Intracranial Arachnoid Cysts - Do They Impair Mental Functions?
Intracranial Arachnoid Cysts - Do They Impair Mental Functions?
Intracranial Arachnoid Cysts - Do They Impair Mental Functions?
Received: 6 April 2008 N Abstract Objectt Arachnoid cysts functions were found to be im-
Received in revised form: 9 May 2008 are benign, congenital malforma- paired, including verbal perception
Accepted: 19 May 2008 tions of the arachnoid with a predi- and memory, more complex verbal
Published online: 28 July 2008 lection for the temporal fossa. Ra- tasks, visuospatial functions, and
diologically, most arachnoid cysts visual attention. In addition, nu-
appear to be expansive lesions, and merous case reports were identi-
functional imaging has shown that fied that link arachnoid cysts to a
arachnoid cysts may cause a reor- wide variety of neuropsychiatric
ganization of cortical functions. and cognitive symptoms. Conclu-
Methods The present article is sions This survey indicates that
K. Wester, MD, PhD based on a literature search for ar- arachnoid cysts may affect cogni-
Section for Neurosurgery
Dept. of Surgical Sciences ticles reporting dyscognition or tion and also that they do so in a
University of Bergen psychiatric symptoms in arachnoid reversible manner, as the patients’
5021 Bergen, Norway cyst patients. Emphasis is placed cognitive impairment seems to im-
K. Wester, MD, PhD (,) on systematic, prospective studies prove after surgical decompression
Dept. of Neurosurgery reporting results from larger series of the cyst.
Haukeland University Hospital of patients. A total of five articles
5021 Bergen, Norway
Tel.: +47-55/975-000
were identified that had investi- N Key words arachnoid cysts ·
Fax: +47-55/975-699 gated cognition in larger series of cognition · dyscognition · mental
E-Mail: kgwe@helse-bergen.no patients. A variety of cognitive functions · psychiatry · psychology
complaints in patients with temporal arachnoid cysts content, in normal persons mostly the brain. An arach-
[31]. noid cyst will create a surplus intracranial space by its
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sheer presence during the growth of the skull. This sur- sion. Such information must be of importance when
plus space may allow the cyst to be present without considering indications for decompressive cyst surgery.
yielding as dramatic symptoms as would be expected The present article is therefore an attempt to summarize
from the radiological appearance. One example of this the present knowledge on the topic.
surplus space is the enlarged middle fossa that often can
be seen in temporal cyst patients. Arachnoid cyst may
not only act as passive surplus fluid volumes; there is Review of the literature
also substantial evidence that cysts in children may grow
considerably, thus indicating an increased intracystic The following review is based on an extensive search in
pressure over time [43, 62, 85]. the PubMed and Web of Science databases. The terms
There appears to be an association between intracys- used in the search were: arachnoid AND cyst AND one
tic pressure and subjective complaints [31]. It therefore of the following terms: “behavio*”, “cognition”, “cogni-
seems reasonable to assume that the patient’s symptoms tive”, “dement*”, “dyscognition”, ”mental*”, ”psychol*”,
are caused by the cyst’s pressure on its surroundings “psychomotor*”, “psychiatr*”, “psychot*”, and “neuro-
(brain tissue, cranial nerves, and/or leptomeninges). It is psychol*”. The review includes all original articles writ-
furthermore not only conceivable, but even probable, ten in English, or articles written in other languages
that the pressure from the cyst may interfere with func- with an informative English abstract that describe cog-
tions of the surrounding brain tissue, including higher nitive or psychiatric symptoms or findings in otherwise
brain functions such as cognition and emotions. A sig- healthy patients with supratentoriall intracranial arach-
nificant thinning of the cerebral cortex adjacent to an noid cyst. Only articles describing what appears to be a
arachnoid cyst has been demonstrated [36], and func- direct association between the cyst and the mental effect
tional imaging has shown that arachnoid cysts may are included in this review.
cause a reorganization of cortical functions [1, 13, 71]. Articles were excluded for the following reasons:
Many reports, either case studies or studies where groups 1. If the mental effects could be explained as a second-
of patients with neuropsychiatric disorders have been ary effect of the cyst, e.g., epileptic seizures, hydro-
radiologically examined, do suggest that arachnoid cysts cephalus caused by obstruction of the normal CSF
indeed may affect mental functions [4, 6, 14–17, 20, 22, drainage, or endocrinological disturbances caused
24, 34, 35, 38, 42, 46, 48, 49, 52, 55, 57, 63, 72, 73, 75, 76, 78, by large suprasellar cysts.
