Consensus recommendations for assessment and management of PNES in children

The ILAE Paediatric Psychiatric Issues Task Force has developed expert-based consensus recommendations for the assessment and management of psychogenic non-epileptic (functional) seizures (PNES) in children. These recommendations were informed by an extensive scoping review following systematic review methodology. We are seeking your comments prior to submitting to a journal for peer review.

The scoping review identified 77 studies. The proposed standards include 23 recommendations. A supplemental file is included also for your convenience.

Draft: Scoping Review and Expert-Based Consensus Recommendations for Assessment and Management of Psychogenic Non-Epileptic (Functional) Seizures (PNES) in Children: A Report from the Psychiatric Pediatric Issues Task Force of the International League Against Epilepsy 

Supplemental File

Public comments are now closed and available for review below.

Comments

7 December 2022

The Functional (Dissociative) Seizures Task Force would like to draw attention to some difficulties to promote a consensual message from the different task forces

Specifics comments

In Introduction:

• 'In the International Classification of Diseases, Tenth Revision (ICD-10), PNES falls under the code F44.5, conversion disorder with attacks or seizures': It would be more appropriate to refer to the ICD-11. In either case PNES falls under 'dissociative' NOT 'conversion' disorders.
• 'For the purpose of the current work, the term PNES is used to acknowledge its previous common use within the International League against Epilepsy (ILAE)': This paper would seem to be more closely aligned with the work of the ILAE Functional / Dissociative Seizure Task Force if this sentence was modified as follows: 'The authors of this paper are aware of the ongoing discussions about a new label for PNES led by the ILAE Functional / Dissociative Seizure Task Force and other groups (including patient organisations). The new term advocated is functional/dissociative seizures ( FDS) but it has not yet been officially recognised or published. So for the purpose of the current work, the term PNES is used to acknowledge its predominance in the recent scientific literature on which this review is based'. We would suggest inserting the following reference after 'other organisations': (1) Wardrope A, Dworetzky BA, Barkley GL, Baslet G, Buchhalter J, Doss J, Goldstein LH, Hallett M, Kozlowska K, LaFrance WC Jr, McGonigal A, Mildon B, Oto M, Perez DL, Riker E, Roberts NA, Stone J, Tolchin B, Reuber M. How to do things with words: Two seminars on the naming of functional (psychogenic, non-epileptic, dissociative, conversion, …) seizures. Seizure. 2021 Dec;93:102-110.

In Method:

• For systematic review, you well detailed all final searches in each data base (supplemental 2). You didn’t use functionnal or /and dissociative seizures, this is a lack that should be discussed within the limits if possible.
• We note that the literature search for this paper was last performed over one year ago. This means that important recent publications are not included (e.g.: Hansen AS, Rask CU, Christensen AE, Rodrigo-Domingo M, Christensen J, Nielsen RE. Psychiatric Disorders in Children and Adolescents With Psychogenic Nonepileptic Seizures. Neurology. 2021 Aug 3;97(5):e464-e475. / Stager L, Morriss S, Szaflarski JP, Fobian AD. Psychiatric and personality factors in pediatric functional seizures: A prospective case-control study. Seizure. 2022 May;98:105-112. / Stager L, Morriss S, McKibben L, Grant M, Szaflarski JP, Fobian AD. Sense of control, selective attention and cognitive inhibition in pediatric functional seizures: A prospective case-control study. Seizure. 2022 May;98:79-86. / Volbers B, Walther K, Kurzbuch K, Erdmann L, Gollwitzer S, Lang JD, Dogan Onugoren M, Schwarz M, Schwab S, Hamer HM. Psychogenic nonepileptic seizures: clinical characteristics and outcome. Brain Behav. 2022 May;12(5):e2567.) This is just a suggestion, perhaps it would be interesting to add a phrase in discussion to refer to this literature.
• Delphi Process (p7): Regarding the sentences reading, 'Five of the 33 studies provided demographic data but did not proceed to the main PNES questions as they indicated that "they were not involved in the care of young people with epilepsy". The level of agreement for consensus was set at 80% (Agree/strongly agree). Participants were encouraged to elaborate on their answer if they "disagreed" or "strongly disagreed" with a statement'.
  • We note that '33 studies' seems to be a typo. The authors mean '33 respondents'. In addition, the decision to eliminate people on the basis that 'they were not involved in the care of young people with epilepsy' reflects a bias towards neurologists. Any psychiatrist or clinical psychologist who treats functional seizures — but not epilepsy — would have been excluded. This bias needs to be made explicit and needs to be included in the limitations.

