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- Personality Subtypes
- Management of Personality Disorders Associated with Epilepsy
This article supports a view that certain personality disturbances in epilepsy should be viewed as associated with the cerebral abnormalities that also lead to seizures. Herein I discuss two main variants: that related to temporal lobe epilepsy and that associated with juvenile myoclonic epilepsy. In view of its controversial nature, I also comment on the link between aggression and epilepsy. Recommendations for treatment include psychological and social therapies with further advice about the use of antiepileptic drugs (AEDs) and psychotropic medications in these conditions.
At one time the link between personality disorders and epilepsy was a controversial topic. The main arguments revolved around whether any personality changes noted were linked with the biology of the epilepsy, in particular associated with temporal lobe lesions, or whether they were secondary to other intrusions such as the consequence of recurrent head injuries, social stigmatization, and/or the long-term prescription of anticonvulsant drugs. Amid the theorizing, several advances in the neurosciences revealed the important role of the frontal lobes in regulating behavior, and frontal lobe syndromes, essentially personality disorders, were described in animals and humans. In addition, a temporal lobe syndrome, namely the Klüver-Bucy syndrome, was described after bilateral damage to the temporal lobes, which presented as an alteration of personality traits, with calming of emotions, visual agnosia, and alterations of sexual behavior (Trimble, 1991).
Many studies in this earlier area used the Minnesota Multiphasic Personality Inventory (MMPI) to detect personality changes, and although some show differences between patients with epilepsy and healthy subjects, many fail to show statistical differences between temporal lobe and generalized epilepsy subgroups (Trimble, 1991). Of interest, several of these “negative” results show nonsignificant but higher levels of psychopathology in atemporal lobe group, and in some studies, scores for psychotic behavior were higher in those with combined psychomotor and generalized seizures or those with bilateral foci.
To overcome the shortcomings of the MMPI, Bear and Fedio (1977) developed a rating scale of 18 behavioral features, drawn from the literature, thought to be associated with temporal lobe epilepsy (TLE). Their scale was given to patients with TLE, neurologic control patients, and healthy controls. They reported that the temporal lobe group scored significantly higher on several subscales, notably humorless sobriety, dependency, obsessionality, and religious and philosophical concerns.
Generally, the data suggest that patients with epilepsy produce abnormal personality profiles, although the main argument relates to whether a subgroup with TLE may be identified (Shetty & Trimble, 1997). Other groups have used a “limbic marker” (such as specific auras or electroencephalography [EEG] findings) to identify patients with limbic epilepsy, and support the view of an association between the latter and such psychopathology (Trimble, 1991). Although the results do not support the concept that a specific “epileptic personality” exists in all patients with TLE, they do suggest that patients with temporal lobe lesions, particularly those with medially sited, limbic lesions, are more susceptible to psychiatric disturbances including personality disorders.
Although the findings can be explained in part by the biologic substrate of the underlying epilepsy (enduring limbic disturbances), other factors should be taken into account, including sociological, biochemical, and neurophysiologic ones.
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- Personality Subtypes
- Management of Personality Disorders Associated with Epilepsy
The concept of a personality disorder has been a source of controversy within psychiatry since the term was introduced in the 19th century, but the concept has become codified in various diagnostic manuals, such as the International Classification of Diseases (WHO, 1992) or the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders (DSM; American Psychiatric Association, 1994), which have gone through various editions with modifications. The classification schemes in use in psychiatry at the present time are based almost entirely on symptoms. However, it is by the personality traits that we get to know a person; the traits are enduring unless distorted by disease or accident. In common parlance, there is little difficulty in understanding a person's personality in these terms. It follows that, over time, people have given certain personality styles names, and that scientists have attempted to quantify various attributes of personality. It is argued that with such assessments, it should be possible to define normality within a population, and hence reflect on abnormality. Therefore, in psychopathology, two ways of defining personality have been used: one is a statistical method based on Gaussian distribution (similar to blood pressure assessments), and the other is based on the assumption of ideal types. The latter, invoking a kind of platonic reality, suggests that ideal pygmalion creations exist in nature; this is the way of DSM-IV. The former is the preferred scientific method, and hence the development of personality rating scales to attempt to quantify individual behaviors.
