Psychogenic non-epileptic seizure

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Psychogenic non-epileptic seizures
Other namespseudoseizures, dissociative non-epileptic seizures, FNEA (Functional Non-Epileptic Attacks), NEAD (Non-epileptic Attack Disorder) [1]
Specialty Neurology, psychiatry

Psychogenic non-epileptic seizures (PNES), which have been more recently classified as functional seizures, are events resembling an epileptic seizure, but without the characteristic electrical discharges associated with epilepsy. [2] [3] PNES fall under the category of disorders known as functional neurological disorders (FND), also known as conversion disorders. [4] These are typically treated by psychologists or psychiatrists. PNES has previously been called pseudoseizures, psychogenic seizures, and hysterical seizures, but these terms have fallen out of favor. [5]

Contents

Incidence

The number of people with PNES ranges from 2 to 33 per 100,000. [6] PNES are most common in young adults, particularly women. [6] The prevalence for PNES is estimated to make up 5–20% of outpatient epilepsy clinics; 75–80% of these diagnoses are given to female patients and 83% are to individuals between 15 and 35 years old. [7]

Children

PNES are seen in children after the age of eight, and occur equally among boys and girls before puberty. Diagnostic and treatment principles are similar to those for adults, except that in children there is a broader differential diagnosis of seizures so that other possible diagnoses specific to children may be considered. [8]

Signs and symptoms

Individuals with PNES present with episodes that resemble epileptic seizures, and most have received a diagnosis of epilepsy and treatment for it. [9] [10] [11] [12] PNES episodes are nearly indistinguishable from epileptic seizures. The main differences between a PNES episode and an epileptic seizure is the duration of episodes. Epileptic seizures typically last between 30 and 120 seconds depending on the type, while PNES episodes typically last for two to five minutes. [13]

Causes and risk factors

The cause of PNES has not yet been established. One hypothesis is that they are a learned physical reaction or habit the body develops, similar to a reflex. The individual does not have control of the learned reaction, but this can be retrained to allow the patient to control the physical movements again. [12] The production of seizure-like symptoms is not under voluntary control, meaning that the person is not faking; [9] [14] symptoms which are feigned or faked voluntarily would fall under the categories of factitious disorder or malingering. [15]

Risk factors for PNES include having a history of head injury, and having a diagnosis of epilepsy. [16] Approximately 10–30% of people diagnosed with PNES also have an epilepsy diagnosis. People diagnosed with PNES commonly report physical, sexual, or emotional trauma, but the reported incidence of these events may not differ between PNES and epilepsy. [17]

Diagnosis

According to the Diagnostic and Statistical Manual of Mental Disorders (version 5) the criteria for receiving a diagnosis of PNES are: [18]

  1. One or more symptoms of altered voluntary motor or sensory function.
  2. Clinical findings provide evidence of incompatibility between the symptom and recognized neurological or medical conditions.
  3. The symptom or deficit is not better explained by another medical or mental disorder.
  4. The symptom or deficit causes clinically significant distress or impairment in social, occupational, or other important areas of functioning or warrants medical evaluation.

Additionally, the specific symptom type must be reported "with attacks or seizures". [18]

Some individuals with PNES have carried an erroneous diagnosis of epilepsy. On average, it takes seven years to receive a proper diagnosis. The differential diagnosis of PNES firstly involves ruling out epilepsy as the cause of the seizure episodes, along with other organic causes of non-epileptic seizures, including syncope, migraine, vertigo, anoxia, hypoglycemia, and stroke. However, 5–20% of people with PNES also have epilepsy. [19] Frontal lobe seizures can be mistaken for PNES, though these tend to have shorter duration, stereotyped patterns of movements, and occurrence during sleep. [20] Next, an exclusion of factitious disorder (a subconscious somatic symptom disorder, where seizures are caused by psychological reasons) and malingering (simulating seizures intentionally for conscious personal gain – such as monetary compensation or avoidance of criminal punishment) is conducted. Finally other psychiatric conditions that may superficially resemble seizures are eliminated, including panic disorder, schizophrenia, and depersonalisation disorder. [20]

The most definitive test to distinguish epilepsy from PNES is long term video-EEG monitoring, with the aim of capturing one or two episodes on both video recording and EEG simultaneously (some clinicians may use suggestion to attempt to trigger an episode). [21] Additional clinical criteria are usually considered in addition to video-EEG monitoring when diagnosing PNES. [22] By recording the event in question on video and EEG simultaneously, a clear diagnosis can usually be obtained. [23]

