ASPERGER'S SYNDROME

What is Aspergerís Syndrome? Aspergerís Syndrome, also known as Aspergerís Disorder or Autistic Psychopathy, is a Pervasive Developmental Disorder (PDD) characterized by severe and sustained impairment in social interaction, development of restricted and repetitive patterns of behavior, interests, and activities. These characteristics result in clinically significant impairment in social, occupational, or other important areas of functioning.

In contrast to Autistic disorder (Autism), there are no clinically significant delays in language or cognition or self help skills or in adaptive behavior, other than social interaction. Prevalence is limited but it appears to be more common in males. Onset is later than what is seen in Autism, or at least recognized later. Motor delays, clumsiness, social interaction problems, and idiosyncratic behaviors are reported. Adults with Aspergerís have trouble with empathy and modulation of social interaction - the disorder follows a continuous course and is usually lifelong. Aspergers is not easily recognizable - in fact, many children are misdiagnosed with other PDDís such as Touretteís Syndrome, or, more seriously, with Autism and/or Attention Deficit (and Hyperactivity) Disorder (ADD & ADHD).

Because it is so new and so difficult to diagnose, our society is ill-equipped to deal with the special educational needs of children afflicted with Aspergerís.

Aspergerís Disorder is a milder variant of Autistic Disorder. Both Aspergerís Disorder and Autistic Disorder are in fact subgroups of a larger diagnostic category. This larger category is called either Autistic Spectrum Disorders or Pervasive Developmental Disorders. In Aspergerís Disorder, affected individuals are characterized by social isolation and eccentric behavior in childhood. There are impairments in two-sided social interaction and non-verbal communication. Though grammatical, their speech is peculiar due to abnormalities of inflection and a repetitive pattern. Clumsiness is prominent both in their articulation and gross motor behavior. They usually have a circumscribed area of interest which usually leaves no space for more age appropriate, common interests. Some examples are cars, trains, French Literature, door knobs, hinges, cappucino, meteorology, astronomy or history.

What is the epidemiology of Aspergerís Disorder? In a total population study of children between ages 7-16 in Goteborg, Sweden minimum prevalence of Aspergerís Disorder was 0.36 % (0.55 % of all boys, and 0.15 % of all girls), and male/female ratio was 4:1. In another total population study, prevalence of Autistic Disorder was 0.024 % in Canada.

What are the differences between Aspergerís Disorder and ĎHigh Functioningí(i.e. IQ > 70) Autism?

It is believed that in Aspergerís Disorder:

  1. The onset is usually later
  2. outcome is usually more positive
  3. social and communication deficits are less severe
  4. circumscribed interests are more prominent
  5. verbal IQ is usually higher than performance IQ (in autism, the case is usually the reverse)
  6. clumsiness is more frequently seen
  7. family history is more frequently positive
  8. neurological disorders are less common

WHAT IS THE BIOLOGY OF ASPERGER'S DISORDER?

Despite the now widely accepted fact that biological factors are of crucial importance in the etiology of autism, so far the brain imaging studies have shown no consistent pattern, no consistent evidence of any type of lesion, and no single location of any lesion in subjects with autistic symptoms. This inconsistency in the results of various brain imaging studies has been attributed to the fact that people with autism represent a highly heterogeneous group in terms of underlying pathology. Therefore there is an ongoing effort to specify more homogenous subgroups among autistic individuals to enhance the accuracy of etiologic inquiry. This approach has been supported with the inclusion of the diagnosis ĎAspergerís Disorderí in the Fourth Edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) of the American Psychiatric Association.

Associated medical conditions such as fragile-X syndrome, tuberous sclerosis, neurofibromatosis, and hypothyroidism are less common in Aspergerís Disorder than in classical autism. Therefore it may be expected that there are fewer major structural brain abnormalities associated with Aspergerís Disorder than with autism. To our knowledge, a very small number of structural brain abnormalities have been so far associated with Aspergerís Disorder, which include left frontal macrogyria, bilateral opercular polymicrogyria, and left temporal lobe damage. On the other hand brain imaging techniques like positron emission tomography (PET), and single photon emission tomography (SPECT) which provide information about the functional status of brain may be more helpful in determining the brain dysfunction in individuals with Aspergerís Disorder. Detailed neuropsychological testing may support these findings providing information about the performances of individual right or left hemispheric brain regions. The first SPECT study in a patient with Aspergerís Disorder was published by the host of this page and his colleagues, and found left parietooccipital hypoperfusion.  Continuation of research in Aspergerís Disorder with various brain imaging techniques in coordination with neuropsychological evaluation in larger samples is clearly needed in this area.