Results:

• 'The median percentage of children with epilepsy who had PNES was 26% (n=44 studies)'. In our opinion, if we well understand should be changed to 'The median percentage of children with PNES who also had epilepsy was 26% (n=44 studies)'.
• Table 3: It is not clear why table 3 was split into 3 sections (all have the same subheading – should there have been different subheadings?).
• 'The scoping review did not identify any studies that examined the utility of a medical history to facilitate the assessment of PNES in children'. In fact there is a body of literature focussing on children and adolescents, which has replicated the diagnostic value of careful analysis of patient’s accounts in adults. Examples include: Cornaggia CM, Di Rosa G, Polita M, Magaudda A, Perin C, Beghi M. Conversation analysis in the differentiation of psychogenic nonepileptic and epileptic seizures in pediatric and adolescent settings. Epilepsy Behav. 2016 Sep;62:231-8. Opp J, Job B, Knerich H. Linguistische Analyse von Anfallsschilderungen zur Unterscheidung epileptischer und dissoziativer Anfälle. Neuropädiatrie in Klinik und Praxis 2015;14(1): 2-10 / Opp, J., Job, B. Dissoziative Anfälle frühzeitig erkennen. Monatsschr Kinderheilkd 170, 77–85 (2022). https://doi.org/10.1007/s00112-021-01355-x / Kinder und Jugendliche als Patienten / Schwabe, Meike. Eine gesprächsanalytische Studie zum subjektiven Krankheitserleben junger Anfallspatienten in pädiatrischen Sprechstunden. V&R unipress: Göttingen 2006 (341 pages). It might be interesting to add one or two sentences to this point if you find it relevant.

Discussion:

• 'With respect to terminology, although PNES is the name most often used in published STUDIES, no consensus was reached based on responses to the Delphi survey'. We would be grateful to you if 'no consensus was reached' could be changed to 'the threshold for consensus on an illness label was not reached in the Delphi survey'. What is more, this would be another opportunity to acknowledge the authors’ awareness of the ongoing efforts by the ILAE FDS Task Force to identify a more broadly acceptable label. Perhaps you could add a few sentences of this nature: 'On the terminology label, unanimity is impossible, there will always be many points of view, opinions, it is a sensitive subject, all the more so for these seizures which are on the border between neurology and psychiatry. This is why it is important that the FDS task force proposes a new terminology, probably FDS (functional and/or dissociative seizures) for move forward a more broadly acceptable label'.
• The limitations section should acknowledge that participation in the Delphi process was by invitation of the Task Force and may not have captured the full range of professional views especially of experts seeing similar / overlapping patients groups (such as experts in syncope or mental health professionals not involved in the treatment of patients with epilepsy, see above). What is more, the voting process (including the ranking of preferred terms) did not involve any patient representatives.
• If the authors are unwilling the update their literature search, they should explicitly discuss that their literature search was last conducted in September 2021 (they could explain that the reason for the lack of repetition of the literature search was the complex review process of the manuscript necessitated by the large number of authors and the ILAE public commenting and approval process).

Thank you very much for your attention to these remarks. We are very grateful for your hard work in advancing this topic. This is a very useful article for professionals to improve the care offered to children with FDS.

Coraline Hingray on behalf of the Functional (Dissociative) Seizures Task Force

3 December 2022

Thank you for developing this much needed consensus recommendations paper.

