Personality Disorders in Epilepsy
Orrin Devinsky
Charles Vorkas
William B. Barr
Bruce P. Hermann
Introduction
At the turn of the 20th century, most of the lay and professional communities believed that people with epilepsy had pathologic personality traits and psychopathologic disorders such as aggression, sociopathy, and psychosis. However, from the medical perspective, this was partly an artifact of the main setting from which epilepsy was viewed: Chronically institutionalized patients, many of whom suffered comorbid disorders (e.g., head trauma, neurosyphilis). The concept of an epileptic personality—a ubiquitous and characteristically negative set of behavioral changes in epilepsy patients—was already established in antiquity, but continued to evolve slowly and relentlessly. Simultaneously, more humanistic and balanced views evolved during the 19th century. Based on extensive experience with a private outpatient population with idiopathic epilepsy, Reynolds87 concluded that epilepsy does not invariably involve abnormal mental change, and Gowers42 also recognized that many epilepsy patients had normal personality and intellect, but that many others could develop intraparoxysmal behavioral changes. He suggested that these changes resulted from many factors, but mainly from epilepsy.
The early 20th century brought diverse views concerning people with epilepsy and their behaviors. Wilson121 offered a progressive psychosocial view: “On epileptic temperament inordinate stress has been laid. Life is difficult for these patients, and much that is attributed to temperament can with greater reason be assigned to chronic invalidism and unlucky circumstance.” Sjobring99 adhered to the negative, pervasive view: “A mental change of a specific nature takes place in individuals suffering from epileptic seizures. They become torpid and circumstantial, sticky and adhesive, effectively tense and suffer from explosive outburst of rage, anxiety, etc.” Kraepelin61 reported aggressiveness in some of his outpatient epilepsy patients: “Almost always an intensification of mental irritability occurs.” The adhesive or viscous personality traits in which the patient has difficulty in disengaging from interpersonal exchanges was reviewed by many observers in the European literature under various terminologies such as the “enechetic constitution,” “ixoid character,” and “glischroid trait.”
The Modern Era
Early in the 20th century, the term “epileptic personality” was used by psychoanalytic theorists to describe a set of character traits associated with epilepsy that focused on impulsivity, egocentricity, and affective viscosity.17 These features were considered to result from hereditary factors, directly from seizures or treatment, or a reaction to painful social situations associated with epilepsy. Others believed that these particular personality features, in individuals with or without seizures, were a direct expression of epilepsy itself. Two coincident developments in the middle of the century helped reintroduce the highly controversial concept of personality changes in epilepsy: Identifying the role of the limbic system in emotion and behavior and localizing the onset of many partial seizures to the temporal lobe.
Papez81 conceived a circuit of interconnected structures comprising the emotion system: Hippocampus—fornix—hy-pothalamus/mammillary bodies—mammillothalamic tract—anterior thalamic nuclei—thalamocingulate fibers—cingulate cortex—amygdala/hippocampus. This emotion circuit theory was based on anatomic connections, sham rage studies, and lesion studies. Yakovlev124 conceived a basolateral circuit modulating emotional behavior, including the amygdala, insula, orbitofrontal cortex, and dorsomedial thalamic nucleus. MacLean67 combined earlier ideas and conceived the limbic system with all the above regions, as well as the septum and nucleus accumbens.
The specific association of temporal lobe epilepsy (TLE) and psychopathology had its major genesis in the 1951 report by Gibbs40 that up to 33% of patients with “psychomotor seizures” of temporal lobe origin exhibited interictal behavioral changes. Gastaut et al.36 in 1954 reiterated common observations on the frequency of emotional viscosity, hyposexuality, hypoactivity, and aggressiveness in epilepsy patients, and first suggested that the stereotypic symptom complex was the antithesis of behaviors in the Kluver-Bucy syndrome (KBS). KBS is characterized by oral exploratory behavior, increased sexual appetite, decreased aggressivity, and continuous environmental exploration as a consequence of bilateral anterior temporal destructive lesions.59
Waxman and Geschwind117 proposed a distinct subset of nonpathologic behaviors associated with TLE, which included deepened emotions, circumstantiality, altered religious and sexual concerns, and hypergraphia. They coined the term “interictal behavior syndrome,” sometimes referred to as Geschwind syndrome or Gastaut-Geschwind syndrome.6 Bear and Fedio4 expanded this syndrome to include the 18 traits based on a literature review. They found an increased frequency of all 18 traits in patients with TLE compared with non-neurologic controls. The interictal behavioral traits described by Bear and Fedio are summarized in Table 1. Some of these traits associated with this syndrome are described below in more detail.
