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NEUROCASE V p Psychology Press

2012, 18(5). 366-376 X Taylor & Francis Group

Perseverations and non-verbal confabulations on the


Rey-Osterrieth Complex Figure Test
in a fronto-temporal dementia single case study

A. SeddaS S. PassonP, and G. Bottini^'^


' Psychology Department, University of Pavia, Pavia, Italy
^Cognitive Neuropsychology Center, Niguarda Ca' Granda Hospital, Milan, Italy

We describe the case of a patient with late onset fronto-temporal dementia (FTD), who presented with typi-
cal personality changes, but also perseverative and confabulatory behaviors while performing the Rey-Osterrieth
Complex Figure Test. We hypothesize that the progressive atrophy of orbitobasal, medial, and dorsolateral frontal
cortices may give rise to both confabulations and perseverations in the non-verbal domain. In agreement with
previous studies, reporting atypical profiles, this case report underlines the clinical heterogeneity of FTD.

Keywords: Confabulations; Rey-Osterrieth Complex Figure Test; Fronto-temporal dementia; Perseveration; Non-
verbal confabulation.

Perseverations and confabulations have been des- Helm-Estabrooks, 1995a), perseverations can be
cribed in association with a variety of neurolog- broadly defined as the incapacity to inhibit the rep-
ical disorders (Hotz & Helm-Estabrooks, 1995a; etition of either all or part of a previous response.
Kern, Van Gorp, Cummings, Brown, & Osato, It has been suggested that the frontal lobes and
1992; Metcalf, Langdon, & Coltheart, 2007). their associated white-matter pathways may play
Perseverations are frequently reported after right a prominent role in perseveration (Hotz & Helm-
brain damage (Bottini et al., 2009). Patients with Estabrooks, 1995a), although there is also evidence
left unilateral neglect, for example, may add unre- that other brain areas such as the basal ganlia
quested writings or drawings on the right half (Bottini et al., 2009) may also give rise to this
of the sheet, while performing cancellation tasks disorder.
(Bottini, Bisiach, Sterzi, & Vallar, 2002; Gainotti Confabulations, occurring in a variety of brain
& Tiacci, 1970). According to the classification disorders such as traumatic brain injury (Dalla
of Sandson and Albert (1984), perseverations can Barba, 1993), dementia (Nedjam, Dalla Barba, &
present as 'stuck-in-set', when a current task cate- Pillon, 2000), and KorsakofTs syndrome (Dalla
gory or framework is inappropriately maintained; Barba, Cipollotti, & Denes, 1990), may be defined
'recurrent', when there is an unintended repetition as statements or actions that involve distortion of
of a previous response to a subsequent stimulus; reality (Metcalf et al., 2007). Although initially
and 'continuous', when a response is inappropri- these behaviors were described in terms of memory
ately repeated without interruption. Although the failure (Berlyne, 1972), over the years the concept
taxonomy widely varies among studies (Hotz & has evolved leading to new hypotheses, suggesting

Address correspondence to A. Sedda, Psychology Department, University of Pavia, piazza Botta 6, Pavia 27100, Italy. (E-mail:
sedda.anna@gmail.com).
Authors declare that they have no conflicts of interest.
© 2012 Psychology Press, an imprint ofthe Taylor & Francis Group, an Informa business
http://www.psypress.com/neurocase http://dx.doi.org/10.1080/13554794.2011.608368
CONFABULATIONS IN AN FTD SINGLE CASE ON THE ROCF 367

