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Neurocase: The Neural Basis of Cognition


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Klüver–Bucy syndrome, hypersexuality, and the


law
a b a
Julie Devinsky , Oliver Sacks & Orrin Devinsky
a
New York University Langone School of Medicine, New York, USA
b
Departments of Neurology and Psychiatry, Columbia University School of Medicine,
New York, USA
Published online: 18 Nov 2009.

To cite this article: Julie Devinsky , Oliver Sacks & Orrin Devinsky (2010) Klüver–Bucy syndrome, hypersexuality, and
the law, Neurocase: The Neural Basis of Cognition, 16:2, 140-145, DOI: 10.1080/13554790903329182

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NEUROCASE
2010, 16 (2), 140–145

Klüver–Bucy syndrome, hypersexuality, and the law


NNCS

Julie Devinsky,1 Oliver Sacks,2 and Orrin Devinsky1


Klüver–Bucy Syndrome, Hypersexuality, and the Law

1
New York University Langone School of Medicine, New York, USA
2
Departments of Neurology and Psychiatry, Columbia University School of Medicine,
New York, USA

A 51-year-old right-handed man developed hypersexuality after a second right temporal lobectomy to treat epi-
Downloaded by [University of Chicago Library] at 06:31 15 September 2013

lepsy. His hypersexuality started with increased marital intercourse and masturbation but he later downloaded
child pornography. Hyperphagia and distractibility, other features of the Klüver–Bucy syndrome, also developed.
Resection of the amygdala and/or temporal lobe neocortical areas that inhibit other limbic areas may contribute
to the pathogenesis of hypersexuality. Neurological factors mitigate the criminal responsibility for hypersexual
activity in patients with Klüver–Bucy syndrome. Most previously reported patients were never charged with a
crime despite uninvited physical contact in some instances. Our patient was convicted and imprisoned.

Keywords: Hypersexuality; Klüver–Bucy syndrome; Temporal lobectomy; Medical-legal.

INTRODUCTION anterior temporal lobectomy in male rhesus mon-


keys (Bucy & Klüver, 1955; Klüver & Bucy, 1939).
In 1888, Sanger Brown and E. A. Schaefer, two The Klüver–Bucy syndrome (KBS) is character-
British experimental physiologists, described a ized by excessive orality and hyperphagia,
variety of behavioral transformations after remov- distractibility (especially for visual stimuli), hyper-
ing both temporal lobes in monkeys (Brown & sexuality and change in sexual preference, visual
Schaefer, 1888). agnosia and loss of aggressive and fearful
responses (Bucy & Kluver, 1955; Weiskrantz,
He no longer clearly understands the meaning of 1956).
the sounds, sights, and other impressions that In humans, partial KBS was first reported in 1955
reach him. His food is devoured greedily, the head after bilateral temporal lobectomy for epilepsy con-
being dipped into the dish, instead of the food trol (Terzian & Ore, 1955). A complete human KBS
being conveyed to the mouth by the hands. He case after encephalitis was reported in 1975 (Mar-
reacts to all kinds of noises, even slight ones – such
lowe, Mancall, & Thomas, 1975). KBS symptoms
as the rustling of a piece of paper – but shows no
may be caused by diverse disorders affecting the tem-
consequent evidence of alarm or agitation and dis-
plays tyrannizing proclivities towards his mate. poral lobe, including head trauma, anoxia,
(Brown & Schaefer, 1888) Alzheimer’s disease and other dementias, adrenoleu-
kodystrophy, unilateral temporal lobectomy, and the
Half a century later, Heinrich Klüver, a German- postictal state (Devinsky & D’Esposito, 2003). Most
American experimental psychologist, and Paul patients have only one or two features, since human
Bucy, an American neuropathologist and neuro- diseases often incompletely destroy anterior temporal
surgeon, described the behavioral effects of bilateral regions. Also, some diseases cause neuronal loss as

There were no sponsors of this study. There are no financial disclosures.


Address correspondence to Julie Devinsky, 97 Westview Road, Short Hills, NJ 07078, USA. (E-mail: julierach@comcast.net).