Laboratory testing can detect rising blood levels of serum prolactin if samples are taken in the right time window after most tonic-clonic or complex partial epileptic seizures. However, due to false positives and variability in results, this test is relied upon less frequently. [20]

Distinguishing features

Some features are more or less likely to suggest PNES but they are not conclusive and should be considered in the broader clinical picture. Features that are common in PNES but rarer in epilepsy include: biting the tip of the tongue, seizures lasting more than two minutes (easiest factor to distinguish), seizures having a gradual onset, a fluctuating course of disease severity, the eyes being closed during a seizure, and side to side head movements. Features that are uncommon in PNES include automatisms (automatic complex movements during the seizure), severe tongue biting, biting the inside of the mouth, and incontinence. [20]

If a person with suspected PNES has an episode during a clinical examination, there are a number of signs that can be elicited to help support or refute the diagnosis of PNES. Compared to people with epilepsy, people with PNES will tend to resist having their eyes forced open (if they are closed during the seizure), will stop their hands from hitting their own face if the hand is dropped over the head, and will fixate their eyes in a way suggesting an absence of neurological interference. [20]

Treatment

Patient understanding of the new diagnosis is crucial for their treatment, which requires their active participation. [24] There are a number of recommended steps to explain to people their diagnosis in a sensitive and open manner. A negative diagnosis experience may cause frustration and could cause a person to reject any further attempts at treatment. Eight points recommended to explain the diagnosis to the person and their caregivers are:

  1. Reasons for concluding they do not have epilepsy
  2. What they do have (describe FND)
  3. Emphasize they are not suspected of "putting on" the attacks, and the symptoms are not "all in their head"
  4. There may be no triggering "stresses"
  5. Maintaining factors
  6. May improve after correct diagnosis
  7. Caution that anticonvulsant drug withdrawal should be done in conjunction with their physician
  8. Describe treatment to help regain control of symptoms

Psychotherapy is the most frequently used treatment, which might include cognitive behavioral therapy or therapy to retrain the physical symptoms and allow the individual to regain control of the attacks (ReACT). There is also some evidence supporting selective serotonin reuptake inhibitor antidepressants. [25]

Cognitive behavioral therapy [26]

Cognitive behavioral therapy (CBT) treatments for PNES typically target fear avoidance and work to reattribute patients' symptoms to psychosocial issues.

Retraining and Control Therapy (ReACT)

ReACT, while new and understudied, has shown extremely promising outcomes for reduction of PNES episodes in pediatric patients. [27] This therapy focuses on the idea that PNES are caused by a learned physical reaction or habit the body develops, similar to a reflex. ReACT aims to retrain the learned reaction (PNES episodes) by targeting symptom catastrophizing and restoring sense of control over symptoms.

Prognosis

Functional seizures have been found to be as disabling and costly as epilepsy. [28] Though there is limited evidence, outcomes appear to be relatively poor with a review of outcome studies finding that two-thirds of people with PNES continue to experience episodes and more than half are dependent on the Social Security program at three-year follow-up. [24] This outcome data was obtained in a referral-based academic epilepsy center and loss to follow-up was considerable; the authors point out ways in which this may have biased their outcome data. Outcome was shown to be better in people with higher IQ, [29] social status, [30] greater educational attainments, [31] younger age of onset and diagnosis, [31] attacks with less dramatic features, [31] and fewer additional somatoform complaints. [31]

For individuals who pursue treatment for PNES, CBT has shown varying rates of success but it has been established as one of the most promising treatments to date. [32] ReACT has shown reduction in symptoms by 100% seven days after treatment and 82% of individuals who completed the therapy remained symptom free for 60 days. A follow-up has not been done to see if the therapy retained its reduction of symptoms beyond the 60 days. [27] In the Cognitive behavioural therapy for adults with dissociative seizures (CODES) trial, the largest regarding CBT treatment for PNES though found no significant reduction in monthly seizures compared to the control arm at 12 months, however there were significant improvements on a number of secondary outcomes, such as psychosocial functioning, and self-rated and clinician-rated global change. [33] A secondary analysis of the CODES trial demonstrated improved frequency of functional seizures at 6 months with CBT.

