J Neurol Neurosurg Psychiatry 1998;65:555–560

555

Autism and visual agnosia in a child with right
occipital lobectomy
I Jambaqué, L Mottron, G Ponsot, C Chiron

Hôpital Saint Vincent
de Paul, Service de
Neuropédiatrie,
Université René
Descartes, Paris,
France
I Jambaqué
G Ponsot
Clinique Spécialisée de
l’Autisme, Hôpital
Rivière des Prairies et
Département de
Psychiatrie de
l’Université de
Montréal, Canada
L Mottron
Hôpital Fréderic Joliot,
Département de
Recherche Médicale
du Centre à l’Energie
Atomique et Inserm U
29, Orsay, France
C Chiron
Correspondence to:
Dr Isabelle Jambaqué,
Hôpital Saint Vincent de
Paul, Service de
Neuropédiatrie, 82 Avenue
Denfert Rochereau, 75 674
Paris, Cedex 14, France.
Telephone 0033 1 40 48 80
48; fax 0033 01 40 48 83 40.
Received 23 June 1997 and
in final revised form 6 March
1998
Accepted 6 March 1998

(J Neurol Neurosurg Psychiatry 1998;65:555–560)

language. However, these models failed to
account for those “high functioning” persons
with autism whose level of verbal production is
average or above average. Deficits in the social
and communication area have convincingly
been attributed to an impairment in the ability
to interpret people’s intentions, beliefs, and
desires, known as “theory of mind”.2
Nevertheless, symptoms of the “repetitive
behaviours and restricted interest” area are difficult to explain through a “theory of mind”
deficit, and an “executive functions deficit”
might be a more appropriate candidate to
explain this cluster of symptoms.3 Recently, it
has been proposed that abnormal perceptual
representations of objects and faces might
account for the social impairments, repetitive
behaviours, and restricted interests that are
commonly seen in persons with autism.4 At a
cognitive level, persons with autism present an
atypical processing of complex visual figures,
including an impairment of movement
perception,5 a deficit in facial perception,6–8 and
atypical part-whole integration of visual
stimuli.9 10
Clinical arguments in favour of a visual perceptual impairment in the aetiology of autistic
disorder originate from the occasional finding
of comorbidity of autism and various visual
deficits.11 12 A pattern of autistic-like behavioural symptoms has long been recognised in
infants with retrolental fibroplasia sequelae.13
Children with delayed visual maturation may
exhibit transient autistic behaviour.14 15 Central
visual disturbances also contribute largely to
the autistic picture seen in children who
develop infantile spasms.16 Futhermore, several
cases of prosopagnosia in childhood suggest an
association between autism and developmental
visual agnosia17–20 although some cases are
clearly non-autistic.21–23 The present paper discusses the case of a 13 year old girl with autistic disorder and developmental apperceptive
visual agnosia due to right occipitotemporal
damage, whose neurofunctional imaging disclosed a left frontal hypometabolism in addition to the right occipitotemporal defect.

Keywords: autism; visual agnosia; executive deficit;
child

Case report

Abstract
Objectives—Autistic disorder is a developmental handicap with an unknown
neurological basis. Current neuropsychological models for autism suggest an
abnormal construction of visual perceptual representation or a deficit in executive functions. These models predict
cerebral lesions in the temporo-occipital
or frontal regions of autistic patients. The
present study aimed at studying the presence of symptoms of autism and visual
agnosia in a 13 year old girl who had a
right temporo-occipital cortical dysplasia
that was surgically removed at the age of
7.
Methods—Neuropsychological evaluation
included Wechsler and Kaufman intelligence scales, a test of word fluency, digit
span, Corsi block, California verbal learning, Trail making, Benton facial recognition, Snoodgrass object recognition tests,
Rivermead face learning subtest, and
developmental test of visual perception.
The ADI-R was used to show current and
retrospective diagnosis of autistic disorder. Neuroimagery included brain MRI,
single photon emission computed tomography (SPECT), and PET.
Results—Brain MRI showed a right occipital defect and an abnormal hyperintensity of the right temporal cortex. PET
and SPECT disclosed a left frontal hypometabolism together with the right
occipital defect. Neuropsychological testing showed a visual apperceptive agnosia
and executive function deficits. Psychiatric study confirmed the diagnosis of autistic disorder.
Conclusions—Although the possibilty that
autism and visual agnosia were dissociable factors in this patient cannot be
excluded, the finding of both deficits supports the possibility that occipitotemporal lesions can predispose to the
development of autism.

