J Neurol (2009) 256:1809–1815
DOI 10.1007/s00415-009-5197-0
ORIGINAL COMMUNICATION
Use of the frontal assessment battery in evaluating executive
dysfunction in patients with Huntington’s disease
Guilherme Riccioppo Rodrigues Æ Carolina Pinto Souza Æ
Roberto Satler Cetlin Æ Daniel Sabino de Oliveira Æ Marcio Pena-Pereira Æ
Liliana Tiemi Ujikawa Æ Wilson Marques Jr. Æ Vitor Tumas
Received: 17 December 2008 / Revised: 17 May 2009 / Accepted: 25 May 2009 / Published online: 18 June 2009
Ó Springer-Verlag 2009
Abstract The frontal assessment battery (FAB) is a
bedside cognitive scale designed to measure executive
functions. Huntington’s disease (HD) is a neurodegenera-
tive disorder characterized by motor, behavioral, and cog-
nitive dysfunction. The aim of this study was to check the
validity of the FAB for the evaluation of cognitive
impairment in patients with HD. Forty-one patients diag-
nosed with HD and 53 healthy controls matched by edu-
cation, sex and age were evaluated with a validated
Brazilian version of the UHDRS, the VFT, the SDMT, the
SIT, the MMSE, and the FAB. The diagnosis of HD was
made by DNA analysis. FAB scores were lower in patients
than in the controls (p \ 0.001) and had significant corre-
lations with the VFT (r = 0.79; p \ 0.05), the SDMT
(r = 0.80; p \ 0.05), the SIT (r = 0.72; p \ 0.05), the
MMSE (r = 0.83; p \ 0.05), the FCS (r = 0.79; p \ 0.05)
and the motor section of the UHDRS (r = -0.80;
p \ 0.05). The FAB differentiated between HD patients in
the initial and later stages of the disease. The one-year
longitudinal evaluation revealed a global trend toward a
worsening in the second score of the FAB. The results
demonstrate that the FAB presents good internal consis-
tency and also convergent and discriminative validity;
therefore it is a useful scale to assess executive functions
and to evaluate cognitive impairment in patients with HD.
G. R. Rodrigues
C. P. Souza
R. S. Cetlin

D. S. de Oliveira
M. Pena-Pereira
L. T. Ujikawa

W. Marques Jr.
V. Tumas (&)
Department of Neurosciences and Behavior Sciences,
Ribeirão Preto School of Medicine, University of São Paulo,
Campus Universitário Monte Alegre,
Ribeirão Preto, SP 14048-900, Brazil
e-mail: tumasv@rnp.fmrp.usp.br
Keywords Huntington’s disease
Executive function

