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Neuropsychological Profile in High Functioning Autism Spectrum Disorders

Article  in  Journal of Autism and Developmental Disorders · December 2012

DOI: 10.1007/s10803-012-1736-0

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J Autism Dev Disord
DOI 10.1007/s10803-012-1736-0
ORIGINAL PAPER
Neuropsychological Profile in High Functioning Autism Spectrum
Disorders
Antonio Narzisi • Filippo Muratori •
Sara Calderoni • Franco Fabbro • Cosimo Urgesi
Ó Springer Science+Business Media New York 2012
Abstract A comprehensive investigation of the neuro-
psychological strengths and weaknesses of children with
autism may help to better describe their cognitive abilities
and to design appropriate interventions. To this end we
compared the NEPSY-II profiles of 22 children with high-
functioning autism spectrum disorders (HFASD) with
those of 44 healthy control (HC) children 2:1 matched by
gender, age, race and education. Results showed that only
Visuospatial Processing was relatively spared in HFASD,
while deficits were observed in Attention and Executive
Functions, Language, Learning and Memory, and Senso-
rimotor Processing. Theory of Mind difficulties were
observed in verbal tasks but not in the understanding of
emotional contexts, suggesting that appropriate contextual
cues might help emotion understanding in HFASD chil-
dren. These widespread neuropsychological impairments
Electronic supplementary material The online version of this
article (doi:10.1007/s10803-012-1736-0) contains supplementary
material, which is available to authorized users.
A. Narzisi
F. Muratori
S. Calderoni
Division of Child Neurology and Psychiatry, Stella Maris
Scientific Institute, University of Pisa, Via dei Giacinti, 2,
56018 Calambrone, Pisa, Italy
A. Narzisi (&)
Division of Child Neurology and Psychiatry, Stella Maris
Scientific Institute, University of Pisa, Viale del Tirreno, 331,
56018 Calambrone, Pisa, Italy
e-mail: antonio.narzisi@inpe.unipi.it
F. Fabbro
C. Urgesi (&)
Department of Human Sciences, University of Udine
and Eugenio Medea Scientific Institute, Via Margreth, 3,
33100 Udine, Italy
e-mail: cosimo.urgesi@uniud.it
reflect alterations in multiple cognitive domains in
HFASD.
Keywords Autism spectrum disorders
NEPSY

Neuropsychology
Theory of Mind
Introduction
Individuals with autism spectrum disorders (ASD) exhibit
core impairments in the socio-communication domain and
have restricted or stereotyped patterns of behaviors. How-
ever ASD vary greatly in the severity of their socio-
communicative impairments (Lord 2011) and in cognitive
and language development. ASD with normal or high IQ
are referred to as high functioning autism (HFA) or high
functioning autism spectrum disorders (HFASD). This
latter term is often used to characterize the broader group
of children with HFA, Asperger syndrome and Pervasive
Developmental Disorders Not Otherwise Specified
(PDDNOS) (Volker et al. 2010; Oliveras-Rentas et al.
2012; Scheeren et al. 2012). These individuals demonstrate
relative strengths in cognitive and language abilities (Klin
et al. 2000), though pragmatic language deficits are
observed (Landa 2000). Thus, they share the two core
features of ASD: social-communication impairment and
circumscribed pattern of behavior and interest, which,
together, contribute to their poor overall social competence
(Attwood 2004).
Several studies have shown that children with HFASD
are also impaired in different other cognitive functions
(Tsatsanis 2005), including deficits in motor functions
(Baranek et al. 2005), high-order language aspects, such as
semantics and pragmatics (Minshew et al. 1995; Tager-
Flusberg 2004), selected executive functions (Liss et al.
123

2001; Ozonoff et al. 2004; Ozonoff and Jensen 1999),
memory for faces (Hauck et al. 1998). On the other hand,
there is a growing literature pointing to a relative sparing of
visual processing (Samson et al. 2012; Joseph et al. 2009;
Ghaziuddin and Mountain-Kimchi 2004), although this is
an area of debate (see Edgin and Pennington 2005),
selected aspects of memory (Minshew and Goldstein
2001), and inhibitory functions (Ozonoff and Strayer
1997). These findings have contributed to a better knowl-
edge of the cognitive characteristics of HFASD; however,
the conclusions that can be drawn from these studies are
limited because they used different tests to measure single
abilities or a subset of cognitive abilities in different
samples of HFASD and control individuals. This makes it
impossible to clarify the profile and the relative strengths
and weaknesses in the same sample, thus introducing an
unknown and uncontrollable amount of variability to the
findings.
The developmental neuropsychological battery NEPSY
(Korkman et al. 1998; 2007) was developed following the
neuropsychological approach of Luria (1962), who con-
siders multiple brain systems at the base of cognitive
functioning, and provides a comprehensive overview of the
child’s neuropsychological functioning. The NEPSY is a
unique, co-normed, and multi-domain neuropsychological
battery designed specifically for preschoolers, children, and
adolescents. Its revised version, the NEPSY-II (Korkman
et al. 2007), includes measures of social perception abili-
ties, thus allowing also the evaluation of the core deficits of
ASD children in comparison to their functioning in other
cognitive domains. Until now, validation data of the
NEPSY (Korkman et al. 1998; Hooper et al. 2006) and
NEPSY-II (Korkman et al. 2007) batteries have provided a
comprehensive evaluation of the neuropsychological
functions of HFASD children, and a few independent
studies have used a selection of subtests of the NEPSY to
evaluate specific cognitive functions (Boneh et al. 2008;
Davidson et al. 2008; Joseph et al. 2005; Miniscalco et al.
2007; Planche and Lemonnier 2011).
The validation study of the first version of the NEPSY
(Korkman et al. 1998) reported that HFASD children pre-
sented lower scores than control individuals in the tests of
executive functions (Tower), memory (Memory for Faces,
Narrative Memory), language comprehension (Compre-
hension of Instructions), sensorimotor functions (Imitating
Hand Positions, and Visuomotor Precision), and line ori-
entation discrimination (Arrows). A reanalysis of these
data, however, controlling for the influence of IQ (Hooper
et al. 2006), showed further impairments of HFASD chil-
dren in the subtests of Phonological Processing, Auditory
Attention and Response Set, and Speeded Naming. In
contrast, the differences between HFASD and control
children in the Comprehension of Instructions and
123
J Autism Dev Disord
Narrative Memory were no longer significant after con-
trolling for IQ.
Similar results were obtained in the validation study of
the NEPSY-II (Korkman et al. 2007). Indeed, HFASD
children presented lower scores in all cognitive domains,
but particularly severe difficulties in the measures of
executive functions (sorting, cognitive flexibility, and
auditory attention) and language. Moreover, verbal and
visuospatial memory, visuomotor coordination, line orien-
tation discrimination and block constructions abilities were
also impaired in HFASD children. Since the latter tests
heavily rely on verbal abilities to understand the task
instructions, it is likely that the deficits of HFASD children
stem from a lack of language mediation. These findings
suggest that the NEPSY can contribute to the neuropsy-
chological description of children with HFASD.
