Psychogenic Non-Epileptic Seizures PNES

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Chapter

Psychogenic Non-Epileptic
Seizures (PNES)
Nirmeen A. Kishk and Mai B. Nassar

Abstract

Psychogenic non-epileptic seizures (PNES) are a common presentation to the


emergency rooms and neurology departments, and they are difficult to discriminate
from epileptic seizures (ES). PNES present as paroxysmal time-limited, alterations in
motor, sensory, autonomic, and/or cognitive signs and symptoms, but unlike epilepsy,
PNES are not caused by ictal epileptiform activity. There is no exact known etiology or
mechanism for PNES so far. The most recognized factors discussed in the literature
include trauma and child adversity, dissociation, somatization, emotional
processing, psychiatric comorbidities, coping styles, and family dysfunction. The
use of a comprehensive assessment model may ease the transition of patient care from
the diagnosing team to the outpatient treatment provider. Recognition of the charac-
teristic clinical features of PNES and utilization of video-EEG to confirm the diagnosis
are critical. Communicating the diagnosis, discontinuation of treatment for epilepsy
(unless comorbid PNES and epilepsy are present), and implementing proper liaison
with a multidisciplinary team with clinical psychologists, neurologists, and psychia-
trists improve patient and healthcare outcome.

Keywords: PNES, DES, definition, etiopathology, management

1. Introduction

According to Hingray et al. [1], between 12 and 20% of adults presenting in


epilepsy clinics have dissociative seizures.
Psychogenic non-epileptic seizures present as paroxysmal time-limited, alterations
in motor, sensory, autonomic, and/or cognitive signs and symptoms that are not
caused by ictal epileptiform activity, and positive evidence for psychogenic factors
that may have caused the seizure is present [2].
PNES were formerly given different names including the name hystero-epilepsy,
pseudo-seizures, and behavioral spells. However, most of these terms became aban-
doned in the literature because of being either vague or pejorative, implying that the
seizures are unreal or fake. So, the accepted terminology in the medical community
became psychogenic non-epileptic seizures (PNES), non-epileptic attack disorder
(NEAD) [3], or dissociative non-epileptic seizures (DES) [4].

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Epilepsy - Seizures Without Triggers

2. Epidemiology

The prevalence of PNES remains somewhat uncertain but has been estimated at up
to 50/1000003; the incidence of video electroencephalography (vEEG)-confirmed
PNES has been determined as 4/100000 per year [5]. However, data from epilepsy
centers estimate a much higher incidence rate.

3. Etio-pathology

Up to now, there is no exact known etiology or mechanism for PNES. Some of


the most commonly presumed factors include trauma and childhood adversity,
dissociation, somatization, alexithymia and defective emotional processing, illness
perception, family dysfunction, psychiatric comorbidities and personality factors,
age, gender, and organicity (including comorbid epilepsy and anti-seizure medica-
tion use) [3, 6, 7].

4. Psychogenesis

Considering the previously mentioned factors, multiple theories for the psycho-
genesis or the mechanism by which PNES operate were hypothesized. All of these
have agreed about the multifactorial nature of PNES that can be explained by differ-
ent models.
One of the convenient proposed models for the psychogenesis of PNES is the one
proposed by Bodde et al. [7]. This model shows five different layers or levels that
highlight how each of these factors represents a heterogeneous group and may have a
differential impact on the causation, development, and prolongation of PNES,
emphasizing that not all factors have a similar impact. The proposed model is as
follows:
Level 1. Psychological etiology
This includes the factors involved in the causation of PNES, such as sexual adver-
sity or other traumatic experiences.
Level 2. Vulnerability
It refers to factors that act as predisposing elements for a person to develop
psychosomatic symptoms like PNES, for example, personality factors, gender, neuro-
psychological impairments, organicity, and age. Many authors have pointed to the
specific vulnerability of patients with PNES in terms of both their emotional “make-
up” and their neuropsychological functioning.
Level 3. Shaping factors
Some factors can specifically shape the symptoms in the direction or form of
“seizures” rather than other forms, for example, movement disorders or headache-
like symptoms. A shaping factor may be a relative with epileptic seizures (symptom
modeling) or the person himself having past history of epilepsy.
Level 4. Triggering factors
These are factors that create circumstances or situations that provoke and precip-
itate PNES, such as factors that refer to primary gain. Psychological mechanisms that
transfer an emotional state into a seizure can be part of these triggering factors, such
as dissociation and somatization. These factors explain why seizures occur on a
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Figure 1.
Model of psychological factors involved in PNES [7].

specific day or in a cluster or why there is a period of remission. This differentiates


PNES from conversion states that have a more predictable presentation.
Level 5. Prolongation factors
The previous factors are specifically important in the development of PNES,
whereas prolongation factors are important in explaining why the seizures persist and
PNES may become a chronic disorder. These factors tailor PNES frequency and
resistance against therapy. Such modulating factors include the coping strategy of the
patient and secondary gain aspects (Figure 1).