Substantial evidence that personality and the brain are closely entwined stems from observations of patients with various neurologic conditions, or patients who have sustained trauma to selected regions of the brain. Generally, any brain lesion may disrupt personality, and some authors refer to an “organic personality change” (Lishman, 1987). This includes irritability and restlessness, lassitude, poor concentration, loss of initiative, and excessive emotionality. There may be poor tolerance for change, withdrawal, insecurity, and anxiety. With more severe damage, extremes of this picture are seen, with social disorganization, loss of interest in the self, explosive irritability, shallowness of affect, and a blunting of emotional responses. In such situations, in which affected patients may also have comorbid epilepsy, an exacerbation of preexisting personality traits is seen, for example, aggressive people exhibiting outbursts of anger, and perhaps violence, with minimal provocation. Although many of these features are nonspecific, their recognition is important in diagnosis and management, and if the clinical problem is largely related to underlying cerebral compromise rather than to epilepsy, the approach may be quite different.
The most direct evidence that the brain is intimately involved in the structure of the personality comes from the observations on patients with frontal lobe damage. The features of various frontal lobe syndromes are well described and well recognized in neurologic practice. In contrast, changes in personality related to temporal lobe pathology are different, and frequently overlooked. However, the potential for patients with temporal, especially limbic lesions, to develop personality changes is in keeping with the known role of temporal lobe function in animal behavior, in particular following the description of the Klüver-Bucy syndrome. In particular, alterations of aggression, affective tone, and sexual activity were a part of the syndrome, and have been described clinically in association with other limbic system conditions where the burden of the pathology is in the temporal lobes, such as encephalitis or some dementias.
Temporal lobe epilepsy
The interictal behavior syndrome of TLE, emphasizing alterations in sexual behavior, hyperreligiosity, and hypergraphia (a tendency toward extensive and often compulsive writing) was described in the 19th century, but became better acknowledged with the work of Gastaut in France and Geschwind and his group in America (Waxman & Geschwind, 1975). The latter authors contrasted this picture both with the frontal lobe syndrome and with the Klüver-Bucy syndrome, having some characteristics almost opposite to the latter with increased attention to the environment and excess social adhesiveness.
The religiosity, the excessive interest in the cosmic and supernatural, the conviction that the person has some special significance in the world or some messianic mission, the hypergraphia, with detailed accounts of daily events being recorded, or with the elaboration of texts often with a moral or religious theme, and disturbed sexuality, are all features of the full syndrome. Viscosity is another feature of this syndrome. This refers to a stickiness of thought processes, a bradyphrenia, but also to an “interpersonal adhesiveness,” or increased social adhesion: patients simply will not leave the consulting room, and tend to prolong interpersonal encounters, for example, with physicians, beyond that indicated by social cues. They can display circumstantiality of thought with slow rambling speech, and have difficulty terminating conversations. Waxman and Geschwind suggested ongoing interictal abnormal electrical activity in the limbic system may be responsible for the behavior changes.
Unfortunately, diagnostic manuals such as the DSM IV are of no help in understanding these syndromes, as they are simply not included in them.
Juvenile myoclonic epilepsy
Although most of the discussion of the personality changes in epilepsy have revolved around the interictal syndrome of TLE, the personality associated with one of the generalized epilepsies was described by Janz (2002). This includes poor sleep habits, going to bed late, and waking late, and a personality profile characterized by “unsteadiness, lack of discipline, hedonism and indifference towards their illness.” These patients were prone to mood swings, were suggestible, and could react with undue sensitivity. These characteristics often led to poor compliance with treatment regimens and made management difficult. Janz drew attention to the differences between the personality profile associated with juvenile myoclonic epilepsy (JME), and that linked with TLE, in keeping with the above descriptions.
Several studies have used rating scales to assess groups of patients with JME, although little has come from them, being that they are few in number and use standardized ratings of psychopathology which, as with the TLE studies, may be inappropriate. It is, however, of interest that studies of cognition in patients with JME suggest that they do less well on frontal lobe tasks than matched patients with TLE, and more recent investigations have revealed subtle frontal lobe structural changes (Trimble, 2000).
