Mirkovic 2019
Mirkovic 2019
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a r t i c l e i n f o a b s t r a c t
Article history: Asperger’s syndrome is a neurodevelopmental disorder which is part of the large family of autism spec-
Received 9 June 2018 trum disorders. People with Asperger’s syndrome have difficulties in social interactions, verbal and
Accepted 11 November 2018 non-verbal communication, and may display behavioural oddities, with stereotypies and limited inter-
Available online 5 February 2019
ests. They show no language delay and their cognitive development is not marked by an overall delay
but by specific impairments in certain areas such as the executive functions. The clinical presentations
Keywords: are very heterogeneous, varying according to age and psychiatric comorbidities. Screening, diagnosis and
Autism spectrum disorders
specialized treatment are not made any easier by the diversity of the clinical manifestations. Asperger’s
Asperger’s syndrome
Developmental disorders
syndrome is often diagnosed belatedly, at 11 years of age on average and even in adulthood in some
ADHD cases. This late diagnosis has a significant impact on the risks of depression and a poor quality of life.
However, in adulthood or in adolescence, certain situations, personality traits and cognitive profiles or
certain comorbidities should suggest the hypothesis of an Asperger-type autism spectrum disorder. We
propose here a review of the clinical situations at different ages of life that could help with the screening
and the referral of patients to specialized clinicians for diagnosis and appropriate treatment.
© 2019 L’Encéphale, Paris.
https://doi.org/10.1016/j.encep.2018.11.005
0013-7006/© 2019 L’Encéphale, Paris.
170 B. Mirkovic and P. Gérardin / L’Encéphale 45 (2019) 169–174
In addition, DSM-V [6] introduces a new diagnostic entity into intimidation and bullying by their peers at school [17]. More
the “language disorders” category: “social (pragmatic) communi- generally, children with cognitive and physical impairments have
cation disorder”. The diagnostic criteria for this disorder partly a greater risk of suffering intimidation and social exclusion than
include those for ASDs, since children diagnosed with a social com- their non-disabled peers. In addition, the disabled children report
munication disorder have a language pragmatics disorder together that peer relations and exclusion from social groups are permanent
with a disorder concerning the “social use of verbal and non-verbal problems throughout their school life [18].
communication”. Nevertheless, the additional presence of obses- Children with ASD-AS often have difficulty deciphering the sub-
sive interests and repetitive behaviours precludes the possibility tleties of non-verbal language such as tone of voice, gestures, facial
of diagnosing a social communication disorder. The presence of expressions and body language. They make a literal interpretation
repetitive behaviours is therefore essential for making a differential of speech and do not understand jokes well. They easily become
diagnosis of autism. a target of mockery and are more vulnerable than others to rejec-
The most common criticism of the new definition of ASDs is tion by their peers. Their contributions to conversations are often
the narrowness of the new criteria, which may prevent a num- inappropriate or out of phase. In addition, their limited and some-
ber of patients from being diagnosed with autism and therefore times unusual interests (trains, science, astronomy, insects, etc.),
limit their access to the services they need [11]. The second contro- their stereotypical behaviour, their conversations in the form of
versial decision in the eyes of the scientific community, including monologues spoken with an unnatural intonation tend to exacer-
the American Academy of Child and Adolescent Psychiatry, is the bate their difference, increase their isolation and make them more
disappearance of the specific diagnostic scheme for Asperger’s syn- vulnerable to intimidation and bullying. It is not unusual to see chil-
drome. Indeed, some Asperger patients do not meet the ASD criteria dren or adolescents with Asperger’s syndrome who have developed
and will therefore not receive treatment, while others claim that significant anxiety and depressive symptoms as a result of their
the new inclusion of Asperger’s syndrome in the category of autism relational difficulties, leading in some cases to them dropping out
spectrum disorders without any distinction has a negative effect on of school. Families are often helpless to cope with these situations
the identity of the persons affected. of school violence and an emergency withdrawal from school, even
However, Asperger’s syndrome is a well-defined clinical entity though it is a poor solution, becomes necessary.