a role of executive control impairments in the gen- highlighting the progression of atrophy from ven-
eration of confabulations (Burgess & McNeil, 1999; tromedial to other frontal areas.
Metcalf et al., 2007; Turner, Cipolotti, Yousry,
& Shallice, 2008). Specifically, it has been sug-
gested that confabulations may be generated due to CASE REPORT
an impairment in the processes required in mem-
ory retrieval (Burgess & McNeil, 1999; Metcalf BM is a 75-year-old right-handed man, with
et al., 2007), attributable to a failure in execu- 11 years of education. He worked as a painter/
tive control and reality monitoring (Turner et a l , decorator, and he was retired at the time of the
2008). Several neuroimaging studies localized these evaluation. He came to our attention through his
strategic retrieval processes in the prefrontal cor- family doctor, due to relevant behavioral changes
tex (Gilboa & Moscovitch, 2002; Rugg & Wilding, because he became more and more irritable, and
2000; Shallice, 2001,2006). due to generic complaint of memory loss over the
previous 2 years. His past medical history was oth-
While perseverations are generally reported
erwise unremarkable except for a benign prostatic
across various domains (Hotz & Helm-Estabrooks,
hyperpiasia, which was treated with Dutasteride.
1995a, 1995b), confabulations are mainly described
Basic neurological examination was normal. The
in the verbal domain (Dalla Barba, 1993; Metcalf
patient was not taking any other drug, and had not
et al., 2007; Moscovitch & Meló, 1997). Although
undergone previous treatment. There was no family
confabulations received fewer attention in the non-
history of note.
verbal domain (Kern et al., 1992), two past studies
described these phenomena (Joslyn, Grundvig, & BM, now divorced, reported that he had been
Chamberlain, 1978; Kern et al., 1992). In par- married, that he has a daughter, and that he was
ticular, visuo-spatial or non-verbal confabulations not having a relationship at the time of evaluation,
have been defined as embellishments of abstract saying he was 'free'. The patient's daughter was not
drawings and they have been proposed as linked present at the examination, neither had she agreed
to verbal confabulations, independently from the for a face-to-face interview in the future. We were
diagnosis of the patients (Joslyn et al., 1978; Kern able to contact her by phone, and she only reported
et al., 1992). These embellishments have also been sporadic aggressive episodes and alcohol abuse of
defined as over-elaboration of the figure details her father in the past. However, given no other
(addition of extra details not present in the orig- relatives were available to gather collateral infor-
inal figure), which is more frequent in fronto- mation, we were not able to verify his daughter's
temporal dementia (FTD) than in Alzheimer dis- statements.
ease (Thompson, Stopford, Snowden, & Neary, Written informed consent was obtained from
2005a). Overall, the key feature of non-verbal BM for all the cognitive evaluations. Neuro-
confabulations seems to be a preserved spatial con- psychological assessment comprised:
figuration, in which unrequired details are embed-
ded. • The Mini Mental State Examination (MMSE;
Probably due to the progression of the atrophy of Folstein, Folstein, & McHugh, 1975; Measso
the frontal structures (Hodges, 2001), FTD patients et al., 1993), a 30-point screening test, which is
frequently present with both confabulatory syn- used to assess the severity of cognitive impair-
dromes (Nedjam et al., 2000) and perseverations ment, sampling various functions such as mem-
(Neary & Snowden, 1996). ory and orientation. The cut-off for the Italian
population is 24/30 (Measso et al., 1993).
Here we report the case of BM, diagnosed with
a probable FTD, the clinical course of whom we • The Raven Coloured Progressive Matrices
had the opportunity to monitor over 2 years. BM (Caffarra, Vezzadini, Zonato, Copelli, &
showed both confabulations and perseverations at Venneri, 2003; Court & Raven, 1995) is a mul-
a number of non-verbal neuropsychological tasks, tiple choice test of abstract reasoning, in which
mainly at the delayed recall of the Rey-Osterrieth the score is the number of correct responses
Complex Figure Test (ROCF) (Osterrieth, 1944; given in 30 minutes.
Rey, 1941). Although with regard to a non-verbal • The Letter Fluency Test and the Categories
test, BM's output was associated with a spe- Fluency Test (Novelli et al., 1986), the first
cific and consistent semantic content. His pat- involving executive functions, while the sec-
tern of confabulation and perseveration on the ond relates more to semantic memory access
ROCF became progressively more evident, possibly (Baldo, Schwartz, Wilkins, & Dronkers, 2006).
368 SEDDA ET AL.