Aggression and epilepsy
Animal and neurophysiologic studies of aggression strongly point to alteration of the limbic system, in particular amygdala function as associated with such behaviors. Because this area of the brain is frequently damaged in patients with TLE, it may be expected that these patients would display more aggression than patients with other forms of epilepsy. This is another area of considerable controversy. Although ictal violence, recorded using videotelemetry, has been clearly described (Delgado-Escueta et al., 1981), the problems of recording and quantifying interictal aggressive behavior, seen almost entirely in an interpersonal setting, has led to considerable variability of results in clinical studies. Hermann and Whitman (1984) noted the high risk factors for aggression in epilepsy, which include organic cerebral disease, low socioeconomic status, and poor upbringing. Brain imaging studies have reported that patients with epilepsy and severe aggression (referred to as Intermittent Explosive Disorder) had a significantly higher incidence of encephalitic brain disease and left handedness, more bilateral EEG abnormality, and less frequent hippocampal sclerosis than controls. In the aggressive patients, a subgroup show severe amygdala atrophy. In a further analysis, voxel-based morphometry revealed reductions of gray matter density over large areas of the left extratemporal neocortex, maximal in the left frontal neocortex (Tebartz van Elst et al., 1999).
A related condition is the episodic dyscontrol syndrome (Monroe, 1970; Fenwick, 1986), a synonym for the Intermittent Explosive Disorder, but usually suggesting some link with neurologic illness. This presents as sudden episodes of spontaneously released violence, often in the setting of minimal provocation, which tend to be short-lived. They may be provoked by small amounts of alcohol, and after the events, patients may feel remorse. Generally, the condition is associated with nonspecific abnormalities that are also seen in epilepsy, with evidence of minimal neurologic damage, soft neurologic signs, and abnormal EEG studies, although there is no evidence that these episodes have the same pathophysiology as epileptic seizures.
Management of Personality Disorders Associated with Epilepsy
- Top of page
- Personality Subtypes
- Management of Personality Disorders Associated with Epilepsy
The first step is to acknowledge that personality disorders are prevalent in the population generally, and are frequently comorbid with epilepsy. The severity obviously varies, and perhaps the more important point is to note personality dispositions, people's approaches to the world and others around them, and to consider how adaptive to a convenient lifestyle such traits are. In other words, many personality attributes can confer advantages, such as the anankastic concerned with the minutiae of detail in the tax office, or the hysteric displaying his or her abilities on the stage. The problem is when the outward behaviors of those with personality disorders lead to suffering of the patient themselves or others around them, and their approach is not adaptive or conducive to a successful lifestyle and the best quality of life they might be able to achieve.
The spectrum of personality disorders briefly reviewed earlier, some of which are peculiar to epilepsy (there are virtually no other neurologic disorders that lead to the Gastaut-Geschwind syndrome), and others of which are encountered in many other situations (such as the Intermittent Explosive Disorder) need to be evaluated alongside the obvious possibility that the personality of the patient has nothing to do with the epilepsy, or even cerebral damage, but is simply one of the recognized personality disorders of the diagnostic manuals. These include the Psychopathic Personality, in DSM-IV referred to as Antisocial Personality Disorder, or the Borderline Personality, hallmarked by an instability of interpersonal relationships, poor self-image, and impulsivity with recurrent attempts at self-harm. Paranoid and variants of the Schizoid Personality are not uncommon, defining people with excessive suspiciousness or sensitivity, or with detachment and eccentricity, respectively. Anxious personalities are common in seizure disorder clinics, as their tendency to have unprovoked panic attacks can lead to a mistaken diagnosis of epilepsy. These distinctions are important as they not only relate to how a patient interacts with physicians, but also color their attitude to any medical disorder.
Management of personality disorders when they present as disruptive to the person's lifestyle can be challenging. Patients often have little or no insight into their behaviors, their motivations, or the repetitive patterns of their interactions. Their approach can only initially be psychotherapeutic, but in the case of the more severe personality disorders, this is often unrewarding for both therapist and patient; a long-haul is needed. Personality profiles will simply not bend to a short-term cognitive behavior therapy (CBT) approach. Intervention in such settings is a specialist area well beyond the neurologic clinic, but with recognition of the need for psychological intervention, appropriate referrals should be made.
With the epilepsy-related comorbid personality disorders, a different approach needs to be taken, especially since they will usually be associated with continuing epilepsy, and the patient will need to be kept within the treating orbit of the neurological clinic. Again, a psychotherapeutic approach is the initial one, but not necessarily one that involves a practicing therapist. Although it takes a while longer for each consultation, empathically interacting with the mildly paranoid, the challenging religiosity, the viscosity, or even the overtly aggressive patient is often all that is required. To reassure them that you understand what they are saying, by listening and tempering the dialogue (sometimes the monologue) with gentle commonsense, and not challenging or appearing disconcerted with overt hostility will allow a rapport to develop. This hangs over to the next consultation, and permits continuing care. The latter aids compliance with treatment regimens, and helps reassure other family members that their concerns (relatives worry more about these behaviors than the patients do) are being addressed, and helps avoid crises.
