Asperger Syndrome
By Stephen
Bauer, MD, MPH
Introduction Asperger syndrome (also called
Asperger disorder) is a relatively new category of developmental
disorder, the term having only come into more general use over the
past fifteen years. Although a group of children with this clinical
picture was originally and very accurately described in the 1940ís
by a Viennese pediatrician, Hans Asperger, Asperger syndrome (AS)
was "officially" recognized in the Diagnostic and Statistical Manual
of Mental Disorders for the first time in the fourth edition
published in 1994. Because there have been few comprehensive review
articles in the medical literature to date, and because AS is
probably considerably more common than previously realized, this
discussion will endeavor to describe the syndrome in some detail and
to offer suggestions regarding management. Students with AS are not
uncommonly seen in mainstream educational settings, although often
undiagnosed or misdiagnosed, so this is a topic of some importance
for educational personnel, as well as for parents.
Asperger syndrome is
the term applied to the mildest and highest functioning end of what
is known as the spectrum of pervasive developmental disorders (or
the Autism spectrum). Like other conditions along that spectrum it
is felt to represent a neurologically-based disorder of development,
most often of unknown cause, in which there are deviations or
abnormalities in three broad aspects of development: social
relatedness and social skills, the use of language for communicative
purposes, and certain behavioral and stylistic characteristics
involving repetitive or perseverative features and a limited but
intense range of interests. It is the presence of these three
categories of dysfunction, which can range from relatively mild to
severe, which clinically defines all of the pervasive developmental
disorders, from AS through to classic Autism. Although the idea of a
continuum of PDD along a single dimension is helpful for
understanding the clinical similarities of conditions along the
spectrum, it is not at all clear that Asperger syndrome is just a
milder form of Autism or that the conditions are linked by anything
more than their broad clinical similarities. Asperger syndrome
represents that portion of the PDD continuum which is characterized
by higher cognitive abilities (at least normal IQ by definition, and
sometimes ranging up into the very superior range) and by more
normal language function compared to other disorders along the
spectrum. In fact, the presence of normal basic language skills is
now felt to be one of the criteria for the diagnosis of AS, although
there are nearly always more subtle difficulties with
pragmatic/social language. Many researchers feel it is these two
areas of relative strength that distinguish AS from other forms of
Autism and PDD and account for the better prognosis in AS.
Developmentalists have not reached consensus as to whether there is
any difference between AS and what is termed High Functioning Autism
(HFA). Some researchers have suggested that the basic
neuropsychological deficit is different for the two conditions, but
others have been unconvinced that any meaningful distinction can be
made between them. One researcher, Uta Frith, has characterized
children with AS as having "a dash of Autism." In fact, it is likely
that there may be multiple underlying subtypes and mechanisms behind
the broad clinical picture of AS. This leaves room for some
confusion regarding diagnostic terms, and it is likely that quite
similar children across the country have been diagnosed with AS,
HFA, or PDD, depending upon by whom or where they are evaluated.
Since AS itself shows a
range or spectrum of symptom severity, many less impaired children
who might meet criteria for that diagnosis receive no diagnosis at
all and are viewed as "unusual" or "just different," or are
misdiagnosed with conditions such as Attention Deficit Disorder,
emotional disturbance, etc. Many in the field believe that there is
no clear boundary separating AS from children who are "normal but
different." The inclusion of AS as a separate category in the new
DSM-4, with fairly clear criteria for diagnosis, should promote
greater consistency of labeling in the future.
Epidemiology The best studies that have been
carried out to date suggest that AS is considerably more common than
"classic" Autism. Whereas Autism has traditionally been felt to
occur in about 4 out of every 10,000 children, estimates of Asperger
syndrome have ranged as high as 20-25 per 10,000. That means that
for each case of more typical Autism, schools can expect to
encounter several children with a picture of AS (that is even more
true for the mainstream setting, where most children with AS will be
found). In fact, a careful, population-based epidemiological study
carried out by Gillberg's group in Sweden, concluded that nearly
0.7% of the children studied had a clinical picture either
diagnostic of or suggestive of AS to some degree. Particularly if
one includes those children who have many of the features of AS and
seem to be milder presentations along the spectrum as it shades into
"normal," it seems not to be a rare condition.
