Psychogenic Nonepileptic Seizures



Psychogenic Nonepileptic Seizures


Selim R. Benbadis



OVERVIEW

Psychogenic nonepileptic seizures (PNES) are routinely seen at epilepsy centers, where they represent 15% to 30% of patients referred for refractory seizures (1,2). They occur fairly often in the general population, with an estimated prevalence of 2 to 33 per 100,000 persons, making this condition nearly as common as multiple sclerosis (MS) or trigeminal neuralgia. In addition to being common, PNES represent a challenge, both in diagnosis and in management.


Terminology

The terminology used to describe PNES is variable and at times confusing. A number of terms have been used, including pseudoseizures, nonepileptic seizures, nonepileptic events, psychogenic seizures, and hysterical seizures. Strictly speaking, terms such as pseudoseizures, nonepileptic seizures, and nonepileptic events include both psychogenic and nonpsychogenic (i.e., organic) episodes that mimic epileptic seizures. Examples of nonpsychogenic episodes include syncope (the most common); paroxysmal movement disorders (e.g., dystonia); cataplexy; complicated migraines; and, in children, breath-holding spells and shuddering attacks. Terms such as psychogenic or hysterical seizures, on the other hand, refer to a subset of nonepileptic seizures with the connotation of a psychological origin. Use of the term hysteria has long since fallen into disfavor. The term psychogenic seizures could possibly be interpreted as epileptic seizures triggered or exacerbated by a psychological factor. For these reasons, PNES is the preferred term (3) and is used throughout this chapter.


The Misdiagnosis of Epilepsy

The erroneous diagnosis of epilepsy is relatively common. Approximately 25% of patients previously diagnosed with epilepsy and who are not responding to antiepileptic drug (AED) therapy are found to be misdiagnosed, both in epilepsy referral clinics (4,5) and in epilepsy monitoring units (1). Most patients misdiagnosed with epilepsy are eventually shown to have PNES (1,2) or, more rarely, syncope (6,7). Occasionally, other paroxysmal conditions can be misdiagnosed as epilepsy, but PNES are by far the most common condition, followed by syncope. Often, electroencephalograms (EEGs) that are interpreted as providing evidence for epilepsy contribute to this misdiagnosis (4,6,8). As is true with other chronic conditions (e.g., MS), whenever a wrong diagnosis of epilepsy has been given, it can be very difficult to “undo.” Unfortunately, once the diagnosis of “seizures” has been made, it becomes easily perpetuated without being questioned, which explains the usual diagnostic delay (9,10) and associated cost (11,12). It is disconcerting that despite the ability to render a diagnosis of PNES with near-certainty, the delay in diagnosis remains long, at about 7 to 10 years (9,10), indicating that neurologists may not have a high enough index of suspicion when AED treatment fails. This chapter begins by reviewing the steps involved in making that diagnosis and then turns to management considerations.


MAKING THE DIAGNOSIS


Suspecting the Diagnosis

PNES are initially suspected in the clinic on the basis of history and examination. A number of “red flags” are useful
in clinical practice and should raise the suspicion that seizures may be psychogenic rather than epileptic. Of course, resistance to AEDs can be the first clue and is usually the reason for referral to an epilepsy center. Most (approximately 80%) of the patients with PNES have been treated with AEDs for some time before the correct diagnosis is made (13). This is because a diagnosis of epilepsy is usually based solely on history and may be difficult, especially for nonneurologists (e.g., emergency department physicians and primary care physicians). A very high frequency of episodes that are completely unaffected by AEDs (i.e., no difference whether on or off medication) should also suggest the possibility of a psychogenic etiology. The presence of specific triggers that are unusual for epilepsy can be very suggestive of PNES, and this should be asked specifically when obtaining the history. For example, emotional triggers (“stress” or “getting upset”) are commonly reported in patients with PNES. Other triggers that are suggestive of PNES include pain, certain movements, sounds, and lights, especially if they are alleged to consistently precipitate a “seizure.” The circumstances under which attacks occur can be very helpful. Like other psychogenic symptoms, PNES tend to occur in the presence of an “audience,” and, for example, occurrence in a physician’s office or waiting room may be predictive of a psychogenic etiology (14). Similarly, PNES tend not to occur in sleep, although they may seem to and may be reported as such (15,16).

If the historian and witnesses are astute enough, the detailed description of the spells often includes characteristics that are inconsistent with epileptic seizures. In particular, some characteristics of the motor (“convulsive”) phenomena are associated with PNES (see “Electroencephalogram-Video Monitoring”). However, witnesses’ accounts are rarely detailed enough to describe the episodes accurately; in fact, even seizures witnessed by physicians and thought to be epileptic often turn out to be PNES. The patient’s medical history can be useful as well. Although it has not been documented, coexisting poorly defined and “fashionable” (probably psychogenic) conditions, such as fibromyalgia, chronic pain, irritable bowel, or chronic fatigue, are associated with psychogenic symptoms. In a population referred for refractory seizures, a history of fibromyalgia or chronic pain has a strong association with a diagnosis of PNES (14). Similarly, a florid review of systems suggests somatization. A psychosocial history with evidence of maladaptive behaviors or associated psychiatric diagnoses should raise the level of suspicion of PNES. The examination, paying particular attention to mental status evaluation, including general demeanor and appropriate level of concern, overdramatization, and hysterical features, can be very telling, often uncovering such histrionic behavior as “give-way” weakness or “tight-roping.” Performing the examination can, in itself, act as an “induction” in suggestible patients, making a spell more likely to occur during the history taking or examination.