Frontal assessment battery
Introduction
The frontal assessment battery (FAB) is a short cognitive
scale, designed to be easily administered at bedside, which
was proposed to be a valid and reliable tool to detect
dysexecutive syndrome in neurodegenerative diseases [10].
This original assumption is corroborated by some studies
that showed that the scale is able to distinguish
Alzheimer’s disease from frontotemporal and vascular
dementia [23, 26], although there are others that did not
find such a competence [21]. In patients with Parkinson’s
disease (PD), FAB scores were shown to be significantly
lower and highly correlated with measures of executive
functions [20].
Huntington’s disease (HD) is a neurodegenerative dis-
order characterized by motor, behavioral and cognitive
symptoms. In general, the cognitive impairment resembles
frontostriatal dysfunction [6], with the first abnormalities
appearing in pre-manifest carriers [34]. As the disease
develops, the neuropsychiatric abnormalities progress to
widespread domains, including memory loss, visuospatial
deficits, aggressive behavior, apathy, mood disturbances,
compulsiveness, and psychosis [5, 8].
The Unified Huntington Disease Rating Scale (UHDRS)
is the most used composite clinical scale for the evaluation
of patients with HD [14]. In order to assess cognitive
operations, it includes the phonetic Verbal Fluency Test
(VFT), the Symbol Digit Modalities Test (SDMT), and the
Stroop Interference Test (SIT) [14]. Apart from the
UHDRS, many studies that addressed the cognitive per-
formance of these patients have also widely used the Mini
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Mental State Examination (MMSE) [3, 9, 25]. To our
knowledge, there are no systematic studies using the FAB
to evaluate patients with HD.
The purpose of using a clinical scale is to estimate with
accuracy and reliability most of the symptoms of specific
diseases. A practical clinical scale must be able to analyze the
most prominent clinical features found in a disease, making
use of brief tests for each symptom. We hypothesize that the
FAB presents good validity in HD, as was demonstrated with
other neurodegenerative disorders [20, 21, 23], and has
become a practical tool to assess executive functions in
patients with HD. Thus the aim of this study was to verify the
validity of the FAB for the evaluation of cognitive impair-
ment in patients with HD. In order to do so we analyzed the
internal consistency, discriminative and concurrent validity
of the FAB in HD patients, as well as its value in assessing the
cognitive decay in HD patients in a 1 year period.
Methods
Patients selection
Patients (n = 41) diagnosed with HD, aged 18 years and
above and who had attended the Movement Disorders Out-
patient Clinic of the Ribeirão Preto School of Medicine
between 2003 and 2008 were included. They were routinely
evaluated with a validated Brazilian version of the UHDRS
including the VFT, SDMT, SIT [30], the MMSE and the
FAB. For comparison, 53 healthy controls matched by age,
sex and education were also selected. Criteria for inclusion as
controls were the absence of any past or present neurological,
psychiatric or metabolic disorders that are known to com-
promise cognition, no drug use, and having normal scores in
the MMSE as defined for the Brazilian population [7].
Demographic data of patients and controls are summarized
in Table 1.
The diagnosis of HD was made by DNA analysis after
genomic DNA was extracted from leukocytes by standard
procedures and the CAG repeat in the IT15 gene was
amplified by PCR [18, 29, 33]. A CAG repeat number [35
was considered diagnostic of HD.
The severity of HD is classified in Stages I–V, as defined
by the Functional Capacity Scale (FCS) (stage I includes
scores from 11 to 13; stage II: from 7 to 10; stage III: from
3 to 6; stage IV: 1 or 2; and stage V: score 0). This study
included seven patients in Stage I, seven in Stage II,
seventeen in Stage III and ten in Stage IV. There were no
patients in stage V. Longitudinal data were ascertained in
those patients (n = 18) who had been submitted to two
examinations, separated by a 1 year interval.
This study has been approved by the local ethics com-
mittee and has therefore been performed in accordance
with the Declaration of Helsinki.

Table 1 Demographic, clinical,

Patients Controls p
and laboratory characteristics of
HD patients and controls Number 41 53 –
Sex M/F 15/26 15/38 0.39#
Schoolinga 6.7 ± 4.5 (0–18) 7.5 ± 4.5 (0–19) 0.49

Age at test a
48.8 ± 13.6 (18–72) 48.25 ± 13.8 (18–72) 0.81
a
Age at onset 40.1 ± 13.1 (11–65) – –
CAG triplet numbera 46.6 ± 6.3 (37–66) – –
UHDRS motor scorea 51.6 ± 25.5 (9–104) – –
Functional Assessment Scalea 11.5 ± 7.4 (1–25) – –
Functional Capacity Scalea 5.5 ± 3.8 (1–13) – –
Independence Scalea 64.3 ± 22.7 (10–100) – –
FABa 6.5 ± 5.0 (0–16) 13.3 ± 3.3 (7–18) p \ 0.001
MMSEa 16.9 ± 8.3 (0–29) 25.8 ± 3.0 (18–30) p \ 0.001

a
VFT Verbal Fluency Test, VFT 8.2 ± 8.6 (0–37) – –
a
SDMT Symbol Digit Modalities SDMT 5.6 ± 1.4 (0–44) – –
Test, SIT Stroop Interference SITa 8.8 ± 10.1 (0–39) – –
Test, MMSE Mini-mental State
Examination, FAB Frontal HD Stageb Number of patients
Assessment Battery I 7 – –
# 2
 II 7 – –
v test; Mann–Whitney;
Student’s t test III 17 – –
a
Data shown in means, IV 10 – –
standard deviation, and range
V 0 – –
b
According to FCS