All studies have agreed
that Asperger syndrome is much more common in boys than in girls.
The reasons for this are unknown. AS is fairly commonly associated
with other types of diagnoses, again for unknown reasons, including:
tic disorders such as Tourette disorder, attentional problems, and
mood problems such as depression and anxiety. In some cases there is
a clear genetic component, with one parent (most often the father),
showing either the full picture of AS or at least some of the traits
associated with AS; genetic factors seem to be more common in AS
compared to more classic Autism. Temperamental traits such as having
intense and limited interests, compulsive or rigid style, and social
awkwardness or timidity also seem to be more common, alone or in
combination, in relatives of AS children. Sometimes there will be a
positive family history of Autism in relatives, strengthening the
impression that AS and Autism are sometimes related conditions.
Other studies have demonstrated a fairly high rate of depression,
both bipolar and unipolar, in relatives of children with AS,
suggesting a genetic link in at least some cases. It seems likely
that for AS, as for Autism, the clinical picture we see is probably
influenced by many factors, including genetic ones, so that there is
no single identifiable cause in most cases.
Definition The new DSM-4 criteria for a
diagnosis of AS, with much of the language carrying over from the
diagnostic criteria for Autism, include the presence of:
• Qualitative impairment in
social interaction involving some or all of the following:
impaired use of nonverbal behaviors to regulate social
interaction, failure to develop age-appropriate peer
relationships, lack of spontaneous interest in sharing experiences
with others, and lack of social or emotional reciprocity.
• Restricted, repetitive, and
stereotyped patterns of behavior, interests, and activities
involving: preoccupation with one or more stereotyped and
restricted pattern of interest, inflexible adherence to specific
nonfunctional routines or rituals, stereotyped or repetitive motor
mannerisms, or preoccupation with parts of objects.
These behaviors must be
sufficient to interfere significantly with social or other areas of
functioning. Furthermore, there must be no significant associated
delay in either general cognitive function, self-help/adaptive
skills, interest in the environment, or overall language
development.
Christopher Gillberg, a
Swedish physician who has studied AS extensively, has proposed six
criteria for the diagnosis, elaborating upon the criteria set forth
in DSM-4. His six criteria capture the unique style of these
children, and include:
• Social impairment with extreme
egocentricity, which may include:
- Inability to
interact with peers - Lack of desire to interact with peers
- Poor appreciation of social cues - Socially and
emotionally inappropriate responses
• Limited interests and
preoccupations, including:
- More rote than
meaning - Relatively exclusive of other interests -
Repetitive adherence
• Repetitive routines or
rituals, that may be:
- Imposed on self,
or - Imposed on others
• Speech and language
peculiarities, such as:
- Delayed early
development possible but not consistently seen - Superficially
perfect expressive language - Odd prosody, peculiar voice
characteristics - Impaired comprehension including
misinterpretation of literal and implied meanings.
• Nonverbal communication
problems, such as:
- Limited use of
gesture - Clumsy body language - Limited or
inappropriate facial expression - Peculiar "stiff" gaze
- Difficulty adjusting physical proximity
• Motor clumsiness
- May not be
necessary part of the picture in all cases
Clinical Features The most obvious hallmark of
Asperger syndrome, and the characteristic that makes these children
so unique and fascinating, is their peculiar, idiosyncratic areas of
"special interest." In contrast to more typical Autism, where the
interests are more likely to be objects or parts of objects, in AS
the interests appear most often to be specific intellectual areas.
Often, when they enter school, or even before, these children will
show an obsessive interest in an area such as math, aspects of
science, reading (some have a history of hyperlexiaórote reading at
a precocious age), or some aspect of history or geography, wanting
to learn everything possible about that subject and tending to dwell
on it in conversations and free play. I have seen a number of
children with AS who focus on maps, weather, astronomy, various
types of machinery, or aspects of cars, trains, planes, or rockets.
Interestingly, as far back as Asperger's original clinical
description in 1944, the area of transport has seemed to be a
particularly common fascination (he described children who memorized
the tram lines in Vienna down to the last stop). Many children with
AS, as young as three years old, seem to be unusually aware of
things such as routes taken on car trips. Sometimes the areas of
fascination represent exaggerations of interests common to children
in our culture, such as Ninja Turtles, Power Rangers, dinosaurs,
etc. In many children the areas of special interest will change over
time, with one preoccupation replaced by another. In some children,
however, the interests may persist into adulthood, and there are
many cases where the childhood fascinations have formed the basis
for an adult career, including a good number of college professors.