History

Hystero-epilepsy is a historical term that refers to a condition described by 19th-century French neurologist Jean-Martin Charcot [34] where people with neuroses "acquired" symptoms resembling seizures as a result of being treated on the same ward as people who genuinely had epilepsy.

The etiology of FND was historically explained in the context of psychoanalytic theory as a physical manifestation of psychological distress and repressed trauma. There is very little supporting evidence for this theory, as there is little research. [35]

The DSM-IV lists conversion disorders instead of the current FND. Additionally, in revision, the DSM-5 was updated to add emphasis to the positive physical signs inconsistent with recognized diseases. The requirement of a history of psychological stressors and that the symptom is not fake was removed as well. [36]

Society and culture

PNES rates and presenting symptoms are somewhat dependent on the culture and society. In some cultures, they, like epilepsy, are thought of as a curse or a demonic possession. [37] In cultures with a solid establishment of evidence-based medicine, they are considered a subtype of a larger category of psychiatric disease.

Terminology

The use of older terms including pseudoseizures and hysterical seizures are discouraged. [38] In the English language, the word "seizure" usually refers to epileptic events, so some prefer to use more general terms like "events", "attacks", or "episodes", as the term "seizures" may cause confusion with epilepsy. [39] [40]

PNES may also be referred to as "non-epileptic attack disorder" "functional seizures", "dissociative convulsions" or "dissociative non-epileptic seizures". These terms are more neutral as to cause, and given that a psychological cause cannot be identified in many cases, they may be more appropriate. Within DSM 5, patients presenting with PNES may meet the criteria for functional neurological disorder and in some cases, somatic symptom disorder, whilst in ICD 10 it may meet the criteria for a conversion disorder. [20]

Related Research Articles

<span class="mw-page-title-main">Epilepsy</span> Group of neurological disorders causing seizures

Epilepsy is a group of non-communicable neurological disorders characterized by recurrent epileptic seizures. An epileptic seizure is the clinical manifestation of an abnormal, excessive, and synchronized electrical discharge in the brain cells called neurons. The occurrence of two or more unprovoked seizures defines epilepsy. The occurrence of just one seizure may warrant the definition in a more clinical usage where recurrence may be able to be prejudged. Epileptic seizures can vary from brief and nearly undetectable periods to long periods of vigorous shaking due to abnormal electrical activity in the brain. These episodes can result in physical injuries, either directly such as broken bones or through causing accidents. In epilepsy, seizures tend to recur and may have no immediate underlying cause. Isolated seizures that are provoked by a specific cause such as poisoning are not deemed to represent epilepsy. People with epilepsy may be treated differently in various areas of the world and experience varying degrees of social stigma due to the alarming nature of their symptoms.

<span class="mw-page-title-main">Seizure</span> Period of symptoms due to excessive or synchronous neuronal brain activity

An epileptic seizure, informally known as a seizure, is a period of symptoms due to abnormally excessive or synchronous neuronal activity in the brain. Outward effects vary from uncontrolled shaking movements involving much of the body with loss of consciousness, to shaking movements involving only part of the body with variable levels of consciousness, to a subtle momentary loss of awareness. These episodes usually last less than two minutes and it takes some time to return to normal. Loss of bladder control may occur.

A convulsion is a medical condition where the body muscles contract and relax rapidly and repeatedly, resulting in uncontrolled shaking. Because epileptic seizures typically include convulsions, the term convulsion is often used as a synonym for seizure. However, not all epileptic seizures result in convulsions, and not all convulsions are caused by epileptic seizures. Non-epileptic convulsions have no relation with epilepsy, and are caused by non-epileptic seizures.

A headache is often present in patients with epilepsy. If the headache occurs in the vicinity of a seizure, it is defined as peri-ictal headache, which can occur either before (pre-ictal) or after (post-ictal) the seizure, to which the term ictal refers. An ictal headache itself may or may not be an epileptic manifestation. In the first case it is defined as ictal epileptic headache or simply epileptic headache. It is a real painful seizure, that can remain isolated or be followed by other manifestations of the seizure. On the other hand, the ictal non-epileptic headache is a headache that occurs during a seizure but it is not due to an epileptic mechanism. When the headache does not occur in the vicinity of a seizure it is defined as inter-ictal headache. In this case it is a disorder autonomous from epilepsy, that is a comorbidity.

