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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.
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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.
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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
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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.
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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.
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