with a rich semiology and relatively characteristic clinical presen- Yet there are initiatives to integrate Asperger children into ordi-
tations. All over the world, teams working on Asperger’s syndrome nary schools which have shown interesting results. However, this
continue to use this diagnosis as a subtype of ASD with no language active approach can be a major dilemma for parents, since there
delay and a normal or superior IQ [12]. The prevalence of Asperger’s is a disparity between the child’s successful adjustment to the
syndrome is estimated to be 0.06% in the general population [13], educational requirements of an ordinary school and the difficul-
psychiatric comorbidities are frequent and the long-term prognosis ties they have with social integration. From a therapeutic point of
is marked by high risk of morbidity and mortality [14]. view, social skills training encompasses a set of strategies aimed
We propose here a review of the clinical situations at differ- at improving interpersonal relationships and understanding social
ent ages of life that could help with the screening and the referral situations by supporting the acquisition of specific skills [19].
of patients to specialized clinicians for diagnosis and appropriate
treatment. For greater clarity, we shall in this paper use the term
Autistic Spectrum Disorder of an Asperger’s Syndrome type (ASD- 2.2. Limited fields of interest and awkwardness-clumsiness
AS).
Some children with ASD-AS have the skills to develop symbolic
play. However, their games are repetitive and stereotypical, and
2. In children and adolescents are not creative. They can play the game of “pretending” with no
modification for days on end. Moreover, play is rarely used with the
2.1. Social isolation and bullying at school aim of social interaction [20]. Their interests are usually limited, in
the sense that they have preoccupations which are abnormal by
Friendship is an important social experience for children and their intensity or their rigor, focusing on one or more stereotyped
adolescents, constituting an essential emotional relationship based interests. Children with ASD-AS are very good at memorizing and
on stable reciprocal interactions in the context of a close and rela- are often attracted to specific subjects such as meteorology, techni-
tively long-term relationship. Friendship offers children a context cal train data, music, mathematics, mechanics, orientation in space,
in which they can develop and practice their social skills, including dinosaurs, or history. These interests may change in content over
concern for others, camaraderie and empathy. Friendship requires the years but the basic pattern remains constant. They are some-
both good social adaptability and a good understanding of emotions times so intense that they prevent accomplishment or they lead
[15]. However, children with an ASD-AS have difficulties with all of to a lack of motivation for other activities, making the youngster
these skills. They will have difficulty initiating play with their peers appear immature and compromising his social relationships. They
and will prefer structured activities that have clear and explicit often collect stamps, insects, and cards of all kinds [21].
rules requiring little interaction. Therefore, they may have limited Although these are not part of the diagnostic criteria, children
experience with their peers because of their lack of social skills and with ASD-AS often have motor coordination disorders. There is,
thus struggle to build friendships. moreover, a diagnostic continuum with children with a specific
In spite of this, research has shown that children with ASD-AS motor development disorder (dyspraxia) [22]. Because of their
are interested in relationships, ask themselves questions as to why motor deficits, young people with ASD-AS have little interest in
they are alone and identify the feeling of loneliness at an early stage sports and competitive games. Indeed, their motor movements
[16]. Some want to have a friend, but do not realize that this neces- are clumsy and uncoordinated. Their posture and their gait seem
sarily involves common interests, not deciding on activities alone, odd. In addition, many of them have difficulty performing activities
not being rigid and letting the friend make choices. which require fine motor skills such as writing, tying laces, fasten-
Given the challenge represented by friendship, children with ing shirt buttons or drawing. On the sensory level, several children
ASD-AS are at greater risk of isolation and relational difficulties with ASD-AS show sensory hypo- or hypersensitivity, which may
with their peers. Several studies have shown that over 40% of lead to difficulties in certain situations where the level of sensory
children with autism spectrum disorders have been victims of stimulation is high.
B. Mirkovic and P. Gérardin / L’Encéphale 45 (2019) 169–174 171
2.3. ADHD children and adolescents with significant relational ability to make inferences and understand the relationship between
difficulties different concepts independently of acquired knowledge). Contrary
to popular belief, not all Asperger’s are geniuses. There is consider-
Attention deficit disorder with or without hyperactivity disorder able inter-individual variability (below and above average) in most
(ADHD) is a common neurodevelopmental disorder, considered to cognitive tasks [29]. ASD-AS individuals have deficits in tasks that
be the most frequent psychic disorder in children and adolescents, require comprehensive treatment, such as the complex Rey figure.
with a prevalence varying from 3.5 to 5%, depending on the authors As regards adults, Bowler et al. [30] showed that adults tend to
[23]. ADHD includes a spectrum of behavioural, cognitive and emo- organise information in a singular way. Encoding and storing word
tional dysfunctions that revolve around three main symptoms: lists appear to be particularly characteristic. They cannot organise
impulsivity, motor hyperactivity and inattention. It is associated the recall of words by semantic or associative links, but only by the
with family, schooling or social impacts, and its evolution is chronic, structure of lists.
and sometimes persistent in adulthood.