5. Predicting PNES: a multivariate approach

All the current research and studies aim to make that leap of “predicting” PNES, to
change PNES from being a “diagnosis of exclusion” to being a “predictable,” early
detected diagnosis.
A multivariate approach may predict the development of PNES and provide useful
markers for early identification of patients with potential PNES [6, 8, 9]. The multi-
variate approach proposed comprises the following:

5.1 The biopsychosocial/3P (BPS/PPP) psychiatric assessment

Multiple studies suggest that the biopsychosocial/3P (predisposing, precipitating,


and perpetuating) model for approaching the diagnosis of PNES is one of the most
comprehensive integrative models for screening and early identification of variables
that can be readily and cost-effectively obtained in patients with non-diagnostic V-
EEG evaluations, or eventually in an outpatient setting, and may prompt more rapid
diagnosis and treatment [10–13]. See Figure 2 [13].
3
Epilepsy - Seizures Without Triggers

Figure 2.
Biopsychosocial conceptualization of PNES [13].

This involves a thorough psychiatric clinical interview to obtain precise history


including demographic characteristics, present and past psychiatric history, medical
history, family history, personal history, current living circumstances, and family
dynamics to identify the possible biological, psychological, and social etiological fac-
tors that may interact as predisposing, precipitating (triggering), or perpetuating
factors for PNES and present them in a BPS/3P (biopsychosocial/predisposing, pre-
cipitating, and perpetuating) formulation to establish a proper individualized treat-
ment plan [10].

5.2 Clinical and neurophysiological assessment

Although none of the clinical signs by themselves carry a strong enough


diagnostic value unless the psychiatric, neurologic, and neurophysiologic
backgrounds are taken into account, the following clinical signs and serological and
EEG findings were claimed to have a predictive value for PNES when integrated
with the other previously mentioned psychological factors and semiological features
[6, 8, 9, 11].

• Self-reported length of the attacks: Patients with PNES have longer (>2 minutes)
events compared to patients with epileptic seizures, where the length of the
attacks is usually less than or equal to 2 minutes [8].

• Age of onset of seizures: Patients with PNES have older age of onset compared
to patients with epileptic seizures, with “30 yrs. old” as the average age of
onset 12.

• Frequency of seizures: PNES have more frequent attacks than epilepsy; diagnosis
entitles a frequency of at least 2 seizures per week [14].

• Duration of illness (years since the first seizure): Shorter duration/less years since
the first seizures is considered a good predictor for PNES with an average of 8 yrs.
since the first seizure [6, 8, 9].

• Occurrence of an episode during clinic visits: In a patient with “refractory epilepsy,”


the occurrence of an episode during clinic visits is a predictor of the episode being
PNES with a high specificity (99%) and positive predictive value (PPV 77%) but
a low sensitivity (3%) [12].

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Psychogenic Non-Epileptic Seizures (PNES)
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• Prolonged PNES or NEPS: Recurrent hospital admissions with prolonged PNES or


NEPS (episode longer than 30 minutes) suggest PNES [6].

• Response to medications: Resistance to at least two anti-seizure medications is a


predictor for PNES [14].

• EEG findings: At least 2 normal EEG studies are required to assume PNES15.

Added to that, the ILAE reported that predictors of PNES include the “rule
of 2s” with an 85% PPV for PNES15. The rule of 2 s suggests that the diagnosis of
PNES requires the following: at least two normal electroencephalography (EEG)
studies with at least two seizures per week and resistance to two anti-seizure
medications [6].

6. Patients’ characteristics

In an attempt to discriminate factors underlying this heterogeneity and detect


important predictors of dissociative seizures, Hingray et al. [1] identified dissociative
seizure patients into three profiles; each had some factors in common, but from a
statistical point of view, participants’ trauma history pattern emerged as the strongest
discriminating feature between these three profiles. Accordingly, Hingray et al. [1]
named the identified patient subtypes according to their trauma history: Group 1,
“No/Single Trauma”; Group 2, “Cumulative Lifetime Traumas”; and Group 3,
“Childhood Traumas” (see Table 1).