<span class="mw-page-title-main">Conversion disorder</span> Diagnostic category used in some psychiatric classification systems

Conversion disorder (CD), or functional neurologic symptom disorder, is a diagnostic category used in some psychiatric classification systems. It is sometimes applied to patients who present with neurological symptoms, such as numbness, blindness, paralysis, or fits, which are not consistent with a well-established organic cause, which cause significant distress, and can be traced back to a psychological trigger. It is thought that these symptoms arise in response to stressful situations affecting a patient's mental health or an ongoing mental health condition such as depression. Conversion disorder was retained in DSM-5, but given the subtitle functional neurological symptom disorder. The new criteria cover the same range of symptoms, but remove the requirements for a psychological stressor to be present and for feigning to be disproved. The ICD-10 classifies conversion disorder as a dissociative disorder, and the ICD-11 as a dissociative disorder with unspecified neurological symptoms. However, the DSM-IV classifies conversion disorder as a somatoform disorder.

Landau–Kleffner syndrome (LKS)—also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder—is a rare childhood neurological syndrome.

<span class="mw-page-title-main">Aura (symptom)</span> Symptom of epilepsy and migraine

An aura is a perceptual disturbance experienced by some with epilepsy or migraine. An epileptic aura is a seizure.

Frontal lobe epilepsy (FLE) is a neurological disorder that is characterized by brief, recurring seizures arising in the frontal lobes of the brain, that often occur during sleep. It is the second most common type of epilepsy after temporal lobe epilepsy (TLE), and is related to the temporal form in that both forms are characterized by partial (focal) seizures.

Dissociative amnesia or psychogenic amnesia is a dissociative disorder "characterized by retrospectively reported memory gaps. These gaps involve an inability to recall personal information, usually of a traumatic or stressful nature." In a change from the DSM-IV to the DSM-5, dissociative fugue is now subsumed under dissociative amnesia.

Abdominal epilepsy is a rare condition most frequently found in children, consisting of gastrointestinal disturbances caused by epileptiform seizure activity. Though a few cases of it have been reported in adults too. It has been described as a type of temporal lobe epilepsy. Responsiveness to anticonvulsants can aid in the diagnosis. Distinguishing features of abdominal epilepsy include (1) Abnormal laboratory, radiographic, and endoscopic findings revealing paroxysmal GI manifestations of unknown origin (2) CNS symptoms (3) Abnormal EEG. Most published medical literature dealing with abdominal epilepsy is in the form of individual case reports. A 2005 review article found a total of 36 cases described in the medical literature.

Juvenile myoclonic epilepsy (JME), also known as Janz syndrome or impulsive petit mal, is a form of hereditary, idiopathic generalized epilepsy, representing 5–10% of all epilepsy cases. Typically it first presents between the ages of 12 and 18 with myoclonic seizures. These events typically occur after awakening from sleep, during the evening or when sleep-deprived. JME is also characterized by generalized tonic–clonic seizures, and a minority of patients have absence seizures. It was first described by Théodore Herpin in 1857. Understanding of the genetics of JME has been rapidly evolving since the 1990s, and over 20 chromosomal loci and multiple genes have been identified. Given the genetic and clinical heterogeneity of JME some authors have suggested that it should be thought of as a spectrum disorder.

Classified as a "conversion disorder" by the DSM-IV, a psychogenic disease is a condition in which mental stressors cause physical symptoms matching other disorders. The manifestation of physical symptoms without biologically identifiable cause results from disruptions in normal brain function due to psychological stress. During a psychogenic episode, neuroimaging has shown that neural circuits affecting functions such as emotion, executive functioning, perception, movement, and volition are inhibited. These disruptions become strong enough to prevent the brain from voluntarily allowing certain actions. When the brain is unable to signal to the body to perform an action voluntarily, physical symptoms of a disorder arise. Examples of diseases that are deemed to be psychogenic in origin include psychogenic seizures, psychogenic polydipsia, psychogenic tremor, and psychogenic pain.

Progressive Myoclonic Epilepsies (PME) are a rare group of inherited neurodegenerative diseases characterized by myoclonus, resistance to treatment, and neurological deterioration. The cause of PME depends largely on the type of PME. Most PMEs are caused by autosomal dominant or recessive and mitochondrial mutations. The location of the mutation also affects the inheritance and treatment of PME. Diagnosing PME is difficult due to their genetic heterogeneity and the lack of a genetic mutation identified in some patients. The prognosis depends largely on the worsening symptoms and failure to respond to treatment. There is no current cure for PME and treatment focuses on managing myoclonus and seizures through antiepileptic medication (AED).