MEDICAL HISTORY

Autism is a developmental syndrome characterised by a triad of behavioural impairments in
the areas of social relationships, communication, and repetitive behaviours or restricted
interests. Early models of cognitive abnormalities in autism focused mainly on language
deficits,1 as many persons with autism present
significant language delays or absence of

This right handed girl, now aged 13, was born
at term after normal vaginal delivery. There
was no family history of neurological or
psychiatric disorder. At 1 month, the child
developed daily partial seizures which became
rapidly intractable to all anticonvulsive drugs.
Seizures were characterised at one month by
left down deviation of her eyes, oculoclonies,
and raising of her arms with cyanosis and

556

Jambaqué, Mottron, Ponsot, et al

(A) Cerebral MRI shows the postoperative right occipital cavity and an abnormal hyperintensity of the temporal cortex at
the junction of the right temporo-occipital sulcus. (B) Single PET shows the right occipital defect and a hypoperfusion in the
left frontal cortex on the slice corresponding to the level +60 mm above the orbitomeatal line.25

chewing. At 3 months of age, the seizures consisted of very brief oculoclonies and occurred
one to five times a day. DiYculties following
light were noted at 8 months of age. Seizure
duration increased progressively from one to
two minutes at the age of 18 months and
included cyanosis, chewing, breathing irregularities, and tonicoclonic jerks of all four limbs.
Neurological examination disclosed left
hemianopsia since the first year of life. At 5
years of age, other neuro-ophtalmological
abnormalities included a rotatory nystagmus, a
marked myopy of the left eye and a mild myopy
of the right eye, a converging strabismus, and a
permanent right deviation of gaze. By contrast
there was no oculomotor paralysis and fundoscopic examination as well as an electroretinography performed at 5 years of age were normal.
Several CT scans and brain MRI at 3, 5, and 6
years of age disclosed nothing atypical. An
EEG performed at 4 years of age showed right
temporo-occipital discharges during weakness
and sleep and secondary diVusion to the frontal lobes. A more precise localisation of the
EEG abnormalities was obtained by stereotaxic
EEG, which established that the seizures originated in the right calcarine occipital region, but
also involved the external part of the right
occipital lobe.
A right occipital lobectomy was performed at
7 years of age because of intractable seizures.
The occipital cortex was removed including the
two edges of calcarine scissure, but the
periventricular white matter of the occipital
horn was spared. Neuropathological study of
the removed occipital lobe showed a characteristic malformative dysplasia.24 The seizures
stopped immediately after surgery and she
remained seizure free for 2 years. At 9 years,
she started to develop brief partial complex

seizures which were not entirely controlled
(two to four seizures a month) with carbamazepine. Her EEG showed slow waves and
rare spikes in the right temporal region.
Cerebral MRI showed an abnormal hyperintensity of the temporal cortex at the junction of
the right temporo-occipital sulcus (figure (A)).
This abnormality could reflect a localised cortical dysplasia which was not removed with the
surgical intervention or postoperative modification due to the occipital resection itself. At
11 years of age, both single photon emission
computed tomography (SPECT) and PET
studies25 showed a postsurgical right occipital
defect, as well as a left frontal hypoperfusion
and hypometabolism (figure (B)).
DEVELOPMENT

The patient has exhibited a mild psychomotor
delay since birth. She reached for objects at 7
months, walked at 17 months, and spoke her
first words at 24 months. At 4 years of age, she
had erratic gaze, often collided with objects,
and did not use pointing gestures. The child
was unable to recognise people and to show or
to name pictures, with the exception of
geometric figures and colours. She was able to
construct sentences of three to four words,
although she did not use pronouns and her
language production included stereotyped
speech and echolalia. Imaginative play was
absent and social interaction was poor, lacking
marks of reciprocity such as shared enjoyment
or interest in children. Just before surgery, at
the age of 7 years, her verbal IQ was 68 on the
Wechsler intelligence scale for children-revised
(WISC-R).26
One year after surgery, there was some
improvement in her attentional capacities and
social interaction, but her IQ remained stable.