However, previous studies did not control for the extent
to which performance of HFASD children in a cognitive
domain may be affected by weaknesses in other but in-
tercorrelated domains independently from general cogni-
tive abilities. The US (Korkman et al. 2007) and Italian
(Urgesi et al. 2011) standardization of the NEPSY-II
showed large inter-correlations between the NEPSY-II
tests both within (in particular for Language tests) and
between domains (in particular for Language and Attention
and Executive Function tests). These between-domain
inter-correlations in healthy individuals were probably
caused by the similarity between the required modality of
stimulus presentation and response and by general effects
of attention resources on task performance. These effects
may suggest the possibility that an impairment in a cog-
nitive domain, for example language, may induce second-
ary impairment in other tasks, for example executive
functions, that involve language mediation to perceive the
stimuli or to provide the response (Russel 1997; Russell
et al. 1999; Joseph et al. 2005).
Here we aimed to provide a comprehensive description
of the neuropsychological profile of children with HFASD
using the NEPSY-II and to explore the possible relations
between the impairments in different cognitive domains. In
keeping with previous studies (Korkman et al. 1998, 2007;
Hooper et al. 2006), we expected to find impaired perfor-
mance by HFASD children in multiple cognitive domains,
with particular deficits in attention and executive functions
and in language and social perception abilities. Impor-
tantly, taking into account the mutual influence between
executive and language impairments, we tested whether or
not the executive deficits in HFASD are secondary to pri-
mary deficits in the ability to use inner speech to control
and guide behaviors (Hughes and Russell 1993). Indeed, in
keeping with the so called language mediation hypothesis
(Russel 1997), the deficits of HFASD children in executive
functions should not be consistent when the relative impact
J Autism Dev Disord
of deficits in language abilities is partialled out. In a similar
vein, we expected to find that, when the influence of
impairments in the attention and executive functions and in
language abilities is controlled for, the impairments in
visuospatial abilities should be attenuated.
Methods
Participants
Twenty-two boys with a diagnosis of HFASD were con-
secutively recruited at the Division of Child Neurology and
Psychiatry of the Stella Maris Scientific Institute (Univer-
sity of Pisa) over a 24 months period. Inclusion criteria
were: (a) diagnosis of Autistic Disorder (AD) or Pervasive
Developmental Disorder Not Otherwise Specified (PDD-
NOS) according to DSM-IV-TR. The diagnosis was certi-
fied by a senior child psychiatrist (F.M.) with more than
25 years of experience in assessing individuals with Aut-
ism; (b) age between 5 and 16 years; (c) a Full Scale
Intelligence Quotient (FSIQ), evaluated through WISC-III,
greater than 80; (d) availability for carrying on several
neuropsychological evaluation sessions. Exclusion criteria
were presence or history of any other axis I mental disor-
der; a history of traumatic brain injury or any other neu-
rological illness. The boys who composed the final sample
had a diagnosis of AD (n = 10) or PDDNOS (n = 12).
The mean age of patients was 9.77 (SD: 3.65; range:
5–16); mean VIQ was 96.14 (SD: 13.73; range: 80–131);
mean PIQ was 103.36 (SD: 16.28; range: 72–141); mean
Total IQ: 99.09 (SD: 14.23; range: 82–126). Four patients
were left-handed.
The neuropsychological performance of HFASD was
compared to that of a group of 44 healthy control (HC)
children matched 2:1 for age, gender, race, and education.
HC children were selected randomly from the standardiza-
tion sample of the Italian Version of NEPSY-II (Urgesi et al.
2011). Two controls were sampled for each HFASD patient
to improve the efficiency of the comparison of the control
sample and the estimation of the expected performance at
each age (Haviland et al. 2007). Five controls were left-
handed. Before inclusion into the standardization sample,
controls were screened with standardized neuropsychologi-
cal tests of verbal (Marini et al. 2007) and non-verbal general
cognitive abilities (Goodenough 1975; Raven 1954) in order
to exclude deficits of verbal or visuospatial abilities. All
HFASD and HC children were native Italian speakers.
Neuropsychological Evaluation
HFASD and HC underwent a full neuropsychological
evaluation (conducted by A.N. and C.U.) using the Italian
Version of NEPSY-II (Korkman et al. 2007; Urgesi et al.
2011). NEPSY-II is a battery of neuropsychological tests
specifically developed for the evaluation of 3–16 years old
children in 6 cognitive domains: Attention and Executive
Functions, Language, Memory and Learning, Sensorimotor
Functions, Social Perception, and Visuospatial Processing.
Here we used the 31 tests of the Italian version of NEPSY-
II (Urgesi et al. 2011) that can be administered to children
aged 5–16 years.
Attention and Executive Functions
Six tests are comprised in this domain. The Visual Attention
assesses visual scanning and selective visual attention abil-
ities. The participant is required to scan an A3 sheet and mark
specified visual targets among several similar distracters
(face drawings). The Design Fluency test accesses behav-
ioral productivity. The participant is required to generate
unique designs by connecting up to five dots presented in a
structured or random array. In the Auditory Attention and
Response Set test the participant listens to a series of words
and points to the appropriate circle when he hears a target
word. Auditory Attention is aimed at evaluating selective
and sustained auditory attention and requires to point to one
circle when one color name is heard, while ignoring other
color names. Response Set is aimed at assessing the ability to
shift and maintain a new and complex response set involving
inhibition of automatic response and alternating between
matching or contrasting stimuli. Inhibition evaluates the
ability to inhibit automatic responses and shift between
congruent or incongruent responses during naming of visual
stimuli. The Naming condition requires to name the shape of
squares and circles or the up or down direction of arrows; the
Inhibition condition requires to provide the opposite naming
response on the same stimuli; the Switching condition
requires to provide the congruent or the incongruent naming
response according to the color of the stimulus. The Clocks
test evaluates planning and organization of visuospatial
perception and response and the concept of time by requiring
to read or draw the times on analogical or digital clocks.
Animal Sorting requires to sort cards into two groups of four
cards each using various self-initiated sorting criteria.
Language
The Language domain includes six tests. The Compre-
hension of Instruction evaluates non-contextual language
and asks to indicate a given sequence of crosses and circles
of different colors in response to oral instructions of
increasing syntactic complexity. In the Speeded Naming
test, the participant is required to name as quickly as
possible a series of letters and numbers, thus allowing to
evaluate the velocity of lexical retrieval and production.
123

The Phonological Processing test assesses phonemic
awareness with tasks requiring the phonological segmen-
tation at the level of syllables and phonemes (substitute a
syllable or a phoneme in a given word to create a new
word). The Word Generation test requires the participant to
produce, within 1 min for each category, as many words as
possible on the basis of a semantic or phonological crite-
rion. The Repetition of Non-sense Words test assesses
phonological encoding and decoding by requiring partici-
pants to repeat nonsense words presented aloud. The
Oromotor Sequences test assesses oromotor coordination
and requires to repeat onomatopoeic sounds and tongue
twisters for five times.