The other major
characteristic of AS is the socialization deficit, and this too,
tends to be somewhat different than that seen in typical Autism.
Although children with AS are frequently noted by teachers and
parents to be somewhat "in their own world" and preoccupied with
their own agenda, they are seldom as aloof as children with Autism.
In fact, most children with AS, at least once they get to school
age, express a desire to fit in socially and have friends. They are
often deeply frustrated and disappointed by their social
difficulties. Their problem is not a lack of interaction or interest
so much as a lack of effectiveness in interactions. They seem to
have difficulty knowing how to "make connections" socially. Gillberg
has described this as a "disorder of empathy," the inability to
effectively "read" others needs and perspectives and respond
appropriately. As a result, children with AS tend to misread social
situations and their interactions and responses are frequently
viewed by others as "odd."
Although "normal"
language skills are a feature distinguishing AS from other forms of
Autism and PDD, there are usually some observable differences in how
children with AS use language. It is the more rote skills that are
strong, sometime very strong. Prosodyóthose aspects of spoken
language such as volume of speech, intonation, inflection, rate,
etc. is frequently unusual. Sometimes the language sounds overly
formal or pedantic, idioms and slang are often not used or are
misused, and things are often taken too literally. Language
comprehension tends toward the concrete, with increasing problems
often arising as language becomes more abstract in the upper grades.
Pragmatic, or conversational, language skills often are weak because
of problems with turn-taking, a tendency to revert to areas of
special interest, or difficulty sustaining the "give and take" of
conversations. Many children with AS have difficulties dealing with
humor, tending not to "get" jokes or laughing at the wrong time;
this is in spite of the fact that quite a few show an interest in
humor and jokes, particularly things such as puns or word games. The
common believe that children with pervasive developmental disorders
are humorless is frequently mistaken. Some children with AS tend to
be hyperverbal, not understanding that this interferes with their
interactions with others and puts others off.
When one examines the
early language history of children with AS there is no single
pattern: some of them have normal or even early achievement of
milestones, while others have quite clear early delays on speech
with rapid catch-up to more normal language by the time of school
entry. In such a child under the age of three years in whom language
has not yet come up into the normal range, the differential
diagnosis between AS and milder Autism can be difficult to the point
that only time can clarify the diagnosis. Frequently, also,
particularly during the first several years, associated language
features similar to those in Autism maybe seen, such as
perseverative or repetitive aspects to language or use of stock
phrases or lines drawn from previously heard material.
Asperger Syndrome Through the Lifespan
In his
original 1944 paper describing the children who later came to be
described under his name, Hans Asperger recognized that although the
symptoms and problems change over time, the overall problem is
seldom outgrown. He wrote that "in the course of development,
certain features predominate or recede, so that the problems
presented change considerably. Nevertheless, the essential aspects
of the problem remain unchanged. In early childhood there are the
difficulties in learning simple practical skills and in social
adaptation. These difficulties arise out of the same disturbance
which at school age cause learning and conduct problems, in
adolescence job and performance problems, and in adulthood social
and marital conflicts." On the other hand, there is no question that
children with AS have generally milder problems at every age
compared to those with other forms of Autism or PDD, and their
ultimate prognosis is certainly better. In fact, one of the more
important reasons to distinguish AS from other forms of Autism is
its considerably milder natural history.
The preschool
child As has been noted, there is no single, uniform presenting
picture of Asperger syndrome in the first 3-4 years. The early
picture may be difficult to distinguish from more typical Autism,
suggesting that when evaluating any young child with Autism and
apparently normal intelligence, the possibility should be
entertained that he/she may eventually have a picture more
compatible with an Asperger diagnosis. Other children may have early
language delays with rapid "catch-up" between the ages of three and
five years. Finally, some of these children, particularly the
brightest ones, may have no evidence of early developmental delay
except, perhaps, some motor clumsiness. In almost all cases,
however, if one looks closely at the child between the age of about
three and five years, clues to the diagnosis can be found, and in
most cases a comprehensive evaluation at that age can at least point
to a diagnosis along the PDD/Autism spectrum. Although these
children may relate quite normally with the family setting, problems
are often seen when they enter a preschool setting. These may
include: a tendency to avoid spontaneous social interactions or to
show very weak skills in interactions, problems sustaining simple
conversations or a tendency to be perseverative or repetitive when
conversing, odd verbal responses, preference for a set routine and
difficulty with transitions, difficulty regulating social/emotional
responses involving anger, aggression, or excessive anxiety,
hyperactivity, appearing to be "in one's own world," and the
tendency to overfocus on particular objects or subjects. Certainly,
this list is much like the early symptom list in Autism or PDD.