Table 1 Proposed Personality Traits by Bear and Fedio | ||||||||||||||||||
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Viscosity
Viscosity is a tendency for prolonged interpersonal contacts; talking repetitively, circumstantially; and pedantically; and not ending conversations and visits after a socially appropriate interval. Bear and Fedio4 reported viscosity to be significantly elevated in both right and left TLE compared to normal and neurologic controls. Brandt et al.10 found increased viscosity among left TLE and generalized epilepsy (GE) patients, with no difference between right TLE and controls. Hoeppner et al.51 presented the “cookie thief” picture from the Boston
Diagnostic Aphasia Examination, which shows a drawing of a boy stealing a cookie, to TLE, GE, and control subjects. Taped responses were reviewed blindly and all four individuals with verbose responses, characterized by trivial, circumstantial, and subjective details, had left temporal foci. A ten-item viscosity scale revealed significantly higher scores in the self-reports of patients with left TLE compared to right or bilateral TLE, absence seizures, panic disorder, or normal subjects.86 Proxy raters reported a trend for increased viscosity scores in the left TLE group, endorsing items such as “when I have a phone conversation with him/her, I always find I am the one who wants to get off first.” Seizure duration and viscosity score were significantly correlated for patients with left TLE.
Diagnostic Aphasia Examination, which shows a drawing of a boy stealing a cookie, to TLE, GE, and control subjects. Taped responses were reviewed blindly and all four individuals with verbose responses, characterized by trivial, circumstantial, and subjective details, had left temporal foci. A ten-item viscosity scale revealed significantly higher scores in the self-reports of patients with left TLE compared to right or bilateral TLE, absence seizures, panic disorder, or normal subjects.86 Proxy raters reported a trend for increased viscosity scores in the left TLE group, endorsing items such as “when I have a phone conversation with him/her, I always find I am the one who wants to get off first.” Seizure duration and viscosity score were significantly correlated for patients with left TLE.
Viscosity may result from some combination of linguistic impairment, social cohesion, mental slowness, and psychological dependence. Language dysfunction associated with left temporal lobe seizure foci may contribute to a verbal style characterized by circumstantiality and excessive discourse. However, there may be independent effects of left temporal foci on social behavior. Viscosity, the personality trait, may also result from an increased desire for interpersonal closeness, a need for affiliation with another being. Discrete limbic lesions can profoundly alter how animals maintain contacts with other members of their own or other species.41,60,73 For example, rats with septal lesions will remain in contact with each other in an open field and, if left alone, will actively approach cats despite expressions of fear.73 It is possible that some biologic effects of the epileptogenic process or recurrent seizures in a minority of patients with temporolimbic epilepsy, especially in the dominant hemisphere, fosters the development of viscosity.
Hyposexuality
Various changes in interictal sexual behavior occur in patients with TLE. Hyposexuality is frequently reported,7,31,35,83,93,98,101,108 with anecdotal reports of hypersexuality38,98 and deviant sexual behavior including exhibitionism,7,52 transvestism,27,84 transsexualism,50 and fetishism.26,74 Isolated cases of unusual sexual behavior occurring in patients with epilepsy could be chance associations. However, some cases develop profound changes in adulthood, shortly after the onset of epilepsy, raising the possibility of an etiologic relationship. Hyposexuality, including decreased libido and impotence, occurs in approximately half of TLE patients without gender bias. In many cases, especially those with seizure onset before puberty, patients may not marry or regard hyposexuality as a problem. Complaints are more likely to come from the spouse or parent who observes lack of interest in the opposite sex. Much of the original literature on hyposexuality from 1954 to 1985 was based on self-report76 without detailed interviews to assess the relative roles of libido, arousal, erectile dysfunction, anorgasmia, and sexual satisfaction, as well as physiologic measures of endocrine and sexual function.
Most studies found higher rates of hyposexuality and sexual dysfunction in TLE than other epilepsy groups, although Fenwick et al.31 did not observe any significant difference in sexual activity related to seizure type, type of epilepsy, or seizure frequency. Among those with TLE, no laterality effects were found for sexual behavior in left- versus right-sided seizure foci.16 A well-designed study of six men with erectile dysfunction found abnormal nocturnal penile tumescence and rigidity in five.44 The pattern of abnormality was consistent with neurogenic, not vasogenic, erectile dysfunction. In a self-report survey of 116 women with epilepsy, partial epilepsy patients experienced more dyspareunia, vaginismus, arousal insufficiency, and sexual dissatisfaction, whereas primary generalized epilepsy (PGE) patients experienced more anorgasmia and sexual dissatisfaction.75 Sexual symptoms were not associated with seizure frequency, antiepileptic drug (AED) exposure, sexual experience, depression, or prepubertal seizure onset.