7. Clinical presentation

i. Semiology: Behavioral seizure manifestation:


According to Hingray et al. [1], cluster analysis data collected on patients with
PNES were categorized using the proposed classification distinguishing five
different semiological profiles, which were simplified to establish three
groups based on categories most frequently used in the previous literature
into hyperkinetic seizures (commonest semiology involves excessive
movement of limbs, trunk, and head), paucikinetic seizures (seizures with
stiffening and tremor), and syncope-like events or seizures (with atonia and
loss of consciousness). The latter is less frequent [15].

ii. Phenomenology: Subjective seizure experience


Many patients describe physical symptoms of panic or hyperventilation
during their seizures without feeling anxious; it has been suggested that panic
symptoms are more common in adolescents with PNES than in adults. Even
in the absence of panic symptoms, most patients experience their seizures as
confusing and beyond their control. At the same time, patients with epilepsy
are more likely to conceptualize their seizure as a hostile agent acting of its
own volition [1].

5
Epilepsy - Seizures Without Triggers

iii. Autonomic seizure manifestations:


More than one-quarter of patients with PNES give a history of ictal
incontinence of urine; fecal incontinence is also reported. Sinus tachycardia is
common but is more gradual in onset, less marked, and less persistent
postictally than in epileptic seizures [15].

8. Confirming the diagnosis: A staged approach

Conversation analysis of history taking (Table 2) [16] and characteristic semio-


logical and clinical features (Table 1 [1]) and Table 2 [16]) may help discriminate
PNES from ES, but individually, they cannot not be a reliable diagnostic discriminator
[17] (Table 3). To provide greater clarity about the process and certainty of the
diagnosis of PNES and improve the care for the patients, the ILAE proposed a staged
approach to confirm the diagnosis of PNES in which levels of diagnostic certainty
were developed (see Table 4).
Key: + means history characteristics consistent with PNES, *PNES = psychogenic
non-epileptic seizures, EEG = electroencephalogram.

9. Delivery of the diagnosis: communication protocol

The process of communicating the diagnosis is one of the most important and
potentially effective therapeutic steps in the management pathway of patients with
PNES with both immediate (within 24 hours of diagnosis presentation) and long-term

Criteria Group 1 No/Single Group 2 Cumulative Lifetime Group 3 Childhood


Trauma Traumas Traumas

Predominant Male Female Female


gender

Educational Low High Intermediate


level

Triggers • Non-identifiable • Identifiable • Identifiable

• Frustration more than • Anxiety (80%) more than • Anxiety (84.1%) more
anxiety frustration (50%) than frustration (31.8%)

Trauma Non-significant Significant Multiple emotional • Significant


history trauma (most common type)
• Childhood onset

• Child sexual abuse and


emotional trauma

PTSD Non-significant PTSD in 33.3% of cases PTSD in 63.6%


Prevalence

Comorbid 43.4% (common) 16.7% (rare) 52.4% (commonest)


epilepsy

Seizures Non-hyperkinetic Hyperkinetic most common Hyperkinetic: Non-


semiology Seizures (paucikinetic hyperkinetic 1:1
42.2%)

Table 1.
Patients’ characteristics in groups [1].

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Psychogenic Non-Epileptic Seizures (PNES)
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Diagnostic, linguistic, and interactional features yielded by conversation analysis

PNES ES

Patients tend to focus on the situations in which **Patients readily focus on the subjective seizure
seizures have occurred or the consequences of their symptoms.
seizures rather than subjective seizure symptoms.

Subjective seizure symptoms may be listed but are **Subjective seizure symptoms are given in detailed
not described in detail. accounts with extensive formulation efforts
(including reformulations, re-starts, neologisms,
and pauses).

When the doctor tries to direct the patient’s **When the doctor tries to direct the patient’s
attention to particularly memorable seizures (e.g., attention to particularly memorable seizures (e.g.,
the first, last, or worst seizure), patients commonly the first, last, or worst seizure), patients readily
show focusing resistance by not providing further provide more information about their subjective
information or by generalizing rapidly to the seizure symptoms in these particular seizures.
description of their events in general.

Patients tend to catastrophize their seizure Patients tend to normalize their seizure experiences
experiences. when talking to a doctor.