557

Autism and visual agnosia in a child with right occipital lobectomy
Table 1 Course of DSM-IV symptoms for diagnosis of autistic disorder according to
autism diagnosis interview scores
DSM-IV items

4–5 y

13 y

Social interaction
Failure to use non-verbal behaviours to regulate social interaction (/6)
Failure to develop peer relationships (/10)
Lack of shared enjoyment (/6)
Lack of socioemotional reciprocity (/10)
Total (cut oV=10)*

3
6
4
5
18

2
3
1
3
9

Communication
Delay in spoken language and failure to compensate through gesture (/8)
Lack of varied spontaneous make believe or social imitative play (/6)
Relative failure to initiate or sustain conversational interchange (/4)
Stereotyped, repetitive, or idiosyncratic speech (/8)
Total (cut oV=8)

4
6
2
1
13

3
4
3
3
13

Repetitive behaviours and stereotyped patterns
Encompassing preoccupation or circumscribed pattern of interest (/4)
Compulsive adherences to non-functional routines or rituals (/4)
Stereotyped and repetitive motor mannerisms (/2)
Preoccupation with parts of objects (/2)
Total (cut oV=3)

4
2
1
2
9

4
2
1
2
9

*Value above which an area is considered suYciently impaired for the diagnosis of autism.

Her phonological and syntactic abilities were
average for her age, but she continued to show
deficits in the communicative use of language
and reciprocal interactions. She learned to read
and therefore used adaptive behaviour such as
shifting the book in front of her eyes. She
remained unable to recognise people without
hearing their voices. Beginning when she was 8
years old, a restricted interest in schedules and
watches pervaded her daily activities, and her
routine could not be modified without anxiety
reaction. This restricted interest was characterised by repetitive drawings of watches and
patches of colour, as well as stereotyped
writings of lists of words or sentences.
Due to the influence of the French classification for infant psychopathology, verbal children are rarely diagnosed with autistic disorder
in France. As a result, the patient’s original
diagnosis at 4 years of age was infantile
psychosis. Current and retrospective diagnosis
of autistic disorder was made at 13 years of age
with the use of the ADI-R,27 a standardised, in
depth interview regarding symptoms associated with autism occurring both in past and
present behaviour. This diagnosis was confirmed through clinical examination by one of
us (LM). The patient scored higher than the
cut oV necessary for a diagnosis of autistic disorder in the three relevant areas at the 4 to 5
years of age period. A comparison of her scores
for past behaviour (4 to 5 years old) with those
of her current behaviour shows that she
improved in the “social” area but remained
clearly above the cut oV for autism in the
“communication” and the “restricted interest
and repetitive behaviours” areas (table ). This
improvement seems to characterise high functioning persons with autism in general,28 and is
not restricted to autistic-like syndrome in the
context of visual impairment.14 16
GENERAL COGNITIVE ASSESSMENT

At the age of 13 years, her full scale IQ on the
WISC-R fell in the range of mild mental
deficiency.26 Nevertheless, the cognitive and
academic assessment disclosed heterogeneities
of performance, with special abilities and
specific impairments. She obtained a verbal IQ
of 63 (mental age of 8 years 7 months). Her

verbal scores were all in the borderline range
(information 4, similarities 5, arithmetic 6,
vocabulary 4) with the exception of her score
on the comprehension subtest (1), on which
she exhibited a decline in performance typical
of persons with autism.29 Her performance IQ
was only 45 due to visual impairment (picture
completion 3, picture arrangement 1, block
design 2, object assembly 1, coding 3).
Remarkably, her academic abilities in arithmetic (mental age 10 years 6 months) and spelling
and reading (mental age 9 years 11 months)
were higher than her intelligence level, both of
which are special abilities often encountered in
patients with autism.30 31
VISUAL AGNOSIA