WHAT IS THE DIAGNOSTIC CRITERIA OF 
ASPERGER'S DISORDER?

DSM-IV DIAGNOSTIC CRITERIA FOR ASPERGERíS DISORDER A. Qualitative impairment in social interaction, as manifested by at least two of the following:

  1. Marked impairment in the use of multiple nonverbal behaviors such as eye-to-eye gaze, facial expression, body postures, and gestures to regulate social interaction.
  2. Failure to develop peer relationships appropriate to developmental level.
  3. A lack of spontaneous seeking to share enjoyment, interests, or achievements with other people (e.g., by a lack of showing, bringing, or pointing out objects of interest to other people).
  4. Lack of social or emotional reciprocity.

B. Restricted repetitive and stereotyped patterns of behavior, interests, and activities, as manifested by at least one of the following:

  1. Encompassing preoccupation with one or more stereotyped and restricted patterns of interest that is abnormal either in intensity or focus.
  2. Apparently inflexible adherence to specific, nonfunctional routines or rituals.
  3. Stereotyped and repetitive motor mannerisms (e.g., hand or finger flapping or twisting, or complex whole-body movements).
  4. Persistent preoccupation with parts of objects.

C. The disturbance causes clinically significant impairment in social, occupational, or other important areas of functioning.

D There is no clinically significant general delay in language (e.g., single words used by age 2 years, communicative phrases used by age 3 years).

E. There is no clinically significant delay in cognitive development or in the development of age-appropriate self-help skills, adaptive behavior (other than in social interaction), and curiosity about the environment in childhood.

F. Criteria are not met for another specific Pervasive Developmental Disorder or Schizophrenia.

GILLBERGíS CRITERIA FOR ASPERGERíS DISORDER

  1. Severe impairment in reciprocal social interaction (at least two of the following):
    1. inability to interact with peers
    2. lack of desire to interact with peers
    3. lack of appreciation of social cues
    4. socially and emotionally inappropriate behavior
  2. All-absorbing narrow interest (at least one of the following)
    1. exclusion of other activities
    2. repetitive adherence
    3. more rote than meaning
  3. Imposition of routines and interests (at least one of the following):
    1. on self, in aspects of life
    2. on others
  4. Speech and language problems (at least three of the following):
    1. delayed development
    2. superficially perfect expressive language
    3. formal, pedantic language
    4. odd prosody, peculiar voice characteristics
    5. impairment of comprehension including misinterpretations of  literal/implied meanings
  5. Non-verbal communication problems (at least one of the following):
    1. limited use of gestures
    2. clumsy/gauche body language
    3. limited facial expression
    4. inappropriate expression
    5. peculiar, stiff gaze
  6. Motor clumsiness: poor performance on neurodevelopmental examination.

    (All six criteria must be met for confirmation of diagnosis.)

WHAT IS THE TREATMENT FOR ASPERGER'S DISORDER?

There is no specific treatment or ďcureĒ for Aspergerís Disorder. All the interventions outlined below are mainly symptomatic and/or rehabilitational. Psychosocial Interventions.

  1. Individual psychotherapy to help the individual to process the  feelings  aroused by being socially handicapped.
  2. Parent education and training.
  3. Behavioral modification.
  4. Social skills training.
  5. Educational interventions.

PSYCHOPHARMACOLOGICAL INTERVENTIONS

  1. For hyperactivity, inattention and impulsivity: Psychostimulants (Methyphenidate, Dextroamphetamine, Metamphetamine, Pemoline), Clonidine, Tricyclic Antidepressants (Desipramine, Nortriptyline)
  2. For irritability and aggression: Mood Stabilizers (Valproate, Carbamazepine, Lithium), Beta Blockers (Nadolol, Propranolol), Clonidine, Naltrexone, Neuroleptics (Risperidone, Haloperidol)
  3. For preoccupations, rituals and compulsions: SSRIs (Fluvoxamine, Fluoxetine), Tricyclic Antidepressants (Clomipramine)
  4. For anxiety: SSRIs (Sertraline, Fluoxetine), Tricyclic Antidepressants (Imipramine, Clomipramine, Nortriptyline).
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