The more difficult problems arise when more severe variants of these personality disorders are encountered. The Gastaut-Geschwind syndrome has a tendency to gradually become more pervasive, with increasing religiosity, and “stickiness” in everyday activities. In some patients the hypergraphia reaches astonishing proportions, such that the neurologist can be bombarded with documents, especially in this era of computer communications. A flurry of seizures can provoke a postictal psychosis when the religiosity becomes more obviously delusional. This can be provoked if the patient, with increasing religious conviction, becomes convinced that AEDs are unnecessary for his care and stops them. The situation can become clinically dangerous, with attempts at self-harm, or even the threat to harm others, as command hallucinations and delusions take over the patient's behavioral demeanor. Suicidal behaviors occur in such settings.
When to intervene when the personality changes are slowly progressive is a difficult judgment, sometimes guided by the patient's relatives' concern over what is happening at home. But the acute situation requires immediate intervention with psychotropic medications. In the case of a postictal psychosis, a short course of benzodiazepines may be sufficient, but antipsychotics will often be necessary. The choice of these is discussed in other articles in this supplement, but atypical agents with the least potential to bring on extrapyramidal side effects are preferred. These may be needed for chronic treatment when the psychotic intensity of the personality disorder remains, as can occur in those with repeated episodes of postictal psychoses. Management of the latter is discussed elsewhere in this supplement, but requires the most careful assessment.
It is important to recognize the different personality profile that accompanies JME. No better description of this and the importance of the behaviors for management of the disorder can be found than that of Janz (2002). He noted in his early description of what became referred to as the Janz syndrome, that the seizures were frequently precipitated by external events, including both emotional and physical stress. Behavioral factors associated either with the onset of the disorder or its maintenance included lack of sleep, excess alcohol consumption, and a rather extreme lifestyle; patients failed to learn from experience the adverse factors that lead to their seizures, and thus the continuation of their disorder.
This leads to interventions that involve sound advice about their lack of self-control, the importance of compliance with medication, and the need for some regularity of daily activities and for sleep hygiene. These reflections of personality immaturity must be met, as Janz puts it “with comprehension and firmness rather than authoritarian exhortations.”
The most difficult behavior problems to manage in epilepsy are the aggressive outbursts, often interlinked with an underlying dysphoric disorder, a touchiness, and irritability that waxes and wanes. The acute episodes may be triggered by the slightest of circumstances, can vary in their intensity, and can be very explosive. These need to be distinguished from an ictal aggression, rare but well observed in telemetric studies (Delgado-Escueta et al., 1981), which tend to cease rapidly and are clinically associated with other manifestations of partial seizures, and from episodes with the release of aggression in a postictal state.
The pathway to care is given in Figure 1 (Tebartz van Elst, 2002).
Episodes of aggression are a feature of several of the personality disorders, in the absence of epilepsy, especially in people with psychopathic or borderline personalities. Therefore, establishing that the patient has epilepsy is sometimes important, in that the aggressive episodes of personality disorders without epilepsy can be misdiagnosed as epilepsy.
Patients need to be assessed for comorbid psychiatric disorders, such as a depressive disorder or a developing psychosis, and managed accordingly, since these can exacerbate the personality tendencies and lead to increasing irritability and aggression. (For treatment of the other psychiatric comorbidities see contributions in this volume.)
Any other medical problems that might be contributing to the behavior disorder need to be addressed (such as infections or endocrinologic disorders), and the drug treatment of the epilepsy reevaluated. Certain antiepileptic drugs (AEDs) tend to increase dysphoria, and are associated with the release of aggression and hostility, especially those that are γ-aminobutyric acid (GABA) agonists (Trimble, 2011). They should be removed slowly from the treatment schedule if possible. Monotherapy regimens are always preferable to polytherapy, not only in aiding compliance, but also in lessening the toxic effects of the AEDs.
In more severe personality disorders in which aggression is a prominent problem for management, various medications can be tried, but there are no studies to support their efficacy. These include lithium, antipsychotic agents, and the institution of certain AEDs that may not have been used in management such as carbamazepine, valproic acid, or lamotrigine.
Needless to say, management problems are even more problematic for those with a learning disability, especially in the more severe range. Often these are dependent on structured behavioral regimens, beyond the scope of this paper, as is the contribution of neurosurgery for epilepsy and the consequent alterations of personality that may ensue.