The left homonymous hemianopia did not
additionally impair her visual performances.
She compensated spontaneously her visual
field cut by head nodding. Her ability to
perceive simple visual features seemed to be
average at 13 years of age. She was able to recognise and copy letters, digits, and simple geometric shapes such as triangles, but had
diYculties copying geometric shapes of two
elements— for example, a square in a circle
(mental age 6 years 3 months). Spatial relations
were slightly better (mental age 7 years 6
months).32 She was unable to perform the simplest object assembly but was able to reproduce
some geometric shapes with blocks.26 Colour
discrimination was preserved.
Face recognition was markedly impaired in
her daily life, although she was able to use the
hairline as a cue. The patient could recognise
family members in photographs but was
unable to recognise her own picture. On the
Benton facial recognition test33 her performance was severely defective (30 of 54, inferior
to 6 years). She failed with faces that were oriented diVerently or under diVerent lighting
conditions. In a matching task, she could not
distinguish between pictures of faces with
diVerent sexes and could only match two out of
10 identical pairs of faces. Face learning was
also severely deficient. When asked to identify a
set of previously seen unfamiliar faces among
new faces, her performance was at the level of
chance (on the Rivermead behavioural
memory battery’s face learning subtest34 her
score was 0, three of 10 faces identified
correctly, three of 10 false positive). She was
also impaired in her ability to recognise
emotional expression. A face game showing
facial expressions as indicators of feeling
created for children aged 4–6 years was
employed. She identified smiling as indicative
of joy because “the child showed his teeth” but
was unable to distinguish between laughing,
astonishment, or anger because “the child
raised his teeth”. In addition, she failed to
identify emotional cues based on the appearance of eyes, and was therefore unable to
distinguish, for example, between a face that
represented sadness and one that was sulky.
Object recognition abilities were also impaired. She successfully named 75% of a set of
line drawings of objects belonging to various
categories.35 Her performance was 70% for

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Jambaqué, Mottron, Ponsot, et al

vehicles, 70% for animals, 70% for fruits and
vegetables, 60% for body parts, 90% for
clothes, and 90% for tools. Impairment of
object recognition was more evident with overlapping realistic and abstract figures,36 and
when asked to reconstruct perceptively degraded objects (Kaufman completion test,
below the age of 2 years 6 months).37 Comparison of small object recognition according to
modalities disclosed a slight superiority of the
tactile modality (90%) over the visual modality
(80%). She spontaneously explained that she
was better in the tactile modality because she
did not see the objects. By contrast, she was
generally able to define and provide functional
information about objects or animals she was
unable to recognise. In sum, the patient had an
apperceptive visual agnosia which was particularly severe for faces.
MEMORY AND EXECUTIVE FUNCTIONS

The patient’s highest score on the WISC-R was
the digit span subtest (11, the norm for 14
years, 6 months), although her performance
was impaired on digits backward (3, normal
mean (SD) 5.9 (1.2)) relative to digits forward
(7, normal mean (SD) 4.5 (1.4)). A clear
dissociation between forward and backward
digit span abilities is indicative of a selective
defect of “central executive functions” among
otherwise intact working memory.38 Her poor
performance on the Corsi block test (3, below
the norm for 5 years) was reflective of a deficit
in visual attention. A computerised memory
battery39 disclosed above average performance
on tests of words and non-word spans, (5 and 4
respectively, the norm for adults). On the California verbal learning test,40 she recalled six
words on trial 1 and improved to 13 words on
trial 5. Her total words recall (trials 1–5) was
49 (mean for 13 years of age). Other scores,
including the number of extra list intrusions
(0), the semantic cluster (13), the learning of
list B (6), and the recall of list A after interference (9) were average for her developmental
level. These scores converge with previous
findings of generally normal to exceptional list
memory
in
patients
with
autism.41
Nevertheless, many perseverative responses
were made by the patient (+3 SD).
Numerous tasks tapping executive dysfunction (Wisconsin card sorting test, Tower of
London) have poor reliability scores in children
under 10 years of age and those with low IQs
due to their high level of diYculty.3 Furthermore, spatial impairment may aVect performance on these tests. As a result, the patient’s
frontal function was measured using more simplistic tasks. Her performance on the test of
word fluency (the norm for 7 years) and the
test of sequencing motor response (the norm
for 6 years)37 was weak. She also showed
significant impairment on the trail making test,
part B (-5 SD), an attentional task on which
weak performance reflects poor mental
flexibility.42 She was unable to achieve the simplest maze of the WISC-R, showing a deficit in
planning (inferior to 6 years). In sum, the
patient’s performance was indicative of several
executive function impairments, including in-