Memory and Learning
Seven NEPSY-II tests evaluate verbal and visuospatial
memory abilities. Four tests have both immediate and
delayed recall conditions to evaluate short- and long-term
memory retrieval. The Memory for Faces test assesses face
discrimination and recognition. The participant looks at a
series of faces in an incidental learning procedure and then,
in immediate and delayed recall conditions, is shown three
photographs at a time from which he selects a face previ-
ously seen. Word List Interference assesses verbal working
memory, repetition, and word recall following interference.
The participant is presented with two series of words and
asked to repeat each sequence following its presentation.
Then, he recalls each series in the order of presentation.
The Memory for Designs test assesses memory for the form
and spatial location of novel visual material. The partici-
pant is shown a series of non-figural abstract shapes placed
on a grid which are, thereafter, removed from view. In
immediate or delayed recall conditions after each presen-
tation, he is required to select the appropriate shapes from a
set of cards and place them onto an empty grid in the
positions in which he had seen them in the sample stimu-
lus. List Memory assesses long term verbal memory for
lists of words. The participant is read a list of words several
times and is asked to recall them. The Memory for Names
test assesses visuo-verbal association learning and requires
participants to learn the name of children drawn on eight
cards. Narrative Memory assesses story recall abilities. The
participant listens to a story and is asked to repeat it. He is
then asked questions to elicit missing details from his or
her recall of the story. In the Sentence Repetition test,
participants are asked to repeat sentences of increasing
complexity and lenghth.
Sensorimotor Functions
Three tests evaluate motor planning and execution abilities
and sensorimotor coordination. The Finger Tapping test
123
J Autism Dev Disord
evaluates motor dexterity in repetitive finger movements and
motor programming in sequences of finger movements.
Imitation of Hand Postures evaluates ideomotor praxis
abilities and requires to imitate, with the dominant and non
dominant hand, various meaningful and meaningless hand/
finger positions demonstrated by the examiner. The Manual
Motor Sequences test assesses manual motor coordination by
requiring to imitate for five times a series of rhythmic hand
movement sequences demonstrated by the examiner.
Social Perception
Two NEPSY-II tests, one with two separate parts, evaluate
social perception. In the verbal items of the Theory of Mind test,
participants are provided with verbal or pictorial descriptions of
some social situations and are, then, asked questions about
those situations which require the understanding of the char-
acters’ point of view. The contextual items of the Theory of
Mind test evaluate the capacity to understand how certain
emotions are linked to given social situations and to recognise
correctly the emotions that the various social settings generate.
Affect Recognition assesses the ability to recognise emotional
expressions (happy, sad, anger, fear, disgust, and neutral) from
photographs of children’s faces in four different tasks. Partic-
ipants need to match the two faces expressing the same emo-
tions among three or more alternatives.
Visuospatial Processing
Six NEPSY-II tests are comprised. Design Copying
assesses constructional abilities asking to copy two-
dimensional geometric figures of increasing complexity.
Block Construction assesses the visuospatial and visuo-
motor ability to reproduce three-dimensional constructions
from two-dimensional drawings. Picture Puzzles assess
visual discrimination and the perception of part-whole
relationships. The participant identifies on a large picture
the sections from which each of four small pictures, pre-
sented separately, were taken. The Geometric Puzzles test
assesses the ability to identify and match geometric shapes
of increasing complexity (design recognition) as well as
mental rotation abilities. The Route Finding test assesses
the knowledge of visual-spatial relations and directionality
and the ability to transfer a route from a simple schematic
map to a more complex one. The Arrows test assesses the
ability to judge line orientation. The participant looks at an
array of arrows arranged around a target and indicates the
arrows that points to the centre of the target.
Data Analysis
The scores obtained by each HFASD and HC participant at
each NEPSY-II test were expressed as scaled scores (mean:
J Autism Dev Disord
10; SD: 3) with respect to the normative values for the cor-
responding chronological ages (Urgesi et al. 2011), thus
improving the approximation of the data to the normal dis-
tribution. It is worth noting that scaled scores were derived
from the normative values with no approximation at the low
or high extremes, thus including also negative numbers for
performance lower than -3.33 SD from the mean. The
scaled scores were entered into series of Multivariate Anal-
yses of Covariance (MANCOVAs) with group as between-
subject factor and MAEVIQ as covariate of interest. The
mental age was estimated from the VIQ for HFASD with the
standard formula (MAEVIQ = VIQ 9 CA/100 and MAE-
PIQ = PIQ 9 CA/100). Although all patients presented a
Full Scale IQ [ 80, they presented different levels of VIQ,
ranging from borderline to above mean levels; to ensure the
effect of verbal cognitive abilities was ruled out from the
between-group comparison, we entered the MAEVIQ as
covariate of interest. Since we did not have a VIQ measure
for HC, we inserted in the MANCOVA their chronological
age, assuming a correspondence between their mental and
chronological ages. The chronological age of controls
(mean: 9.77 years; SD: 3.61) was not significantly different
from the equivalent mental age of HFASD patients for both
VIQ (MAEVIQ; mean: 9.95 years; SD: 4.24; t64 = -0.245;
p = 0.807) and PIQ (MAEPIQ; mean: 10.65 years; SD:
4.28; t64 = 0.448; p = 0.655).
Separate domain-specific MANCOVAs or ANCOVAs
were used for the NEPSY-II tests that were administered to
all 5–16 years old children or only to 7–16 or 5–12 years old
children. Post-hoc univariate ANCOVAs were performed
for each test to explore how they contributed to significant
between-group differences in each cognitive domain. In
keeping with the within-domain correlations of the US
(Korkman et al. 2007) and Italian (Urgesi et al. 2011) nor-
mative studies, all the variables entered in each MANCOVA
were only moderately correlated (0.14 \ r \ 0.67).
Normative studies showed higher levels of correlation
between the tests that are comprised in the same cognitive
domain when compared with those comprised in different
cognitive domains (Korkman et al. 2007; Urgesi et al.
2011). Furthermore, moderate levels of correlations were
also observed between some tests of the Attention and
Executive Functions and Language domains and the tests
of other domain. To analyze how HFASD children differed
from HC in each cognitive domain independently from
their performance in other cognitive domains, six domain
scores were obtained by averaging individual scaled scores
for all the tests comprised in each cognitive domain. All the
domain scores were moderately correlated with each other
(0.34 \ r \ 0.6). The scores of the tests comprised in each
cognitive domain were entered into separate MANCOVAs
with group as between-subject factor and the domain
scores of other cognitive domains as covariates of interest.
A significance threshold of p \ 0.001 was set for all
parametric and non parametric statistical tests, applying a
Bonferroni correction to control for false discoveries in
multiple comparisons (36 neuropsychological variables).
Results
(1). HFASD versus HC
Table 1 shows mean and standard deviation (SD) of the
scores obtained by HFASD and HC and the ANCOVAs
results controlling for MAEVIQ.