© 2009 Psychology Press, an imprint of the Taylor & Francis Group, an Informa business
http://www.psypress.com/neurocase DOI: 10.1080/13554790903329182
KLÜVER–BUCY SYNDROME, HYPERSEXUALITY, AND THE LAW 141

well as hyperexcitability, which can cause irritability, with impaired right hemisphere memory (1/6 items)
aggressiveness and paranoia (Cummings & Duchen, and preserved left memory (6/6). A one-stage right
1981; Hooshmand, Sepdham, & Vries, 1974; Lilly, temporal lobectomy was performed after mapping
Cummings, Benson, & Frankel, 1983; Pilleri, 1966) the ‘musical areas’ in the cortex to avoid damage to
rather than the loss of aggressive and fear responses. these. Pathology showed a ganglioglioma. Following
We report KBS-related hypersexuality in a man surgery, he was seizure free for 9 months, until he
with medically uncontrolled seizures whose behav- self-tapered off phenobarbital and had a convulsion.
ior changed after an extension of a prior right tem- Complex partial seizures became refractory to ther-
poral lobectomy. His hypersexuality started with apy and began with a ringing sound, foul smell, and
increased marital intercourse and masturbation a ‘feeling of thickness in my tongue’. Despite seizures
but expanded to the viewing and downloading of and medication side effects, he continued to work
child and adult internet pornography for which he full-time as a pharmaceutical researcher and volun-
was convicted and imprisoned. The literature on teered to provide support at programs and fundraise
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hypersexuality associated with temporal lobe for the local Epilepsy Foundation.
lesions is reviewed, and medical and legal implica- At age 39, repeat videoEEG monitoring showed
tions of these disorders are discussed. a right posterior temporal seizure focus. A two-
stage subdural grid/strip electrode surgery was per-
formed, with a posterior temporal resection. He
CASE REPORT was seizure free for more than 4 years, but then par-
tial seizures recurred. However, approximately a
This 51-year-old man first suffered attacks of déjà month after surgery, behavioral changes of irrita-
vu at age 19. They became much more frequent –as bility, hyperphagia and hypersexuality (including
many as 20 attacks a day – and much more com- coprophilia) developed. He became more sexually
plex, the déjà vu now being followed by ‘a cascade’ active with his wife and masturbated more often.
of other symptoms: sharp pains in the chest and Compulsively, he began to watch adult porno-
sensations of breathlessness; alterations of hearing; graphic images and videos on the internet when his
occasional musical hallucinations – he would wife slept. Some websites solicited him to view and
always hear a particular song ‘as clearly as if it purchase child pornography. He became obsessed
were being played in the next room’; and in the with this and eventually purchased and down-
severest attacks, olfactory and gustatory hallucina- loaded pornographic images of prepubescent
tions (‘a pungent smell and a taste corresponding females engaged in sexual activities from the inter-
to the smell’) followed by vomiting. There were net. He was ashamed and secretive about these
occasional ‘out of body’ experiences too. In Christ- activities, not discussing the pornography or mas-
mas of 1976 he had a tonic-clonic seizure. turbation with his wife or with anyone else. In 2006,
A work-up at this time showed a seizure focus in he was arrested by federal authorities for down-
the right temporal lobe. Antiepileptic drugs were loading child pornography. He was charged with
started, but it was never possible to achieve good
seizure control. He started to develop certain insid- knowingly and willfully possess[ing] material
ious personality changes – he became (in his own which contained at least three images of child por-
words) ‘more spiritual’, ‘more creative’ (he started nography, as defined in 18 U.S.C. § 2256, which
to write a great deal), and, specifically, ‘more were shipped and transported in interstate and
musical’ –strumming his guitar ‘obsessively . . . for foreign commerce, including by computer, and
hours and hours’ – though plucking the strings, he were produced using materials that were shipped
found, could induce a seizure. But he maintained an and transported in interstate and foreign com-
unbroken work record – he was trusted and well- merce, including by computer.
liked by his colleagues and loved by his wife. He was
‘a good husband’, his wife told us, not given to any Following his arrest, he was treated by a psychia-
inordinate or perverse sexual practices, and always trist with quetiapine and sertraline and his obses-
sensitive and responsive to her needs and feelings. sive sexual thoughts abated. His wife confirmed
On evaluation at age 33, his MRI showed a right that his incessant demands for sexual relations
mesial temporal lesion and videoEEG revealed right stopped and he became hyposexual.
temporal seizure onsets. An intracarotid amobarbi- The patient described his own behavioral
tal test revealed left hemisphere language dominance changes as follows:
142 DEVINSKY, SACKS, DEVINSKY