According to the Larson et al. [24] survey, over two-thirds of 2.5. Sensory and perceptual particularities
ADHD children and adolescents have an associated psychopatho-
logical disorder or learning disability. Comorbidities should be Unusual sensory experiences are very frequently observed in
systematically investigated; they are frequent and may affect the autistic subjects, with over 90% of them [31] seeming to have sen-
prognosis. It is thus important to note that neither ICD-10 [25] nor sory peculiarities which, according to certain authors, could explain
DSM IV-TR [26] accepted the diagnostic association of autistic dis- many of their basic symptoms. Abnormal sensory perceptions could
orders with ADHD. The distinctive characteristics of inattention lead to serious anxiety, resulting in repetitive and stereotyped
and/or hyperactivity/impulsivity sometimes observed in subjects behaviours. Sensory disorders may affect all senses, perceptions
with an ASD must then be directly attributed to the autistic disor- may be increased or decreased and there is, furthermore, consid-
der itself, and vice versa. Nevertheless, in everyday practice, strict erable disparity between different subjects. The high variability
adherence to this category-related algorithmic rigidity leads to and fluctuation of sensory disorders require an informed diagnostic
numerous diagnostic but also therapeutic shortcomings for these approach [32].
subjects. Thus, and despite this restriction, many studies have long Hypersensitivity is frequently observed in the fields of hearing
since shown these co-occurring phenomena. On the basis of these (noisy environments, vacuum-cleaning, etc.), taste (food selectiv-
findings, the DSM-V integrated the possibility of a diagnostic co- ity), touch (strongly reacting to the contact of certain types of
occurrence into its decision algorithm and minimally included a clothing, to affectionate physical contacts, or to having their hair
dimensional perspective. cut) and smell (body odour). There is also a significant deterioration
In addition, several symptoms overlap in ADHD and Asperger’s in movement performance and in proprioceptive and vestibular
syndrome. Both disorders may lead to a decrease in attention level: processing in AS subjects [33]. It is likewise possible to find all these
e.g. high distractibility for ADHD sufferers and a lack of mental flex- examples as manifestations of hyposensitivity, such as lower pain
ibility for ASD-AS subjects. Similarly, for relational difficulties, the thresholds (with certain injuries going unnoticed and becoming
explanations vary according to the main diagnosis. ADHD subjects infected), indifference to temperature (wearing a t-shirt in winter)
are impulsive, impatient, have trouble waiting for their turn or lis- or vestibular symptoms (rocking or circular movements).
tening to others and as a result, adjusting their behaviour becomes These sensory disorders are considered as normal by the sub-
difficult. jects themselves, and awareness of them may sometimes occur at
ASD-AS subjects, have a difficulty of treatment of the informa- a very late stage, around the age of 20.
tion of the emotion so they do not response as it is expected, that According to Ozonoff et al. [34], autistic subjects focus on details
can lead to complications in social interactions. rather than the overall picture, on a level which is more conceptual
Patients with either disorder often show the special ability to than perceptual. Sensory information is received with a super-
engage their interests very deeply: it is typical for ASD-AS subjects abundance of simultaneous details, thus leading us to speak of
to develop “special interests”, while ADHD patients tend to “hyper ‘Gestalt perception’. Gestalt perception may be invasive and lead to
focus” on subjects that interest them. many kinds of deformation in the processing of information, such
In the end, the two types of situations (“overlap of symptoms” as fragmented or distorted perception, hyper- or hyposensitivity,
or “association of the two disorders”) mean that, when diagnosing fluctuation or sensory agnosia.