Compared to those children, however, the child with AS is more
likely to show some social interest in adults and other children,
will have less abnormal language and conversational speech, and may
not be as obviously "different" from other children. Areas of
particularly strong skills may be presen t, such as letter or number
recognition, rote memorization of various facts, etc.
Elementary
School The
child with AS will frequently enter kindergarten without having been
adequately diagnosed. In some cases, there will have been behavioral
concerns (hyperactivity, inattention, aggression, outbursts) in the
preschool years. There may be concern over "immature" social skills
and peer interactions, and the child may already be viewed as being
somewhat unusual. If these problems are more severe, special
education may be suggested, but probably most children with AS enter
a more mainstream setting. Often, academic progress in the early
grades is an area of relative strength; for example, rote reading is
usually good, and calculation skills may be similarly strong,
although pencil skills are often considerably weaker. The teacher
will probably be struck by the child's "obsessive" areas of
interest, which often intrude in the classroom setting. Most AS
children will show some social interest in other children, although
it may be reduced, but they are likely to show weak friend-making
and friend-keeping skills. They may show particular interest in one
or a few children around them, but usually the depth of their
interactions will be relatively superficial. On the other hand,
quite a number of children with AS present as pleasant and "nice,"
particularly when interacting with adults. The social deficit, when
less severe, may be under appreciated by many observers.
The course through
elementary school can vary considerably from child to child, and
overall problems can range from mild and easily managed to severe
and intractable, depending upon factors such as the child's
intelligence level, appropriateness of management at school and
parenting at home, temperamental style of the child, and the
presence or absence of complicating factors such as
hyperactivity/attentional problems, anxiety, learning problems, etc.
The upper
grades As
the child with AS moves into middle school and high school, the most
difficult areas continue to be those related to socialization and
behavioral adjustment. Paradoxically, because children with AS are
frequently managed in mainstream educational settings, and because
their specific developmental problems may be more easily overlooked
(especially if they are bright and do not act too "strange"), they
are often misunderstood at this age by both teachers and other
students. At the secondary level, teachers often have less
opportunity to get to know a child well, and problems with behavior
or work/study habits may be misattributed to emotional or
motivational problems. In some settings, particularly less familiar
or structured ones such as the cafeteria, physical education class,
or playground, the child may get into escalating conflicts or power
struggles with teachers or students who may not be familiar with
their developmental style of interacting. This can sometimes lead to
more serious behavioral flare-ups. Pressure may build up in such a
child with little clue until he then reacts in a dramatically
inappropriate manner.
In middle school, where
the pressures for conformity are greatest and tolerance for
differences the least, children with AS may be left out,
misunderstood, or teased and persecuted. Wanting to make friends and
fit in, but unable to, they may withdraw even more, or their
behavior may become increasingly problematic in the form of
outbursts of noncooperation. Some degree of depression is not
uncommon as a complicating feature. If there are no significant
learning disabilities, academic performance can continue strong,
particularly in those areas of particular interest; often, however,
there will be ongoing subtle tendencies to misinterpret information,
particularly abstract or figurative/idiomatic language. Learning
difficulties are frequent, and attentional and organizational
difficulties may be present.
Fortunately, by high
school, peer tolerance for individual variations and eccentricity
often increases again to some extent. If a child does well
academically, that can bring a measure of respect from other
students. Some AS students may pass socially as "nerds," a group
which they actually resemble in many ways and which may overlap with
AS. The AS adolescent may form friendships with other students who
share his interests through avenues such as computer or math clubs,
science fairs, Star Trek clubs, etc. With luck and proper
management, many of these students will have developed considerable
coping skills, "social graces," and general ability to "fit in" more
comfortably by this age, thus easing their way.