Functional disorder is an umbrella term for a group of recognisable medical conditions which are due to changes to the functioning of the systems of the body rather than due to a disease affecting the structure of the body.

Migralepsy is a rare condition in which a migraine is followed, within an hour period, by an epileptic seizure. Because of the similarities in signs, symptoms, and treatments of both conditions, such as the neurological basis, the psychological issues, and the autonomic distress that is created from them, they individually increase the likelihood of causing the other. However, also because of the sameness, they are often misdiagnosed for each other, as migralepsy rarely occurs.

<span class="mw-page-title-main">Epilepsy in children</span>

Epilepsy is a neurological condition of recurrent episodes of unprovoked epileptic seizures. A seizure is an abnormal neuronal brain activity that can cause intellectual, emotional, and social consequences. Epilepsy affects children and adults of all ages and races, it is one of the most common neurological disorders of the nervous system. As well as, this condition is more common among children than adults affecting about 6 out of 1000 US children that are between the age of 0 to 5 years old. The epileptic seizures can be of different types depending on the part of the brain that was affected, seizures are classified in 2 main types partial seizure or genralized seizure.

Forced Normalization (FN) is a psychiatric phenomenon in which a long term episodic epilepsy or migraine disorder is treated, and, although the electroencephalogram (EEG) appears to have stabilized, acute behavioral, mood, and psychological disturbances begin to manifest. If, or when, treatment for the disorder is halted, the disturbances go away, but the episodic spikes on the EEG reappear. H. Landolt coined the term 'Forced Normalization' in 1953 in response to a change he witnessed in epileptic EEGs, which monitor electrical activity in the brain. These changes were followed by abrupt behavioral changes in the patient. Landolt concluded that forced normalization is "the phenomenon characterized by the fact that, with the occurrence of psychotic states, the electroencephalography becomes more normal or entirely normal, as compared with previous and subsequent EEG findings." Forced normalization, as described by Landolt, was therefore an electrophysiological phenomenon with the electroencephalograph at its helm.

A neonatal seizure is a seizure in a baby younger than age 4-weeks that is identifiable by an electrical recording of the brain. It is an occurrence of abnormal, paroxysmal, and persistent ictal rhythm with an amplitude of 2 microvolts in the electroencephalogram,. These may be manifested in form of stiffening or jerking of limbs or trunk. Sometimes random eye movements, cycling movements of legs, tonic eyeball movements, and lip-smacking movements may be observed. Alteration in heart rate, blood pressure, respiration, salivation, pupillary dilation, and other associated paroxysmal changes in the autonomic nervous system of infants may be caused due to these seizures. Often these changes are observed along with the observance of other clinical symptoms. A neonatal seizure may or may not be epileptic. Some of them may be provoked. Most neonatal seizures are due to secondary causes. With hypoxic ischemic encephalopathy being the most common cause in full term infants and intraventricular hemorrhage as the most common cause in preterm infants.

Functional neurologic disorder or functional neurological disorder (FND) is a condition in which patients experience neurological symptoms such as weakness, movement disorders, sensory symptoms, and blackouts. As a functional disorder, there is by definition no known disease process affecting the structure of the body, yet the person experiences symptoms relating to their body function. Symptoms of functional neurological disorders are clinically recognisable, but are not categorically associated with a definable organic disease.

Musicogenic seizure, also known as music-induced seizure, is a rare type of seizure, with an estimated prevalence of 1 in 10,000,000 individuals, that arises from disorganized or abnormal brain electrical activity when a person hears or is exposed to a specific type of sound or musical stimuli. There are challenges when diagnosing a music-induced seizure due to the broad scope of triggers, and time delay between a stimulus and seizure. In addition, the causes of musicogenic seizures are not well-established as solely limited cases and research have been discovered and conducted respectively. Nevertheless, the current understanding of the mechanism behind musicogenic seizure is that music triggers the part of the brain that is responsible for evoking an emotion associated with that music. Dysfunction in this system leads to an abnormal release of dopamine, eventually inducing seizure.

References

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