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Materials
The FAB is a six item scale designed to evaluate frontal
and subcortical deficits. FAB tasks include conceptualiza-
tion (Similarities test), mental flexibility (Lexical fluency),
motor programming (Luria’s ‘‘fist–edge–palm’’ test, con-
flicting instructions and Go–No go tests) and environ-
mental autonomy (Prehension behavior). The sequence of
subtests is: (1) Similarities test; (2) Lexical fluency; (3)
Luria’s sequence; (4) Conflicting instructions; (5) Go–No
go; (6) Prehension behavior. Each item is graduated from 0
to 3, and the best performance score is 18 [10]. The MMSE
comprise subtests of orientation, language, memory, visual
ability, attention and calculation, and is applied to assess
general cognitive function [11]. The Stroop Test (ST) is
used to measure attention and processing speed [28]. The
UHDRS version of the ST consists of 3 parts. In the first
part the patient received a small chart with several colored
boxes and read out the names of the colors displayed. In the
second part the patient read out the names of colors written
in black ink. In the third part, where the color names were
different to the color of the ink used, the patient had to read
out the color of the ink used and not the written word. For
each subtest the patient had 45 s. The SDMT is used to
evaluate attention through a visual tracking task. The
patient received a reference key containing numbers and
geometric figures and had 90 s to pair a sequence of spe-
cific numbers with given geometric figures [27]. In the
VFT the patient said as many words as possible whose first
letter is F, A or S, with 1 min to complete each subtest. The
total score was given by the sum of the number of words in
each subtest. The VFT is used to evaluate mental flexibility
[4].
This Brazilian version of the FAB was first indepen-
dently translated by two neurologists both fluent in
English; these translations were then compared, minor
inconsistencies resolved, and a final version was produced.
The final translation is similar to another version validated
in Brazil while our data were being collected [2].
Data analysis
Descriptive statistics were used in demographic data. The
Shapiro–Wilk test was used to assess the normality of
continuous variables. The Student’s t test was used to
compare means with a normal distribution and Mann–
Whitney or Wilcoxon tests were used for those with a
non-normal distribution. The ability of the scales to dis-
criminate patients in different stages of disease was
determined by ANOVA or Kruskal–Wallis tests; Tukey or
Schaich and Hamerle Post hoc tests were employed when
necessary. The best discrimination threshold between
patients and controls was assessed by analysis of a
1811
Receiver Operating Characteristic (ROC) Curve. General
agreement among ratings was assessed using Spearman’s
correlation coefficient. The Cronbach’s coefficient of alpha
was calculated for patients and controls, as a measure of
internal consistency of the FAB in our sample. Bonferroni
correction was performed in multiple-dependent measures
to keep the type-I error stable. Statistical analysis was
carried out with SPSS version 10.0 (SPSS, Inc., Chicago,
IL, USA).
Results
Influence of age and education
The performance on the FAB was worse in individuals
with fewer years of education. In our sample, age did not
influence the FAB scores. Data is summarized in Table 2.
Discriminative validity
FAB scores were significantly lower in patients with HD
(6.5 ± 5.0) than in controls (13.3 ± 3.3) (p \ 0.001). The
area under the ROC curve was 0.84 (p \ 0.05), and the
best cut-off score was 10/11, with a sensitivity of 75.6%
and specificity of 79.2% (Fig. 1). To verify whether the
FAB is useful to discriminate HD patients in the early
stages of the disease from controls, we analyzed data from
14 patients from stages I and II. Their mean score was
10.6 ± 4.5 (p = 0.055 vs. controls), not significant when
compared with controls but suggesting a trend toward a
discriminative power that might become statistically sig-
nificant if more patients had been evaluated.
Considering the stages of the disease (I–V), the FAB
was able to demonstrate a decline in the performance of
patients in the initial (levels I and II) and advanced stages
(III and IV) of the disease. This ability was observed to a
Table 2 Influence of age and education on FAB scores
Number of controls FAB score
Age (years)*
\45 21 14.0 ± 3.3
46–60 23 13.1 ± 3.4
[60 9 12.1 ± 2.7
Schooling (years)
\5 18 10.7 ± 2.9
5–9 18 13.6 ± 2.5
[9 17 15.7 ± 2.4
* p = 0.3 (ANOVA)

p \ 0.001 between groups ‘‘\5’’ and ‘‘[9’’ (ANOVA, Tukey’s
post hoc test)
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Table 4 Spearman’s correlation test among the motor section of the

UHDRS, and functional and cognitive assessment scales
FCS VF SDM SIT MMSE FAB

VFT 0.67
SDMT 0.67 0.82
SIT 0.64 0.81 0.79
MMSE 0.74 0.83 0.76 0.81
FAB 0.79 0.79 0.80 0.72 0.83
UHDRS-m -0.77 -0.76 -0.78 -0.73 -0.83 -0.8
All results: p \ 0.002, significant after Bonferroni’s correction
FCS Functional capacity scale, VF Verbal Fluency Test, SDMT
Symbol Digit Modalities Test, SIT Stroop Interference Test, MMSE
Mini-mental State Examination, FAB Frontal Assessment Battery,
UHDRS-m motor section of the Unified Huntington Disease Rating
Scale

