Psychogenic Nonepileptic Seizure Case File

Psychogenic Nonepileptic Seizure Case File

Eugene C. Toy, MD, Ericka Simpson, MD, Pedro Mancias, MD, Erin E. Furr-Stimming, MD

CASE 17

A 27-year-old young professional was at the office late one night preparing for a presentation for a board meeting the next day. He told his coworker he was not at all worried about the presentation. Suddenly, he stopped talking, stared at the wall, and slid off of his chair onto the floor. His arms and legs suddenly began shaking uncontrollably with asynchronous movements of fluctuating intensity. He closed his eyes and complained about the pain during the event. He remained responsive during the event, and afterward he began mumbling incoherently. He was never incontinent of urine or stool, and he did not bite his tongue or injure himself when he fell from the chair. He was quickly taken to the emergency room by his coworker.

▶ What is the most likely diagnosis?

▶ What is the next diagnostic step?

▶ What is the most likely useful consultation?

ANSWERS TO CASE 17:

Psychogenic Nonepileptic Seizure                                        

Summary: A 27-year-old man suddenly “seized” with all four extremities involved with sudden asynchronous movements of fluctuating intensity, remained conscious with eyes closed, and complained about pain during the event. He did not experience any confusion after the event.

• Most likely diagnosis: Psychogenic nonepileptic seizure (PNES).
• Next diagnostic step: A thorough neurologic and psychiatric assessment. Video electroencephalography (EEG) is the gold standard for diagnosis when available. Laboratory studies, brain imaging, and prolonged video EEG monitoring may need to be obtained.
• Consultation: Initiate interaction with a neurologist and recognize that patients with PNES may also have true epileptic seizures. Furthermore, without EEG monitoring during an event, it can be impossible to distinguish between some epileptic seizures and nonepileptic events. After a thorough neurologic evaluation has been performed and a diagnosis of PNES is made, a consultation with a psychiatrist should be obtained.

ANALYSIS

Objectives

1. Know diagnostic approach to PNESs.
2. Understand that PNESs reflect psychodynamic issues and can occur in patients who also have true epileptic seizures.

Considerations

PNESs were formerly referred to as pseudoseizures. This term is misleading and should not be used to describe or diagnose these events, as it suggests that the events are not real. PNESs are one of the most misunderstood areas in neurology. A good example that helps clarify this situation is the physical symptoms of distress that many people experience when in an anxiety-provoking situation (such as speaking to a large audience). A person may experience nausea, decreased appetite, and indigestion that resolve when the stressor is removed. They do not have any underlying gastrointestinal disease, but they experience the symptoms of it. Diagnostic tests and procedures would be unlikely to reveal abnormalities. The fact that the symptoms were produced secondary to the stressful situation in the setting of a normal and healthy gastrointestinal system does not mean the person’s symptoms were any less real. The symptoms and the experience were real, but the gastrointestinal system was healthy; it was responding physically to psychological stress. Conversion disorders are another example of an aberrant neurologic response to psychological distress.

In PNES, patients have seizure-like events that occur as an expression of psychological stress but are not produced by an abnormal cortical electrical discharge in the brain. These patients are not consciously pretending to have a seizure, the events are very real to them, and they should be carefully evaluated and appropriately treated. There are clinical characteristics that help distinguish these PNESs from epileptic seizures. The patient, in this case, had generalized (all four extremities) motor activity yet was conscious and aware of his surroundings. This level of consciousness is inconsistent with bilateral cerebral hemisphere epileptic activity. He also had sudden asynchronous movements of fluctuating intensity, which is not characteristic of the movement produced from cerebral electrical activity.

APPROACH TO:

Psychogenic Nonepileptic Seizures                                        

DEFINITIONS

PNES: An attack clinically resembling an epileptic seizure without any abnormal cerebral electrical activity with a purely psychological cause.

Malingering: Intentional production of false or exaggerated symptoms motivated by external incentives, such as obtaining compensation or drugs, avoiding work or military duty, or evading criminal prosecution. Malingering is not considered a mental illness.

Conversion disorder (functional neurologic symptom disorder): One or more motor or sensory symptoms which cause psychosocial impairment, distress, or warrant medical evaluation, which are not intentionally produced, are not of conscious control, and are clinically incompatible with the examination findings.