my appetite for food and sex increased dramati- LEGAL PROCEEDINGS


cally. I had greater mood swings. I wanted sex
constantly. Every day. I was very easily stimulated The patient was arrested at his home and his com-
and began to touch myself regularly. I began to puters were confiscated. He pleaded guilty to pos-
request sex daily from my wife. If I wasn’t having
sessing at least three images of child pornography
sex with my wife, I masturbated. This behavior
that were transported in interstate or foreign com-
increased over time. I became more emotionally
labile, obsessive–compulsive, but on the other merce. He was sentenced in the US District Court.
extreme became disinterested or unable to initiate The treating physician (OD) summarized the
things I needed or was supposed to do. patient’s neurological history and the KBS, focus-
ing on hypersexuality after temporal lobe damage.
I raged for hours at inappropriate things at home
Several cases from the literature were cited to
(no socks, no rye bread, perceived criticisms).
Driving home from work a driver ‘squeezed me’
exemplify the range of behaviors in KBS: an eld-
on a merge. I accelerated and cut him off. I rolled erly patient with frontotemporal dementia who
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my window down, gave him the finger and began sexually approached minors (Mendez, Chow,
screaming at him, and threw a metal coffee mug Ringman, Twitchell, & Hinkin, 2000) and a 14-
and hit his car. He called police from his cell. I was year-old girl who masturbated in public after a
pulled over and ticketed. temporal lobectomy (Ozmen, Erdogan, Duvenci,
I become distracted so easily that I can’t get any- Ozyurt, & Ozkara, 2004).
thing started or done. It was argued that KBS caused or was the major
contributing factor to the patient’s hypersexuality
His wife’s account tallies with this: and viewing of child pornography. Hypersexuality
almost immediately followed his second surgery; it
He started to gain weight and his pants changed 3 was a sudden and dramatic behavioral change and
sizes within 6 months. His appetite was out of con- associated with other KBS symptoms of hyper-
trol. He would get up in the middle of the night phagia and distractibility. The prosecutor asked
and eat an entire bag of cookies or a block of how, since there are only rare cases of pedophilia
cheese with a large box of crackers. He asked that reported in relation to KBS, a relation could be
I serve the same foods every day. He also had to established with medical certainty? The response
wear the same sets of clothing. I always had to do was that there is a wide range of hypersexual behav-
laundry every night so his two favorite shirts and iors in KBS, from marked increase in heterosexual
two favorite pants were in a closet with 25 other
intercourse and excessive masturbation to fetishism,
sets that he not would wear.
homosexuality, pedophilia, zoophilia, coprophilia,
He wanted to have sex all the time. He went from and other changes in sexual appetite that were never
being a very compassionate and warm partner to previously evident. These changes in sexual behav-
just going through the motions. He didn’t ior could occur separately or clustered in a so-called
remember having just been intimate. He said it polymorphous perversity. The temporal relation to
didn’t seem like a current memory or emotion.
surgery and reports from the patient and wife were
He wanted sex constantly after his surgery until typical of KBS hypersexuality.
the day the officers showed up, at least 5 to 6 times The prosecutor argued that the patient’s hyper-
a day. He also gave up on foreplay. He would sexual behavior in some situations but not others
always want to get right to it. [He] was constantly was evidence for volitionally controlled criminal
grasping with his hands, coming up behind me and
behavior; that it was incompatible with a neurolog-
pressing himself against me while I was doing
ical cause. For example, he downloaded and
chores or talking on the phone. If [he] came to bed
in the middle of the night, he would ply me for sex. viewed child pornography at home but not at
work. We responded that patients with neurologi-
Two years ago, after his arrest, he was given cal disorders may restrain their urges when in
Zoloft and Seroquel and became much warmer socially inappropriate places and only give vent to
and loving but the medications shut off his libido.
them in selected settings. For example, a Parkin-
As with much of his obsessions and activities that
were ‘all or nothing’, sex became non-existent. His
son’s patient in a hyperdopaminergic state may
emotions to me, however, have reverted back to compulsively masturbate in his bedroom but not in
loving and compassionate and his outburst of public. Neurological disease often changes the
anger and rage have disappeared. It is as if a faulty threshold for abnormal behavior, but does not
switch was turned off. make behavior indiscriminate.
KLÜVER–BUCY SYNDROME, HYPERSEXUALITY, AND THE LAW 143