I have following small suggestions:

1. The appropriate terminology would be Paroxysmal Non-Epileptic Events (PNEE). It would not be right to use 'psychogenic' as it tends to generalise the causality, whilst the fact is that not all non-epileptic events have a known psychogenic basis. Secondly using 'seizures' in the term PNES can be unintentionally misleading, whilst using the term 'events' would be more reassuring to parents that non-epileptic events would not be detrimental to brain/organ systems unlike epileptic seizures.
2. A table showing phenomenology based differentiation of PNES from epileptic seizures would be useful for non-neurology paediatricians/physicians.
3. Frontal lobe onset hyperkinetic seizures can be easily misdiagnosed as PNES. Frontal lobe onset seizures can have a normal ictal EEG too, depending on the seizure onset zone. It would be useful to put these caveats.
4. From psychology point of view, review of evidence for psychological interventions, where an obvious psychogenic cause has not been identified also needs to be undertaken, as it would be relevant for return to normal activity by the individual especially if PNEE are frequent and intrusive. This is also relevant as upto 50-60% cases of PNES do not have an identifiable psychogenic stressor to target management to.

Best wishes,

Dr Anil Israni

30 November 2022

My comment relates to the recommendation about attempting to confirm the diagnosis of PNES with vEEG (when available) - further guidance about the duration of vEEG would be helpful, particularly as clinicians often have to balance the benefits of capturing an episode during EEG to make a 'confirmed' diagnosis with the inconvenience/distress associated with prolonged EEG studies. Specifically, would the recommendation to obtain a video EEG constitute a 1 hour vs. 24 hour study, vs. longer 3-5 days studies?

Dr. Azara Singh

23 November 2022

Very informative paper.

I suggest to rename the condition as: Psychogenic Non-Epileptic Episode/Event, as it doesn’t satisfy the definition of seizure as describe by ILAE.

Anis Jamil

16 November 2022

Many thanks for this nice and helpful paper.
I however have few comments.

You may have epileptic seizures without EEG correlates especially in hyperkinetic seizures and in sleep-related hypermotor epilepsy. It is not exceptional that hyperkinetic seizures have been considered for years as psychogenic because explosive semiology and no epileptiform abnormalities on EEG during the seizure. The EEG is helpful but not mandatory. vEEG is sometimes difficult to interpret because of artifacts and could be over-read in case of interictal epileptiform activities especially in epilepsy children with PNES. Homemade videos are often quite more helpful. We have to remember that the diagnosis of epileptic seizures is essentially clinical. In Box 1, point 4 gives a too strong value to the vEEG. If vEEG does not detect epileptiform activity during a typical event, 'clinically' and 'documented' should be reserved only in non artifact recordings. Furthermore, the ictal recording could be unreadable, but the postictal EEG may give sometimes some information (diffuse slowing, asymmetry of slow waves, etc).

NEAD is a useful term when you start to speak about diagnosis: you can explain organic and functional problems and progressively you arrive with the diagnosis of PNES. For me, PNES is a part of the broader term NEAD. However for numerous parents, the term 'psychogenic' is like an aggression because of the old conception (but still quite alive) that psycho-something is the same as madness or worse as factitious disorder. For that reason I prefer the term 'functional non-epileptic attack disorder'. Functional = non organic, attack is not a seizure. NEAD alone is not sufficient because, i.e., convulsive syncopes may be included. The term 'pseudo' must be avoided because somewhere it is a true attack and because the family has already the tendency to think that if it is not epilepsy, then it is comedy.

These last few years/months, functional disorders are highly frequent in children coming from a war region, and unfortunately, they arrive to my epilepsy consultation because the diagnosis of epilepsy has been previously made and the seizures were not improved by ASM. The treatment is often difficult because of language barrier (at least at the beginning) and the family is also profoundly traumatized. Specific recommandations should be developed for these situations probably more and more frequent.

van Rijckevorsel Kenou

14 November 2022

Thank you for excellent work.

I support comments from Dr Kate Riney re use of word psychogenic and implication of universal causality particularly with changes in DSM5.