The score of each test is given by the number of as attentional skills, visuo-motor planning, sus-
elements produced in 1 minute. tained attention, and working memory. Part A
• The Short Story Recall (Spinnler & Tognoni, requires the patient to join numbers, from 1 to
1987) assesses long-term episodic verbal 25, randomly distributed in space, while in part
memory. The number of elements recalled from B the subject is asked to alternately join a set of
a short article-like story gives the score. Recall numbers (from 1 to 13) and a set of letters (from
is assessed immediately and after a 10-minute A to L). The time taken to complete each part
delay. of the test is used as score.
• The Corsi block-tapping task (Corsi, 1972; • The Frontal Assessment Battery (AppoUonio
Orsini et al., 1987) assesses short-term visuo- et al., 2005; Dubois, Slachevsky, Litvan, &
spatial memory span. The score is given by Pillon, 2000) is a short but reliable battery for
the number of blocks in the longer sequence the assessment of executive functions. It consists
identified correctly. of 6 subtests investigating conceptualization,
• The Digit Span Forward Task (Orsini et al., mental flexibility, motor programming, sensi-
1987) is a common measure of short-term mem- tivity to interference, inhibitory control, and
ory verbal span, in which the score is given by environmental autonomy. Maximum total score
the longest list of digits repeated correctly. is 18 and higher scores indicate a better perfor-
• In the Rey-Osterrieth Complex Figure Test mance.
(ROCF) (Osterrieth, 1944; Rey, 1941), subjects • The Cornell Depression Scale (Alexopoulos,
are asked to reproduce a complex and abstract Abrams, Young, & Shamoian, 1988) assesses
line drawing, first copying it and then drawing signs and symptoms of major depression. Cut-
it from memory after a delay of some minutes. off for depression is 9 (Italian validation of the
This test involves not only constructional abili- scale for demented patients, Gruppo di Ricerca
ties but also planning and programming (exec- Geriatrica di Brescia, http://www.grg-bs.it).
utive functions), visuo-spatial abilities, mem-
ory and attention. For the administration and For all the tests, we applied the Italian published
scoring procedure we referred to Caffarra, norms. Scores have been corrected for age and edu-
Vezzadini, Dieci, Zonato, and Venneri (2002). cational levels and, when possible, transformed in
The original ROCF was used. The delay inter- equivalent scores.
val between the copy and the reproduction
from memory was 10 minutes (Caffarra et al.,
2002), with a verbal distracting task in the mid- 26 September 2006 - First cognitive
dle. In the scoring, 2 points are given when evaluation
the element is correctly reproduced, 1 point
when the reproduction is distorted, incom- At this time BM showed a performance in the nor-
plete but placed properly, or complete but mal range on all neuropsychological tests apart
placed poorly; a 0.5 point is credited when from the Semantic Fluency (Table 1, first evalua-
the element is distorted or incomplete and tion). Moreover, he performed poorly (below the
placed poorly. A zero score is given when the 25th percentile) on the FAB and on the ROCF
unit is absent or not recognizable (Caffarra recall. In detail, BM was impaired in the motor
et al., 2002). The ROCF includes 18 units, programming (Luria's series) and inhibition control
thus the maximum raw score for each of (interference sensitivity) sections of the FAB, result-
the two tasks (direct and delayed copying) is ing in an equivalent score of 2. His ROCF copy-
36. ing was normal, although he spontaneously repro-
• The Attentional Matrices (Spinnler & Tognoni, duced part of the geometrical elements (triangles)
1987) are used to assess subjects' ability to detect of the ROCF itself in the space surrounding the
visual targets among distracters. The test is com- copied ROCF (Figure la). However, BM's delayed
posed by 3 matrices of numbers, in which the figure recall was impaired (equivalent score of 1):
targets have to be crossed out as fast as possi- although he was still able to reproduce most of
ble. The score is given by the overall number of the individual elements of the figure, he was inca-
targets crossed out within 45 seconds. pable to preserve the global geometric pattern
• The Trail Making Test ~ parts A and B (Figure lb). During the clinical interview he pre-
(Giovagnoli et al., 1996; Reitan, 1958), is a sented with tangential speech. Neither visuo-spatial
measure of different cognitive components, such (Corsi Span) (Spimiler & Tognoni, 1987) nor verbal
CONFABULATIONS IN AN FTD SINGLE CASE ON THE ROCF 369

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370 SEDDA ET AL.