hibition of previously encountered material
(perseverations), planning of motor strategies,
planning of search strategies in long term
memory, and manipulation of information in
working memory.
Discussion
The patient is a child with autistic disorder who
presented with developmental visual apperceptive agnosia associated with a right occipitotemporal lesion. In addition, hypometabolism
of the left frontal lobe was demonstrated by
functional brain imaging in our patient, who
also presented an executive deficit. The cognitive deficits and localisation of brain lesions
have a likely causal relation with her symptoms
of autism.
Although surgery improved control of her
seizures, she still had right temporal lobe
epilepsy up to the last evaluation. The hypothesis that temporal lobe dysfunction plays a part
in autistic disorder was strongly supported by
the description of cytoarchitechtonic abnormalities in the limbic structure of patients with
autistic disorder43 and the finding of unilateral
or bilateral complete temporal lobe destruction
in a few cases of such patients.44–48 Behavioural
regression similar to that seen in patients with
autistic disorder has also been reported in limbic pathology and epilepsy.49–54 Thus, temporal
lesions from various aetiologies appear as the
most frequent localisation involved in autism.
First models of temporal lobe involvement in
autism45 55 56 propose that early bilateral damage to the medial temporal lobe produces a
major amnesic syndrome in adults, which may
take the form of severe mental retardation with
autistic-like behaviour in children. However,
some studies have discarded the hypothesis of
an amnesic syndrome in autism,57 and recently
a few cases of amnesia due to very early
hippocampal pathology were reported with
relatively preserved cognitive and social
development.58 The present case suggests that
severe dysfunction of the temporo-occipital
cortex, rather than the mesial temporal lobe
itself, may predispose to the development of
autistic disorder.
Even though there are often visual perceptual abnormalities in those with autistic
disorder,5–10 the relation between developmental visual agnosia and autism remains debated.
A retrospective analysis of published cases supports strongly such a relation but only one case
with established early temporo-occipital brain
lesion has been recently documented.20 Autism
does not occur, however, when such a lesion
occurs later during development.21 When
present early, visual agnosia, and particularly
prosopagnosia, leads to severe impairment
which leaves little room for recuperation.59 60 It
is therefore tempting to link the deficit in interpreting facial cues to the failure to interpret
other people’s intentions, beliefs, and desires:
the “theory of mind”.11 61 Visual agnosia for
faces may be responsible for many behaviours
or absence of behaviours identified as critical
symptoms for autistic disorder in the “social”
area—for example, absence of emotional reciprocity (absence of direct gaze, limited range of

