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REVIEW ARTICLE COPYRIGHT © 2018 THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES I

REVIEW ARTICLE COPYRIGHT © 2018 THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES INC. An Overview of Psychogenic Non-Epileptic Seizures: Etiology, Diagnosis and Management Ángela Milán-Tomás, Michelle Persyko, Martin del Campo, Colin M. Shapiro, Karl Farcnik ABSTRACT: The purpose of this review is to provide an update of the research regarding the etiology, diagnosis and management of psychogenic non-epileptic seizures (PNES). A literature search using Pubmed, Ovid MEDLINE and EMBASE database was performed from 2000 up to August 2017. We have evaluated the different factors leading to PNES as well as the diagnostic approach and management of this disorder which continue to be very difficult. The coexistence of epilepsy and PNES poses special challenges and requires the coordinated efforts of the family physicians, psychiatrists, psychologists and neurologists. Although this condition has an overall poor prognosis, a multidisciplinary approach in the diagnosis and management of this disorder would likely improve the outcomes. We have proposed a diagnostic and treatment algorithm for PNES and suggested a national registry of patients suffering from this condition. The registry would contain data regarding treatment and outcomes to aid in the understanding of this entity. RÉSUMÉ: Vue d’ensemble des crises psychogènes non-épileptiques: étiologie, diagnostic et prise en charge. L’objectif de cet article est de présenter une mise à jour des activités de recherche qui concernent l’étiologie, le diagnostic et la prise en charge des crises psychogènes non-épileptiques (CPNE). Pour ce faire, nous avons mené de 2000 à août 2017 une recherche bibliographique au moyen des bases de données suivantes : PubMed, Ovid MEDLINE et Embase. Nous avons procédé à l’évaluation des divers facteurs causant les CPNE, des approches diagnostiques et de la prise en charge de ce trouble, laquelle continue à être très difficile. La coexistence de l’épilepsie et des CPNE présente aussi des défis particuliers et exige des efforts coordonnés de la part des médecins de famille, des psychiatres, des psychologues et des neurologues traitants. Bien que les pronostics au sujet de cette condition soient généralement réservés, une approche multidisciplinaire dans l’établissement d’un diagnostic et la prise en charge des CPNE contribueraient probablement à améliorer l’évolution de l’état de santé des patients. À cet égard, nous avons proposé, en plus d’un registre national des patients atteints de ce trouble, un algorithme de diagnostic et de traitement pour les CPNE. À noter que ce registre contiendrait des données portant sur les modalités de traitement et leur résultat afin de favoriser la compréhension du cadre clinique des CPNE. Keywords: Psychogenic non-epileptic seizures, PNES, Pseudoseizures, Non-epileptic attacks doi:10.1017/cjn.2017.283 Can J Neurol Sci. 2018; 45: 130-136 INTRODUCTION Psychogenic non-epileptic seizures (PNES) are a common phenomenon and there is no absolute consensus in defining this condition, so several terms have been used to describe these events over the years.1 A well-accepted notion is that they represent a sudden event characterized by paroxysmal changes in consciousness, move- ment and/or behavior that are similar to the ones seen in epileptic seizures. However, they are not associated with epileptiform activity as detected through video electroencephalography (VEEG)2 and there are psychologic underpinnings and causes that lead to the seizure.3 The term pseudo-seizures has been frequently used in the past4 but because it implies that the individual is “faking” it, alternative descriptors have been suggested. Accepted by some5,6 and adop- ted more widely1,7 the term PNES has been chosen as we feel it best describes these events. The incidence of PNES has been estimated in 4.9/100,000/year8 and its prevalence varies widely between 2 and 33/ 100,000 population,9,10 probably due to variation in diagnostic criteria, the inherent complexity of PNES and misdiagnosis perhaps due to lack of proper training in this area. Although in the majority of cases the distinction between PNES and epileptic seizures can be made,11 to add more intricacy, PNES can be present in some patients with epilepsy. Some authors2,11,12 have reported that 5% to 15% of out-patient populations referred to epilepsy clinics and between 20% and 40% of in-patient populations admitted at epilepsy centers have PNES. In a recent retrospective study6 it was found that from 65 patients diagnosed with PNES, 11.1% also had epileptic seizures. In previous reports it has been described that 75%–80% of the patients with PNES are female.13 Although this disorder can occur at any age, Duncan et al.14 reported that the mean age of onset is 30.5 years (SD ±13.7 years) (see Table 2). PNES have been known for at least 2,500 years,15 however, it continues to be a poorly understood disorder. The diagnosis of PNES is controversial and there is no definitive treatment approach. The overall prognosis of this condition is poor7 and it is From the Department of Neurology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada (AM-T); Private Practice (MP); Department of Neurology, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada (MDC); Department of Psychiatry, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada (CMS, KF). Correspondence to: A. Milan-Tomas, Sunnybrook Health Sciences Centre – Neurology, 2075 Bayview Ave, Toronto, ON, Canada M4N 3M5. Email: angela.milantomas@ sunnybrook.ca RECEIVED JULY 20, 2017. FINAL REVISIONS SUBMITTED SEPTEMBER 21, 2017. DATE OF ACCEPTANCE OCTOBER 15, 2017. 