Attention and Executive Functions
HFASD children had an overall lower performance
(F5,59 = 11.13; p \ 0.001; gp2 = 0.485); the effect of
MAEVIQ was marginally significant but did not reach the
Bonferroni corrected threshold (F5,59 = 3.34; p = 0.01;
gp2 = 0.221). A similar pattern of results was obtained for
the tests that were administered only to children aged
7–16 years, with a significant effect of group (F4,48 = 13.2;
p \ 0.001; gp2 = 0.524) and no significant effect of
MAEVIQ (F4,48 \ 1, gp2 = 0.051). Post-hoc univariate
ANCOVAs revealed that HFASD children, independently
from MAEVIQ, had significant lower performance for all the
subtests except for Visual Attention and Design Fluency.
Language
The MANCOVA revealed a significantly lower perfor-
mance of HFASD as compared to HC (F6,56 = 4.87;
p \ 0.001; gp2 = 0.343), with non-significant effect of
MAEVIQ (F6,56 = 1.85; p = 0.105; gp2 = 0.166). Post-
hoc univariate analyses showed that HFASD children
tended to show lower performance than controls in all tests
but the difference was significant only in the Oromotor
Sequences test. No between group difference (F1,49 =
3.04, p = 0.087, gp2 = 0.058) and no effect of MAEVIQ
(F1,49 \ 1, gp2 \ 0.001) was observed for the Phonologi-
cal Word Generation test, which was administered only to
7–16 years old children.
Memory and Learning
The MANCOVA revealed significant lower performance
of HFASD compared to HC (F5,59 = 11.08; p \ 0.001;
gp2 = 0.484), with non-significant effect of MAEVIQ
(F5,59 = 2.28; p = 0.03; gp2 = 0.27). Univariate ANCO-
VAs showed that the MAEVIQ had a significant effect on
Memory for Designs (F1,63 = 18.51; p \ 0.001), which is
likely due to the influence of verbal intelligence on the
123
 J Autism Dev Disord

Table 1 Mean (SD) NEPSY-II scaled scores (mean: 10; SD: 3) of HFASD and HC and comparison with the univariate ANCOVAs, controlling
for verbal mental age equivalent, and the non parametric Mann–Whitney U test
NEPSY-II test, variable Age HFASD HC HFASD vs. HC
ANCOVA Mann–Whitney U test

Attention and executive functions

Visual attention 5–16 9.47 (5.59) 9.96 (3.55) F1,63 = 0.32, p = 0.572 z = -0.22, p = 0.822
Design fluency 5–16 7.81 (4.31) 9.94 (3.11) F1,63 = 6.04, p = 0.017 z = -2.39, p = 0.017
Auditory attention 5–16 -0.8 (16.35) 10.41 (2.17) F1,63 = 20.04, p \ 0.001* z = -4.72, p \ 0.001*
Auditory response set 7–16 3.33 (10.43) 9.78 (2.54) F1,51 = 12.19, p = 0.001* z = -2.5, p = 0.013
Inhibition, naming 5–16 3.1 (7.49) 9.3 (1.85) F1,63 = 27.94, p \ 0.001* z = -4.23, p \ 0.001*
Inhibition, inhibition 5–16 1.82 (10.88) 9.64 (2.34) F1,63 = 22.63, p \ 0.001* z = -2.88, p = 0.004
Inhibition, switching 7–16 4.99 (5.78) 10.29 (3.24) F1,51 = 19.27, p \ 0.001* z = -3.32, p = 0.001*
Clocks 7–16 -4.84 (18.84) 10.23 (2.35) F1,51 = 22.62, p \ 0.001* z = -4.24, p \ 0.001*
Animal sorting 7–16 5.18 (3.67) 9.68 (2.75) F1,51 = 26.21, p \ 0.001* z = -4.17, p \ 0.001*
Language
Comprehension of instructions 5–16 7.22 (3.56) 9.12 (3.08) F1,63 = 4.35, p = 0.041 z = -2.16, p = 0.031
Speeded naming 5–16 1.27 (29.67) 10.2 (2.95) F1,63 = 4.05, p = 0.049 z = -0.61, p = 0.545
Phonological processing 5–16 6.36 (6.86) 9.9 (2.5) F1,63 = 9.25, p = 0.003 z = -2.78, p = 0.005
Word generation, semantic 5–16 7.71 (4.78) 9.97 (4.32) F1,63 = 3.91, p = 0.053 z = -2.93, p = 0.003
Word generation, phonological 7–16 7.66 (3.92) 9.48 (3.32) F1,51 = 3.04, p = 0.087 z = -2.1, p = 0.036
Repetition of non-sense words 5–16 6.21 (8.32) 10.24 (2.18) F1,63 = 8.71, p = 0.004 z = -1.5, p = 0.133
Oromotor sequences 5–16 3.17 (7.36) 9.96 (2.76) F1,63 = 27.88, p \ 0.001* z = -3.63, p \ 0.001*
Memory and learning
Memory for faces 5–16 7.58 (3.29) 10.33 (2.64) F1,63 = 13.31, p \ 0.001* z = -3.24, p = 0.001*
Word list interference 7–16 3.32 (5.85) 9.96 (2.73) F1,51 = 35.43, p \ 0.001* z = -4.06, p \ 0.001*
Memory for designs 5–16 5.16 (7.48) 9.85 (2.31) F1,63 = 17.6, p \ 0.001* z = -2.67, p = 0.008
List memory 7–16 1.30 (4.48) 9.57 (2.31) F1,51 = 82.66, p \ 0.001* z = -5.43, p \ 0.001*
Memory for names 5–16 7.95 (4.02) 10.02 (2.56) F1,63 = 6.59, p = 0.013 z = -1.9, p = 0.057
Narrative memory 5–16 4.95 (5.97) 10.03 (2.74) F1,63 = 22.17, p \ 0.001* z = -3.91, p \ 0.001*
Sentence repetition 5–16 4.09 (6.81) 10.59 (3.29) F1,63 = 27.19, p \ 0.001* z = -4.77, p \ 0.001*
Sensorimotor functions
Finger tapping 5–16 6.94 (8.65) 10.54 (2.04) F1,63 = 7.13, p = 0.01 z = -1.46, p = 0.142
Imitation of hand postures 5–16 -1.05 (9.14) 8.53 (8.74) F1,63 = 17.67, p \ 0.001* z = -5, p \ 0.001*
Visuomotor precision 5–12 11.62 (1.81) 10.73 (2.23) F1,42 = 1.49, p = 0.229 z = 1.97, p = 0.048
Manual motor sequences 5–16 3.17 (6.16) 9.31 (2.68) F1,63 = 32.65, p \ 0.001* z = -4.37, p \ 0.001*
Social perception
Theory of Mind, verbal 5–16 4.02 (6.15) 9.43 (3.08) F1,63 = 22.55, p \ 0.001* z = -3.67, p \ 0.001*
Theory of Mind, contextual 5–16 9.83 (3.31) 10.57 (3.09) F1,63 = 0.81, p = 0.373 z = -1.05, p = 0.292
Affect recognition 5–16 6.54 (6.76) 10.05 (2.87) F1,63 = 8.99, p = 0.004 z = -3.03, p = 0.002
Visuospatial processing
Design copying 5–16 5.33 (8.57) 11 (2.55) F1,63 = 16.42, p \ 0.001* z = -3.8, p \ 0.001*
Block construction 5–16 9.86 (4.65) 9.67 (2.63) F1,63 = 0.08, p = 0.784 z = 0.13, p = 0.897
Picture puzzles 7–16 9.16 (3.95) 9.96 (2.71) F1,51 = 0.77, p = 0.384 z = -0.49, p = 0.627
Geometric puzzles 5–16 7.83 (4.75) 10.15 (3.14) F1,63 = 5.55, p = 0.022 z = -1.86, p = 0.062
Route finding 5–16 9.48 (4.03) 10.06 (2.75) F1,63 = 0.47, p = 0.494 z = -0.69, p = 0.492
Arrows 5–16 6.11 (4.39) 10.61 (2.41) F1,63 = 28.68, p \ 0.001* z = -4.26, p \ 0.001*
* Significance threshold

ability to understand the task instructions which are par- HC in the Memory for Designs test. Significant differences
ticularly complex for this test of visuospatial memory. between the two groups, with no MAEVIQ effect were also
HFASD children had significantly lower performance than obtained for Narrative Memory, Sentence Repetition, and

123
J Autism Dev Disord
Memory for Faces tests. The analysis of the Word List
Interference and List Memory tests, that were administered
only to 7–16 years old children, showed lower perfor-
mance of HFASD (F2,50 = 46.84; p \ 0.001; gp2 =
0.652) at both the Word List Interference and List Memory
tests, with non-significant effect of MAEVIQ (F2,50 =
5.84; p = 0.005; gp2 = 0.189).