Fig. 1 Receiver operating characteristic (ROC) curve discloses the

diagnostic accuracy of the FAB in assessing cognitive impairment in
HD patients (area under the ROC curve 0.84; p \ 0.05). Legend: x equation is (FCS = 1.07 ? 0.52FAB ? 0.063MMSE
axis = Sensitivity; y axis = 1 - Specificity (r2 = 0.62; p \ 0.001)).

lesser degree in the other scales studied—data are sum-
marized on Table 3.
The FAB did not present a significant ceiling effect
since no patients scored 17–18 in the initial stages of the
disease (I and II), and only 9/53 (17%) of the healthy
controls scored 17 or 18. However, there was a clear floor
effect at stage IV, where 8 out of 10 patients scored zero.
The worst floor effect was observed in the SDM test, with
9/10 patients at stage IV scoring 0.
Convergent validity and Regression analysis
The FAB scores presented significant and strong correlations
with the VFT, SDMT, SIT, MMSE, the motor section of the
UHDRS (UHDRS-m), and the FCS (Table 4). Furthermore,
a regression analysis model revealed that the FAB is a good
predictor of FCS scores (FCS = 1.602 ? 0.6034 FAB
(r2 = 0.60; p \ 0.001)) (Fig. 2). When the MMSE scores
are included in this model, the multiple linear regression
Internal consistency
The Cronbach’s coefficient of alpha between the 6 subtests
of the FAB for 41 patients was 0.83, and for 53 controls
was 0.76, suggesting a good internal consistency in this
sample.
Longitudinal assessment
The data from 18 patients were included in the longitudinal
analysis. The analysis of 1 year interval evaluations
revealed a global trend toward a worsening in the second
score of the FAB, however, only the MMSE and VFT
reached statistically significant values (Table 5).
FAB subtests
In the HD patients, the lowest score was reached in the
Lexical fluency subtest (mean = 0.615 ± 0.14), and the

Table 3 Summary of HD patients’ performance in different tests, classified by FCS-based severity stage
Stage p
I II III IV

FAB* 13.1 ± 2.8 8.1 ± 4.7 6.3 ± 2.9 1.2 ± 2.8 1,2,3 9 4; 1 9 2; 1 9 3 (p \ 0.05)
SDMT 17.2 ± 13.2 5.5 ± 8.2 3.2 ± 5 1.5 ± 4.7 1 9 3 (p \ 0,01); 1 9 4 (p \ 0.001)
VFT 17.2 ± 10.4 9.5 ± 9.4 8 ± 6.4 1.5 ± 3.2 1 9 4 (p \ 0.01)
MMSE 24.2 ± 3 21.7 ± 4.8 17.5 ± 5.1 7.5 ± 8.8 1 9 4 (p \ 0.01); 2 9 4 (p \ 0.05)

SIT 22.1 ± 12.2 7.2 ± 6.4 7.3 ± 7.2 3 ± 7.5 1 9 4 (p \ 0.01)
Data shown in means and standard deviation
*ANOVA, Tukey’s post hoc test

Kruskal Wallis, Schaich-Hamerle’s post hoc test

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those obtained from other scales intended to measure

executive functions, and also exhibited the best correlation
with measures of functional activities. Furthermore, from
the scales studied, the FAB presented the best performance
in discriminating different stages of the disease, although it
was not able to discriminate the cognitive decay within
1 year of follow-up. However, the longitudinal part of this
study enrolled a small number of patients and this negative
finding must be confirmed in larger groups of patients.
It is very important to evaluate the longitudinal decay in
scores of cognitive function to follow the progression of the
disease. In our study, the MMSE and VFT appeared to be the
most useful tests in this regard. However, other previous
longitudinal studies have found that the SDM is usually the
Fig. 2 Regression analyses model shows that the FAB score is a most sensitive instrument to follow up the cognitive decline
good predictor of the Functional Capacity Scale score in these patients and also in presymptomatic carriers [16, 19].
A possible explanation for the discrepancy of our findings in
this respect could be that most of our patients were in an
Table 5 One-year longitudinal evaluation of the motor section of the
UHDRS, Functional Capacity Scale and cognitive tests in HD patients advanced stage of the disease, where their performances in
the SDMT reached a floor effect.
Initial score Follow-up score p
The cognitive decline in patients with HD appears to
UHDRS-m 57.7 ± 25.3 62.9 ± 16.4 0.87
start before the clinical onset of the disease and is char-
FCS 4.31 ± 3.3 3.5 ± 2.6 0.076
acterized by memory loss and executive dysfunction
MMSE 14.79 ± 8.7 12.47 ± 8.48 0.003
[12, 15, 32, 34]. However, it is not clear whether the FAB
VFT 6.74 ± 7.2 4.37 ± 5.93 0.006 would be a good tool to differentiate patients in the initial
SDMT 4.32 ± 7.5 2.84 ± 6.04 0.19 stage of the disease from controls in our population.
SIT 8.84 ± 12.3 5.47 ± 9.16 0.10 Cognitive impairment has a negative impact on the
FAB 5.42 ± 4.59 4.5 ± 4.14 0.21
quality of life of patients with HD; the functional decline
may be associated more with cognitive decay than with some
Data shown in means and standard deviation