86–89]. Lang et al. [47] were the first to report on cogni- 2. If the mental deterioration was believed to be caused
tion in a larger series of cyst patients. They found im- by the congenital metabolism disorder glutaric acid-
paired cognition in 9 of 10 patients with temporal or uria type 1, where the disease itself is the cause of the
temporofrontal cysts and concluded that one should not brain damage, not the bitemporal cysts that often are
regard arachnoid cysts as being of no functional signifi- associated with this disease [39, 51], or other condi-
cance unless extensive psychological testing had been tions known to be associated with cerebral dysfunc-
performed. Contrary to their findings, Kunz et al. did not tion, e.g., cortical dysplasia or syndromes known to
find a general dyscognition in patients with temporal yield mental retardation.
cysts when they were tested with several tests for intel- 3. Pure review articles without original observations.
ligence, memory, and visuospatial functions. However, 4. A few articles reporting on patients with cyst of non-
in the dichotic listening test, they observed a unilateral arachnoid origin, e.g., ependymal or porencephalic
deficit in four patients [45]. cyst, were also excluded.
Only a few studies have looked upon cognition in
larger series of cyst patients before and after surgical A total of 198 articles were identified by the search. Of
decompression in a systematic fashion. They all seem to these, 142 were excluded for the reasons mentioned
confirm that arachnoid cysts may impair cognition and above, leaving 56 relevant articles for this review.
more importantly, the preoperative dyscognition tended
to be normalized following surgical decompression [28,
60, 69, 83, 84]. These observations of a preoperative im- Case reports
pairment followed by a postoperative improvement in-
dicate that the pressure from the cyst may not necessar- N Psychiatric symptoms
ily cause a permanent destruction of brain tissue; it
appears more likely that it causes a reversible suppres- Over the years, many case reports have been published
sion of brain functions, as suggested by Ræder et al. that describe psychiatric symptoms in cyst patients,
[60]. most often in the form of a psychosis. These symptoms
As indicated above, there is accumulating evidence include alexithymia [6], attention deficit hyperactivity
that arachnoid cysts may cause cognitive or even psychi- disorder (ADHD) [55, 80], delirium [15], depression
atric symptoms that are reversible after cyst decompres- [22], delusion of hallucinations and persecution [4, 18,
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27, 38, 87], religious delusions [38], insomnia and irrita- arachnoid cyst patients in a population of 168 psychotic
bility [77], organic dementia syndrome [14, 15, 25, 40], patients. As referred to above, Kohn et al. [38] studied
schizophrenia-like symptoms or other psychotic disor- eight cyst patients that presented with a primary psychi-
ders [16, 17, 23, 38, 42, 46], anorexia nervosa combined atric diagnosis. Some of their patients also had neuro-
with guilt delusions and catatonia [86], suicidal attempts psychological abnormalities and it was found that surgi-
[38], and even aggression [77, 80] and homicide [57, cal decompression resolved the psychiatric manifestations
63]. in both cases that were operated.
Very few of these patients were reported operated for
their cyst. Only six reports give an account of the post-
operative functional results; five of them demonstrate Systematic studies in patient series
postoperative normalization of the symptoms [14, 41,
42, 78, 89]. In addition, Kohn et al. [38] studied eight pa- To our knowledge, only six studies have been published
tients with intracranial cysts presenting with primary that have systematically investigated mental functions
psychiatric diagnoses. They report that surgical inter- in populations of arachnoid cyst patients. Lang et al. [47]
vention resolved the psychiatric manifestations in both were the first to systematically investigate cognition in
cases that were operated. Arai et al. [2], however, did not cyst patients. In a series of ten adult patients with an
find any postoperative improvement in behavioral prob- arachnoid cyst in the anterior and middle cranial fossa,
lems. with no overt cognitive problems, they nevertheless dis-
closed dyscognition in nine. They concluded that there
was a functional relationship between the cysts and the
N Cognition cognitive deficits in these patients, and that one should
not regard such cysts as being without functional sig-
Dyscognition has also been demonstrated in several nificance unless extensive psychological testing had
case reports on arachnoid cyst patients. Some of these been performed. The question of whether surgical de-
patients had clinically overt cognitive problems [12, 19, compression of the cyst might improve the dyscognition
20, 34, 52, 61, 72, 73, 75] in others the dyscognition was remains, however, unanswered after this report, as their
revealed only after neuropsychological testing [35, 44, patients were not operated.
