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revue neurologique xxx (2016) xxx–xxx

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General review

Psychogenic non-epileptic seizures (PNES)

C. Hingray a, J. Biberon b, W. El-Hage c,d, B. de Toffol b,c,*


a
Service de neurologie, CHRU de Nancy, 29, avenue du Maréchal-de-Lattre-de-Tassigny, 54000 Nancy, France
b
Service de neurologie et de neurophysiologie clinique, CHU Bretonneau, 2, boulevard Tonnellé, 37044 Tours cedex,
France
c
Inserm U 930, 10, boulevard Tonnellé, 37000 Tours, France
d
Clinique psychiatrique universitaire, rue du Coq, 37540 Saint-Cyr-sur-Loire, France

info article abstract

Article history: Psychogenic non-epileptic seizures (PNES) are defined as change in behavior or conscious-
Received 5 October 2015 ness resembling epileptic seizures but which have a psychological origin. PNES are cate-
Accepted 23 December 2015 gorized as a manifestation of dissociative or somatoform (conversion) disorders. Video-EEG
Available online xxx recording of an event is the gold standard for diagnosis. PNES represent a symptom, not the
underlying disease and the mechanism of dissociation is pivotal in the pathophysiology.
Keywords: Predisposing, precipitating and perpetuating factors should be carefully assessed on a case-
Psychogenic non epileptic seizures by-case basis. The process of communicating the diagnosis using a multidisciplinary
Epilepsy approach is an important and effective therapeutic step.
Dissociative seizures # 2016 Elsevier Masson SAS. All rights reserved.
Psychic trauma

differential diagnosis of epilepsy. Search for the psychogenic


1. Introduction origin of the events should be conducted by specialized
psychiatrists/psychologists. In the DSM-5 [3], PNES belong to
Psychogenic non-epileptic seizures (PNES) designate a disease the category of ‘‘conversion disorders’’ under the heading
defined symptomatically with no etiological implication. PNES ‘‘functional neurological symptom disorders’’. In the ICD-10,
are described as changes in a subject’s behavior, perceptions, PNES are categorized as ‘‘dissociative disorders’’ [4]. PNES are
thoughts or feelings occurring for a limited period of time and autonomous yet multifactorial psychopathological disorders
resembling epileptic seizures, but without the concomitant that are becoming better understood through a neurobiolo-
electro-physiological pattern associated with epileptic seizu- gical approach to the phenomenon of psychic dissociation.
res [1]. In routine practice, patients have a first-line diagnosis
of epilepsy and are referred for neurological assessment with
video-EEG. The recording reveals an event not involving an 2. Epidemiology
epileptic seizure. The term PNES was adopted by the
international community in 2012 [2] and should be preferred It is estimated that 5 to 10% of ambulatory patients receiving
over the former term of pseudoseizure perceived as offensive. care for epilepsy have PNES. In tertiary centers, this proportion
Moreover, PNES is a positive diagnosis and not simply a would reach 20 to 40% of patients, both in developed and

* Corresponding author. Service de Neurologie et de Neurophysiologie clinique, CHU Bretonneau, 2, boulevard Tonnellé, 37044 Tours
Cedex, France.
E-mail address: bertrand.detoffol@univ-tours.fr (B. de Toffol).
http://dx.doi.org/10.1016/j.neurol.2015.12.011
0035-3787/# 2016 Elsevier Masson SAS. All rights reserved.