Dr Ann Bye

10 November 2022

I congratulate the working group for their effort and commitment to a topic that becomes more relevant every day in daily practice. I think it is important to consider three aspects:

1. Just as the effort was made in the description of epileptic syndromes about what to do in places with limited resources, I think that some recommendation should be made when these resources are not available with respect to the PNES.
2. The recommendations correctly emphasize the care of children and adolescents. I think that an emphasis should be placed on a 'comprehensive' approach, but not as a standard package for everyone, but with an emphasis on the individual needs of each case.
3. Most recommendations on therapeutic interventions fall exclusively on the patient, when in fact the cause of PNES is external to the patient in many cases. If external causes of PNES are identified (bullying, high academic demands, family dysfunction, abuse, etc.), not only the patient is treated, but also her/his environment (school, parents, group of friends, etc.) is intervened. It is possible that what is mentioned is covered under the term 'comprehensive', but the same term does not necessarily explicitly state that the intervention is according to the characteristics of each individual case, nor to factors external to the patient. If the external factor of the PNES is not intervened, it is possible that, despite psychological or psychiatric treatment, the situation does not improve in the patient.

Jaime Carrizosa Moog

8 November 2022

Our experience using the term seizure in PNES when educating and treating patients leads many parents, family, support workers and even doctors to mistake them for an as yet undetected underlying organic cause. These events do not meet the ILAE definition of a seizure.

Angela Spencer

8 November 2022

Concerns regarding the ongoing involvement of specialist in epilepsy after diagnosis. Have found this counter productive especially where PNES is only diagnosis, understand the need for this where epilepsy and PNES occur concurrently. For many families this appears to reinforce that this is still related to epilepsy (as does use of word seizure in PNES) despite clear explanation and signposting. The main problem is a lack of appropriate mental health support and understanding among local services.

Kathryn Coleman

8 November 2022

Thank you for this excellent work. I would ask that you consider using brackets around the word 'psychogenic' in the term used throughout this article ie (Psychogenic) Non-Epileptic (Functional) Seizures, (P)NES. This allows the flexible use of PNES, NES or Functional Seizures. My rationale is that mandating the term 'psychogenic' in the title in children infers that there IS a psychogenic cause in all cases (anxiety, depression, trauma). The term 'psychogenic' means there is a psychological cause rather than a physical one. This is often harmful to families/children as a concept and contributes significantly to disengagement during attempts at explanation in cases where this is not a major factor. The studies you have reviewed have confirmed these are frequently present, and may be a causal factor for some children, but not in every case. In many cases these are consequences of the attacks rather than causing them, and psychological health can be worsened by how patients are treated/managed in the health service - patients are often excluded from usual health care supports and treated as if they are malingering. In some children, developmental factors can be the main underlying factor - learning difficulties, ADHD, ASD phenotypes (or alternatively high IQ brains are predisposed). These are not 'psychogenic' disorders, they are biological developmental disorders. There is a wealth of clear published literature confirming that brain networks in individuals at risk of functional neurological symptom disorder (including seizure-like attacks) are different biologically (physically or functionally), predisposing them to these attacks, so continuing to mandate use of 'psychogenic' in the term (inferring that there is no biological cause, only a psychological one in all cases) makes it difficult for future research to develop in this space, as researchers have to grapple with a major conflict between the disorder they are studying with a proven biological differences in how these brains work, and a term that implies the opposite. Evidence base for functional disorders has moved on, DSM-5 criteria for these disorders was updated to remove a requirement for psychological stressors/comorbidities as this was not proven in all cases and the neurobiological differences confirmed in studies. It would be good for this ILAE paper to reflect this better in 2022.

For neurobiological basis see for example: www.ncbi.nlm.nih.gov/pmc/articles/PMC7293766/

I noted that the survey you completed on the term did not result in any positive agreement to continue using a term with 'psychogenic' (there was no positive argument to do so).

I was particularly concerned through the article that in many places the term PNES was replaced simply by the word 'psychogenic' alone. For example 'if events are psychogenic in nature', 'events thought to be psychogenic in origin'. It would be better to replace 'psychogenic' in these locations (four times, I believe) with PNES/(P)NES, again for the same reason - there is no mandatory requirement for or proven psychological cause in all cases.

Lastly, you have described the term 'functional' as synonymous with 'non physiological', 'functional (and not physiologic)'. Functional does not mean 'not physiological'. Functional neurological symptom disorder has a clear definition, and as noted above has increasingly identified physiological basis, perhaps the correct definition of FNSD is best addressed properly in the article to avoid confusion.

With much appreciation for an otherwise excellent piece of work, with a small adjustment requested to improve clarification and avoid confusion for this complex but common entity in our practice.

Kate Riney