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THE LANCET

Seminar

The autistic spectrum

Lorna Wing

The autistic spectrum consists of a group of disorders of Panel 1: ICD-10 classification into subgroups
development with life-long effects and that have in F84 Pervasive developmental disorders
common a triad of impairments in: social interaction, F84·0 Childhood autism
communication, imagination, and behaviour (narrow, F84·1 Atypical autism
and repetitive pattern of behaviour).1,2 The spectrum F84·2 Rett’s syndrome
includes, but is wider than, the syndromes originally F84·3 Other childhood disintegrative disorder
described by Leo Kanner3 and Hans Asperger.4 F84·4 Overactive disorder associated with mental
The triad can occur on its own but is often retardation and stereotyped movements
accompanied by other features. It can be found together F84·5 Asperger’s syndrome
with any level of ability, from profound general learning F84·8 Other pervasive developmental disorders
disability to average or even superior cognitive skill in F84·9 Pervasive developmental disorder, unspecified
areas not directly affected by the basic impairments. It
can occur with any other physical, psychological, or simple practical skills and social adaptation from early
psychiatric condition. childhood and that abnormalities were recognisable
from the second year of life. Many clinicians diagnose
Clinical pictures on their interpretation of Asperger’s clinical
Each element of the triad has a range of manifestations. descriptions, rather than insisting on normal
The clinical picture is also affected by the individual’s development before 3 years in the areas mentioned.7
level of ability, and the number and type of additional In this article, a simple system of subgrouping based
features or other disorders. Age, sex, personality, and solely on a description of the type of social impairment
temperament, the social and physical environment, and will be used,1,8–10 which avoids the confusion inherent in
educational, psychological, and medical interventions all trying to identify the named syndromes. This simple
influence behaviour. subgrouping has proved helpful in clinical practice even
The spectrum overlaps with but covers a wider range though the groups are no more sharply differentiated
than the category of pervasive developmental disorders and permanent than in any other existing method of
in the tenth edition of the International Classification classification in the specialty.2
of Diseases (ICD-10).5 In ICD-10, pervasive
developmental disorders are divided into several sub- Aloof group
groups (panel 1). The fourth edition of the American The most easily recognised variant of the spectrum is
Psychiatric Association’s Diagnostic and Statistical seen in children who appear aloof and indifferent to
Manual (DSM IV) has definitions of pervasive others, especially their age peers, though they may
developmental disorders and sub-groups that are almost accept physical affection from familiar people. Some
the same as those in ICD-10.6 individuals with autism are profoundly disabled and
Current attempts to identify specific syndromes have little or no speech and no skills in any area apart
within the autistic spectrum, including those used in from gross motor function, though there are some who
ICD-10 and DSM IV, are unsatisfactory. The criteria are not mobile. Their repetitive behaviour usually
for distinguishing subgroups tend to be arbitrary, and consists of odd movements of limbs and body, known as
are difficult to apply and unhelpful in clinical practice. motor stereotypies, and fascination with simple sensory
The clinical picture can change with increasing age and stimuli, such as bright lights. In some, self-injury is a
in different environments. The ICD-10 classification major problem.
system aims to increase reliability by defining Asperger’s The aloof children who fit most closely into the
syndrome with the same criteria for social impairment popular picture of autism and the ICD-10 definition of
and repetitive routines as for autism but with no delay childhood autism tend to have moderate or mild
in development of speech, self help, adaptive skills, and learning disabilities, with higher levels of skill in specific
curiosity up to the age of 3 years. However, this has areas, especially visuospatial tasks or rote memory.
moved away from Asperger’s account of his syndrome. Some autistic children fit Kanner’s syndrome of early
His clinical descriptions closely resemble the group with infantile autism, narrowly defined as social aloofness
active but odd social interactions (see below), in and resistance to change in their elaborate repetitive
contrast to the aloofness and indifference of typical routines. A few of these children have cognitive ability
autism. He noted that there were difficulties in learning in the normal or even the superior range. They may
have no speech but, more typically, speech is delayed in
Lancet 1997; 350: 1761–66 onset and shows characteristic abnormalities including
The Centre for Social and Communication Disorders, Elliot House, immediate echolalia (parrot-like copying of other
113 Masons Hill, Bromley, Kent, BR2 9HT, UK people’s utterances) or delayed echolalia (repetition of
(Lorna Wing FRCPsych) words or phrases in a stereotyped way). This type of