48, 76, 88]. Several aspects of dyscognition have been re- In 1995 Wester and Hugdahl [83] investigated pre-
ported: agraphia [34], organic dementia syndrome [14, and postoperative verbal perception and verbal memory
15, 25, 33, 58] mental retardation [12, 20, 52, 56, 74, 75], functions in 13 adult patients with a left temporal arach-
delayed language development and learning problems noid cyst. The patients were tested with a dichotic listen-
in children [18, 20, 35, 50, 73], verbal learning, visual per- ing technique with simultaneous presentation of differ-
ception, constructional skills, conceptual shifting, psy- ent auditory stimuli to the two ears. In the preoperative
chomotor speed [69], and memory deficits [35, 44, 69, 72, memory test, the patients showed impaired total recall
76] . compared with healthy control subjects, and moreover,
Apparently, very few of these cases were operated. recall from the right ear was significantly impaired. The
Only five articles report on the functional results after patients also performed poorly in a forced attention task
cyst decompression; they all describe normalization of consisting of dichotic presentations of brief consonant-
the cognition, including developmental delays [19, 35, vowel syllables. The cyst was decompressed in all the
56, 69, 76] even with only minimal reduction of the vol- patients. The surgical procedures led to improvements
ume of the decompressed cyst [69]. However, a well-es- in both perception and memory. Overall memory per-
tablished mental retardation does not seem to improve formance was enhanced, mainly because of an improved
[2]. recall from the right ear. This normalization of memory
function was found as early as four hours after the op-
eration. The results indicate that arachnoid cysts in the
Arachnoid cysts in patient populations left temporal fossa may impair verbal cognition and,
with mental disorders more importantly, that this impairment is not perma-
nent, as surgical cyst decompression restores the cogni-
Only a few studies have tried to estimate the prevalence tion, even in adults.
of arachnoid cysts in patients with mental disorders. Left-handedness and right hemisphere speech domi-
Zeegers et al. [89] found that 8.5% of children under the nance may follow early, localized damage to the develop-
age of 3 years with developmental disorders had an ing brain. It is therefore conceivable that a congenital
arachnoid cyst. Lingam et al. [49], on the other hand, arachnoid cyst may interfere with the functional organi-
found a much smaller fraction of cysts in a CT study in zation of the cerebral hemispheres, causing a so-called
76 mentally subnormal children, most of them with se- “pathological” left-handedness syndrome and a speech
vere retardation. In adults, Gewirtz et al. [24] found three dominance of the right hemisphere. This possibility was
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tested in a relatively large series of 51 consecutive pa- In the last study from that group, 27 patients with
tients with temporal or frontal arachnoid cysts [84]. Ver- temporal or frontal cyst and 27 healthy control subjects
bal laterality was measured with the dichotic listening were tested with two different visual attention para-
(DL) technique, a non-invasive, reliable technique for digms [28]. The patients were tested before and mini-
hemispherical speech localization. In this test, with si- mum three months after decompressive surgery, with
multaneous presentation of different auditory stimuli to the same test-retest interval for the controls. It was found
the two ears, about three quarters of a normal popula- that both automatic and effortful visual attention was
tion will perceive better the sounds delivered to the right impaired, and that these attention impairments were
ear. This normal superiority of the right ear is often re- significantly improved after surgery, also when control-
ferred to as a Right Ear Advantage (REA). ling for learning and practice effects from pre- to post-
The distribution of handedness was normal in the surgery testing. Moreover, the effects appeared depen-
cyst patients, and thus not affected by the cyst. In the dent on cyst location, as patients with right hemisphere
preoperative DL test, however, the cyst patients displayed cysts were impaired for shift of attention, whereas pa-
a response pattern indicating that a hemispherical reor- tients with a left hemisphere cyst were impaired for vi-
ganization of verbal perception in fact had taken place. sual search. Again, it was concluded that intracranial
Only 51 % of the patients showed the normal superiority arachnoid cysts seem capable of causing a cognitive im-
of the right ear (REA), significantly different from the pairment that is reversible after surgical decompression,
REA frequency (74 %) in a normal reference group. Pa- and that these facts should be taken into account when
tients with a left temporal, or a frontal cyst had a sig- considering the indications for decompressive cyst sur-
nificantly lower preoperative REA frequency than the gery.
reference group, whereas patients with a right temporal Recently, Spansdahl and Solheim [70] have demon-
cyst did not differ from the controls. Three to six months strated in a large population of arachnoid cyst patients
after decompressive surgery, the cyst patients showed that they have a higher anxiety level than normal per-
normalization, as the REA frequency in the patients sons have.