Autism and visual agnosia in a child with right occipital lobectomy

facial expressions) and failure to develop peer
relationships (lack of interest in children, lack
of shared enjoyment, lack of socioemotional
reciprocity). On the other hand, an impairment
of movement perception may cause lack of
pointing and social imitation gestures and failure to use non-verbal behaviours. Central
visual impairment supports a model in which
restricted interests are directly determined by
what it is perceived, the reduction of interest
diversity being attributed to the reduction of
the visual input.4 In this context, the abilities of
our patient to recognise and name a restricted
range of visual stimuli, specifically colours and
simple geometric shapes, might explain her
“special interests” for drawing of colour
patches and watches. It is, however, likely that
early visual agnosia is not suYcient to generate
the full range of symptoms of autism. Indeed,
persons with autism are shown to present various frontal cognitive deficits, including planning deficit and an inability to switch cognitive
programming.62 Evidence of frontal brain
lesions is, however, rare in autism, and claims
of such frontal lobe involvement are generally
based on functional studies.16 63–66 Hypometabolism of the left frontal lobe was also shown
by functional brain imaging in our patient, who
exhibited deficits in manipulation of information, verbal fluency, planning, and motor
sequencing. The ritualisation of her behaviour
and her marked tendency to perseverations was
also likely due to frontal dysfunction.3 Although we cannot exclude the possibility that
autism and visual agnosia were dissociable factors in our patient, our finding raises the
intriguing possibility that occipitotemporal
lesions may predispose to the development of
autism. These lesions are, however, not suYcient to produce by themselves the full range of
autistic symptoms. The disconnection from
other cortical regions, in particular the frontal
lobes, may be crucial in the development of
autistic disorder67 by disorganising the integrations of cognitive functions.
We thank Patrick Aubourg for his constructive discussion on
this case and his work on the manuscript; Sylvie Belleville for her
participation to the neuropsychological study, and Beth
Randolph for editing.
1 Bartak L, Rutter M, Cox A. A comparative study of infantile
autism and specific developmental receptive language
disorder. 1. The children. Br J Pychiatry 1975;126:127–45.
2 Baron-Cohen S, Leslie AM, Frith U. Does the autistic child
have a “theory of mind”? Cognition 1985;2:37–46.
3 Pennington BF, OzonoV S. Executive functions and
developmental psychopathology. J Child Psychol Psychiatry
1996;37:51–87.
4 Mottron L, Belleville S. Study of perceptual analysis in high
level autistic subject with exceptional graphic abilities.
Brain Cogn 1993;23:279–309.
5 Gepner B Mestre D, Masson G, De Schonen S. Postural
eVects of motion vision in young autistic children. Neuroreport 1995;6:1211–14.
6 Tantam D, Monagham L, Nicholson H, et al. Autistic children’s ability to interpret faces: a research note. J Child
Psychol Psychiatry 1989;30:623–30.
7 Hobson RP, Ouston J, Lee A. What’s in a face? The case of
autism. Br J Psychol 1988;79:441–53.
8 Boucher J, Lewis V. Unfamiliar face recognition in relatively
able autistic children. J Child Psychol Psychiatry 1992;33:
843–59.
9 Shah A, Frith U. An islet of ability in autistic children: a
research note. J Child Psychol Psychiatry 1983;24:613–20.
10 JolliVe T, Baron-Cohen S. Are people with autism and
Asperger syndrome faster than normal on the embedded
figures test? J Child Psychol Psychiatry 1997;38:527–34.
11 Young AW, Ellis HD. Visual perception. In: Segalowitz SJ,
Rapin I, eds. Handbook of neuropsychology. Vol 7. Child neuropsychology. Amsterdam: Elsevier, 1992:1–14.