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speech may be meaningless or may be used as a request. tractors, train timetables, mathematical calculations,
One child, for example, always said “Go for ride to dinosaurs, lamp posts, or species of birds. Some become
shops in Daddy’s green car”, to request any kind of intensely interested in a particular person, real or
outing, even when the father bought a new blue car. The fictional. Any topic can become a special interest and
tendency to echo often leads to reversal of pronouns; a the focus may change from time to time. A small
child may say, for example, “Do you want juice” when proportion tend to fantasise about their special interests,
they mean, “I want juice” because they echo the words telling muddled stories about events that never
they hear when juice is offered. Some autistic children occurred. They seem to have difficulty distinguishing
develop large vocabularies but do not use their speech these inventions from reality.
for reciprocal conversation. They do not use non-verbal Poor gross motor coordination, most noticeable when
body language to accompany or substitute for spoken attempting to play team games, is particularly common
speech. Gestures tend to be confined to pulling people in the active but odd group. Odd responses to sensory
along to use them as tools to obtain some desired object. stimuli and motor stereotypies are less common, but can
Eye contact is inappropriate for the social situation. Play occur. Behaviour difficulties, including temper tantrums
is absent or limited to a repetitive sequence of actions. and physical and verbal aggression, arise from the
The pattern of activities is dominated by repetitive egocentricity and stubborn resistance to doing anything
routines, such as putting objects into long straight lines other than their own preferred activities. The child’s
or patterns, insisting on following the same routes for repetitive talking on a favourite theme and the constant
journeys, and demanding a lengthy, unchanging bedtime asking of the same questions regardless of any replies are
routine. These routines can come to dominate family life very wearing for parents. The range of cognitive ability
because the child has terrifying temper tantrums if any in this group is wide but levels tend to be higher than for
change is introduced. the aloof children, and a larger proportion have average
In addition to the triad, other features include: or superior intelligence as measured on standardised
unusual responses to sensory stimuli, especially over tests.
sensitivity to some sounds; disturbance of sleep pattern;
refusal to eat all but a few items of food; odd posture; Loners
odd gait, such as tiptoe walking; motor stereotypies,
The most subtle form of the triad, described by Sula
such as hand and arm flapping or twisting; poor
Wolff,11 is found in people of average, high, or
attention span in general but with the ability to attend
outstanding ability, including fluent speech, who tend to
intensely to one or a few activities.
prefer to be alone, lack empathy, and be concerned with
their own interests regardless of peer-group pressures.
Passive group
Their schooldays are often stressful and difficult because
A somewhat different picture is seen in children whose they will not conform to the demands of teachers or of
social impairment presents as passivity. On ICD-10
fellow pupils. Most are happier as adults and may follow
criteria, some might be classified as childhood autism or
successful careers, sometimes of high academic
atypical autism, while others fit Asperger’s syndrome.
distinction. Some learn the rules of social interaction by
Such children do not socially interact spontaneously but
rote, while others remain solitary by choice. Some
passively accept approaches from others. They have the
marry, but partners may feel the lack of emotional
impairments of communication and imagination and
rapport. A minority develop psychiatric illnesses or
additional features similar to the aloof group, but
break the law in pursuit of their special interests. The
usually in less florid form. They tend to be more
additional features often associated with the triad are
amenable in behaviour and less obviously upset at
negligible or absent in this group, which overlaps with
interference with their repetitive routines. Some have
Asperger’s descriptions of his syndrome.
ability in the average or high range and manage to
survive mainstream school, at least in the primary years. High-functioning people with autistic disorders have
The diagnosis may be missed until problems with been able to describe their experiences of the world as a
learning and with social interaction with age peers begin confusing and frightening place.12
to emerge in secondary school.
Aetiology
Active but odd group As yet, autism spectrum disorders can be defined on
A third type of social impairment is seen in children who behavioural criteria only. There is a wide range of
make active social approaches that are naive, odd, conditions that can be associated with autistic disorders,
inappropriate, and one-sided. They tend to fit though, in the present state of knowledge, a specific
Asperger’s clinical descriptions of his syndrome. Their aetiology can be found in only a minority of cases. Even
speech is often fluent with good grammar and when a cause can be identified, this does not map onto
vocabulary but repetitive and not used for reciprocal the behaviourally defined subgroup, whatever
conversation. In a small minority, there is no history of classification system is used.
delay in speech development or adaptive skills and they In the majority of cases, parents recognise that their
could be classified as Asperger’s syndrome on the ICD- child has been different from birth or the first few
10 criteria. These children may have quite complicated months of life. However, some report a set-back, usually
play but observation shows that it is concerned only with in the second or third year of life, though occasionally
one or a few themes and usually not shared with other later, after a period of apparently normal development.13
children. Some like to watch particular videos and act The regression, or failure to progress, affects language,
out the same scenes over and over again. play, and social interaction and occasionally other skills.
The repetitive routines take the form of fascination In such cases, skilled, specific questioning about social
with and talking about particular topics, such as behaviour before the set-back often reveals