Sensorimotor Functions
The MANCOVA showed significant lower performance of
HFASD (F3,61 = 14.93; p \ 0.001; gp2 = 0.423) with
non-significant effect of MAEVIQ (F3,61 = 1.21; p =
0.312; gp2 = 0.056). Post-hoc univariate analyses showed
that HFASD children had lower performance than HC in
Imitation of Hand Postures and Manual Motor Sequences,
but not in Finger Tapping. No effects were also observed
between HFASD and 5–12 years old control children
for the Visuomotor Precision test (group: F1,42 = 1.49,
p = 0.229, gp2 = 0.034; MAEVIQ: F1,42 \ 1, gp2 =
0.023).
Social Perception
The MANCOVA showed significantly lower performance
of HFASD children (F3,61 = 8.25; p \ 0.001; gp2 =
0.289), with no effect of MAEVIQ (F3,61 \ 1; gp2 =
0.044). The effect of group was due to the scores in the
verbal items of the Theory of Mind test, while no differ-
ence was observed for the Theory of Mind contextual items
and for the Affect Recognition test.
Visuospatial Processing
The MANCOVA showed lower performance in HFASD
(F5,59 = 9.04; p \ 0.001; gp2 = 0.434), with non-signifi-
cant effect of MAEVIQ (F5,59 = 1.28; p = 0.285;
gp2 = 0.098). Post-hoc univariate analyses showed that
HFASD children had lower performance than HC in
Design Copying and Arrows. On the Design Copying test,
the deficits of HFASD children were apparent in the Motor
(F1,63 = 27.3; p \ 0.001; gp2 = 0.302) and Global Pro-
cessing (F1,63 = 19.23; p \ 0.001; gp2 = 0.234) while no
difference was observed for the Local Processing
(F1,63 = 5.03; p = 0.028; gp2 = 0.074). Non-significant
effects of group (F1,51 \ 1, gp2 = 0.015) and MAEVIQ
(F1,51 \ 1, gp2 \ 0.001) were obtained for the Picture
Puzzles test in 7–16 years old children.
Non-Parametric Tests
Inspection of the data shown in Table 1 reveals that the
HFASD had lower mean scores and higher SD in most tests
in which they had a significantly impaired performance. This
suggests that, while all HC individuals had scores close to the
group mean, which corresponded to the norm-based expec-
ted value (Urgesi et al. 2011), HFASD individuals had more
variable performance, with some individuals showing more
impairments than others. Importantly, more extreme
impairments were obtained by different HFASD children in
different tests, reflecting individual weakness rather than
overall outlier performance. To ensure that our results are not
influenced by violations of the homogeneity of variance
assumption, we conducted a supplementary nonparametric
analysis, comparing the performance of HFASD and HC
children on each test with the Mann–Whitney U Test. As
shown in Table 1, the nonparametric comparisons confirmed
a significantly impaired performance of HFASD children on
most tests that show significant between-group effects in the
corresponding ANCOVA. The only discrepant results con-
cerned the Auditory Response Set, Inhibition, and Memory
for Designs tests, in which the nonparametric analysis
detected only a non-significant trend toward impaired per-
formance of HFASD children, probably because of reduced
power. Furthermore, no differences were observed between
the NEPSY-II performance of children with a DSM-IV-TR
diagnosis of AD vs. PDDNOS (-1.7 \ all zs \ 1.7,
p [ 0.8), with the exception of a non significantly worse
NEPSY-II performance of children with PDDNOS on the
verbal items of the Theory of Mind test (AD: 7.16 ± 4.33 vs.
PDDNOS: 1.39 ± 6.36; z = 2.31, p = 0.021).
(2). Specificity of each neuropsychological domain in
HFASD children
The MANCOVA on the Attention and Executive
Functions domain entering the other domains as covariates
of interest showed that the difference between HFASD and
HC was still significant after removing the variance in
common with the other cognitive domains (F5,55 = 5.02,
p \ 0.001, gp2 = 0.313). The effects of the other cogni-
tive domains were non-significant (all Fs5,55 \ 4.5,
p [ 0.002, gp2 \ 0.29). This suggests that the deficits of
HFASD children in attention and executive functions were
independent from other neuropsychological domains. In
contrast, no effects of group (F4,44 = 2.65, p = 0.046,
gp2 = 0.194) and of the other neuropsychological domains
(all Fs4,44 \ 3.8, p [ 0.01, gp2 \ 0.26) were obtained for
the Attention and Executive Functions tests that were
administered only to 7–16 years old children.
The MANCOVA on Language domain showed signifi-
cant effects of Attention and Executive Functions
(F6,52 = 5.59, p \ 0.001, gp2 = 0.392) and of Memory
and Learning (F6,52 = 8.57, p \ 0.001, gp2 = 0.497),
with no effects of the other domains and no between-group
difference (all Fs6,52 \ 3.9, p [ 0.003, gp2 \ 0.31). Thus,
the impairments of HFASD children in language abilities
123

were not consistent after removing the variance in common
with the other cognitive domains and in particular with
Attention and Executive Functions and Memory and
Learning abilities, which strongly affected performance in
language tasks.