Patients prefer metaphors depicting their seizures as Patients tend to describe their seizures as acting
a place or space they traveled through or to which independently (and often as doing something to the
they were confined. patient).
**features that revealed statistically significant differences between PNES and ES patients.

Table 2.
Conversation analysis diagnostic features in PNES and ES [16].

Signs that favor PNES Evidence from primary studies

Long duration Good

Fluctuating course Good

Asynchronous movements Good*

Pelvic thrusting Good*

Side-to-side head or body movement Good**

Closed eyes Good

Ictal crying Good

Memory recall Good

Signs that favor ES Evidence from primary studies

Occurrence from EEG-confirmed sleep Good

Postictal confusion Good

Stertorous breathing Good

Other signs Evidence from primary studies

Gradual onset Insufficient

Nonstereotyped events Insufficient

Flailing or thrashing movements Insufficient

Opisthotonus “Arc de cercle” Insufficient

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Epilepsy - Seizures Without Triggers

Signs that favor PNES Evidence from primary studies

Tongue biting Insufficient

Urinary incontinence Insufficient


*Frontal lobe partial seizures excluded.**Convulsive events only.

Table 3.
Summary of evidence that supports the signs used to distinguish PNES from ES [6].

Diagnostic History Witnessed event EEG


level

Possible + By witness or self-report or self- No epileptiform activity in routine or


description sleep-deprived interictal EEG

Probable + By clinicians who reviewed recording No epileptiform activity in routine or


or in person, showing semiology sleep-deprived interictal EEG
typical of PNES

Clinically + By clinician experienced in diagnosis No epileptiform activity in routine or


established of seizure disorders (on video or in ambulatory ictal EEG during a typical
person), showing semiology typical of ictus/event in which the semiology
PNES while not on EEG would make ictal epileptiform EEG
activity expectable during equivalent
epileptic seizures

Documented + By clinician experienced in diagnosis No epileptiform activity immediately


of seizure showing semiology typical of before, during, or after ictus captured
PNES while on video EEG on ictal video EEG with typical PNES
semiology

Table 4.
Proposed diagnostic levels of certainty for PNES [6].

reduction of PNES [18]. The summary of four reasonably detailed communication


strategies that have been published [19–22] is shown in Table 5 [23].

10. Treatment of PNES

I.Treatment of the underlying etiological factors and comorbidities:


Recognition and treatment of the “3Ps” (predisposing, precipitating, and
perpetuating factors) are almost always necessary for symptom resolution. It
may even be sufficient to treat the comorbid condition in conjunction with
proper presentation of the diagnosis [12, 13].

II.Patient engagement:
Brief psychoeducation of the patient and motivational interviewing after presenting
diagnosis can reduce ambivalence about treatment and facilitate behavioral
change in favor of the patient’s health and give the patient a sense of control
(internal locus of control) [20]. Motivational interviewing can be particularly
useful in patients who find it difficult to trust their claimed diagnosis and thus
recurrently seek new healthcare providers despite previous findings
documenting PNES [24].
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Covered topic Communication points delivered to patient

Negative diagnosis What you do not have (i.e., epilepsy)


What you do not need (i.e., treatment with AEDs*) – unless needed for
other indications

Diagnostic method How diagnosis was made (i.e., video-EEG* captured typical event)
“It is common!,” frequently seen in long-term monitoring units

Genuine symptoms Symptoms are real, not fabricated

Explanatory model Role of accumulating risk factors over time and automatic functional brain
(positive diagnosis) patterns

Suggestion Some patients improve with reassurance that their events are not epileptic
and once diagnosis is explained

Treatment and expectations There are effective treatments


Psychotherapy works though skills learning, “brain re-training”
There is no sudden cure; treatment requires time and training
*AEDs = antiepileptic drugs, EEG = electroencephalogram.

Table 5.
Diagnosis delivery: Summary of communication protocol [23].

III.Psychotherapeutic interventions:

A. Cognitive behavioral therapy


In a randomized controlled trial that compared cognitive behavioral
therapy (CBT) to standard medical care, individual CBT was evaluated
with a significant reduction in monthly event frequency after 12 sessions
[25]. The following concepts were addressed in the CBT sessions: (1)
treatment engagement; (2) reinforcement of independence; (3)
distraction, relaxation, and refocusing techniques when episode is
imminent; (4) graded exposure to avoided situations; (5) cognitive
restructuring; and (6) relapse prevention.