(73 %) no longer was different from that of the reference
group.
It was concluded that, although such cysts are con- Discussion
genital, the pressure from the cyst on the adjacent brain
is not strong, nor persistent enough to cause a patho- In neurosurgery, as well as in neurology, neurological
logical left-handedness. It can further be concluded that deficits have been important as indications for surgery,
the arachnoid cysts are capable of interfering with corti- but also in the evaluation of patient outcome. To a sur-
cal processes involved in verbal perception. However, prisingly little degree have neurosurgeons been inter-
the observed postoperative normalization indicates that ested in mentall functions, such as cognition and emo-
a real reorganization of cortical language areas into the tions. Cognitive deficits may be subtle and not perceived
right hemisphere had nott taken place. More likely, the as such by the patient or the family, and they may often
cyst had only suppressed d cortical functions involved in be detected only if looked for by specific, psychological
speech perception in a reversible manner, rather than tests [47, 48]. The present survey reveals a number of
destroyed them, thus allowing a functional normaliza- publications, mostly case reports, on possible mental ef- f
tion after decompressive surgery. fects exerted by intracranial arachnoid cysts. As arach-
In another study, 54 patients and 32 healthy controls noid cysts have been reported to occur in as much as 1 %
were assessed pre- and postoperatively on four different of the normal population [79], the mere co-existence of
cognitive tests for visuospatial and complex verbal tasks a cyst and relatively common mental symptoms cannot
(Benton Visual Retention Test, Street Gestalt Test, Stroop be taken as evidence of a causal relationship between the
Test and Trails Test A and B) [60]. On the first test occa- two phenomena. Although few however, nearly all the
sion (preoperatively), the patients performed worse case reports that describe the effect of surgical cyst de-
than the controls for all four tests. Three to six months compression on the co-existing mental deficits seem to
after surgical decompression the patients, but not the report a postoperative improvement [14, 19, 35, 38, 41,
controls, improved significantly on three out of the four 42, 56, 69, 76, 78, 89], thus constituting a somewhat stron-
tests, with a trend towards improvement also on the ger indication of a causal relationship. The strongest
fourth test. For two of the tests, the patients’ test perfor- evidence comes from the systematic investigation with
mance seemed to be influenced by the degree of postop- standardized neuropsychological tests in relatively large
erative reduction in cyst volume. The study showed that series of patients [28, 47, 60, 83, 84]. They all describe
patients with intracranial arachnoid cysts may have impairment over a wide spectrum of cognitive func-
concealed deficits in a wide variety of cognitive func- tions, such as visuospatial functions, verbal perception
tions, and that these deficits may be reversed by surgical and memory, as well as visual attention. Only four of
decompression. these studies describe the effect of cyst decompression
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on cognition, reporting a significant improvement. Un- first time a neuropsychological finding in itself may con-
fortunately, it is a clear weakness with the present survey stitute, or add to, the indication for neurosurgery. How-
that all these systematic, prospective studies showing ever, if surgery should be considered on this indication
preoperative impairment and postoperative improve- alone, it is important to notice that neurosurgery with
ment come from the same research group, namely ours. the aim of improving life quality only should be under-
We would therefore encourage and welcome similar sys- taken if the surgical risk is very low. In our experience, it
tematic and prospective studies on different aspects of is [30, 32].