Figure 1. ROCF copy (a) and delayed reproduction (b) (first evaluation). Geometrical insertions are evident below the figure in the copy
condition.

memory (Short Story Recall) (Novelli et a l , 1986)


deficits emerged at this time.

First follow-up (17/03/2008) - Second


cognitive evaluation

Eighteen months after the first evaluation, BM's


behavioral symptoms worsened, although he Figure 2. Copy of a geometric pattern. The initial task is lost
appeared to be less aware of this, not complaining and original elements are substituted with spontaneous and
unrequested productions.
anymore of memory losses or other cognitive
disturbances. On direct questioning the patient
reported that his memory was as good as it ever At the Raven Coloured Progressive Matrices
had been. He was confabulating about his auto- (Court & Raven, 1995), BM stated that he could
biographical information, claiming not to have not see well and that 'These are as table cloths
enough time to complete the assessment, because burnt by an iron', and refused to perform the test.
he had an appointment with his brother (he has no On the ROCF, although clearly instructed to copy
relatives except his daughter). Moreover, he stated all the details, he 're-interpreted' the original pat-
to have a degree in architecture and a broken right tern as a fish. Furthermore, he gave a rich verbal
arm, which were false information. description, commenting on all the elements of the
At the neuropsychological testing, the patient original figure as possible parts of the drawn fish,
showed a general worsening, most of the tests with the exception of the 'cross' that he could not
now being outside the normal range (Table 1, fit in, as it was not coherent with the rest. He stated
second evaluation). Moreover, he produced many as follows: 'I do not understand how a cross can fit
perseverations on the Phonemic and Semantic fiu- in a fish! However, I will copy it as these are your
encies (Novelli et a l , 1986). A dense perseverative instructions'. He finally completed the 'fish' adding
behavior was also evident at the copy of a geometric a number of new elements, such as the tail and
pattern (Figure 2). the fins (Figure 3a). On the ROCF delayed recall.
CONFABULATIONS IN AN FTD SINGLE CASE ON THE ROCF 371

Figure 3. ROCF copy (a) and delayed reproduction (b) (second evaluation). Geometrical insertions are no longer present and the patient
completely substitutes the original ROCF with a fish, both during the copy and the delayed reproduction.

when asked to reproduce the figure, he said: 'Oh behavior did not allow him to perform a more
yes, the fish with the cross that does not fit in at all, exhaustive neuropsychological evaluation. At this
I will draw a fish again but now it will be a shark' stage, BM has been diagnosed as FTD following the
(Figure 3b). Lund and Manchester groups diagnostic criteria
When tested with the practice trials of the Trail (1994). Moreover, given the absence of a caregiver,
Making Test (Reitan, 1958), instead of simply we required the intervention of a social worker.
connecting the numbers, BM added some graphic
elements in order to make the pattern looking like a
fish (Figure 4). On the MMSE, he said an incorrect Second follow up (7/04/2008) - Third
year (2006) and he interpreted the sentence 'No cognitive evaluation
ifs, ands or buts' as 'there is no total traffic light'.
Moreover, his spontaneous written sentence was One month after the second evaluation, we found a
incomplete and indicative of disinhibition: 'hospi- clear persistence of the 'fish' behavior while copy-
tal evaluation only troubles'. On the Attentional ing the ROCF (Figure 5), and the patient clearly
Matrices, BM read aloud all the numbers he remembered to have drawn a fish before. Pointing to
crossed, although being advised not to do it. His the different parts ofthe figure, he commented: 'The
cross that does not fit in at all'. At this time, BM was
even more productive: he drew more fish and added
new elements to the original pattern (Figure 5a).
In the delayed recall the patient drew a number of
small circles at the bottom of the sheet, saying that
they were eggs the fish being pregnant (Figure 5b).
BM was cheerful and satisfied of how his memory
was functioning, as he was able to remember the
fish with all its details.
At this time, we tried to administer the patient
with other cognitive tests, but he was unable to
follow the instructions and started confabulating
Figure 4. Trail Making Test - part A; practice trials. Instead of about the stimuli. For instance, on the Raven
performing the requested conjunctions, BM draws a fish, using Matrices BM interpreted each item in terms of
as landmarks the Trail Making circles. familiar objects (A3: a table suite; AB 10 n.5:
372 SEDDA ET AL.