The defense attorney presented the opinion of Blumer, 1970; Christianson et al., 1995; Cogen
Judge Mansmann in US v. McBroome: ‘an indi- et al., 1979).
vidual’s average or above average mental capacity The pathological qualities of our patient’s sexu-
in one aspect of his or her affairs is not necessarily ality – its all-or-noneness and its insatiability –
relevant to a determination about the individual’s developed only after the second lobectomy. ‘All-
mental capacity in another aspect’ (USA vs. McB- or-none’ or ‘on/off’ reactions are characteristic of
roome). The defense then quoted from a letter impaired central control systems; they occur for
from OS: ‘. . . a man of superior intelligence and of example, in parkinsonian patients maintained on
real moral delicacy and sensibility, who at one L-Dopa (Sacks, 1990). Normal control systems
point was driven to act out of character under the have a middle ground and respond in a modulated
spur of an irresistible physiological compulsion fashion. The patient’s wife also observed that he
resulting from his brain injury. A recurrence of wanted more sexual activity within seconds of
such behavior is extremely unlikely given his char- orgasm – that there were only fleeting moments of
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acter and insight. It is important to note that even satiety before he was again overtaken by sexual
when his Klüver–Bucy syndrome was at its worst, urges. Normally, when one has eaten enough or
[he] never acted, never had any actual inappropri- climaxed sexually, there follows a peaceful period
ate contact with children or anyone else. He is of consummatory fulfillment. Our patients’ sexual
strictly monogamous’. and hunger appetitive systems were continually on
The patient faced up to a 20-year maximum ‘GO’; there was never any sense of satisfaction,
prison sentence with a 5-year minimum term. only the drive for more and more.
Despite the reports and testimony of physicians, The role of amygdala lesions in causing hyper-
the prosecutors sought the maximum sentence sexuality remains uncertain. In monkeys, the amy-
allowable by law because they saw the patient as a gdala is activated during sexual and aggressive
pedophile with an unrelated neurological disorder. social interaction (Kling, Steklis, & Deutsch, 1979).
The judge found that KBS was a mitigating factor In humans, amygdala stimulation can elicit sexual
and he received the shortest sentence allowable: 26 experiences (Gloor, 1990) and functional imaging
months in prison, 25 months of home confinement, reveals amygdala activation during sexual arousal
and then a 5-year period of supervision. (Karama et al., 2002). In monkeys, bilateral amy-
gdala lesions cause some features of KBS behaviors
(decreased fear and aggressive responses, hyperoral
DISCUSSION behavior) but they do not alter sexual behavior
(Aggleton & Passingham, 1981; Spies et al., 1976;
Hypersexuality after unilateral temporal lobec- Weiskrantz, 1956). Similarly, in humans, bilateral
tomy (Baird, Wilson, Bladin, Saling, & Reutens, amygdalotomy for behavioral disorders transiently
2002; Blumer, 1970; Anson & Kuhlman, 1993; alters sexual behavior, causing either hypo- and
Christianson, Silfvenius, Saisa, & Nilsson, 1995; hypersexuality, in less than 5% of patients (Baird,
Cogen, Antunes, & Correll, 1979) can occur as an Wilson, Bladin, Saling, & Reutens, 2007).
isolated symptom of KBS or, as in our patient, can The paradoxical role of amygdala lesions in
be accompanied by increased appetite and distract- hypersexuality probably reflects intersecting effects
ibility. Hypersexuality after temporal lobectomy of temporal regions that are resected, preserved,
occurs in both sexes, often with a latency averaging and irritated. In monkeys, hypersexuality with KBS
1–6 months (Blumer, 1970; Cogen et al., 1979). requires bilateral amygdala and neocortical resec-
Hypersexuality can involve ideation and fantasy, tions. In our patient, the first temporal lobectomy,
use of obscene language, demands for sexual activ- in which the amygdala and anterior temporal neo-
ity by a spouse, frequency and duration (up to 2 h) cortex was resected, did not result in hypersexual-
of erections, masturbatory behavior, changes in ity. This suggests that resection of additional
sexual preference (from heterosexual to homosex- posterior neocortical temporal regions was critical.
ual, etc.), and unprecedented interest in sexual Other patients developed hypersexuality after either
activity with children (Anson & Kuhlman, 1993; limbic or neocortical temporal lesions (Ozmen
Blumer, 1970; Christianson et al., 1995; Cogen et al., 2004). Patients who become hypersexual after
et al., 1979) Hypersexuality after temporal lobec- temporal lobectomy have larger contralateral
tomy lasts from several months to more than 5 amygdala than those without changes in sexual
years (Anson & Kuhlman, 1993; Baird et al., 2002; behavior (Baird, Wilson, Bladin, Saling, & Reutens,
144 DEVINSKY, SACKS, DEVINSKY