Please cite this article in press as: Hingray C, et al. Psychogenic non-epileptic seizures (PNES). Revue neurologique (2016), http://dx.doi.org/
10.1016/j.neurol.2015.12.011
NEUROL-1618; No. of Pages 7

2 revue neurologique xxx (2016) xxx–xxx

developing countries [5]. In a study of the general population few details. They have difficulty concentrating on one particular
in Scotland, incidence of PNES was estimated at 4.9/100,000/yr attack, for instance the last one. A sociolinguistic analysis of the
[6], or for comparison, around the same level as multiple modalities used to describe attacks has successfully distin-
sclerosis or Parkinson’s disease [7]. Estimated prevalence is 2 guished between the two entities [17].
to 33/100,000 inhabitants [8]. A retrospective analysis of the One-quarter of PNES patients have prolonged attacks
patient population in our center revealed that 17.4% of (several minutes or hours) with the same psychopathology
patients with a diagnosis of epilepsy who underwent as in subjects with intermittent PNES [18]. Motor signs
explorations before potential surgery had PNES [9]. associated with a prolonged state of altered responsiveness
are suggestive of status epilepticus and lead to inappropriate
intensive care (sedation, intubation). In the emergency room,
3. Diagnosis persistent symptoms despite intravenous administration of
high-dose benzodiazepines contrasting with an excellent
Approximately 75% of patients are female [10]. A first peak of respiratory tolerance are suggestive of the diagnosis [19].
PNES incidence is observed between the ages of 15 and During and EEG recording, hyperventilation and intermit-
24 years, with no age-related effect on clinical presentation tent photic stimulation can trigger PNES [20]. Verbal sugges-
[11]. A second peak is observed after 60 years [12], constituting tion, massage of the temples, or placebo injection are
a distinct diagnostic group defined by seizure characteristics sometimes used to trigger attacks [21]. Seizure induction by
[13] and psychopathological mechanisms [14]. Video-EEG hypnotic suggestion has 44% sensitivity and 95% specificity
recordings are required for certain diagnosis of PNES. In [22]. These sensitization techniques raise however a certain
2011, Hubsch et al. [15] proposed a descriptive classification number of ethical issues and may compromise the therapeutic
based on 22 clinical variables observed in a series of 52 patients alliance [23].
presenting 145 episodes of PNES. Five groups of events can be The rule of ‘‘2’’ proposed by LaFrance et al. in 2013 [24] to
described. screen patients for video-EEG may be difficult to implement–at
Dystonic attacks with primitive gestural activity with least two normal EEG recordings, at least two seizures per
emotional content (32% of events). Typically, these attacks week, at least two antiepileptic drugs–but has 85% positive
last about 5 min, frequently with dystonic postures and motor predictive value for the diagnosis of PNES. On average,
behavior with strong emotional implications (anger, fear). diagnostic delay has been 7 years [25], but more recently
Prolonged hyperkinetic attacks (12%): has been reduced to 2 years in dedicated PNES clinics [17].