ADHD with serious relational difficulties, ASD-AS should be sought. Visually, for example, changing a minor detail will cause a
change in the entire scene (Gestalt). This may explain the intoler-
2.4. Heterogeneous cognitive assessment ance to small changes observed in people with autism. In terms of
hearing, autistics with Gestalt perception will have great difficulty
The heterogeneity of the neurocognitive profile appears to be a concentrating on auditory stimuli in a noisy environment.
constant in children with AS with significant inequalities. The ver- In other words, they have trouble with sorting, as they cap-
bal IQ is generally clearly higher than the performance IQ [27]. In ture all stimuli indiscriminately. It is thus easy to understand that
the past, these significant disparities concerning standard assess- this operating mode will lead to sensory overload that may quickly
ment tools have led some to put forward aberrant interpretations become unbearable and give rise to atypical behaviours and avoid-
such as “weak central coherence” and incompatibility with true ance strategies.
human intelligence. In recent years these assumptions have been
seriously questioned through surveys using the standard Wech-
sler scales and Raven Progressive matrices, an important marker of 3. In adult population
intelligence which is more suitable for ASD patients [28]. The intel-
ligence of the autistic spectrum is atypical, but also authentic and In practice, the diagnosis of ASD-AS in the adult population is
generally underestimated. difficult and is rarely mentioned in general medicine. There are
It appears that ASD-AS subjects have deficiencies in their exec- several obstacles:
utive functions (planning or mental flexibility), theory of mind and
overall information processing, while other strengths are usually • treating physicians lack information about developmental his-
observed in the areas of problem solving or fluid intelligence (the tory;
172 B. Mirkovic and P. Gérardin / L’Encéphale 45 (2019) 169–174
• the lack of knowledge of autistic disorders, but also the lack of seemingly hermetic discourse dotted with strange neologisms and
reference to international classifications; explanations may appear similar to the thought disorder which is
• the complexity of symptoms and clinical services; characteristic of schizophrenia.
• entanglement with other psychiatric comorbidities (depres- However, despite the clear distinction between the two disor-
sion, social anxiety, schizoid or schizotypal personality disorder, ders, ASD-AS subjects may display transient psychotic episodes,
chronic tics, emotional lability with aggressiveness, atypical eat- with a sense of depersonalisation and derealisation, which may be
ing disorders, ADHD, dyspraxia). accompanied by transient hallucinatory experiences. In the event
of repeated occurrence of these episodes, antipsychotic treatment
There is a significant delay in diagnosis and it is not uncom- may be justified. In a French study of 122 adult ASD patients, 67
mon for the diagnosis to be made in adulthood. One of the possible of whom were Asperger’s, 12% had a comorbid psychotic disorder
reasons for this delay can be explained by the fact that ASD-AS (lifetime prevalence) [35]. Interestingly, in this same study, nearly
individuals have succeeded in partially compensating for their 50% had an anxiety disorder or a mood disorder, 43% had ADHD,
social difficulties. Indeed, they are generally of medium or supe- 24% had obsessive-compulsive disorder, and 20% had chronic tics.
rior intelligence and are able, to a certain extent, to mask their Concerning schizoid and schizotypal personality disorders, the
deficit in social communication skills through stereotyped learn- distinction is equally complex due to overlapping symptoms. It
ing processes of explicit situational rules. Thus, 50 to 80% of people is important to note that people with these two personality dis-
with ASD live independently, nearly 80% have been through higher orders may not meet the diagnostic criteria for schizophrenia: in
education, and almost 50% have had intimate interpersonal rela- particular, they have no hallucinations or delusions.
tionships [35,36]. However, as the social environment becomes In the study by Barneveld et al. [41], 40% of a group of ASD
more and more complex, this mode of operation is difficult to main- patients also had the criteria for a schizotypal personality disor-
tain and becomes increasingly complicated, especially in terms of der. Schematically, schizotypal subjects are described as eccentric,
anxiety and depression. with cognitive distortions and feelings of persecution due to a ques-
In addition, the compensatory strategies eventually fail because tionable tendency to “over-interpret” details and social cues such
of their excessive rigidity and do not withstand the significant as voice intonation, mimicry, facial expressions and gesticulation.
changes that occur during the course of life (leaving the parental Conversely, Asperger subjects tend to “under-interpret” these same
home, starting a professional activity or living in a couple). cues.