Asperger
children grown up It is important to note that we
have limited solid information regarding the eventual outcome for
most children with AS. It has only been recently that AS itself has
been distinguished from more typical Autism in looking at outcomes,
and milder cases were generally not recognized. Nevertheless, the
available data does suggest that, compared to other forms of
Autism/PDD, children with AS are much more likely to grow up to be
independently functioning adults in terms of employment, marriage,
and family, etc.
One of the most
interesting an useful sources of data on outcome comes indirectly
from observing those parents or other relatives of AS children, who
themselves appear to have AS. From these observations it is clear
that AS does not preclude the potential for a more "normal" adult
life. Commonly, these adults will gravitate to a job or profession
that relates to their own areas of special interest, sometimes
becoming very proficient. A number of the brightest students with AS
are able to successfully complete college and even graduate school.
Nonetheless, in most cases they will continue to demonstrate, at
least to some extent, subtle differences in social interactions.
They can be challenged by the social and emotional demands of
marriage, although we know that many do marry. Their rigidity of
style and idiosyncratic perspective on the world can make
interactions difficult, both in and out of the family. There is also
the risk of mood problems such as depression and anxiety, and it is
likely that many find their way to psychiatrists and other mental
health providers where, Gillberg suggests, the true, developmental
nature of their problems may go unrecognized or misdiagnosed.
In fact, Gillberg has
estimated that perhaps 30-50% of all adults with AS are never
evaluated or correctly diagnosed. These "normal Aspergers" are
viewed by others as "just different" or eccentric, or perhaps they
receive other psychiatric diagnoses. I have met a number of
individuals whom I believe fall into that category, and I am struck
by how many of them have been able to utilize their other skills,
often with support from loved ones, to achieve what I consider to be
a high level of function, personally and professionally. It has been
suggested that some of these highest functioning and brightest
individuals with AS represent a unique resource for society, having
the single mindedness and consuming interest to advance our
knowledge in various areas of science, math, etc.
Thoughts on Management in the School
The most
important starting point in helping a student with Asperger syndrome
to function effectively in school is for the staff (all who will
come into contact with the child) to realize that the child has an
inherent developmental disorder which causes him/her to behave and
respond in a different way from other students. Too often, behaviors
in these children are interpreted as "emotional," or "manipulative,"
or with some other term that misses the point that they respond
differently to the world and its stimuli. It follows that school
staff must carefully individualize their approach for each of these
children; it will not work to treat them just the same as other
students. Asperger himself realized the central importance of
teacher attitude from his own work with these children. In 1944 he
wrote, "These children often show a surprising sensitivity to the
personality of the teacher" They can be taught, but only by those
who give them true understanding and affection, people who show
kindness towards them and, yes, humour "The teacher's underlying
emotional attitude influences, involuntarily and unconsciously, the
mood and behavior of the child."
Although it is likely
that many children with AS can be managed primarily in the regular
classroom setting, they often need some educational support
services. If learning problems are present, resource room or
tutoring can be helpful, to provide individualized explanation and
review. Direct speech services may not be needed, but the speech and
language clinician at school can be useful as a consultant to the
other staff regarding ways to address problems in areas such as
pragmatic language. If motor clumsiness is significant, as it
sometimes is, the school Occupational Therapist can provide helpful
input. The school counselor or social worker can provide direct
social skills training, as well as general emotional support.
Finally, a few children with very high management needs may benefit
from the assistance of a classroom aide assigned to them. On the
other hand, some of the higher functioning children and those with
milder AS, are able to adapt and function with little in the way of
formal support services at school, if staff are understanding,
supportive, and flexible.
There are a number of
general principles of school management for most children with PDD
of any degree which apply to AS, as well:
• The classroom routines should
be kept as consistent, structured, and predictable as possible.
Children with AS often don't like surprises. They should be
prepared in advance, when possible, for changes and transitions,
including things such as schedule breaks, vacation days,
etc.
• Rules should be applied
carefully. Many of these children can be fairly rigid about
following "rules" quite literally. While clearly expressed rules
and guidelines, preferably written down for the student, are
helpful, they should be applied with some flexibility. The rules
do not automatically have to be exactly the same for the child
with AS as for the rest of the students–their needs and abilities
to conform are different.