By contrast, the presence of certain symptoms argues in favor of epileptic seizures and should warrant caution. These include significant postictal confusion, incontinence, and, most important, significant injury (17, 18, 19, 20, 21). Although some injuries have been reported in PNES, data that describe injuries in patients with PNES are based largely on patients’ self-reports (22). In particular, tongue biting is highly specific to generalized tonic-clonic seizures (18) and thus is a very helpful sign when present.


Confirming the Diagnosis


EEG and Ambulatory EEG

Because of its low sensitivity, routine EEG is not very helpful in diagnosing PNES. However, the presence of repeated normal EEGs, especially in light of frequent attacks and resistance to AEDs, certainly can be viewed as a red flag (23). Ambulatory EEG is increasingly used, is cost-effective, and can contribute to the diagnosis of PNES by recording the habitual episode and documenting the absence of EEG changes. However, because of the difficulties involved in conveying this diagnosis (see “Management”), it should always be confirmed by video-EEG monitoring.


Video-Electroencephalogram Monitoring

This is the gold standard for diagnosis of PNES (2,3,9,15, 16, 17, 18, 19,21), and, in fact, is indicated in all patients who continue to experience frequent seizures despite the use of AEDs (24). In the hands of experienced epileptologists, the combined electroclinical analysis of both the clinical semiology of the ictus and the ictal EEG findings allows a definitive diagnosis in nearly all cases. If an attack is recorded, the diagnosis is usually easy, and it is unusual that this question (i.e., PNES versus epilepsy) cannot be answered.

The principle of video-EEG monitoring is to record an episode and demonstrate that (a) there is no change in the EEG during the clinical event, and (b) the clinical spell is not consistent with seizure types that can be unaccompanied by EEG changes. Ictal EEG has limitations because it may be negative in simple partial seizures (25,26) and in some complex partial seizures, especially frontal ones (21). Ictal EEG may also be uninterpretable or difficult if movements generate excessive artifact.

Analysis of the ictal semiology (i.e., video) is at least as important as the ictal EEG, as it often shows behaviors that are obviously nonorganic and incompatible with epileptic seizures. Certain characteristics of the motor phenomena are strongly associated with PNES, including a very gradual onset or termination; pseudosleep; discontinuous (stop-and-go) activity; and irregular or asynchronous (out-ofphase) activity side-to-side head movement, pelvic thrusting, opisthotonic posturing, stuttering, and weeping (15, 16, 17,19,21,27, 28, 29, 30). A particularly useful sign is preserved awareness during bilateral motor activity, which is relatively specific for PNES. This is because unresponsiveness is almost always present during bilateral motor activity, with the notable exception being supplementary motor area seizures (31,32).



Inductions

Provocative techniques, also known as activation procedures, or “inductions,” can be extremely useful for the diagnosis of PNES, particularly when the diagnosis remains uncertain and no spontaneous attacks occur during monitoring. Many epilepsy centers use some sort of provocative technique to aid in the diagnosis of PNES (33,34). Some variability exists among the methods used. Although intravenous (IV) saline injection has traditionally been the most common (35, 36, 37, 38), a number of other techniques have been described (39, 40, 41, 42), which may be preferable (see below).

The principle behind provocative techniques is suggestibility, which is a feature of somatoform disorders in general. For example, in psychogenic movement disorders, where the diagnosis rests solely on phenomenology (i.e., there is no equivalent of the EEG), response to placebo or suggestion is considered a diagnostic criterion for definite psychogenic mechanism (43).

There are many advantages to the use of provocative techniques. First, when carefully studied and used simultaneously with EEG, their specificity approaches 100% (44). Second, difficult situations exist in which the combination of semiology (video) and the EEG does not allow one to conclude that an episode is psychogenic in origin. As mentioned earlier, two relatively common scenarios are (a) the ictal EEG is uninterpretable because of movement-related artifacts, and (b) the ictal EEG is normal, but the symptoms are consistent with a “simple partial” seizure. In these situations, the very presence of suggestibility (i.e., suggestion triggers the episode in question) is the strongest argument to support a psychogenic etiology. Third, at least theoretically, nonepileptic is not quite synonymous with psychogenic. The combination of a recorded attack and a normal ictal EEG qualifies as a nonepileptic spell but cannot in itself be categorized as psychogenic. On the other hand, a positive induction does stamp the episode as psychogenic, and even difficult-to-convince laypersons and attorneys understand this concept. Fourth, there is a strong economic argument for the use of these techniques, especially with the constraints imposed by third-party payers. When spontaneous attacks do not occur in the allotted time for monitoring, the evaluation may be inconclusive. In such situations, provocative techniques often turn an inconclusive evaluation into a diagnostic one.

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Oct 17, 2016 | Posted by in NEUROLOGY | Comments Off on Psychogenic Nonepileptic Seizures

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