CLINICAL APPROACH

Etiologies and Clinical Presentation

PNESs have been described previously as hysterical attacks, pseudoseizures, and psychogenic seizures. These latter terms should not be used to refer to the events, as they imply that the event is not real and this can have a negative impact on the treating physician’s relationship with the patient and the support the patient receives from caregivers. These events are not the result of intentional production, as in malingering, but rather, these are the result of a somatoform disorder, such as conversion disorder.

PNES has been found to have a higher prevalence in females, but males may have more frequent episodes and the diagnosis may be delayed. Most of the patients will have a history of physical, sexual, or other abuse, a dysfunctional family relationship, psychiatric comorbidities such as anxiety, posttraumatic stress disorder (PTSD), or cluster B personality disorder, attention-seeking behavior, or a combination of these features. It is important to speak with the patient in private and inquire about present or past abuse.

Clinically, PNES may be very difficult to distinguish from true epileptic seizures; thus, a period of prolonged video EEG monitoring is the current gold standard to reach the correct diagnosis. The differences between epileptic seizures and PNES are difficult to recognize, even for trained medical professionals. The features suggestive of PNES include the following:

• (Forceful) eye closure during the event
• Asynchronous convulsive movements (ie, bicycle kicking) that may fluctuate in intensity or start/stop suddenly
• Pelvic thrusting
• Side-to-side head movements and opisthotonus (arching of the back)
• Controlled falling
• Frequent generalized events without any type of serious injury
• Predictable occurrence in the presence of a witness and in response to a psychological trigger
• Prolonged unresponsiveness during an event (with a normal EEG)
• Longer duration of events (most true epileptic seizures last <3 minutes, and most PNESs last >10 minutes)

Patients with PNES are less likely to report or exhibit postictal confusion. Falls during these psychogenic attacks rarely physically traumatize the patient. Tongue biting and urinary incontinence can occur but are not common.

Bilateral seizure activity without confusion or unconsciousness (ie, able to talk coherently to the examiner while their arms and legs are shaking) is rarely organic. Bilateral seizure activity in the brain is usually associated with altered consciousness because both hemispheres in the brain are compromised. PNES should be considered when a patient with seizures has a normal neurologic evaluation/assessment (often including normal EEG recording during the seizure), and the seizures are not only refractory to treatment but are also influencing family members and impacting the patient’s life (this can also occur in organic seizures) in areas with psychodynamic meaning/importance.

It should be noted that some frontal lobe seizures can produce bizarre bilateral movements with preservation of consciousness, so it should not be assumed a patient has PNES without completing a thorough assessment. When the episodes are recorded on video EEG, PNES is not associated with electrical abnormalities in the brain, as is the case with epileptic seizures.

PNES and epilepsy are not mutually exclusive conditions. Approximately 1% to 2% of patients with PNES also have true epileptic seizures or a prior history of them. PNES should be distinguished from malingering, which can be difficult. The boundaries between conscious and subconscious behavior in psychogenic seizures can be difficult to detect. True malingerers can prove quite resourceful in pretending to have focal abnormalities on examination, even producing voluntary Babinski signs. As opposed to malingering, PNES is distressing to patients, and these patients want treatment. The proper diagnosis of PNES is important, as it will prevent patients from being prescribed unnecessary antiepileptic medications, many of which carry serious adverse effects.

Physicians believe PNESs are either psychological defense mechanisms or maladaptive processing induced by stress or episodes of severe emotional trauma. It is estimated that 20% to 30% of patients referred to an epilepsy center for monitoring are found to have PNES. The diagnosis of nonepileptic seizures has become more prevalent with a better understanding of the psychological issues related to these events and correlation of these changes with normal brain activity. It is important for the physician to recognize that these patients want help, and it is inappropriate to view them as trying to “fake out” the doctor.