The MANCOVA on Memory and Learning domain
showed a significant effect of Attention and Executive
Functions (F9,51 = 5.14, p \ 0.001, gp2 = 0.476), while
the effects of the other cognitive domains and of group were
non-significant (all Fs9,51 \ 2.4, p [ 0.025, gp2 \ 0.3). In
contrast, when the scores in the Word List Interference and
List Memory tests were analyzed, the effect of group resulted
significant (F4,44 = 5.71, p \ 0.001, gp2 = 0.342), with
non-significant effects of the other cognitive domains (all
Fs4,44 \ 2, p [ 0.12, gp2 \ 0.15). Thus, HFASD impair-
ments in memory for lists of words were consistent even after
removing the portion of variance in common with other
neuropsychological functions.
The MANCOVA on Sensorimotor Functions domain
showed only a significant effect of Language (F3,57 =
6.25, p \ 0.001, gp2 = 0.248), while the effect of group
and of the other cognitive domains were not significant (all
Fs3,57 \ 2.8, p [ 0.049, gp2 \ 0.13). That the HFASD
impairments in Sensorimotor Functions were not signifi-
cant after removing the variance in common with Lan-
guage tests was likely due to the fact that many language
tasks require motor planning and monitoring abilities
similar to those required by the sensorimotor tasks. In
particular, the analysis of the intercorrelations between the
NEPSY-II tests revealed highly significant correlation
between the Oromotor Sequences and Manual Motor
Sequences tests (Urgesi et al. 2011).
The MANCOVA on Social Perception domain was only
influenced by Attention and Executive Functions (F3,57 =
5.93, p = 0.001, gp2 = 0.238), while the other effects were
not significant (all Fs3,57 \ 2.2, p [ 0.09, gp2 \ 0.11). This
suggests that the deficits of HFASD children in the social
perception abilities evaluated with NEPSY-II were associated
to attention and executive functions deficits.
In a similar vein, the MANCOVA on Visuospatial Pro-
cessing domain was influenced only by Attention and
Executive Functions (F5,55 = 5.58, p \ 0.001, gp2 =
0.336). Although HFASD children tended to show a rela-
tively lower performance in visuospatial tests, the effect of
group did not reach the Bonferroni corrected significance
threshold (F5,55 = 2.73, p = 0.029, gp2 = 0.199) after
removing the effects of the other cognitive domains.
Discussion
The main aim of the present study was to describe the
neuropsychological profiles of HFASD children across
123
J Autism Dev Disord
different cognitive domains, ruling out the effects of gen-
eral cognitive abilities. Controlling for verbal general
cognitive abilities, several impairments were observed
across different cognitive domains in HFASD. Our data
seem to support the notion that NEPSY-II is able to
highlight the profile of neuropsychological functioning of
HFASD. While the results from the initial validation study
reported in the NEPSY-II manual (Korkman et al. 2007)
were largely replicated, some differences were noted.
Within the Attention and Executive Functioning
domain, we confirmed the deficits of HFASD children in
the measures of Inhibition, Animal Sorting and Auditory
Attention and Response Set. Furthermore, the pattern of
Attention and Executive Function deficits in HFASD is
largely consistent with prior research in HFASD using the
first version of the NEPSY (Joseph et al. 2005; Hooper
et al. 2006; Korkman et al. 1998). In particular, in the
Joseph et al. (2005)’s study, children with autism exhibited
deficits compared to the comparison group across all the 3
domains of executive functions that were assessed,
including working memory, inhibitory control and plan-
ning. Several studies, indeed, have demonstrated that
HFASD children present executive dysfunctions as com-
pared to age-matched typical developing individuals and
other clinical control groups (Ozonoff et al. 1991; Ozonoff
1997; Bennetto et al. 1996; Ozonoff and McEvoy 1994;
Ozonoff 1995; Ozonoff and Jensen 1999; Sergeant et al.
2002; Geurts et al. 2004; Verté et al. 2005). In a similar
vein, in other studies that evaluated planning abilities
(Hughes et al. 1994; Ozonoff et al. 2004; Pennington and
Ozonoff 1996; Bennetto et al. 1996; Robinson et al. 2009;
Joseph et al. 2005; Lopez et al. 2005; Happé et al. 2006),
HFASD were impaired relative to HC. The NEPSY subtest
‘Statue’ was also used to study 2 components of motor
control: motor response inhibition and motor persistence in
24 HFASD aged 7–13 years (Mahone et al. 2006). HFASD
achieved lower scores than controls only on measures of
motor persistence, with no concomitant impairment on
motor inhibition tasks, in keeping with prior research
demonstrating relatively spared motor inhibition in autism.
We also confirm the lower performances of HFASD
children in Language domain (Hooper et al. 2006;
Korkman et al. 2007). In particular, the impairments in the
Phonological Processing, Oromotor sequences and Repe-
tition of non-sense words are consistent with Tager-
Flusberg (2004), who suggested that children with autism
and phonological deficits may represent a selected subtype
of autism. Whitehouse et al. (2008) suggested that only
some children with autism demonstrate poor non-sense
word repetition, while Schmidt et al. (2008) pointed out
that problems in phonological processing and repetition of
non-sense words might be characteristic of the broad aut-
ism phenotype. Accordingly, some studies have found that
J Autism Dev Disord
HFASD have an impairment in the repetition of non-sense
words as compared to controls (Botting and Conti-Rams-
den 2003; Riches et al. 2011) and this deficit might reflect a
general phonological processing impairment in HFASD
(Bishop et al. 2004; Hooper et al. 2006).
HFASD had lower performance than HC also in all tasks
of the Memory and Learning domain. These results are
consistent with the Korkman et al. (2007)’s findings and
with other studies showing impaired immediate memory
performance in HFASD. In particular, we confirm that
HFASD performance on immediate verbal recall of com-
plex material, such as a stories or sentences, is significantly
impaired (Boucher and Warrington 1976; Boucher 1981;
Fein et al. 1996; Bennetto et al. 1996; Minshew et al. 1997;
Korkman et al. 1998; Minshew and Goldstein 2001;
Williams et al. 2005, 2006; Hooper et al. 2006). Previous
studies have found that individuals with HFASD may
display a similar pattern of list learning across 5 repetitions
of the same list words presented in the same order (Bowler
et al. 2009). However, the impairments of HFASD indi-
viduals in verbal learning become particularly manifest
under conditions that require the organization of the
learning material to improve the performance (Bowler
et al. 2008, 2010). Such organization of the information is
also required by the List Memory and Word List interfer-
ence tests of the NEPSY-II, in which an interferential list is
presented and participants need to organize the words
correctly in the two lists avoiding interference. Thus, the
deficits observed in list learning in our HFASD sample
may be due mainly to difficulties in organizing the verbal
materials and to resist interference, rather than to word
learning per se. Our results are also consistent with previ-
ous evidence that performance in the immediate recall of
complex designs or forms is impaired in ASD children
(Ameli et al. 1988; Minshew et al. 1992; Minshew and
Goldstein 2001; Rumsey and Hamburger 1990; Williams
et al. 2006). We confirmed also the marked deficits in face
memory which has been widely reported in the literature.
