British Journal of Neurosurgery

ISSN: 0268-8697 (Print) 1360-046X (Online) Journal homepage: http://www.tandfonline.com/loi/ibjn20

Kluver-Bucy Syndrome Following Recovery from
Transtentorial Herniation
Eugene Rossitch, Enrique J. Carrazana, Richard Ellenbogen & Eben Alexander
To cite this article: Eugene Rossitch, Enrique J. Carrazana, Richard Ellenbogen & Eben
Alexander (1989) Kluver-Bucy Syndrome Following Recovery from Transtentorial Herniation,
British Journal of Neurosurgery, 3:4, 503-506, DOI: 10.3109/02688698909002837
To link to this article: http://dx.doi.org/10.3109/02688698909002837

Published online: 06 Jul 2009.

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Date: 16 March 2016, At: 18:37

British Journal of Neurosurgery (1989) 3 , 503-506

SHORT REPORT

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Kluver-Bucy Syndrome Following Recovery from
Transtentorial Herniation
EUGENE ROSSITCH, JR* ENRIQUE J. CARRAZANA,** RICHARD ELLENBOGEN*
& EBEN ALEXANDER III*

Divisions of Neurosurgery* and Neurology** Brigham and Women’s Hospital Haruard Medical
School Boston, Massachusetts, U.S.A.

Abstract
Kluver-Bucy syndrome is an uncommon constellation of behavioral abnormalities resulting from bilateral temporal lobe
damage. The syndrome is rare in humans. In this paper we present a patient who developed the Kluver-Bucy syndrome
following recovery from an intracerebral haemorrhage and transtentorial herniation.

Key words: Kluver-Bucy syndrome, temporal lobe, transtentorial herniation

Introduction
A century ago, Brown and Schafer described
behavioral changes occurring after bilateral
temporal lobectomy in primates’. However, it
was through work of Kluver and Bucy that a
new constellation of symptoms now known as
the Kluver-Bucy syndrome (KBS) was identified2*3.
In primates, the complete expression of the
syndrome consists of the following:
1. ‘Psychic blindness’ which is defined as the
inability to use visual clues to appreciate the
significance of different objects.
2. An increase in the frequency and diversity
of sexual activity.
3. Changes in emotional behaviour.
4. Oral tendencies- the animals continuously examine objects by placing them in their
mouth.
5. ‘Hypermetamorphosis’- an excessive tendency to take notice of and to react to every
visual stimulus.

6 . Increased appetite.
7. Placidity.
8. Memory difficulties may also developKluver and Bucy observed that monkeys had
difficulty learning form-board tasks.
The first identification of a human KBS was
made in 1955 by Terzian and Dalle Ore in a
seizure patient treated with bilateral temporal
lobectomies4. Over 100 cases of ‘partial’ or
‘complete’ KBS have now been reported in
adults following bilateral damage to the temporal lobes. Etiologies have included herpes
encephaliti~~-~,
head traurna6q8, Alzheimer’s
Disease6, Picks D i ~ e a s e ~ adreno-leukodys.~,
trophy’O, acute intermittent porphyia”, and
Huntington’s ChoreaI2. In children, there have
been only seven reported cases of KBSS,13-16.
The most common etiology in this group is
hypoxia. One of us recently reported the case
of a child with a partial KBS and bitemporal
arachnoid cysts”. We now report a case of
KBS that occurred following recovery from an

503

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Eugene Rossitch et al.

intracerebral haemorrhage and transtentorial
herniation.

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Case Report

E.G. is a 42-year-old Hispanic male who was
brought to our institution following a generalised seizure. In the emergency room, he was
bradycardic and hypertensive with a blood
pressure of 220/ 140. Neurologic examination
revealed an unresponsive male who did not
move his left side even to painful stimulation.
A brain C T scan showed a large, primary
intracerebral haemorrhage located with its
center in the right putamen (Fig. la). There
was marked mass effect with effacement of the
suprasellar and quadrigeminal plate cisterns. A
small amount of blood was visualised in the
third and fourth ventricles. Carotid angiography revealed no aneurysm or arteriovenous
malformation.
In spite of hyperventillation and manitol,
the patient deteriorated over the next 24 h and
developed fixed, dilated pupils bilaterally. A
repeat C T scan showed increasing dilation of
the left temporal horn. The basilar cisterns
were no longer visualised (Fig. lb). From this
scan, the diagnosis of impending transtentorial
herniation was made.
E.G. was immediately taken to the operating
room where he underwent a right frontal
craniotomy for evaluation of the haematoma
and placement of a ventricular catheter. The
patient tolerated the procedure well and had an
uncomplicated postoperative course.
One month following the haemorrhage, E.G.
was alert and aware of his surroundings.
Neurologic examination was remarkable for a
left homonymous hemianopsia, a dense left
hemiparesis and left hyperreflexia. Language
modalities were intact although at times the
content of his speech was bizarre. Interestingly, the patient's speech was soft and monotonous, having lost the musical quality of the
Central American accent. Short-term memory
was impaired (the patient was unable to repeat
a simple three-sentence story), but long-term
memory seemed relatively intact. At this time,

a follow-up C T scan was performed and is
shown in Fig. lc.
In general, the patient was placid and
unconcerned, rarely showing any signs of
emotion. He had a very short attention span
and was easily distracted. E.G. did not seem to
pay special attention to anyone, apparently he
was unable to recognise relatives or friends.
The patient ate voraciously and tended to
place all objects within reach into his mouth
(e.g. bedsheets, curtains, spoons, faeces, fingers). E.G. made numerous inappropriate sexual advances towards the nurses taking care of
him. These advances were limited to persons
of the opposite sex. The patient almost constantly pulled on his foley catheter or stroked
his genitals. Masturbation was a frequent
occurrence.
The unusual behaviour persisted at the time
of discharge to a rehabilitation center two
months after admission.

