Psychogenic Non-epileptic Seizures (PNES)

Case
A 12-year-old child was brought in with a complaint of repeated episodes of "seizures" of 4 years duration. The episodes were characterized by tonic-clonic movements of all four limbs associated with blinking of eyes. The child was "unresponsive" and had urinary incontinence during each of the episodes. There was no history of tongue biting, falling or sustaining injuries. The child had 8-10 episodes per day and there were no episodes during nights. Though majority of episodes occurred in front of others, a few occurred when none was around. He was previously treated with various antiepileptic drugs and was on maximum therapeutic doses of phenytoin, carbamazepine and clobazam at presentation. Clinical examination was normal. MRI scan of the brain and EEG were normal. Video-EEG monitoring showed that the childs attacks were non-epileptic in origin as during the attacks, he would stand up and jerk his limbs in a bizarre fashion and pass urine. He would maintain eye contact but would not answer questions. The episode would last for about 5-30 minutes and concomitant EEG recording was normal except for movement artifacts.

Introduction
Epileptic seizure: a transient occurrence of signs and/or symptoms due to abnormally excessive or synchronous neuronal activity in the brain
Characteristic epileptiform changes seen on video electroencephalography (vEEG).

Epilepsy: a condition characterized by recurrent, unprovoked seizures.

At least 2 unprovoked seizures 24 hours apart

Introduction
Psychogenic non-epileptic seizure (PNES): sudden, time-limited disturbances of motor, sensory, or altered consciousness without epileptiform change on vEEG monitoring
Symptoms mimic epileptic seizures Not associated with physiological CNS dysfunction, but are instead psychogenic in origin DSM-IV: classified as a somatoform disorder, specifically a type of conversion disorder

 Other names:
Pseudoseizures Non-epileptic attack disorder Functional seizures Hysterical seizures Hysteroepilepsy

Epidemiology
Estimated prevalence: 2 - 33 per 100,000 Most frequent non-epileptic condition seen in epilepsy centers Among patients referred to outpatient epilepsy centers:
5-25% of patients are believed to have PNES 25-40% of patients evaluated in inpatient epilepsy monitoring units for intractable seizures are diagnosed with PNES

Affects patients of all age groups Most commonly presents in the 3rd decade of life
Patients with learning disabilities may have a relatively younger age of onset

Female predominance Race, marital status, and years of education do not appear to influence the prevalence of PNES

Etiology
Physical symptoms occur in response to psychosocial stress, but are not intentionally produced Psychosocial stressors often precipitate or exacerbate PNES episodes, although the patient may not knowingly connect the two
Bereavement, job pressure, unwanted pregnancy, ongoing physical, verbal, or sexual abuse, lawsuits, job pressure, financial difficulties, impending divorce, domestic conflicts, and assault Children: separation anxiety, school avoidance, divorce, familial discord

Differential Diagnosis
Epileptic seizures Substance withdrawal seizures: alcohol withdrawal, BZD withdrawal Syncope Migraine Panic attacks Episodic dyscontrol Sleep disorders
Narcolepsy, nocturnal paroxysmal dystonia, night terrors

Paroxysmal movement disorders:

Dystonia, ballism, chorea, hemifacial spasm, blepharospasm, Meige syndrome

Clinical Features
No single feature is either sensitive or specific for PNES Compared to epileptic seizures:
Events tend not to occur during sleep Most episodes occur in front of witnesses Episodes are often frequent most patients report at least daily episodes Longer duration (> 2 minutes) Ictal movements more often asynchronous, variable, and wax and wane over the course of the ictus Specific movements such as writhing, thrashing, pelvic thrusts, opisthotonus (arched back), and jactitation (rolling from side to side) Ictal eye closing, crying, stuttering, and vocalizations Absence of tongue biting, falling, incontinence Incomplete loss of consciousness during the episode

 Post-ictal symptoms:
Absence of post-ictal confusion Rapid alerting and reorientation Post-ictal headache are rare

Resistance to antiepileptic drugs (AEDs)

Psychiatric conditions associated with PNES

Depression Anxiety Somatoform disorder PTSD Dissociative disorder Personality disorders
Especially borderline personality disorder (but also narcissistic, histrionic, and anti-social personalities)