cognition in arachnoid cyst patients. Some neuroimaging studies have shown that the
Thus, the present survey appears to deliver clear in- temporal lobe adjacent to an arachnoid cyst is smaller
dications that arachnoid cysts indeed affect mental and less metabolically active than the contralateral tem-
functions, and also that they in many cases do so in a poral region [88], that cortical language areas within the
reversible manner, i.e., the patients’ cognition seems to left hemisphere may be displaced by an arachnoid cyst
improve after surgical decompression of the cyst. This [71], and that there is a thinning of cortical tissue in the
fact should be taken into account when considering de- vicinity of an arachnoid cyst [36]. Most importantly, it
compressive cyst surgery. The data presented here also has also been demonstrated that a cyst may reduce the
underlines the importance of using psychological tests perfusion and metabolism in the surrounding cortical
in these patients, as stated by Soukup et al.: “Cognitive regions [19, 35, 53, 66, 76, 87], and that these changes are
measures may provide an alternative, functional index reversible after the cyst has been decompressed, thus
of outcome efficacy, and reliance on traditional outcome paralleling the cognitive improvements seen in the same
measures (i.e. anatomical decompression or resolution patients [19, 53, 66, 76]. These findings are of the utmost
of clinical symptoms) may underestimate the efficacy of importance, as they demonstrate so clearly the associa-
surgical intervention for these patients” [69]. tion between a functional improvement and a normal-
Most arachnoid cysts are considered congenital. Con- ization of the metabolism in the corresponding cortical
sequently, it seems reasonable to assume that the effects area following the surgical decompression of a cyst.
exerted by the cyst upon the neighboring brain tissue Studies in animals and humans have shown that there
must have been life-long. The findings of the prospective are critical periods or time windows in a young individ-
studies presented here, both single-case reports and on ual’s development, i.e., the optimal time during which
larger series of patients, with a postoperative cognitive the brain is in the optimal condition to learn or achieve
improvement even in adults, suggest that the pressure specific abilities, intellectual as well as emotional, and
from the cyst on the surrounding brain parenchyma during which deprivation of stimuli that are important
does not necessarily cause a permanent destruction of for the brain’s development can cause permanent defi-
brain tissue, but more likely a reversible suppression of cits [3, 7–11, 26, 29, 65, 67].
brain functions. These observations are important, as Animal experiments indicate that these time win-
this potential for cognitive improvement must be taken dows are rather absolute [3, 7–10, 27, 58]; experience in
into account when considering the indications for de- humans indicates that a human child may have the ca-
compressive cyst surgery. Some studies estimate the pacity to compensate to some extent, even after that pe-
prevalence of arachnoid cysts in the population to be as riod has elapsed [26]. It is reasonable to assume, how-
high as 1 % [79]. Whether similar effects on mental func- ever, that the learning after the closure of the “time
tions can be found in all these persons remains un- window” is much more difficult than when the neural
known, as all the patients in our series were operated networks of the developing brain still is susceptible to
because of distinct clinical symptoms, mainly headache new impulses.
and dizziness. Thus, there is a potential danger that an arachnoid
Most of the studies have used standard neuropsycho- cyst in childhood may not only cause acute symptoms in
logical tests. It remains uncertain to which degree these some patients. More importantly, it may interfere during
tests and findings reflect practical problems for the pa- these vulnerable phases with neural processes that are
tient. However, the tests have disclosed deficits in so crucial for the normal development of the brain, thereby
many aspects of cognition, including verbal perception giving rise to permanent, irreversible learning difficul-
and memory, complex verbal tasks, attention, and visuo- ties and behavioral problems. This may particularly be
spatial functions that it is difficult to imagine that they the case in children with middle fossa cysts, the most
do not interfere with the daily life, even in patients who common location for arachnoid cysts [21, 30, 32, 64, 81,
are not aware of any dyscognition. 82], as the temporal lobe is one of the most important
The knowledge that arachnoid cysts may cause cog- parts of the developing brain. A substantial number of
nitive deficits, and that this dyscognition normalizes our pediatric patients with temporal cysts were referred
after cyst decompression, constitute in our view evi- because of behavioral and learning problems. In our
dence in favor of surgical decompression, however not view, the probability that a cyst may hamper the normal
necessarily on an individual basis. This, we believe, is the mental development should be taken into account when
1118
considering early surgical decompression of arachnoid ner, as the patients’ cognitive impairment seems to im-
cysts in pediatric patients. An early decompression might prove after surgical decompression of the cyst. These
relieve the pressure on the surrounding cerebral tissue observations are important, as this potential for cogni-
during critical periods for learning, thus allowing these tive improvement should also be taken into account
children a more normal development of the brain. when considering the indications for decompressive
cyst surgery.
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