 with axial involvement, progressive onset, prolonged dura-


tion (> 5 min), flexion/extension movements of the trunk 4. Coexistence of PNES and epileptic seizures
(possibly opisthotonos), tonic limb postures, possible pre-
monitory sensation, possible hyperventilation, fluctuating About 10% of PNES patients [26] also have epilepsy, the
evolution; proportion going up to 36% depending on the report [27].
 without axial manifestation (with hyperventilation), pro- Frontal epilepsy is over-represented in these cases of PNES
gressive onset, prolonged duration (> 5 min), no axial plus epileptic seizures. Certain PNES have been observed late
manifestation, various and fluctuating motor signs involv- after successful epilepsy surgery and have been interpreted as
ing the limbs (dystonia, tremor, clonic movements), possible the expression of emotional vulnerability that existed in an
premonitory sensation, frequent hyperventilation, fluctuat- organized manner around the status of being an epileptic
ing evolution. person. Since this type of association is a possibility, proper
assessment of the gravity of authentic epilepsy requires video-
Paucikinetic attacks with preserved responsiveness (23%), EEG for confirmation. It is important to detail all of the
progressive onset, variable duration, preserved responsive- different seizures experienced by a given subject, interrogat-
ness, rare or discrete motor signs (for example distal tremor), ing friends and family. PNES and epileptic seizures are not
axial immobility, possible premonitory sensation. mutually exclusive so that several events must be recorded in
Pseudosyncope or dialeptic seizures (17%), sudden onset, the same patient.
duration < 5 min, altered responsiveness (often eyes closed),
motor signs typically clonic movements, tremor, myoclonia,
possible denegation movements, inconstant hyperventilation. 5. Somatic comorbidities
Intense prolonged axial tonic hyperkinetic attacks (16.4%)
(opisthotonos) with vocalization and hyperventilation. Several somatic conditions are over-represented in PNES
This classification appears to be culture-independent patients compared with the general population including
since is successfully classified 95% of patients in a series epilepsy, fibromyalgia, tension headache, irritable bowel
from India [16]. syndrome, chronic pelvic pain, and certain intermittent
A clinician familiar with PNES can make the diagnosis at symptoms such as migraine, asthma and gastroesophageal
history taking. Epileptic patients describe their attacks as a reflux [28]. A correlation has been observed between the
strange experience breaking in from outside and provide a occurrence of PNES and learning impairment in 10 to 20% of
precise and detailed description of aura. PNES patients describe subjects; a history of minor head trauma has also been found
a malaise arising within themselves, interpreting the attacks as in about 30% of subjects [29]. As about 20% of PNES patients
internal emotional states using vague descriptions with very exhibit abnormal brain images and/or non-specific anomalies

Please cite this article in press as: Hingray C, et al. Psychogenic non-epileptic seizures (PNES). Revue neurologique (2016), http://dx.doi.org/
10.1016/j.neurol.2015.12.011
NEUROL-1618; No. of Pages 7

revue neurologique xxx (2016) xxx–xxx 3

on the EEG, it is important to take a global clinical approach in emotions through visual expressions or the analysis of
all circumstances [30]. attentional disorders. Responses to emotional tasks are
abnormal compared with healthy controls, with a trend to
avoidance of emotional stimuli [39]. The neurobiology of
6. Psychiatric comorbidities emotional regulation in PNES is reviewed elsewhere [40].

PNES are associated with various psychiatric disorders


identifiable in variable proportion across studies, but in at 8. Etiopathogenesis: PNES, emotion and
least 70% of patients. The 12-month prevalence is as follows: dissociation