í/

Figure 5. ROCF copy (a) and delayed reproduction (b) during the last follow-up (third evaluation). The fish pattern is still present.
Moreover, other confabulatory items, related to the fish, are added to the picture, during the copy and the delayed reproduction.

Pharmacy sign; Al, N.3: the flag when Schumacher Initially BM's performance may be described as
wins; A7, N. 5: the Swedish flag) claiming that none a visual perseveration, as he repeats part of the geo-
of the available choices was the correct one. He was metrical elements (triangles) of the ROCF in the
able to complete only one stimulus, scoring 1 of 36. space surrounding the copied figure (Sandson &
Albert, 1984). The repetition of the fish pattern in
the third evaluation and on the Trail Making Test
DISCUSSION may be seen as well as a perseveration of the fish
visuo-spatial confabulation. Similarly, in the third
Confabulations and perseverative behaviors may evaluation the patient adds more little fish, which
be typical disorders of FTD when compared to can be a perseveration focused on the visuo-spatial
other dementias (Rozzini, Lussignoli, Padovani, confabulation he had previously produced.
Bianchetti, & Trabucchi, 1997; Thompson, However, at the second assessment, BM's per-
Stopford, Snowden, & Neary, 2005b). We had the formance on the ROCF suites the definition of
opportunity to monitor the neuropsychological visuo-spatial confabulation (Figures 2 and 3), as he
symptoms of BM, a patient diagnosed as probable develops a meaningful embellished figure, related
FTD. to the ROCF original abstract drawing, rather than
Interestingly, our patient presented with a dense simply adding perseverative strokes or repeating the
non-verbal confabulation, starting from his second same elements of the figure. He adds details to the
neuropsychological evaluation. BM's performance abstract pattern of the ROCF, drawing a fish, but
on the ROCF initially showed an intrusion of geo- maintains the original spatial configuration (i.e., the
metrical shapes (first evaluation. Figure 1) devel- fish has the same orientation of the ROCF, of which
oping in the following controls a more complex part of the original elements are elaborated), in
re-interpretation of the original pattern (the ROCF) agreement with non-verbal confabulations descrip-
as a fish (second and third evaluations. Figures 2 tions from previous studies (Joslyn et al., 1978;
and 3). Moreover, this flsh pattern persisted enough Kernetal., 1992).
to spread across the Trail Making Test, the stim- We believe that BM presents a mix of non-verbal
uli of which have been re-interpreted as a fish as confabulation and perseveration, which we can
well. speculate is related to the disease progression and
CONFABULATIONS IN AN FTD SINGLE CASE ON THE ROCF 373