559

12 Brown R, Hobson P, Lee A, et al. Are there “autistic-like”
features in congenitally blind children? J Child Psychol Psychiatry 1997 ;38:693–703.
13 Keeler WR. Autistic patterns and defective communication
in blind children with retrolental fibroplasia. In: Hoch PH,
Zubin J, eds. Psychopathology of communication. New York :
Grune and Stratton, 1958:64–84.
14 Goodman R, Ashby L. Delayed visual maturation and
autism. Dev Med Child Neurol 1990;32:814–81.
15 Cass H D, Sonksen PM, McConachie HR. Developmental
setback in severe visual impairment. Arch Dis Child
1994;70:192–6.
16 Jambaqué I, Chiron C, Dulac O, et al. Visual inattention in
West syndrome: a neuropsychological and neurofunctional
imaging study. Epilepsia 1993;34:692–700.
17 Mc Conachie R. Developmental prosopagnosia: a single
case report. Cortex 1976;12:76–82.
18 Kracke I. Developmental prosopagnosia in Asperger’s
syndrome: presentation and discussion of an individual
case. Dev Med Child Neurol 1994;36:873–86.
19 Mottron L, Décarie JC, Mineau S, et al. Overlapping
between symptoms of autism and visual agnosia: a case
study. Brain Cogn 1996;2:339–41.
20 Mottron L, Mineau S, Décarie JC, et al. Visual agnosia with
temporo-occipital brain lesions in an autistic child: a case
study. Dev Med Child Neurol 1997;39:699–705.
21 Young AW, Ellis HD. Childhood prosopagnosia. Brain Cogn
1989;9:16–47.
22 De Haan EHF, Campbell R. A 15 year follow up of a case of
developmental prosopagnosia. Cortex 1991;27:489–509.
23 Ariel R, Sadeh M. Congenital visual agnosia and prosopagnosia in a child: a case report. Cortex 1996;32:221–40.
24 Taylor DS, Falconer MA, Bruton CJ, et al. Focal dysplasia of
the cerebral cortex in epilepsy. J Neurol Neurosurg Psychiatry 1971;34:369–87.
25 Chiron C, Jambaqué I, Nabbout R, et al. The right brain is
dominant in human infants. Brain 1997;120:1057–65.
26 Wechsler D. Echelle d’Intelligence de Wechsler pour Enfants,
forme Révisée. Paris: CPA, 1981.
27 Rutter M, Le Couteur A, Lord C. Autism diagnosis
interview-AR, research. (Traduction française de Plumet
MH, Recasens C, Waller D, et al.) Paris: Ed Inserm, 1993.
28 Piven J, Harper J, Palmer P, et al. Course of behavioral
change in autism: a retrospective study of high-IQ
adolescents and adults. J Am Acad Child Adolesc Psychiatry
1996;35:523–9.
29 Lincoln AJ, Courchesne E, Kilman BA, et al. A study of
intellectual abilities in high functioning people with autism.
J Autism Dev Disord 1988;18:505–24.
30 Aram DM, Rose DF, Horwitz SJ. Hyperlexia; developmental reading without meaning. In: Malatesha RN, Whitaker
HDA, eds. Dyslexia, a global issue. The Hague: Martinus
NijhoV, 1984, 517–31.
31 Aram DM, Healy JM. Hyperlexia: a review of extraordinary
word recognition. In: Obler LK, Fein D, eds. The
exceptional brain: neuropsychology of talent and special abilities.
New York: The Guilford Press, 1988:70–102.
32 Hammill DD, Pearson NA, Voress JK. Developmental test of
visual perception. Austin: TX Pro-Ed 2e Ed, 1993.
33 Benton AL, de Hamsher K, Varney NR, et al. Facial recognition. Contributions to neuropsychological assesment. New York:
Oxford University Press, 1983:30–43.
34 Wilson B, Cockburn J, Baddeley AD. The Rivermead behavioural memory test manual. SuVolk: Thames Valley Test
Company, 1985.
35 Snoodgrass JG, Vanderwatt M. A standardized set of 260
pictures: norms for name agreement, image agreement,
familiarity and visual complexity. Journal of Experimental
Psychology Human Learning and Memory 1980;6:174–215.
36 Agniel A, Joanette Y, Doyon B, et al. Protocole MontréalToulouse d’Evaluation des Gnosies Visuelles. Paris: Ortho Edition, 1993.
37 Kaufman AS, Kaufman NL. Batterie pour l’examen psychologique de l’enfant. K-ABC. Paris, ECPA, 1993.
38 Bender MB. Defect in reversal of serial order of symbols.
Neuropsychologia 1979;17:125–38.
39 Chatelois J, Pineau H, Belleville S, et al. Batterie
informatisée de l’évaluation de la mémoire inspirée de
l’approche cognitive. Canadian Psychology 1993;34:45–53.
40 Delis CD, Kramer JH, Kaplan E, et al. California verbal
learning test. San Antonio: The Psychological Corporation,
1987.
41 Minshew NJ, Goldstein G, Taylor G, et al. Academic
achievement in high functioning individuals. J Clin Exp
Neuropsychol 1994;16:261–70.
42 Reitan RM. Trail making test results for normal and braindamaged children. Percept Mot Skills 1971;33:575–81.
43 Bauman ML, Kemper TL. Limbic and cerebellar
abnormalities: consistant finding in early infantile autism. J
Clin Exp Neuropsychol 1988;47:369.
44 Ghaziuddin M, Tsai LY, Eilers L, et al. Brief report: autism
and herpes simplex encephalitis. J Autism Dev Disord 1992;
22:107–13.
45 DeLong GR, Bean SC, Brown FR. Acquired reversible
autistic syndrome in acute encephalopathic illness in
children. Arch Neurol 1981;38:191–4.
46 Gillberg C. Onset at age 14 of a typical autistic syndrome. A
case report of a girl with Herpes simplex encephalitis. J
Autism Dev Disord 1986;16:369–75.
47 Gillberg IC. Autistic syndrome with onset at age 31 years:
Herpes encephalitis as a possible model for childhood
autism. Dev Med Child Neurol 1991;33:912–29.