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Diagnosis
There are no physical tests for autistic
disorders. A patchy profile on cognitive
testing, tests showing absence or
impairment of theory of mind, and those
showing poor ability to comprehend or use
body language, can indicate the presence of
an autistic disorder, though good
performance on such measures in the
artificial situation of a formal test does not
exclude the diagnosis.24 Some children with
autistic disorders seen in a structured, one-
to-one situation, such as a school, clinic, or
during psychological testing, may behave
very well, smile, and look at the examiner
and show none of the overt features of

Steve Hickey
autism. If the same children are observed in
unstructured real-life settings, especially in
a group of age peers, the autistic behaviour
A pupil and teacher from a school run by the National Autistic Society becomes apparent.
Diagnosis is made by obtaining a detailed
abnormalities that the parents did not recognise, but developmental history (from infancy), from parents or
there are some children whose early development seems other informants, with particular emphasis on the
to have been entirely normal before the gradual or elements of the autism triad and repetitive behaviour.
sudden change. Semistructured interview schedules have been designed
Early theories that autism was due to being reared by for this purpose but specific training and experience is
cold and distant parents have been disproved.14 There is needed.25,26
strong evidence for a genetic cause in many, perhaps When the information has been collected, if it is to be
most, cases of typical autism.15 Available evidence used for research, the ICD-10 research criteria27 can be
suggests that genetic factors may be involved in any of applied to assess whether a pervasive developmental
the spectrum disorders.16 However, it is also clear that disorder is present and to which subgroup the individual
conditions affecting brain function—eg, tuberous belongs. However, clinically, the most important point
sclerosis17 or maternal rubella1—can be associated with to establish is whether the triad of impairments is
the autism triad.18 Epilepsy or an epileptiform present. For this purpose, clinical experience is a surer
electroencephalogram (EEG) have been noted in a guide than the rigid application of a set of rules that are,
significant minority of children with a reported set-back in any case, open to different interpretations.
in development as described above, and in a smaller Differential diagnosis includes generalised learning
minority of those without such a history.13 By the time of disabilities and specific disorders affecting language,
adult life, about a third of individuals with autistic reading, number work, motor coordination, hearing, or
disorders have had at least one epileptic seizure. These vision. Any of these conditions can occur alone but also
are most likely to occur among children and adults with in association with an autistic-spectrum disorder. When
severe learning disabilities and motor deficits as well as any aspect of development is delayed or deviant, it is
an autistic disorder. Immunisation against whooping appropriate to consider whether the autistic triad of
cough and, more recently, the triple vaccine for mumps, impairments is also present.28 An autistic disorder can
measles, and rubella have been suggested as causes, but be missed because attention is given to only one aspect
scientific evidence either for or against has not, to my of development. So-called semantic-pragmatic disorder,
knowledge, been published. Serious work in this field in which a child has fluent speech, that is used for
must be based on sound epidemiological principles, not repetitive talking about the child’s own concerns and
on anecdotes. not for reciprocal conversation, is a particular problem.
Various specific developmental syndromes can be Many workers believe that this does not occur on its
associated with some or all of the features found in own but is always part of an autistic-spectrum
autistic disorders. For example, children with Rett’s disorder.29 Attention deficit and hyperactivity,
syndrome often go through an autistic phase in the early Tourette’s syndrome, and obsessive-compulsive
years but may become more sociable later.19 The typical disorder affect movement and behaviour. They also can
behaviour pattern in the fragile-X syndrome includes occur alone or together with the triad of impairments.28
delayed echolalia, hand flapping, repetitive speech, and In adolescents and adults autistic disorders, especially
gaze avoidance, but not the social and symbolic in higher-functioning individuals, can be misdiagnosed
impairments of autistic disorders. However, the full as any kind of psychiatric condition, or they can be
picture of autism can occur in this condition.20 The missed if a psychiatric condition is superimposed on the
similarities to and differences from autistic-spectrum developmental disorder. The history and detailed
disorders may eventually provide clues to the assessment of the pattern of skills, disabilities, and
neuropathology. Neurobiological studies of individuals behaviour are required for correct diagnosis.28
with autism suggest abnormalities at the cellular level, When making a diagnosis, it is also necessary to
but not in gross brain structure.21 Necropsy studies have consider whether there are any physical abnormalities,
shown unusually large brain size in some individuals.22 including epilepsy and other specific syndromes.
Biochemical studies are as yet inconclusive.23 A common mistake made by those lacking relevant