 22–84% for somatoform disorders; PNES involve multiple complex mechanisms [41]. No neces-
 22–91% for dissociative disorders; sary or sufficient causal factor has been identified [17]. In the
 35–49% for post-trauma stress disorders (PTSD); DSM-5, PNES belong to the category of somatoform (conver-
 57–85% for depression disorders; sion) disorders and in the ICD-10 to the category of
 11–50% for anxiety disorders; dissociative disorders. A psychic trauma underlying the
 25–67% for personality disorders [31,32]. dissociation is very often found in subjects forming the
largest group of patients. The DSM-5 describes a subtype of
Quality-of-life is significantly less satisfactory in persons PTSD that associates two types of symptoms, i.e. dissociative
with PNES compared with persons with epilepsy [33]. depersonalization symptoms (persistent or recurrent disem-
bodiment experiences, the feeling of being an outside
observer of one’s own mental processes or body; dreamlike
7. Risk factors experiences; feelings of the unreality of oneself or one’s body;
feelings of time slowdown) and derealization symptoms
The predominant risk factors are psychic trauma, psychic (persistent or recurrent feelings of the unreality of the
dissociation (see below) and disorders of emotional regulation. environment, e.g. belief the surrounding world is unreal,
Exposure to psychic trauma is found in variable propor- dreamlike, far away, or deformed). Dissociation thus involves
tions across reports and can reach 90% (average 75%). Psychic a discontinuation of consciousness that, in the normal state,
trauma is at the heart of the psychopathology [34,35]. Psychic controls motor activity and behavior. The dissociation
trauma is defined as exposure to death, threatening death, or involves detachment from the environment with deperso-
sexual violence, in one (or several) of the following manners: nalization/derealization symptoms in response to a trauma-
direct exposure; direct witness of exposure; learning that tic event and compartmentalization by defective integration
someone close was exposed to one or several of these of different functions of consciousness, causing a somato-
traumatic events; repeated exposure in an occupational form disorder (with identifiable physical symptoms) or
context. (For death of someone close to be considered as dissociative amnesia [42]. PNES could thus result from a
traumatic, it must have been violent or accidental). PTSDs are dissociative process involving several sequences: an initial
characterized by the association of the traumatic event with dissociative process in response to a traumatic event then
any of the following: perpetuation or accentuation of the phenomenon in response
to triggering events [43]. PNES would reflect dissociated
 recurrent involuntary invasive symptoms; mental content. This process would be favored by the
 persistent avoidance coping; neurobiological vulnerability of dissociation or by add-on
 negative alteration of cognition and mood; brain damage. Almost all of the theories on PNES implicate
 altered consciousness and responsiveness. emotions: dissociative (conversion) responses occur when
the emotional discharge can be controlled only by suppres-
Patients with PNES regularly report experiencing repeat- sion or inversely by somatic expression. The hyperarousal of
ed multiple traumatic events that began in childhood (often PTSD could be related to the desire to avoid all emotional
in a context of emotional negligence) and continue in stimuli [44]. Disorders of emotional control could take the
adulthood. These traumatic events are generally reported form of incapacity to experience emotions if they exceed a
less frequently by men with PNES, but are widely described certain level of intensity. PNES may be triggered or exacerba-
in the USA by war veterans [36]. The traumatic experience ted by emotions such as shame, guilt, or anger. In the absence
generates dissociative symptoms (see etiopathology below). of any particular emotion, the factor triggering PNES might be
Emotional regulation is abnormal in PNES patients. It is a conditioning process [43]. In a context of hyperarousal, the
described as difficulty perceiving, identifying and describing particularly strong emotional content of threatening events
emotions (alexithymia). Patients with alexithymia are less may coexist with an increased desire to avoid emotional
‘‘conscious’’ of their emotional state and have little stimuli. The dissociation would thus take on the form of a
inclination to accept their emotional problems as the cause disconnection between different psychic components that
of their symptoms [37]. PNES patients also tend to have an process emotions. The incapacity to control emotional
‘‘external health locus’’, i.e. they tend to attribute their excitation could exacerbate cognitive and behavioral impair-
health status to outside causes rather than to their own ments and finally trigger motor programs taking the form of
behavior or emotions [38]. Disturbed emotional regulation PNES. Another hypothesis is that emotions could trigger
can be studied experimentally using tasks that identify motor symptoms by short-circuiting control centers [45].

Please cite this article in press as: Hingray C, et al. Psychogenic non-epileptic seizures (PNES). Revue neurologique (2016), http://dx.doi.org/
10.1016/j.neurol.2015.12.011
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4 revue neurologique xxx (2016) xxx–xxx