B. Psychodynamic psychotherapy
Psychodynamic psychotherapy has not been examined as frequently as
CBT, but favorable results have been demonstrated in uncontrolled studies
using individual and group formats [26, 27].

C. Family therapy
Family therapy may be indicated when family system dysfunction is
present since it is a contributor to symptoms of depression and to a poorer
quality of life in PNES [28].

D.Mindfulness techniques
Mindfulness techniques promote the challenging of experiential avoidance
while delineating personal values. In a case series that utilized a
mindfulness-based treatment protocol, event reduction was attained using
mindfulness techniques [29].

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Epilepsy - Seizures Without Triggers

IV.Pharmacotherapy:

A. The pharmacologic treatment of patients should begin with early tapering


and discontinuation of the anti-epileptic drugs (AEDs).

B. In people with mixed epileptic seizures (ES) and PNES, reduce high doses
of AEDs or polytherapy if possible.

C. Use psychopharmacologic agents to treat comorbidities.

Protocol of personalized psychological interventions in PNES

Triage:
• Patient’s thoughts on diagnosis and potential treatment (locus of control, attributions, and perceived
responsibility for recovery)
• Seizures occurrence and response to seizures (seizure description, frequency, hospital contact, and
medications)
• Onset factors (home, work, and life events in the months prior to onset)
• Current circumstances (home, family, work, pastimes, and social support)
• Past history (other illness, traumatic events, and long-term life history)
Treatment:
• Treatment approach was based on a psychological formulation developed with the patient.
• The broad outline of the treatment covered the following: psychoeducation to patients and their
families to develop an understanding of PNES* and awareness of triggers, both external and internal;
considering the context that may both prevent and perpetuate attacks; and identifying the attack
prodromal phase and how to take remedial action.
• While the models used were integrative and varied according to the formulation, intervention was
predominantly delivered in a CBT* framework; other approaches were used on a case-by-case basis (see
below).
• Session 1: assessment and formulation
• Sessions 2–10: interventions are used according to treatment targets that emerge from formulation:
1. When social factors predominate in cause and maintenance:
Family therapy
Interpersonal therapy
Social interventions
2. When internal thought processes/personal conscious behavior predominate in cause and
maintenance:
Cognitive behavioral therapy
Behavioral management advice
3. When internal conflicts such as grief or reaction to past trauma predominate in cause and
maintenance:
Mindfulness and compassionate mind
Acceptance and commitment therapy
Counseling
Focused analytic therapy
Dialectical behavioral therapy
4. When physiological states, current health problems, or habitual reactions to these problems
predominate in cause and maintenance:
Psychological treatment for sleep dysregulation
Cognitive assessment remediation
Behavioral management advice
*CBT = cognitive behavioral therapy, PNES = psychogenic non-epileptic seizures.

Table 6.
Protocol for psychological interventions in PNES.

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Psychogenic Non-Epileptic Seizures (PNES)
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In an attempt to reach a consensus on a specific protocol of psychological inter-


ventions when dealing with PNES, Duncan et al. [30] proposed a protocol (see
Table 6).

11. Evidence-based guide for management of PNES

The ILAE proposed the following management algorithm shown in Table 6 [6] in
an attempt to provide an evidence-based protocol for the management of PNES
(Table 7).

Treatment steps Direct evidence Indirect evidence

Diagnosis X
Consider early X
Investigate (vEEG)

Assessment X
Characterize: X
Neurologic comorbidity X
Psychiatric comorbidity
Social/family conflict

Communication of diagnosis X X
Explain: X
What PNES are not
What PNES are

Psychiatric/psychological treatment X X
Patient engagement X X
Psychotherapy: CBT for PNES X X
Family therapy

Antidepressants X X

Case management X

Rehabilitation X
Note: vEEG = video electroencephalogram, CBT = cognitive behavioral therapy, PNES = psychogenic non-epileptic
seizures.

Table 7.
Management of psychogenic non-epileptic seizure and evidence basis [6] (updated from [31]).

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Epilepsy - Seizures Without Triggers

Author details

Nirmeen A. Kishk1* and Mai B. Nassar2

1 Department of Neurology, School of Medicine, Cairo University, Giza, Egypt

2 Department of Psychiatry, School of Medicine, Cairo University, Giza, Egypt

*Address all correspondence to: [email protected]

© 2022 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of
the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided
the original work is properly cited.
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DOI: http://dx.doi.org/10.5772/intechopen.108418

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