Discussion
The expression of KBS in humans is somewhat
different than in animals. As expected, symptoms such as psychic blindness, hypermetamorphosis, hyperorality and aberrent sexuality
are manifested differently in humans. Psychic
blindness in humans includes the inability to
distinguish significant persons such as family
members from strangers. Hyperorality often
takes the form of hyperphagia, bulimia, or
ingestion of inedible objects. Hypermetamorphosis in humans consists of extreme distractibility and frequent exploratory behaviour.
Alterations in sexual behaviours are also expressed differently in monkeys and humans. In
humans, true copulation or masturbation is
infrequent. Rather, sexual overtures and attempted physical contact are more prominent.
Finally, complete KBS is rarely observed in
humans.
Bilateral temporal lobe damage in humans is
usually associated with severe disorders of
language, memory, and learning'*. Not surprisingly, memory and language dysfunction
occurs commonly in patients with KBS. This
defect is obviously not as easy to detect in

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Kluver-Bucy Syndrome

505

FIG. l(a) The brain C T scan performed on admission
showing a large, primarily intracerebral haemorrhage
located with it’s centre in the right putamen. There is
marked mass effect with effacement of the suprasellar and
quadrigeminal plate cisterns. (b) A repeat C T scan
performed approximately 24 h later after the patient’s
condition deteriorated. From this scan, the diagnosis of
impending transtentorial herniation was made. (c) Followup C T scan obtained five weeks after initial presentation.

monkeys, however, the animals observed by
Kluver and Bucy had difficulty learning formboard tasks, suggesting memory difficulties.
Our patient had impaired short-term memory
as well as some problems with language.
In 1975, Marlowe et al.’ reported a case of
humans KBS that was unique in that the
patient exhibited all components of the syndrome as defined in the experimental animal:
placid demeanor with flat affect, impaired

recognition of persons and objects, a pattern of
oral exploration, hyperbulimia, alteration of
sexuality, and stereotyped motor mannerisms.
In addition, the patient exhibited memory
difficulties. In most other reports of human
KBS, there is only partial expression of the
syndrome. For example, in the paper by Lilly
et aL6, cases were included if they manifested
simultaneously, a minimum of only three KBS
elements. Our patient appears to exhibit the
full syndrome.
Transtentorial herniation is the end result of
downward displacement of the hemispheres,
compressing and then displacing the midbrain
through the tentorial notchI9. In our patient,
the rapid evacuation of the hematoma probably
prevented the brain stem damage that is seen in
completed transtentorial herniation. However,
it appears that his temporal lobes were damaged by the process as evidenced by his

506

Eugene Rossitch et al.

memory and language difficulties as well as the
development of the full Kluver-Bucy syndrome.

8 Gerstenbrand F, Poewe W, Archner F, Saltuari L.

Kluver-Bucy syndrome in man: Experience with posttraumatic cases. Neurosci Biobehav Rev 1983;
7~413-17.
in
9 Cummines IL. Duchen LW. Kluver-Bucv< svndrome
*
Picks disease: Clinical and pathological correlations.
Neurology 1981; 3 1:1415-22.
10 Powers JM, Schaumburg HH, Gaffney CI. KluverBucy syndrome caused by adreno-leukodystophy.
Neurology 1980; 30:1131-2.
11 Guidotti T L , Charnes ME, Lamon JM. Acute intermittent porphyria and the Kluver-Bucy syndrome. J
Hopkins Med J 1979; 145233-5.
12 Janati A. Kluver-Bucy syndrome in Huntington’s
chorea. J Nerv Ment Dis 1985; 173:632-5.
13 Hooshmand H, Sepdbam T, Vries JK. Kluver-Bucy
syndrome: Successful trreatment with carbamazepine.
JAMA 1974; 229:1782.
14 Longman J, Valente MI. Disturbios neuropsiquiatricos
por lesoes bilateralis do lobo temporal. Sindrome de
Kluver e Bucy. Arq Neurosiquiatr 1957; 1540-57.
15 Tonsgard JH, Harwicke N, Levine SC. Kluver-Bucy
syndrome in children. Pediatr neurol 1987; 3:162-5.
16 Sawa M, Ueki Y, Arita M, Harada T. Preliminary
report on the amygdalordectomy on the psychotic
patient with interpretation of oral-emotional manifestations in schizophrenics. Folia Psychiatr Neurol Japan
1954; 7~309-29.
17 Rossitch E, Oakes, WJ. Kluver-Bucy syndrome in a
child with bilateral arachnoid cysts: Report of a case.
Neurosurgery 1989; 24:llO-2.
18 Milner B, Penfield W. The effect of hippocampal
lesions on recent memory. Trans Am Neural Assoc
1955; 8042-4.
19 Scheinker IM. Transtentorial herniation of the brain
stem; a characteristic clinicopathologic syndrome; pathogensis of hemorrhages in the brain stem. Arch
Neurol Psychiatry 1945; 53:289-98.
“ . I

Address for correspondence: Eben Alexander
111, M.D., Division of Neurosurgery, Brigham

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and Women’s Hospital, 75 Francis Street,
Boston, Massachusetts 021 15, U.S.A.
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