possibly to the anatomical involvement of different establish the primary causal role. As highlighted
frontal areas. Our hypothesis is that the progressive by his neuropsychological profile, both his ver-
atrophy of orbitobasal, medial, and dorsolateral bal and graphical confabulations increased together
frontal cortices may give rise to both confabulations with the executive control decline. At the second
and perseverations. FTD has been reported to ini- evaluation, in fact, when BM's 'fish' behavior
tially involve the ventromedial and orbitobasal por- became more apparent, his performance at a num-
tions of the frontal cortices, implying emotional ber of frontal tests (FAB, Verbal Fluency, and
dysfunctions, dishinibition, poor impulse control, Attentional Matrices; Spinnler & Tognoni, 1987)
and stereotypes (Hodges, 2001; Neary, Snowden, became impaired together with a decline of his
& Mann, 2005). With the disease progression, dor- memory functions as shown by the Digit and the
solateral frontal cortices are affected as well, and Corsi Span scores. Curiously enough, his perfor-
planning, organization, executive functions in gen- mance on the Short Story Recall was nearer to
eral are also compromised (Hodges, 2001). In sup- the normal range, possibly suggesting a greater role
port of this view, BM initially showed a perse- of executive control rather than memory storage
verative behavior, and his confabulations increased impairments per se in arising BM's confabulations
together with the worsening of his executive func- (Metcalf et al., 2007; Turner et al., 2008).
tions. It was only during the second evaluation that Independently from the mechanism responsi-
tests other than the FAB showed a clear impairment ble for the impairment, the strict application of
of executive control. BM's personality changed dur- clinical criteria in FTD diagnosis may be mis-
ing the follow-ups, from a mildly impulsive behav- leading in some atypical cases in which memory
ior to an increasing irritability, passing through impairment or other peculiar deficits are present,
a depressive mood. just because of the well-known heterogeneity of
FTD is known to be characterized by a strong FTD (Graham et a l , 2005). BM himself may rep-
clinical heterogeneity: each patient experiences dif- resent a typical case of FTD, as he presented
ferent symptoms, depending on the frontal and with classical personality and behavioral changes
temporal areas of the brain initially involved in the during its initial stages. However, he also pre-
atrophy (Graham et al., 2005). Typically, during sented with perseverations and confabulations in
the initial stages, personality and behavior may be the non-verbal domain and initially complained
compromised, while memory is still intact. Patients about memory impairments, partially confirmed by
may become extroverted and lose their inhibition or his performance on the Semantic Fluency Test.
alternatively they may manifest apathy and become Moreover, his disease onset was late in life. For
withdrawn. Patients may also develop fixed and FTD, the age of onset has been established typ-
obsessive routines and change their food prefer- ically between 45 and 65 years, although rarely
ences towards sweet tastes. However, otherwise typ- onset can occur after 75 years of age (Manes et al.,
ical cases of FTD have already been reported in 2010).
the literature. For instance, Graham et al. (2005) It is puzzling that, despite the general tendency
even evaluated FTD patients with memory dis- of our patient to confabulate, he actually did not
turbance as the presenting feature. Manes et al. confabulate while recalling the short story, unlike
(2010) described the case of a FTD patient, whose previously reported FTD patients (Nedjam et al.,
initial feature was a pathological gambling behav- 2000; Nedjam, Devouche, & Dalla Barba, 2004;
ior. Based on clinical experience, absence of 'early, Thompson et al., 2005b). It may also be possible
severe amnesia' as an exclusion criterion for the that BM produces 'spatial' confabulations referring
diagnosis of FTD has been recently questioned: sev- to a semantic domain (fish) to facilitate his recall
eral studies demonstrated that patients with FTD process using this re-interpretation of an unfa-
may complain of memory loss and memory under- miliar geometric pattern as a known object. The
performance (Binetti, Locascio, Corkin, Vonsattel, attempt to refer the individual components of the
& Growdon, 2000; Graham et al., 2005). It is ROCF rather than to the global pattern is typi-
still debated if these memory problems are a 'pure cally observed in brain-damaged patients (Trojano
storage' deficit (Hodges, 2001) or an impairment et al., 2004). This 'piecewise approach' (Trojano
in strategic retrieval (Turner et al., 2008). In our et al., 2004) may facilitate the interpretation of
patient, confabulations and cognitive dysfunctions the single elements as more familiar things such
were in parallel; however, it is very difficult to as a house or a cross. To store information in
374 SEDDA ET AL.

long-term memory, consolidation processes, allow- in frontal lobe lesions. Previous studies (Joslyn
ing reorganization within representations of stored et al., 1978; Kern et al., 1992) on non-verbal
information, have been shown to be important confabulations, although limited, are meaningful.
(Shin, Park, Park, Seol, & Kwon, 2006). Executive Non-verbal confabulations, nicely revealed by the
functions help to organize diverse materials into a ROCF, may be a relevant part of the clinical profile
meaningful cluster, so that the memory consolida- of FTD and a distinctive sign useful for a differen-
tion can be facilitated. Organizing the ROCF into tial diagnosis from Alzheimer's disease.
a meaningful perceptual unit, during the Copy con-
dition, has been shown to enhance its subsequent Original manuscript received 17 November 2010
recall from memory (Shin et al., 2006). Thus, BM's Revised manuscript accepted 24 July 2011
better performance on the delayed recall in the sec- First published online 6 December 2011
ond evaluation (equivalent score 2; first evaluation,
equivalent score 1) may be explained thanks to the
fish pattern, which is, in fact, a helpful, meaningful REFERENCES
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