Several studies, indeed, found that ASD, as compared to IQ
matched controls, are particularly impaired in this task (de
Gelder et al. 1991; Boucher and Lewis 1992; Gepner et al.
1996; Korkman et al. 1998; Hauck et al. 1998; Klin et al.
1999; Blair et al. 2002; Williams et al. 2005; Hooper et al.
2006) (for a theoretical explanation see Carver and Dawson
2007). However, our study allows to document that the
visuospatial memory impairments in HFASD are not lim-
ited to faces, but they extend to abstract geometric forms
(Memory for Designs).
According to Hooper et al. (2006), ASD demonstrated
deficits in the Sensorimotor Functions domain. In particu-
lar, while no difference between HFASD and control
children were observed in the Visuomotor Precision and
Finger Tapping tests, specific impairments were found in
the Imitation of hand postures and in the Manual motor
sequences tests, thus in those tasks requiring the imitation
of complex movement sequences. Following Joseph et al.
(2005), these tasks require children to combine working
memory and inhibitory control so as to withhold a domi-
nant motor response by maintaining a conflicting response
rule (e.g., to knock with one hand while the other hand taps
and vice versa) in active memory. In keeping with our
finding, Korkman et al. (2007) and Hooper et al. (2006)
showed no significant differences between HFASD versus
controls in the Finger Tapping task, suggesting the absence
of deficits in low level sensorimotor control.
The findings in Social Perception domain confirm the
Korkman et al. (2007)’s results with lower scores for
HFASD than controls in Affect recognition and Theory of
Mind Verbal tasks. Within the Social Perception domain an
important result of our work was the intact performances of
HFASD in the contextual items of the Theory of Mind test.
These trials evaluate the capacity of children to understand
how certain emotions are linked to given social situations
and to recognize correctly the emotions that the various
social settings generate. The child is shown an illustration
of a social situation and he/she has to choose which of four
facial expressions illustrates appropriately the emotional
state of one of the characters shown in the photograph (see
Fig. 1). Our results contrast with those of Da Fonseca et al.
(2008) that revealed that children with HFASD are able to
use contextual cues to recognize objects but not emotions.
However, differently than in the task of Da Fonseca et al.
(2008), in which the children have to choose the appro-
priate emotional state between smile illustrations, in the
NEPSY-II tasks the children have to choose the appropriate
emotional state from real facial photos. Our findings may
suggest that: (1) contextual elements (the social situation)
seem to facilitate the processing of the corresponding
emotional state (photo of human face); (2) the human face,
in contrast to smiles illustration (Da Fonseca et al. 2008),
could facilitate the activation of an isomorphic process—
identity between the level of observed experience and that
of the physiological processes underlying it—in HFASD
children (Eagle and Wakefield 2007; Gallese 2003); (3) the
systematizing inclination of HFASD to analyze details
(Baron-Cohen 2010) could facilitate the trend toward
greater attention to contextual cues of social situations by
facilitating the recognition of appropriate emotional states.
Our HFASD sample displayed a relative sparing of the
visuospatial abilities when compared with the deep neu-
ropsychological deficits observed in the other domains. In
this domain, only the Arrows and Design Copy tests
showed a significant difference between the HFASD and
HC children, with scores corresponding to borderline per-
formance. The deficits in the line orientation discrimination
(Arrows) have been found also in previous studies
123

Fig. 1 Example of contextual item of the Theory of Mind test
(Korkman et al. 2007; Hooper et al. 2006). On the other
hand, the deficit in the Design Copying test is consistent
with the initial validation study of the NEPSY-II (Korkman
et al. 2007), but not with the validation study of the NEPSY
(Hooper et al. 2006). This discrepancy between the two
tests might be due to the improvement of the sensitivity of
the Design Copy test in the NEPSY-II, with the inclusion
of more complex items. Importantly, only the Motor and
Global Processing scores of the Design Copying test were
lower in HFASD than HC children, while no difference
was found in the local processing score. Thus, while
HFASD children presented difficulties in fine-motor con-
trol, causing inaccurate drawing, and in representing the
overall configuration of the design, they accurately repre-
sented the design features. This suggests a bias of HFASD
children towards a local strategy of stimulus processing
(Baron-Cohen 2010) which may be detected by analyzing
the specific processing scores of the NEPSY-II Design
Copying test. Recently, Planche and Lemonnier (2011)
have used the NEPSY and WISC-III tests to study visuo-
spatial abilities in HFASD and found non-significant dif-
ferences between HFASD and controls. Nevertheless, as
compared to controls, HFASD children showed a different
response strategy in the visuospatial tasks with motor
coordination components, i.e. the ‘‘object assembly’’ and
‘‘block design’’ tests of the WISC-III, but not in tasks
requiring similar visuospatial processing in the absence of
any motor coordination ability, i.e. the ‘‘arrows’’ NEPSY
test and the ‘‘picture completion’’ WISC-III test. These
results might suggest that HFASD children have spared
visuospatial processing abilities, but their difficulties in
sensorimotor functions facilitate the use of local rather than
configural processing strategies when the visuospatial tasks
require motor coordination. Thus, the study by Planche and
Lemonnier (2011) suggests that, in keeping with the Luria
(1962)’s approach, the performance of HFASD children in
a given cognitive domain may be affected by their weak-
nesses in other domains.
A further aim of the present study was to determine how
the neuropsychological impairments of HFASD children in
123
J Autism Dev Disord
a given domain were influenced by their weaknesses in
other domains. A comprehensive neuropsychological
investigation of the main cognitive functions with the
NEPSY-II is particularly adept to this purpose, allowing to
identify specific patterns of primary and secondary deficits
(Luria 1962). To analyze how HFASD children differed
from controls in each cognitive domain independently from
their performance in the others, MANCOVAs were per-
formed for each set of neuropsychological variables
entering the others domain scores as covariates of interest.
These analyses allowed to show how the performance of
children in a given set of neuropsychological variables is
influenced by their abilities in other cognitive domains and
how much the impairments of HFASD in each neuropsy-
chological function are associated to their deficits in other
domains. We found that the impairments of HFASD chil-
dren in attention and executive functions and verbal
memory were not associated, at least partially, to the other
neuropsychological functions. In turn, a widespread influ-
ence of executive deficits was observed on the performance
in the other domains. Furthermore, verbal memory affected
performance in language tests. The deficits of HFASD
children in language, visuospatial memory, sensorimotor
functions, social perception and visuospatial processing
were associated to executive and verbal memory deficits
and did not survive after removing the variance in common
with other domains. It is worth noting, however, that since
the variability in the performance at the different NEPSY-
II tests was not random in the two groups, being strongly
influenced by having or not HFASD, the interpretation of
these results should be cautious. They cannot indicate that
HFASD children are not impaired in a given cognitive
function apart from deficits in other cognitive domains, but
that their deficits in a given domain are associated to, and
possibly influenced, by deficits in other cognitive domains
(Joseph et al. 2005). In other words, individuals with
greater language, sensorimotor and social perception defi-
cits also had greater impairments in verbal memory or
executive functions.