The pathogenetic role of temporal lobe seizures in hyposexuality is supported by animal models30 and observations that sexual activity can increase following successful seizure control with AEDs83 and temporal lobectomy.7 In some postlobectomy subjects, marked hypersexuality similar to the Kluver-Bucy syndrome can develop occasionally.9 However, AEDs modulate hypothalamic-pituitary-gonadal axis hormone activity and can directly inhibit sexual behavior.64 Barbiturates may cause the greatest decrease in libido and sexual dysfunction.70 Other hepatic enzyme–inducing AEDs are also associated with decreased testosterone levels and diminished libido and function.49 Valproic acid is associated with menstrual disorders, hyperandrogenism, and polycystic ovaries.54,78
Religiosity
The ancient association between epilepsy and mystical/religious phenomena is paradigmatic of the difficulty reconciling dramatic anecdotes and long-standing medical opinion with limited clinical studies. Hippocrates began his monograph On the Sacred Disease by refuting the association between epilepsy and the divine. Despite his modern insights, religious and magical treatments of epilepsy predominated throughout the Middle Ages and Renaissance.109 In the 19th century, psychiatrists stressed the religiosity of epilepsy patients and observed that Siberian medicine men preferred epileptic pupils.64 Classic monographs on religious mysticism noted that “among the dread diseases that afflict humanity there is only one that interests us quite particularly; that disease is epilepsy.”64 Intense religious experiences and beliefs are reported frequently by people with epilepsy. Many prominent religious figures allegedly had epilepsy, including prophets and founders of several religions.116 The evidence supporting epilepsy in these people varies. Intense religious experiences can occur in association with seizures.13,25,53,66,102
Dewhurst and Beard23 reported six TLE patients who underwent sudden religious conversions. There was a clear temporal
relationship between conversion and increased seizure activity in five patients; one patient had a marked decrease in seizure frequency prior to conversion (she attributed her improved seizure disorder to the Almighty). Increased religious conviction and practice is not a consistent behavioral feature in patients with epilepsy. There is little evidence that epilepsy or TLE patients as a group are hyperreligious, although a subgroup may have unusually strong religious beliefs. Two studies with questionnaires on religion failed to differentiate patients with right versus left TLE, TLE versus GE, or epilepsy patients and controls.110,120 However, one study found that patients with smaller right hippocampi had significantly higher ratings on a religiosity scale.123
relationship between conversion and increased seizure activity in five patients; one patient had a marked decrease in seizure frequency prior to conversion (she attributed her improved seizure disorder to the Almighty). Increased religious conviction and practice is not a consistent behavioral feature in patients with epilepsy. There is little evidence that epilepsy or TLE patients as a group are hyperreligious, although a subgroup may have unusually strong religious beliefs. Two studies with questionnaires on religion failed to differentiate patients with right versus left TLE, TLE versus GE, or epilepsy patients and controls.110,120 However, one study found that patients with smaller right hippocampi had significantly higher ratings on a religiosity scale.123
Hypergraphia
Hypergraphia is not characteristic of interictal behavior among TLE or GE patients. However, several studies support that the subgroup manifesting this behavior most intensely are those with temporal lobe foci. Waxman and Geschwind116 reported seven TLE patients with hypergraphia, which is a tendency toward extensive and sometimes compulsive writing. There was a striking preoccupation with detail—words were defined and redefined and underlined, parentheses were used to make word meaning absolutely clear—and the writers accorded great importance to their material. In four patients, the writings focused on moral and religious concerns. Hypergraphia was viewed as a component of the deepened emotions and especially viscosity of interictal behavioral changes.
Utilizing a mailed standard questionnaire, Sachdev and Waxman92 demonstrated that TLE patients responded frequently and extensively (mean 1,301 words) as compared to other epilepsy patients (mean 106 words). Hermann et al.47 replicated the higher response rates and longest letters in the TLE group, but they did not find that the average response was longer in TLE patients compared to other seizure patients. Duration of epilepsy, hypomania, and number of significant life events during the past year positively correlated with hypergraphia.47,48 Hypergraphia occurs in 7% to 10% of TLE patients.47,48
Dostoyevsky was the most famous hypergraphic TLE patient,39 although his prolific writing also reflected his pay per page and financial troubles. As a person, he was deeply emotional, irritable, angered over minor provocations, guilt ridden, depressed, and tortured over the question of God’s existence. He described the relation between his writing and epilepsy in a letter to his brother (August 27, 1849): “Whenever formerly I had such nervous disturbances, I made use of them for writing; in such a state I could write much more and much better than usual.”24
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