560

Jambaqué, Mottron, Ponsot, et al
48 Hoon AH, Reiss AL. The mesial-temporal lobe and autism:
Case report and review. Dev Med Child Neurol 1992;34:
252–65.
49 Bolton PF, GriYths PD. Association of tuberous sclerosis of
temporal lobes with autism and atypical autism. Lancet
1997;349:392–5.
50 Gonzales RC, Welsh JT, Sepulveda AC. Autismo en la
esclerosis tuberosa. Gaceta Med Mexico 1994;130:374–9.
51 Curatolo P, Cusmai R, Cortesi F, et al. Neuropsychiatric
aspects of tuberous sclerosis. Ann New York Acad Sci 1991;
615:8–16.
52 Deonna T, Ziegler AL, Moura-Serra J, et al. Autistic
regression in relation to limbic pathology and epilepsy:
report of two cases. Dev Med Child Neurol 1993;35:166–76.
53 Chugani HT, Da Silva E, Chugani DC. Infantile spasms: III.
Prognostic implications of bitemporal hypometabolism on
positron emission tomography. Ann Neurol 1996;39:643–9.
54 DeLong G, Heinz ER. The clinical syndrome of early-life
bilateral hippocampal sclerosis. Ann Neurol 1997;42:11–17.
55 Bachevalier J. An animal model for childhood autism:
memory loss and socioemotional disturbances following
neonatal damage to the limbic system in monkey. In: Tamminga NA, Schultz SC, eds. Advances in neuropsychiatry and
psychopharmacology schizophrenia research. New York:
Raven, 1991:129–40.
56 Bachevalier J. Medial temporal lobe structures and autism: a
review of clinical and experimental findings. Neuropsychologia 1994;32:627–48.
57 Bowler DM, Matthews NJ. Asperger’s syndrome and
memory: similarity to autism but not amnesia. Neuropsychologia 1997;35:65–70.

58 Vargha-Khadem F, Gadian DG, Watkins KE, et al.
DiVerential eVects of early hippocampal pathology on episodic and semantic memory. Science 1997;277:376–80.
59 Mancini J, de Schonen S, Deruelle C, et al. Face recognition
in children with early right or left brain damage. Dev Med
Child Neurol 1994;36:156–66.
60 Schiavetto A, Décarie JC, Flessas J, et al. Childhood visual
agnosia: a seven-year follow-up. Neurocase 1997;3:1–17.
61 Baron-Cohen S,Wheelwright S, JoliVe T. Is there a language
of the eyes ? Evidence from normal adults, and adults with
Autism and Asperger syndrome. Visual Cognition 1997;4:
311–31.
62 Stuss DT, Shallice T, Alexander MP, et al. A multidisciplinary approach to anterior attentional functions. Ann N Y
Acad Sci 1995;769:191–211.
63 Horwitz B, Rumsey J, Grady C, et al. Interregional correlations of glucose utilization among brain regions in autistic
adults. Ann Neurol 1987;22:118.
64 Jambaqué I, Cusmai R, Curatolo P, et al. Neuropsychological aspects of tuberous sclerosis in relation to epilepsy and
MRI findings. Dev Med Child Neurol 1991;33:698–705.
65 Georges MS, Costa DC, Kouris K, et al. Cerebral blood
flow abnormalities in adults with infantile autism. J Nerv
Ment Dis 1992;180:413–17.
66 Zibolvicius M, Garreau B, Samson Y, et al. Delayed maturation of the frontal cortex in childhood autism. Am J
Psychiatry 1995;152:248–52.
67 Damasio AR, Maurer RG. A neurological model for
childhood autism. Arch Neurol 1978;35:77–86.