A similar account has been used by Joseph et al. (2005)
in a study attempting to verify the hypothesis of Russel
(1997) that the functional deficit underlying poor perfor-
mance by children with autism on executive function tasks
(Hughes and Russell 1993) could be an impairment in the
ability to use inner speech to maintain arbitrary response
rules in working memory and to guide behavior accord-
ingly. In keeping with the language mediation hypothesis
of the executive dysfunctions in autism, studies have found
that the deficits of HFASD children on executive functions
tasks disappeared when the lower scores of the autistic
group in verbal IQ (Liss et al. 2001) and in lexical retrieval
and access measures (Joseph e al. 2005) were controlled for
using ANCOVAs. However, since the HFASD group was
J Autism Dev Disord
impaired in both executive functions and language tasks,
these data could not determine the origin of the association.
In other words, it could not be established whether a lack of
language mediation may explain the executive function
deficits or whether language and executive functions are
simultaneously impaired in HFASD. On the other hand, our
findings show that the deficits of HFASD in attention and
executive functions as compared to HC were still signifi-
cant after removing the effects of verbal IQ and of neu-
ropsychological linguistic abilities favor a primary
dysfunction of executive functions in HFASD.
The widespread impairments in different cognitive
domains in our HFASD sample might suggest a deficit in
general cognitive abilities rather than in specific neuro-
psychological functions. However, since we only tested
HFASD individuals with IQ values within the normal
range, we can rule out that a deficit of general cognitive
abilities may explain the results. On the other hand,
although we did not have the IQ values of HC individuals
and cannot rule out definitively that we selected partici-
pants with general cognitive abilities higher than expected
from chronological age, it is unlikely that this may explain
the neuropsychological deficits of HFASD individuals.
Indeed, the scaled scores of HC individuals in all NEPSY-
II tests were about 10, thus they were comparable to the
values expected from the Italian normative study (Urgesi
et al. 2011). Conversely, the scaled scores of HFASD
participants in the texts showing a between-group differ-
ence ranged from 6 to -4 and were less than 2 SD from the
expected value in many tests (see Table 1) Furthermore,
low correlations have been reported between the NEPSY-II
scores and the IQ values (measured with the WISC-III test
in a subsample of children) in both the US (Korkman et al.
2007) and the Italian (Urgesi et al. 2011) normative studies.
This suggests that performance on the NEPSY-II tests was
partially independent from general cognitive abilities as
measured with the WISC-III tests (Korkman et al. 2007;
Urgesi et al. 2011). Relatively higher correlations were
found for the Attention and Executive Functions domains,
where HFASD individuals showed an impaired perfor-
mance as compared to HC, but also for the Visuospatial
Processing domain tests, where HFASD showed a relative
sparing. The intact performance in many tests (e.g., Visual
Attention, contextual items of Theory of Mind, Block
Construction, Picture Puzzle and Route Finding) and in
some performance indexes of other tests (e.g., intact Global
Processing score but impaired Local Processing score in
the Design Copying tests) points to alterations in multiple
cognitive subsystems rather than to general cognitive
deficits.
Our results could be consistent with the emerging view
of autism as a disorder of the interactions among multiple
brain systems (Frith 2004; Mundy and Sigman 2006). This
hypothesis is also corroborated by recent findings derived
from HFASD neuroimaging studies, in which brain alter-
ations are not limited to specific regions or circuits, but
rather are indicative of a global cerebral impairment
(Müller 2007). Indeed, structural magnetic resonance
imaging (MRI) investigations consistently report an abnor-
mal enlargement of total brain volume in patients with
HFASD that involves gray and white matter (Courchesne
et al. 2001; Hazlett et al. 2005). Those alterations are
particularly conspicuous in frontal and temporal regions of
children with autism (Calderoni et al. 2012; Carper and
Courchesne 2005; Hazlett et al. 2006), with a relatively
spare of the occipital lobe. Similarly, studies using Diffu-
sion Tensor Imaging (DTI) to investigate the whole brain
confirm a diffuse and atypical connectivity organization in
HFASD children (Bloemen et al. 2010; Shukla et al. 2010).
Evidence of extensive brain impairment in HFASD
patients comes also from functional-connectivity MRI data,
that point to an altered connectivity among several brain
areas during the execution of different complex tasks (Just
et al. 2004, 2007; Koshino et al. 2008; Mason et al. 2008).
The above mentioned anatomical and functional brain
alterations are consistent with the disrupted cortical con-
nectivity model (Kana et al. 2011), which claims that the
autistic deficit in higher level cognitive processes may
reflect reduced connectivity among key brain areas. Also,
the widespread and global neuropsychological impairments
found in HFASD patients could resemble the neurocogni-
tive profile of negative symptoms in patients with chronic
schizophrenia (Frith 1995; Fagerlund et al. 2006; Kravariti
et al. 2005; Rhinewine et al. 2005). This adds to the
recent literature data showing shared features between
these two conditions (King and Lord 2011), that involve
genetic (Burbach and van der Zwaag 2009; Crespi et al.
2010), anatomical (Cheung et al. 2010; Pinkham et al.
2008) and clinical (Couture et al. 2010; Craig et al. 2004)
aspects.
The results of this study should be interpreted in the
light of important limitations. First, the relatively small
sample size of HFASD children may limit the generaliza-
tion of the finding to the general population of HFASD.
Second, the inclusion of children with PDDNOS who had
comparable social dysfunctions but did not satisfy all
DSM-IV-TR criteria for AD should be considered when
comparing the results to studies testing only children who
satisfy the more stringent criteria for AD. However, this
limitation is lessened by the absence of significant differ-
ences on the NEPSY-II profile when children with AD are
compared to PDDNOS.
Finally, the results of the analyses comparing the per-
formance of HFASD and HC children on each cognitive
domain when the variance in common with the other
domains is partialled out should be cautious, since we
123

cannot establish whether associated impairments simply
co-occur in HFASD children or are dependent from the
same primary deficit.
In conclusion, our study supports the utility of NEPSY-
II as a sensitive evaluation tool to describe the neuropsy-
chological profile of HFASD children. It can identify
weaknesses and strengths of their cognitive abilities and to
disentangle between primary and secondary deficits. Fur-
thermore, our findings, in particular the lower perfor-
mances on verbal versus contextual items of Theory of
Mind, may pave the way for empirical studies on larger
sample of HFASD. Indeed, a deep investigation of the
neuropsychological impairments in HFASD might help the
identification of the pathophysiological mechanisms asso-
ciated with the disorder and to design appropriate inter-
ventions aimed at improving cognitive capacities.
Acknowledgments This work was supported by the Italian Ministry
of Health under Strategic Program ‘Inquiry into Disruption of Inter-
subjective Equipment in Autism Spectrum Disorder in Childhood
(IDIA)’ (Principal Investigator F.M.) and partially by grant from the
IRCCS ‘‘E. Medea’’ (Ricerca Corrente, Ministry of Health to C.U.).
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