Treatment

The earlier in the syndrome the patient is diagnosed, the better the chances for complete recovery. After a neurologist has ruled out epilepsy, the patient should be informed that the condition is not organic in nature and will not require antiepileptic medications. The patient should then be referred to a psychiatrist for psychoeducation and cognitive behavioral therapy (CBT). CBT addresses the maladaptive coping thinking, skills, and behaviors that are thought to manifest as PNES. The psychiatrist should also assess for and treat comorbidities (anxiety, depression, PTSD) if present. A multidisciplinary approach is the best management for patients to help resolve both the old and new stresses in their lives, and a significant portion of the patient’s symptoms can be eliminated. Cognitive behavioral therapy has been shown to be effective.

CASE CORRELATION

• See Case 15 (Absence Versus Partial Complex Seizures) and Case 16 (Cardiogenic Syncope)

COMPREHENSION QUESTIONS

17.1 A 35-year-old man is suspected to have PNES. Which of the following is the best method to confirm the diagnosis?

A. Routine EEG monitoring

B. Initiation of antiepileptic therapy and observation

C. Psychiatric evaluation

D. Video EEG monitoring

17.2 A 23-year-old man is noted to have tonic-clonic activity while yelling and screaming for a fire extinguisher after a stressful event. Which of the following is the most likely etiology?

A. Malingering

B. Psychological defense mechanism to a significant traumatic event

C. Hypertensive encephalopathy

D. Complex partial seizure

17.3 Which of the following is most suggestive of PNESs?

A. Cocaine found on urine drug screen

B. Oxygen saturation level of 80%

C. Alert with generalized (bilateral) convulsions

D. History of diabetes mellitus

ANSWERS

17.1 D. About 1% to 2% of patients with epilepsy also have PNES. Therefore, patients often require invasive and/or noninvasive video EEG monitoring to determine whether true epileptic events are present. For the vast majority of patients with PNES, termination of antiepileptic therapy is recommended.

17.2 B. PNESs, like many psychoneurologic syndromes, have a psychological origin and are often associated with a past history of a significant emotional or physical traumatic life event(s). PNESs are distinguished from factitious or malingering in that the former is subconscious and the latter is purposeful and on the conscious level.

17.3 C. Generalized convulsions or bilateral convulsions are typically associated with loss of consciousness or significantly impaired alteration of consciousness, which can last several minutes after the ictal event. Thus, being conscious during a generalized seizure is most suggestive of a psychogenic disorder.

    CLINICAL PEARLS    

▶ PNESs should be considered when patients bilaterally “seize” or have bilateral tonic-clonic activity but maintain normal consciousness. ▶ Patients with PNES do not have volitional control over their episodes of seizure-like activity and are not malingering. ▶ Sexual abuse and head injury are important risk factors of PNES and are reported in approximately one-third of patients. ▶ PNESs can coexist with organic seizures in 5% of patients with epileptic seizures. Remember, patients with epileptic seizures can have nonepileptic events, and patients with nonepileptic events can have epileptic seizures. ▶ Pseudoneurologic or nonorganic syndromes can mimic almost any neurologic disease. Presenting syndromes can include pseudoparalysis, pseudosensory syndromes, PNES, pseudocoma, psychogenic movement disorders, and pseudoneuroophthalmologic syndromes.

REFERENCES

Bazil CW, Morrell MJ, Pedley TA. Epilepsy. In: Rowland LP, ed. Merritt’s Neurology. 11th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2005:990-1014. 

Bodde NM, Brooks JL, Baker GA, et al. Psychogenic non-epileptic seizures—definition, etiology, treatment and prognostic issues: a critical review. Seizure. 2009;18(8):543-553. 

Gedzelman ER, LaRoche SM. Long-term video EEG monitoring for diagnosis of psychogenic nonepileptic seizures. Neuropsychiatr Dis Treat. 2014:10;1979-1986. 

Jain SK, Ettinger AB. Psychogenic nonepileptic events imitating epileptic seizures. In: Panayiotopoulos CP, ed. Atlas of Epilepsies. London, UK: Springer Science & Business Media; 2010:597-609. Neidermeyer E. Nonepileptic attacks. In: 

Niedermeyer E, Lopes da Silva F, eds. Electroencephalography: Basic Principles, Clinical Applications, and Related Fields. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2005:621-630. 

Takasaki K, Diaz Stransky A, Miller G. Psychogenic nonepileptic seizures: diagnosis, management, and bioethics. Pediatr Neurol. 2016;62:3-8.

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