characterize psychopathological profiles amendable to indi-


9. Clinical and etiopathogenic heterogeneity vidualized treatment.

There are various risk factors for PNES and, consequently,


PNES populations are not homogenous. There appears to be 11. Therapeutic management
two distinct categories [46] depending on the presence or
absence of psychic trauma. The presence of psychic trauma is Although all experts recommend psychological care, consi-
associated with significant psychiatric comorbidity and pre- dered in a broad sense [24], the precise modalities remain to
dominant dissociative phenomena. This population is pre- be defined. No consensus has been reached, but communi-
dominantly female. If the psychic trauma is not present, the cation of the diagnosis should be the first step of psycholo-
psychiatric comorbidity is moderate or absent and few gical care conducted by a multidisciplinary team including
dissociative phenomena are noted. This population is pre- neurologists, psychiatrists and psychologists. The diagnosis
dominantly male, presenting learning impairments and low is generally established by the neurologist who performed the
intelligence levels and seizures triggered by frustration or video-EEG, but knowledge of psychopathological profiles
contrariety. PNES in older subjects form another clinical identified by the psychiatrist is very useful for early-stage
category where the main etiological factors are serious health diagnosis based on structured interrogation and specific
problems observed in about half of patients [12]. questionnaires [41]. This evaluation phase, before or after
This heterogeneity corresponds to different etiopathogenic diagnosis, should be conducted by the team’s psychiatrist or
mechanisms that would involve an indirect dissociative psychologist specialized in PNES. For the patient, the
pathway and a direct non-dissociative pathway. These two neurologist remains the pivotal reference during the transi-
mechanisms might occur successively in certain patients tion to psychological care. The multidisciplinary care path-
during the time-course of the disorder. In subgroups of way should be coordinated by the neurology unit in order to
patients with strong dissociative tendencies, PNES would increase the probability of adhesion to the proposed psy-
correspond to episodes of dissociation in response to uncon- chological follow-up. Otherwise, there is a risk the patient will
trolled panic attack, fear, frustration, guilt, fatigue. . . [47]. feel rejected, abandoned or stigmatized by the orientation
Direct mechanisms of emotional expression could intervene towards psychiatric care [50]. It has been shown that only half
in the subgroup of patients without dissociative tendency. For of patients with functional neurological symptoms will begin
example, high levels of emotional intensity or particularly psychological follow-up [51]. Ideally, the patient should be
aversive self-threatening emotions could invade the emotion rapidly evaluated by a psychiatrist or psychologists trained in
management system and give rise to experiences and the management of PNES patients in order to ensure proper
behaviors such as PNES without dissociation. A model has referral and care pathway. Due to the lack of information and
been described [48] according to which emotions would be a certain degree of unawareness about this psychopathology,
triggered directly by motor symptoms such as PNES [48]. the patient is often confronted with contradictory informa-
tion or even excluded from care. Thus if the patient is
managed in an outpatient setting (general practitioner,
10. Multilevel model psychiatrist), the managing physician should be provided
with detailed information and specific documentation on
In order to establish a hierarchy of risk factors, causes, and PNES.
modalities of onset, Reuber and Elger [27] proposed a model Communication of the diagnosis is a fundamental step of
based on interaction of the 3Ps–predisposing, precipitating, the initial phase of treatment. The announcement is usually
and perpetuating factors. This model was later completed by made by the neurologist and conditions the prognosis [52]. It
Bodde et al. [49] who distinguished five levels of assessment. should be made in the presence of a member of the family, if
The first level corresponds to a psychogenic etiology and the patient agrees. The neurologist details the conclusions of
evaluates factors causing PNES, e.g. sexual abuse. The second the clinical observations with empathy and corrects the
level evaluates vulnerability or predisposing factors such as erroneous diagnosis of epilepsy [53]. In practice, it is essential
personality, gender, age, or the presence of comorbid somatic to confirm the reality of the disease, avoiding any presentation
disorders. The third level is termed shaping to take into of the symptoms as trivial or stigmatizing. The disease should
account factors that shape the symptoms into seizures rather be named and the prevalence explained in detail, insisting on
than a somatoform disorder. Shaping factors could include the difficulty in establishing the diagnosis. The neurologist
for example the subject’s knowledge of epilepsy in someone should discuss the causes, provide an explanatory model of
close. The fourth level evaluates precipitating factors taking the seizures, and propose a therapeutic management scheme.
into account the occurrence of PNES at a precise moment, e.g. The important messages are: ‘‘this is not a simulation’’; ‘‘we
stress or frustration. The fifth level analyzes factors that believe you’’; ‘‘this is a real disease’’; ‘‘this is good news’’;
make PNES become a chronic condition, e.g. inappropriate ‘‘antiepileptic drugs are ineffective for this type of seizures’’. In
management or secondary benefits. All of these factors France, a video teaching film for announcing the diagnosis of
interact with each other and any given psychological factor PNES is available on the web: (http://www.canalu.tv/video/
can intervene at the different levels. This model provides a canal_u_medecine/annonce_d_un_diagnostic_de_crises_non_
plan for hierarchical psychopathological analysis. A structu- epileptiques_psychogenes.12726). The clinician’s expertise in
ral approach is indispensable in order to identify different announcing the diagnosis of PNES improves acceptation by
predisposing, precipitating and perpetuating factors and the patient and family.