2004). The volume of the contralateral amygdala cor- parkinsonism, anoxia, multiple sclerosis, post-
related with the degree of postoperative hypersexual- neurosurgical and head trauma (Mendez et al.,
ity. Amygdala dysfunction may impair sexual 2000). These lesions predominantly affected the
arousal; removal of the diseased amygdala can right frontal and temporal regions.
improve libido if the contralateral amygdala func- Hypersexuality and pedophilia from brain disor-
tions normally (Ellison, 1982). Humans with KBS ders often conflict with social rules and laws. Our
hypersexuality often have irritative lesions such as case highlights the complex medical-legal issues
encephalitis and tumor that cause seizures. Hypersex- regarding responsibility for behavior in the setting
uality after temporal lobectomy is not usually corre- of KBS. Many patients focused their sexual inter-
lated with seizure activity (Baird et al., 2002), as in our ests and activities on their spouses, leading some
patient. Rarely, hypersexuality abated immediately patients to request that their spouses have temporal
after the first postoperative seizure (Blumer, 1970). lobe surgery and occasionally leading spouses to
Functional imaging studies in normal subjects request the operation themselves, in order to ‘keep
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and clinical observations in epilepsy patients sug- up’ (Baird et al., 2002). Other postsurgical patients
gest that the right hemisphere dominates the per- have violated criminal statutes. One woman, fol-
ception, activation and regulation of sexual lowing a left temporal lobectomy, had postictal
behavior (Fisher, Aron, & Brown, 2005; Janszky voracious eating, masturbating in public and solic-
et al., 2002; Ozkara et al., 2006; Remillard et al., iting family and neighbors for sex (Anson & Kuhl-
1983; Tiihonen et al., 1994). Visual sexual stimuli man, 1993). After one seizure she was brought to an
selectively activate right temporal regions on func- emergency room and after 30 min was found per-
tional imaging studies (Fisher et al., 2005). During forming fellatio on an elderly cardiac patient
male orgasm, there is a larger increase in blood (Anson & Kuhlman, 1993). This case and others
flow in the right frontal regions (Tiihonen et al., involved actual physical contact or an expressed
1994). Ictal sexual automatisms, ictal orgasm, and desire to engage in sexual relations with another
orgasm-induced seizures are more frequent with individual as a result of KBS, but none was crimi-
right temporal seizure foci (Janszky et al., 2002; nally charged (Anson & Kuhlman, 1993; Blumer,
Ozkara et al., 2006; Remillard et al., 1983). 1970; Mendez et al., 2000). Among more than 35
Unilateral lesions, usually involving the tempo- cases of pedophilia associated with neurological
ral lobes, can cause hypersexuality or hyposexuality. disorders, an arrest was only reported in two cases.
Increased libido is more frequent after right than left One man with a right frontal arteriovenous malfor-
hemisphere strokes (Baird et al., 2002; Kalliomaki, mation extending into the septum had sex with a
Markkanen, & Mustonen, 1961). Another group juvenile (Fisher et al., 2005). Another man with
found hyposexuality significantly more often with bilateral hippocampal sclerosis, (Mendez et al.,
left hemisphere lesions and hypersexuality with right 2000) (see above) molested a 5-year-old boy (Miller
hemisphere lesions (Braun, Dumont, Duval, Hamel, & Cummings, 1991). In contrast, our patient was
& Godbout, 2003). These observations suggest that prosecuted by federal authorities and now serves a
the right hemisphere inhibits sexual thoughts and 19-month prison sentence for downloading porno-
impulses. Although hypersexuality occurs equally graphic images of children. The KBS was a critical
after right and left temporal lobectomy, other fac- factor in driving his hyersexuality. In light of this
tors such as contralateral amygdala size may influ- mitigating factor, was he criminally responsible?
ence symptoms (Baird et al., 2004). Did his behavioral actions warrant imprisonment?
Pedophilia and other changes in sexual prefer- We believe the answer is no to both questions.
ence can follow brain damage. A 41-year-old man
developed hypersexuality 5 weeks after a right tem- Original manuscript received 1 August 2009
poral lobectomy (Blumer, 1970). In addition to Revised manuscript accepted 8 September 2009
obscene language at work, he desired homosexual First published online 18 November 2009
relations with his 15-year-old nephew. Pedophilia
developed in one man with frontotemporal demen-
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