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Panel 2: Sources of information and help area can refer to specialists or one of the small number
UK: The National Autistic Society (393 City Road, London, of special centres so that a full diagnosis and assessment
EC1V 1NE, UK; tel +44 (0)171 903 3599; fax +44(0)171 can be done.
833 9666) is a voluntary body with parent and professional Examinations for possible causes and for additional
membership. It provides a wide range of services and physical or psychological disorders should be
information on diagnostic centres, schools, residential undertaken if there are clinical indications, such as a
homes for adults, publications in the field, and many other clear history of regression in development. Clinical
matters of interest to professionals, families, and to people practices vary but, in some clinics, EEG and
with autistic disorders. The National Society also has details chromosomal investigations, including investigations for
of all local autistic societies and other relevant fragile X, are done routinely. Epileptic activity is most
organisations.
likely to be seen in a prolonged sleep EEG.13
Europe: The International Association Autism Europe, Rue E
The other part of the medical role is the treatment of
Van Becelaere 26B, Bte 21, B-1170 Bruxelles, Belgium; tel
+32 (0)2 675 7505; fax +32 (0)2 675 7270.
any associated conditions, including epilepsy, visual or
USA: Autism Society of America, 7910 Woodmont Avenue, hearing impairments, and intercurrent illnesses.
Suite 650, Bethesda, Maryland 20814, USA; tel +1 301 657 The sudden onset of disturbed behaviour may be due
0881. to physical factors, including epilepsy. Discomfort or
pain due to a physical condition may affect behaviour.
One young adult, for example, began to bite people
experience is to assume that the child’s behaviour is due though he had no previous history of aggression. This
solely to parental mishandling, especially since the continued until a dental abscess was diagnosed and
children often have an alert and attractive appearance. treated. The possibility of a physical cause for a change
For accurate diagnosis it is essential to ask the right in behaviour should always be considered.
questions systematically, listen to the parents with great Disturbed behaviour that is due to psychological
care, and treat them and their information with respect. rather than physical causes often responds to
Autistic-spectrum disorders merge into what can be appropriate environmental and behavioural
called eccentric normality and there is no clear cut-off management, which is the treatment of choice.28 If
point. In clinical practice, a diagnosis of an autistic
psychotropic medication is needed—eg, to reduce the
disorder is considered only if help is sought by parents
level of physical aggression—this should be given in
or by individuals themselves for difficulties with
addition to environmental and behavioural treatment
everyday life. In these circumstances, it is appropriate to
and for as short a time as possible. The effects should be
investigate whether, among other possibilities, the
carefully monitored because there is great variation in
difficulties are due to the types of impairments in
individual response.
psychological function that underlie autistic disorders. A
Superimposed psychiatric conditions may respond to
diagnosis in those who are managing well and are happy
removal of adverse factors in the environment but may
with their lives is of purely academic interest.
also require the relevant type of drug treatment.
Education, management, and treatment
Prevalence
There is as yet no curative treatment for autistic
disorders. Many therapies have been promulgated on A review of 16 epidemiological studies using various
anecdotal grounds but scientific evidence for their definitions of typical autism gave rates ranging from 3·3
efficacy is lacking. Controlled studies of auditory to 16·0 per 10 000.32 Those adopting the original narrow
integration training and facilitated communication have definition of Kanner found prevalences of around five
been published and these have not substantiated the per 10 000. Two further studies investigated the rates
claims made.30 for children with other autistic-spectrum disorders
The most effective way of helping the children to combined with learning disabilities and found
maximise their abilities and minimise behaviour approximately 15 per 10 000.1,33 There is only one
disturbance is through structured education designed to published study of children in mainstream schools, done
take account of their impairments and special skills.31 in Sweden, which suggests a rate of 36 in 10 000 for the
Some cope with support in mainstream schools or picture described by Asperger and 35 in 10 000 for
schools for a mixture of disabilities but many need other high functioning spectrum disorders.34 More
specialised education in schools for autistic disorders. studies are needed to evaluate the accuracy of these
Parents require information and guidance in order to estimates. One difficulty in interpreting the published
provide an organised, predictable environment at home rates is the fact that the most intensive studies have
(panel 2). After school age the adults who do not been done on small populations and the confidence
become independent need accommodation, occupation, limits are very wide, so there may be more overlap than
and leisure activities tailored to their special needs. The is often appreciated.
basic principles underlying education and management Boys and men are affected perhaps three or four times
are the same for the whole autistic spectrum, though more often than girls and women. Among those with
details differ depending on levels of ability and the type severe or profound disabilities, the male excess is less
of social impairment. pronounced than for those of higher ability.
The medical role is limited but important and most There is currently an impression that the incidence of
effective when the doctor is part of a team with other autistic-spectrum disorders is rising. This may be due to
relevant professions. The first priority is diagnosis of the wider diagnostic criteria, greater awareness of these
autistic-spectrum disorder so that the available sources disorders, or a genuine change in incidence.35 It would
of help can be contacted as early in the child’s life as be a lengthy and expensive task to do a study that could
possible. Doctors who have no particular interest in this provide the answers.