The warning signs are most evident in the field of social inter-
actions:
3.2. Atypical sexuality
• the resort to university medicine or occupational physicians for
In terms of sexuality, the prevalence of homosexuality and
failures to understand the relational world and in particular social
bisexuality among young people with ASD appears to be identi-
codes;
cal to that of other male adolescents and young adults [42]. This
• the frequency of the impressions and realities of harassment,
suggests that sexuality in individuals with ASD-AS should not be
particularly at the workplace;
considered to be significantly different from that in subjects with
• the incomprehension of humour, implicit meaning, or irony;
normal development. It is important to remember that sexual and
• social withdrawal which is incompatible with staying in work,
romantic desires exist in people with autism. Although they seek
whereas the individual would be intellectually capable of doing
to establish love relationships, they do not know how to go about
so.
it and their attitude may be seen as bizarre or disturbing. They lack
the social cues that would enable them to understand what can and
Given the lack of specificity of the warning signs in adults,
cannot be done in public, such as undressing, masturbating or talk-
it is important, considering the subject’s persistent difficulties in
ing about sex inappropriately. Their lack of understanding of social
social adaptation and communication with his environment, to also
situations leads to inappropriate attempts at seduction, which may
envisage the possibility of an ASD-AS. A number of diagnostic tools
be perceived as harassment by some.
are used in practice for screening. The Autism Spectrum Quotient
Moreover, the sexual development of ASD subjects can also be
(AQ) [37], the Empathy Quotient (EQ) [38], and the Systemizing
influenced by repetitive, stereotyped behaviours and sensory fas-
Quotient Revised (SQ-R) [39], can be used with high-level autistic
cinations [43]. This can lead to compulsive masturbation, possibly
subjects as a screening questionnaire, pending confirmation of the
with an object, as in fetishism, obsessions with sexual references, or
diagnosis.
abnormal sexual fears. According to a Dutch study, clinics receiving
transgender subjects showed a higher prevalence of ADS subjects
3.1. Asperger’s syndrome or schizophrenia?
[44].
ASD-AS individuals who have not received help to adapt their
The differential diagnosis between schizophrenia and
sexual behaviour so as to be socially acceptable may find them-
Asperger’s syndrome is not always easy to make [40]. People
selves in illegal situations with a risk of prosecution and thymic
with Asperger’s syndrome are singular in their style of contact
complications.
because of their difficulties in expressing their emotions in an
appropriate way. The apparent absence of affects and the lack of
visual contact and facial expressiveness may give an impression of 3.3. At work
oddity that can be mistaken for the discordance of schizophrenia.
Similarly, the alteration of social interactions may be difficult to In spite of developing linguistic skills in terms of grammar or
distinguish from the withdrawal periods observed in schizophre- vocabulary, ASD-AS subjects lack understanding of the pragmatic
nia. However, Asperger subjects are more likely to develop (implicit or implied) and semantic (irony or metaphor) content, so
conversations when it comes to their favorite subject. Their speech that communication tends to be very formalised. They are direct
is well organised around precise topics and is not delusional, but and do not seek to avoid offending when reporting mistakes and
it abounds in details and turns into an interminable monologue. blunders to their colleagues or supervisors, particularly since they
Finally, the impairment of communication skills in Asperger’s fail to understand the unspoken and implicit aspects of their social
syndrome, with singular, uninformative spoken language and a relations.
B. Mirkovic and P. Gérardin / L’Encéphale 45 (2019) 169–174 173
They display a number of weaknesses that are frequently prob- 5. Search strategy and selection criteria
lematic in the professional sphere:
We identified references by searching PubMed and PsycINFO,
for articles published from January, 2000, to June 2017. We used
• social naivety leading to a risk of being manipulated, scapegoating
the search terms “autism”, “autism spectrum disorder”, “perva-
by their colleagues and the risk of being a victim of calumny in
sive developmental disorder”, “Asperger syndrome”, and “child”,
various forms;
“adolescent” and “adult”. We identified relevant earlier articles by
• the refusal to stand up for themselves and compete with others;
searching the reference lists of retrieved papers.
• reluctance towards sudden changes: doing an unexpected task
can be a source of anxiety.
Disclosure of interest
These weaknesses require precautions to be taken as to the The authors declare that they have no competing interest..
choice of certain occupations and the suitability of work-positions:
difficulties with teamwork (preference for positions where they
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