Please cite this article in press as: Hingray C, et al. Psychogenic non-epileptic seizures (PNES). Revue neurologique (2016), http://dx.doi.org/
10.1016/j.neurol.2015.12.011
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revue neurologique xxx (2016) xxx–xxx 5

The second phase of treatment focuses on psychoeduca-


tion [54,55]. Several modalities are proposed, e.g. interven- 12. Course and outcome
tion of a consultation-liaison psychiatry nurse during the
hospital stay for the video-EEG, four individual psychoedu- Most studies examining the prognosis of PNES consider that
cation sessions conducted by an experienced therapist, or reduction or elimination of seizures is the treatment objective,
participation in psychoeducation group therapy. No struc- despite the absence of a clear correlation with improvement in
tured program of this type has been developed in France. The quality-of-life or psychopathology [60]. Approximately 40% of
first session should take place shortly after communication patients become seizure-free after diagnosis, communicated
of the diagnosis. The diagnosis was announced with a large with an explanation of their clinical condition [61]. One
volume of information delivered in an emotionally intense retrospective study found that seizures were totally elimina-
context and must be explained again. Care must be taken to ted in 60% of patients and reduced in frequency by 18% in 50%,
ensure good comprehension and proper analysis of the an average 21 months after diagnosis [62]. Nevertheless, an
impact of the diagnosis, beliefs and doubts. Detailed answers average 11 years after the first manifestations and four years
must be given to all questions raised. The different after diagnosis, two-thirds of patients still had seizures and
emotional and psychological aspects of PNES–influence of 40% were still taking antiepileptic treatments [63]. In more
emotions and stress on behavior; presentation of the recent studies, outcome has been better. In a cohort of 188
predisposing, precipitating and perpetuating factors of PNES; patients followed for 5–10 years after diagnosis of PNES, 32%
therapeutic aspects focusing on the inefficacy of anti- consulted for seizures during the first six months and 13%
epileptic drugs and the usefulness of psychiatric follow- attended the emergency room, but only 11% without epilepsy
up–should be addressed during the psychoeducation ses- comorbidity were still taking antiepileptic drugs; 40% were
sions. Certain programs combine coping techniques and taking antidepressants and 26% had psychiatric follow-up [64].
relaxation exercises. Psychoeducation leads to remission in Factors of good prognosis are care started early after
30% of participants [54]. symptom onset, absence of comorbid epilepsy, short diagnostic
Antiepileptic treatments should be progressively disconti- delay, good acceptation of the psychogenic etiology, supportive
nued in PNES patients with no comorbid epileptic element. environment, occupational activity, absence of a history of
There is no urgency; the risk of withdrawal-related seizures trauma or psychiatric comorbidity, and care attendance.
should be avoided. For patients with co-existing epilepsy, a
simplified antiepileptic regime taking into account the
respective involvements should be discussed on a case-by- Disclosure of interest
case basis. In France, no medication has marketing authoriza-
tion for the indication of PNES [56]. Programs of cognitive The authors declare that they have no competing interest.
behavioral therapy have proven efficacy [57]. Group therapies
may also be contributive. Patients with PNES have different
references
profiles so management should take into account each
individual case. Each psychopathological profile should be
carefully assessed using an analysis of the predisposing,
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10.1016/j.neurol.2015.12.011
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