Monday, February 20, 2017

Psychogenic paroxysmal nonepileptic events in children

Morgan LA, Buchhalter J. Psychogenic Paroxysmal Nonepileptic Events in Children: A Review. Pediatr Neurol. 2015 Jul;53(1):13-22.

Paroxysmal nonepileptic events are common in children. Events with a psychological basis, historically referred to as pseudoseizures, are a large subset of paroxysmal nonepileptic events.
A review of the relevant pediatric and adult literature was performed.
It was found that these events have many semioloigc features similar to epileptic events and can be challenging to correctly identify. The use of a detailed history in combination with video encephalography and knowledge of psychogenic paroxysmal nonepileptic events will facilitate making the correct diagnosis. Paroxysmal nonepileptic events are important to identify as comorbid disorders such as depression, anxiety disorder, family discord, and school issues are frequent. In addition, prior sexual, emotional, and/or physical abuse may be present.
Pediatric patients with paroxysmal nonepileptic events need to be recognized in order to avoid unnecessary antiepileptic drugs and emergency department or hospital visits and to facilitate appropriate psychological intervention to address the underlying etiologies. This review will focus on evaluation and identification of paroxysmal nonepileptic events, in addition to reviewing the various comorbidities, effective treatments, and outcomes for pediatric patients. The key differences between pediatric and adult patients with paroxysmal nonepileptic events are addressed.

From the article

In the pediatric population, 76.8%-80% of PNEs have an abrupt start, and 68% end abruptly.  Eye closure during the entire attack occurs in 22%-45% and tremor in 25% in upper instead of lower extremities.  Furthermore, the episodes can be classified as only unresponsiveness, unresponsiveness with subtle motor activity, or unresponsive episodes of motor activity not typical of known types of epileptic seizures such as generalized limb jerking, thrashing of bodies and extremities, swooning, and generalized arrhythmic flailing of extremities.  In one pediatric series in which “nonresponsive” was specifically mentioned, the event was associated with generalized violent, thrashing, and uncoordinated movements in 44%, generalized trembling in 26%, and only nonresponsiveness in 9%.  Swoon type can be misdiagnosed as a vasovagal event, but swoon type events prolonged over a minute should raise suspicion for PNEs. 

Several studies note that older children and adolescents have more motor manifestations, whereas younger school-age children more frequently exhibit staring.  When motor manifestations are present, they most commonly mimic generalized tonic-clonic movements (60%), followed by focal clonic like movements (10%), and akinetic (10%).  There are conflicting data as to whether children more typically demonstrate a single PNE semiology or multiple semiologies.  To best group the various semiologies, a proposal of five major categories was created, allowing the entire study cohort to be classified. These include: (I) abnormal motor comprising (IA) hypermotor, (IB) partial motor, (II) affective/emotional behavior phenomena, (III) dialeptic, (IV) “aura,” and (V) mixed pattern. 

Correctly distinguishing PNEs from epileptic seizures remains a diagnostic challenge, more so because up to 78% of PNEs in pediatric patients are stereotypic and repetitive in nature.  One study demonstrated a sedentary, dialeptic form of events more frequently in younger children (less than 14 years) than adults (29% versus 11%).  Adults typically retain awareness, even during generalized convulsions  and have no discrete gaps of missing memory.  Both pediatric and adult patients are usually at their baseline level of responsiveness immediately following the event without displaying postictal confusion or sleepiness.

Emotional manifestations are more common in adults than in children and may include combativeness, crying, yelling, and vulgar language.  In adult patients, weeping is relatively common and strongly suggestive of PNEs.  Pelvic thrusting is uncommon in children, unlike in adults, although it has been documented at a frequency of 8.9% in children.   Ictal eye closure, convulsions longer than 2 minutes, postictal speech change, and vocalizations during the tonic-clonic phase all occur significantly more in adults than children.   Ictal eye closure can still be noted at any age and is more common in PNEs compared with epileptic seizures.  Pupillary dilation is thought to be more specific to epileptic seizures, yet dilation of pupils may occur from increased sympathetic discharge from a stress response and thus may be seen with PNEs.  Some historically classic signs for PNEs, such as opisthotonos, postictal whispering, and biting the tip of the tongue, appear to be rare in both children and adults. 

PNEs vary in length, lasting from 30 seconds to upwards of 10 minutes, with events typically longer than 3 minutes,  whereas epileptic seizures have a duration of less than 3 minutes.  One pediatric study documented the mean duration of PNEs to be 269 ± 549 seconds (4 minutes, 29 seconds ± 9 minutes, 9 seconds) compared with epileptic seizure duration of 83.2 ± 222.4 seconds (1 minute, 32.2 seconds ± 3 minutes, 42.4 seconds).    In a separate study of 10 children presenting to the emergency department, PNEs were documented with a broad range including 1 minute in three patients and 20-180 minutes in seven patients.  Another pediatric study reported PNE durations of 10-35 minutes; and those events characterized by unresponsiveness have been reported to last as long as 40 minutes. 

When PNEs were first described, it was thought that patients would not suffer injurious behavior or have incontinence. Many adult and pediatric studies to date have refuted this early belief, reporting injuries such as tongue biting, patients injuring themselves against the bed guard rails, bruises, fractures, clawing at the face, intubation, not responding to painful stimuli, and urinary incontinence and defecation.  In adult patients, caregiver reporting and self-reporting of injuries is as high as 30.8% and includes tongue biting, lacerations, limb fractures, and dental injury.   In patients with injury, the frequency of violent shaking movements, nocturnal PNEs, and urinary incontinence is higher.  46  In addition, injury is more common during PNEs in patients who have attempted suicide previously (61% versus 30%)  8  and those with a history of physical abuse.  In adults, urinary or fecal incontinence, or both, may occur up to 20% of the time.  Overall, injury is not a common feature in children.  34 36  In a study of 10 children seen in the emergency department with PNE, none had incontinence and none were injured, but nine had invasive procedures performed or laboratory tests obtained by the emergency department or paramedics.  

1 comment:

  1. Takasaki K, Diaz Stransky A, Miller G. Psychogenic Nonepileptic Seizures: Diagnosis, Management, and Bioethics. Pediatr Neurol. 2016 Sep;62:3-8.

    The diagnosis and management of psychogenic nonepileptic seizures (PNES) is often challenging and fraught with discord and disagreement between patients, parents, and physicians. Furthermore, there are ethical challenges when making the diagnosis, communicating this information, and instituting management.
    We reviewed the current body of knowledge regarding the characteristic differences between epileptic seizures and PNES, and the high incidence of psychiatric comorbidities. An ethical analysis was made of diagnosis and management based on ethical principles, virtue ethics, and the social contract that health professionals have with patients.
    Key distinctions between PNES and epilepsy lie in both patient and seizure characteristics. Long duration, eye closure, asynchronous movements, frequent recurrence in the same context, intra-ictal awareness, and lack of post ictal state are useful in helping establish the diagnosis. Psychiatric comorbidities, history of abuse, cognitive impairment, and multiple non specific somatic complaints are some salient patient features that should increase suspicion for the diagnosis of PNES. However, definitive diagnosis rests on capturing the events on video EEG.
    Effective diagnosis and management of PNES requires the use of video EEG and an early collaborative approach between pediatricians, neurologists, psychiatrists, nursing staff, and other professional colleagues. Ethical questions that may arise should be addressed with the virtues of competence, courage, compassion, prudence, and honesty; and the principles of respect beneficence, and the avoidance of unnecessary harm.