Sexual or physical abuse Dysfunctional family relationships

Poor communication or support, interpersonal conflict, trauma

History
Detailed description of the event as perceived by the patient and as witnessed by others
Prodromal and post-ictal features

Precipitants & circumstances in which episodes occur Episode frequency &duration Factors that reduce seizure frequency or attenuate an episode

Physical exam
Physical examination of patients with PNES may be:
Completely normal Demonstrate neurologic abnormalities associated with unrelated neurologic disorders Exhibit classic signs of conversion disorder

Diagnosis
Diagnosis of exclusion
Exclusion of neurological disease Exclusion of feigning Determination of a psychological factors assoc. w/ initiation or exacerbation of symptoms

Video EEG is the gold standard test for the diagnosis of PNES, and should be performed in all patients in whom this diagnosis is suspected
Despite the ability to diagnose PNES with high certainty by using vEEG monitoring, the time to diagnosis is long (~ 7-10 years)

Spell induction: administer IV saline or rubbing alcohol on the skin of the patient and telling the patient that this may induce an episode, with adjunctive vEEG monitoring.
Helpful if the patient fails to have a spontaneous event during prolonged video-EEG monitoring Between 67- 90% of patients with PNES will have a typical event in this setting

 Outpatient ambulatory EEG: ideal with concomitant video, if prolonged vEEG and induction techniques do not produce any episodes Advise family members to have a video camera ready to record on stand-by Serum prolactin testing: prolactin levels are often elevated after an epileptic seizure, depending on the seizure type
Prolactin level elevations (2x baseline level) occur in 60% of generalized tonic clonic seizures and 46% of complex partial seizures Prolactin levels are unlikely to rise after a PNES

Treatment
Communicate the diagnosis of PNES to the patient, only once the clinician feels very definite about the diagnosis.
State that we do not consider the episodes to be caused by epileptic discharges, but rather represent "the mind playing tricks on the body. Emphasize that this problem is as serious as one caused by epilepsy and deserves the attention and treatment that is accorded any illness. Discuss how stress afflicts each of us to variable degrees and in different ways. We note that "the body needs to blow off steam" in some fashion; some people release this stress with the development of headaches, others with tremors, and some with non-epileptic seizures. Emphasize that the episodes are experienced as real and disabling, and that although they relate to emotional or psychological causes, we do not dismiss the problem, and we do not consider the patient to be "crazy. State that their disorder warrants different treatments than those administered for epilepsy. Tell the patient that we expect that they may react in many ways to this diagnosis. Many patients become angry in response to receiving the diagnosis. Some may acknowledge these feelings, while others may not. An angry reaction may forebode a poor prognosis. In our experience, it is useful to anticipate these feelings at the time of revealing the diagnosis. State that physicians have found that patients may exhibit exacerbations of their episodes after the diagnosis is revealed. Warn the patient not to be surprised if this were to occur.

Treatment
Neurologic and psychiatric follow-up
Close dialogue should also be developed and maintained between neurologic, psychiatric, and PCP

Pharmacotherapy:
Cautious discontinuation of AED therapy
Episodes may increase during this time period In some cases, epileptic seizures as well as PNES emerge during this interval

Antidepressants & anxiolytics may better address psychiatric comorbidities of depression and anxiety, rather than the underlying causative psychiatric disorder.

Psychotherapy:
Cognitive behavioral therapy Psychodynamic interpersonal therapy

In some cases, patients may begin to recover spontaneously.

Implications
It is important that clinicians consider PNES when evaluating patients with episodic seizure-like symptoms. Missing the diagnosis of PNES may result in inappropriate treatment with antiepileptic drugs that are associated with potential morbidity, especially if drug toxicity occurs in the attempt to suppress episodes Prolonged PNES episodes are often treated with toxic doses of AEDs, intubation, and iatrogenically induced coma. When PNES occur during pregnancy, these treatments can pose additional risks to the fetus. Uncontrolled or unrecognized PNES results in recurrent emergency room visits and hospitalizations, placing a high cost burden on the healthcare system. Failure to recognize underlying psychiatric issues can prolong the persistence of conversion symptoms and deny the patient necessary psychiatric interventions.

References
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