wave activation in sleep (D/EESWAS)

I particularly liked its new nomenclature but in the description its written "The EEG pattern previously required for this syndrome was known as continuous spike-wave in sleep (CSWS) and the clinical correlate previously known as electrical status epilepticus in sleep (ESES). Isn't it the other way round? The EEG correlate was called ESES(obviously as term electrical is used) and the clinical correlate was called Epileptic encephalopathy with CSWS.

Regarding EEG in this syndrome its written "SWAS is usually diffuse but may occur more focally (typically
frontally) or multifocally"

I would like to point out that we have analysed EEG of more than 35 children of ESES and followed them for more than 3 years with serial EEG and neurocognitive assessment and their EEG were analysed in special source localisation and mapping software and we found that these apparently "diffuse" looking pseudo generalised spikes are infact in 100 % cases due to “almost”synchronous bilateral activation of spikes (with a lead-in of 20-40 ms from one hemisphere with shifting asymmetry ) with a tangential/oblique dipole .The rules of mapping localised all these spikes around Rolandic region(anterior and posterior walls).Morover the frontal predominance which you have mentioned is also seen due to the wrong interpretation of the spikes originating from posterior margins of the rolandic sulcus which has frontal negativity and tempero-occipital positivity with a positive phase reversal.

We have sent this study and accepted for poster presentation in AOEC 2021.I hope you consider this finding before concluding that the EEG pattern in this syndrome as "diffuse with frontal predominance".

One more thing which I wanted to add is that significance of the so called "developmental epileptic encephalopathy " in this syndrome should be infact more stressed. This in fact comprises the entity which we previously called symptomatic ESES(structural causes) and its more and more recognised than before.

3. Regarding Self-Limited Epilepsy with Autonomic Seizures (SeLEAS), "generalized discharges may also be seen"

It's seen that most of these "generalised" discharges are infact the bilateral occipito-frontal spikes which is seen in this syndrome and I hope you add this as an EEG finding in this syndrome. Also sometimes bilateral occipito-frontal spikes seen in this syndrome sometimes mimic ESES pattern if we are not aware of the clinical history.Therfore I feel that this valuable EEG finding be added in description.

Neetha

 


5 May 2021

This series of four position papers represent a monumental contribution to the establishment of well defined epilepsy syndromes.

Corrective Feedback:
The Onset in Childhood paper has a typo on Page 60 (Figure 1) in that Occipital Spikes currently shows 4 to 7 years, but should be 4 to 17 as in the narrative. 

Regards, John McGinley