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Prognosis the pressures of daily life. Most of those with average or


Disturbed behaviour is often evident from age 2 to 5 high ability will become independent as adults but a
years, tends to improve from 6 to 10 years, but may re- minority will remain dependent to some extent, needing
appear in adolescents and young adults, eventually support in daily living, or work, or both.
calming down in middle and later life. At any age, the To my knowledge, there are no published studies of
problems can be diminished by appropriate education longevity in autistic-spectrum disorders. Life expectancy
and behavioural and environmental management. would be affected by the presence of other conditions
As noted above, psychiatric illnesses can occur in with implications for physical health, such as epilepsy or
adolescents and adults.36 Depression is particularly tuberous sclerosis.
common in response to awareness of problems in social Autistic-spectrum disorders have profound effects on
interaction and there is a risk of suicide. Catatonic states the lives of the individuals who are affected and their
of varying degrees of severity develop in a small families. Their difficulties are often intensified by lack of
proportion of cases for unknown reasons.26 A very small recognition, even denial that autism exists, among some
minority of more able individuals break the law in medical and other professional workers. The problems
pursuit of their special interests or because of paranoid are made worse by the hostility of some lay people when
feelings arising from social rejection, whether apparent observing autistic behaviour. Even the autistic
or real.36,37 individuals with high levels of ability suffer because their
The outcome for independence in adult life is closely struggle to comprehend social rules is so often
linked to overall level of ability.38 Individuals with severe misunderstood. Appropriate advice and support for
learning disabilities will remain dependent all their lives, families, specialised education, occupation, leisure and,
requiring total support and supervision in living when required, residential provision can reduce distress
accommodation, occupation, and leisure activities. and help the affected individuals develop potential skills
Those with mild learning disabilities may achieve some and lead more contented lives. The essential first step to
degree of independence in living or working but most obtaining these services is early recognition of the
are likely to need some support to help them cope with presence of the autistic triad of impairments.

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