• Staff should take full
advantage of a child's areas of special interest when teaching.
The child will learn best and show greatest motivation and
attention when an area of high personal interest is on the agenda.
Teachers can creatively connect the child's interests to the
teaching process. One can also use access to the special interests
as a reward to the child for successful completion of other tasks
or adherence to rules or behavioral expectations.
• Most students with AS respond
well to the use of visuals: schedules, charts, lists, pictures,
etc. In this way they are much like other children with PDD and
Autism.
• In general, try to keep
teaching fairly concrete. Avoid language that may be misunderstood
by the child with AS, such as sarcasm, confusing figurative
speech, idioms, etc. Work to break down and simplify more abstract
language and concepts.
• Explicit, didactic teaching of
strategies can be very helpful, to assist the child gain
proficiency in "executive function" areas such as organization and
study skills.
• Insure that school staff
outside the classroom, such as physical education teachers, bus
drivers, cafeteria monitors, librarians, etc., are familiar with
the child's style and needs and have been given adequate training
in management approaches. Those less structured settings where the
routines and expectations are less clear tend to be difficult for
the child with AS.
• Try to avoid escalating power
struggles. These children often do not understand rigid displays
of authority or anger and will themselves become more rigid and
stubborn if forcefully confronted. Their behavior can then get
rapidly out of control, and at that point it is often better for
the staff person to back off and let things cool down. It is
always preferable, when possible, to anticipate such situations
and take preventative action to avoid the confrontation through
calmness, negotiation, presentation of choices, or diversion of
attention elsewhere.
A major area of concern
as the child moves through school is promotion of more appropriate
social interactions and helping the child fit in better socially.
Formal, didactic social skills training can take place both in the
classroom and in more individualized settings. Approaches that have
been most successful utilize direct modeling and role playing at a
concrete level (such as in the Skillstreaming curriculum). By
rehearsing and practicing how to handle various social situations,
the child can hopefully learn to generalize the skills to
naturalistic settings. It is often useful to use a dyad approach
where the child is paired with another student to carry out such
structured encounters. The use of a "buddy system" can be very
useful, since these children relate best 1-1. Careful selection of a
non-Asperger peer buddy for the child can be a tool to help build
social skills, encourage friendships, and reduce stigmatization.
Care should be taken, particularly in the upper grades, to protect
the child from teasing both in and out of the classroom, since it is
one of the greatest sources of anxiety for older children with AS.
Efforts should be made to help other students arrive at a better
understanding of the child with AS, in a way that will promote
tolerance and acceptance. Teachers can take advantage of the strong
academic skills that many AS children have, in order to help them
gain acceptance with peers. It is very helpful if the AS child can
be given opportunities to help other children at times.
Although most children
with AS are managed without medication and medication does not
"cure" any of the core symptoms, there are specific situations where
medication can occasionally be useful. Teachers should be alert to
the potential for mood problems such as anxiety or depression,
particularly in the older child with AS. Medication with an
antidepressant (e.g., imipramine or one of the newer serotonergic
drugs such as fluoxetine)may be indicated if mood problems are
significantly interfering with functioning. Some children with
significant compulsive symptoms are ritualistic behaviors can be
helped with the same serotonergic drugs or clomipramine. Problems
with inattention at school that are seen in certain children can
sometime be helped by stimulant medications such as methylphenidate
or dextroamphetamine, much in the same way they are used to treat
Attention Deficit Disorder. Occasionally, medication may be needed
to address more severe behavior problems that have not responded to
non-medical, behavioral interventions. Clonidine is one medication
that has proven helpful in such situations, and there are other
options if necessary.
In attempting to put a
comprehensive teaching and management plan into place at school, it
is helpful for staff and parents to work closely together, since
parents often are most familiar with what has worked in the past for
a given child. It is also wise to put as many details of the plan as
possible into an Individual Educational Plan so that progress can be
monitored and carried over from year to year. Finally, in devising
such plans, it can sometimes be helpful to enlist the aid of outside
consultants familiar with the management of children with Asperger
syndrome and other forms of PDD, such as behavioral consultants,
psychologists, or physicians. In complex cases a team orientation is
always advisable. |