motor symptoms such as chorea and dystonia [13, 22, 24].
Wilcoxon’s test


Our regression model demonstrated that FAB scores are
Paired Student’s t test
good predictors of functional impairment in symptomatic
patients with HD, corroborating previous findings and sug-
highest in the Prehension behavior test (mean = 2.3 ± gesting that cognitive targeted therapeutics must be consis-
0.19). tently addressed in patients with HD.
The best correlation with FCS scores was found in the In our study the FAB and MMSE presented a good
Luria’s sequence subtest (r = 0.78, p \ 000.1 (Spearman’s correlation with functional abilities. However, the multiple
correlation test)), and the worst in the Similarities and linear regression model showed that there is no advantage
Prehension behavior subtests (r = 0.52, p = 0.001). In the in adding the MMSE to the FAB in the prediction of
longitudinal assessment arm, the Lexical fluency subtest functional impact in HD patients.
exhibited the highest decline during the 1 year interval Since the FAB is an instrument designed to assess
(mean 1 = 0.75; mean 2 = 0.31; p = 0.07 (Paired frontal-subcortical deficits which are the initial and most
Student’s t test)). The subtest that best differentiated HD prominent cognitive dysfunctions in patients with HD
patients from controls was the Luria’s sequence, with a [19, 34], and because the FAB presented the best correla-
sensitivity of 88.6% and specificity of 76.9% tion scores with FCS, we suggest using the FAB instead of
(AUC = 0.89 ± 0.04). the MMSE to evaluate cognitive impairment in HD
patients.
In the original description [10] the authors found that
Discussion FAB scores were not influenced by MMSE performance.
Furthermore, Appolonio et al. [1], in a normalization study,
We examined a group of HD patients and our results found that the MMSE was significantly correlated with
showed that the FAB is a useful tool for assessing their FAB raw scores, but this correlation disappeared following
executive abilities. FAB results were highly correlated with age and education adjustments. However, several studies

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using different populations [2, 17, 20, 21] have found a
correlation between the FAB and the MMSE. Our data
corroborate these studies and can be explained by the
partial overlap of FAB and MMSE constructs, as can be
demonstrated in some MMSE subtests such as Attention,
3-stage command, and Language, which also explore
frontal related functions. The high correlation between
FAB and UHDRS-m scores was expected since both are
measures influenced by disease progression.
The analysis of the FAB subtests provided interesting
data related to HD dementia. The Luria’s fist–edge–palm
test presented the best correlation with FCS scores, sug-
gesting that programming tasks have a deep impact on
functional abilities. The low scores obtained from the
Lexical fluency test suggest that HD patients prematurely
decreased in verbal fluency. Furthermore, this test also
showed a considerable decline after the 1 year evaluation.
This corroborates the idea that the VFT is a sensitive test to
be carried out as a screening test in preclinical or initial
stages of the disease [12]. As testing verbal fluency was not
the purpose of this study, we did not include asymptomatic
patients, and neither was the VFT performed on the control
group.
A potential flaw in this study is that depression is
more common in HD patients than in the general
population [31]. Thus it is possible that the prevalence of
depression among our patient group was higher than in
controls. Since depression may negatively affect cogni-
tive status it is possible that part of our data may be
biased. Future studies evaluating cognitive status of HD
patients should verify and control for the presence of
depression.
The FAB was shown to have good inter-rater reliability,
internal consistency and discriminative validity; it is an
easily administered scale that requires little training and
approximately 10 min to perform [10]. Our data corrobo-
rated the competence of the FAB in the assessment of
executive functions and proved its usefulness in the eval-
uation of cognitive impairment in patients with HD. Based
on its simple application, strong correlation with functional
performance, and good metric properties, we believe that
the FAB is a useful cognitive scale to be used in clinical
practice as well as in scientific research.
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