130 https://www.cambridge.org/core/terms. https://doi.org/10.1017/cjn.2017.283 Downloaded from https://www.cambridge.org/core. IP address: 114.122.102.180, on 23 Dec 2021 at 05:29:35, subject to the Cambridge Core terms of use, available at associated with a high level of disability and reduced quality of life (QOL).16 As far as we know, there is no data regarding the prevalence and incidence of this disorder in Canada; therefore, a database including the characteristics and outcomes of PNES would help clinicians to develop guidelines for the management of patients suffering from this condition (see Table 1 for the key points of this review). METHODS We present a comprehensive narrative review of PNES. To cover a wide range of topics related with PNES and add value to our manuscript we reviewed different sources including Pubmed, MEDLINE and EMBASE databases using OVID with literature pertaining PNES from the year 2000 and up to August 2017. We used the terms “PNES” as well as “pseudoseizures” and “psy- chogenic non-epileptic attacks” with the different following medical subheadings to identify relevant articles: “prevalence”, “diagnosis”, “management,” “prognosis” and “outcomes”. The abstracts and bibliographies of relevant studies were reviewed to identify additional articles. We included only articles written in English and the evidence ranged from expert opinion to clinical trials, systematic reviews, meta-analyses and a Cochrane review was also included in this paper. PSYCHOPATHOLOGY OF PNES Various risk factors have been described in adults and children with PNES. In a study evaluating psychiatric comorbidities encountered in epilepsy monitoring units at Veterans Affairs Medical Centers in the USA,17 post-traumatic stress disorder (PTSD) preceded the diagnosis of PNES in 58% of the patients. Major depression and alcohol abuse were common diagnoses in PNES and there is a well-documented association between chronic pain, psychiatric illness (especially depression, anxiety and personality disorders) and PNES (see Table 3).18,19 In children and adolescents parental discord or divorce, sexual or physical abuse, trauma, school difficulties, having epilepsy or family history of epilepsy among others are significantly associated factors.9,11,20,21 In a more comprehensive systematic review22 authors described different theoretical models of PNES: (1) PNES as a dissociative phenomenon, reflecting a defensive process that prevents individual from becoming overwhelmed by emotional adversity (seen for instance, as a somatic flashback in PTSD); (2) PNES as a “hard- wired” reflex (tendency to have a defensive reflex as occur in other animals); (3) PNES as a learned behavior (maintained by positive and negative reinforcement); and (4) PNES as a disturbance of cognitive control (resulting and maintained by factors that increase activation of seizure programs). PNES has been classified in the Diagnostic and Statistical Manual of Mental Disorders, third revised edition (DSM-III-R), as a dissociative disorder.19 However, according to the DSM-5 and International Statistical Classification of Diseases and Related Health Problems (ICD)-10 manual, PNES is generally con- sidered a conversion type of somatoform disorder.23,24 In a recent published study analyzing dissociative symptoms experienced by patients suffering from PNES;25 those with PNES compared with controls reported significantly more psychological and somatoform dissociative symptoms, trauma, perceived impact of trauma and post-traumatic symptoms. The relationship between traumatic brain injury and PNES has also been described,26 but the causality of this association should be further evaluated in prospective studies. In addition, other disorders such as psychogenic movements disorders (PMD) have shown to share common risk factors (e.g. similar age and sex distribution; relatively frequent co-occurrence of psychiatric disorders or similar psychological profiles and high rates of chronic pain and other somatization disorders),27 com- plicating the understanding of the psychopathological process in PNES further. PNES and PMD may represent two ends of a continuum, where different clinical presentations explain referral to either the epilepsy or movement disorder specialists.27 DIAGNOSIS The diagnosis of PNES is challenging and misdiagnosis of PNES as epilepsy can have deleterious consequences.28 Syncope, panic/anxiety, hyperventilation attacks, parasomnias, migraine with aura and transient ischemic attacks are some of the conditions that may give the appearance of both epileptic and non-epileptic seizures.29,30 Sometimes accurate diagnosis uploads/Sante/ an-overview-of-psychogenic-non-epileptic-seizures-etiology-diagnosis-and-management.pdf