How is an autism spectrum disorder treated

Asperger's Syndrome - an autism spectrum disorder

Archive2 / 2009Asperger's Syndrome - an Autism Spectrum Disorder

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introduction
Asperger's syndrome is a profound developmental disorder that is characterized by a characteristic pattern of social, communicative and stereotypical, repetitive behaviors.

Methods
Review article on the diagnosis, etiology and therapy of Asperger's Syndrome based on the guidelines of the German Society for Child and Adolescent Psychiatry and a selective review of the literature by the authors.

Results
The disorder exists from childhood and persists into old age. Despite the undoubted biological pathogenesis, a conclusive model for the etiology and genesis is still lacking. However, the results available so far suggest the involvement of genetic factors, brain damage and dysfunction, associated physical illnesses, biochemical abnormalities, neuropsychological deficits and the interaction of these factors. A number of studies are already available for behavioral interventions that indicate the effectiveness of these approaches.

Conclusions
There is no causal treatment for Asperger's Syndrome and there are no verified standards in treatment.

keywords
Asperger's Syndrome, Autism Spectrum Disorder, Autistic Psychopathy, Autism
Pervasive developmental disorders begin in early childhood and are characterized by delay and variance in development. According to the two common classification systems (ICD-10 and DSM-IV), they are characterized by 3 features: qualitative impairments in mutual interaction, communication and a restricted, stereotypical, repetitive repertoire of interests and activities. These qualitative impairments are a fundamental functional characteristic of those affected and show up in all situations - however, they vary in severity.

The most important profound developmental disorders according to ICD-10 are in Box 1 (gifppt). Autism spectrum disorders include, in particular, early childhood autism (F84.0), Asperger's syndrome (F84.5) and atypical autism (F84.0). In a further definition, all profound developmental disorders are subsumed under it. This concept is based on the assumption that different autistic disorders cannot be categorically differentiated from one another, but are to be arranged in one dimension. This means that they differ from one another only quantitatively, but not qualitatively. In Table 1 (gifppt) the similarities and differences between the various autism spectrum disorders are listed.

The autism spectrum disorders encompass a wide variety of symptoms, a wide spectrum of clinical manifestations, and a wide range of degrees of expression. Autism spectrum disorders are considered to be developmental disorders of the central nervous system ("neurodevelopmental disorders") and are associated with impairments of basic brain functions that affect the ability to communicate.

Epidemiology
If it was previously assumed that autism spectrum disorders are relatively rare, more recent studies (1) show higher prevalence rates. Most epidemiological studies are available on early childhood autism; the data situation is less favorable for atypical autism and Asperger's syndrome.

More recent data exist for the area of ​​intelligence: While until a few years ago the rule was that three quarters of all autistic people had an intellectual disability, recent studies show that this is not the case (2, 3) (Table 2gifppt). The ratio of the sexes (male: female) is around 3: 1, whereby the autistic disorder in affected girls is usually associated with significant mental retardation. In Asperger's syndrome, the gender ratio between boys and girls is around 8: 1.

Symptoms
Asperger's syndrome is characterized by a pronounced contact and communication disorder and, in addition to this basic disorder, has some distinctive features that distinguish it from early childhood autism: On the one hand, language development and intellectual development are not delayed. On the other hand, many people with Asperger's syndrome - perhaps precisely because of their higher intelligence (compared to those with other autistic disorders), which they cannot adequately use - show highly specialized and pronounced special interests that they pursue monomaniacally and that they consider to be in their environment Make extreme oddities appear, for example memorizing timetables, the melting points of all metals or the paragraphs of the Basic Law. According to the ICD-10 (F84.5), the following features are required to diagnose Asperger's syndrome:

Qualitative impairment of social interaction
The children and adolescents concerned are conspicuous both in their non-verbal behavior (significantly reduced gestures, facial expressions, gestures, eye contact) and in their inability to establish informal relationships with their peers or the elderly. However, this is not necessarily due to the desire of those affected to withdraw from society, but rather to their inability to understand the unwritten rules of social interaction and to behave accordingly. There is a marked inability to grasp the feelings of others and to resonate emotionally. This difficulty is often referred to as a "disorder of empathy" or a lack of "theory of mind". They can also be described as extreme self-centeredness, whereby the extreme isolation from the environment, which is usually associated with early childhood autism, is much less in the foreground in Asperger's syndrome. People with Asperger's Syndrome make multiple, but inappropriately, contact with the environment. They like to talk a lot with other people, talk extensively and at length about their interests, but do not pay attention to whether their behavior is appropriate to the situation and how the other person reacts to it.

Communication abnormalities
Asperger's syndrome lacks the speech development delay characteristic of early childhood autism. Other language disorders are typical of early childhood autism and rarely occur in Asperger's syndrome, such as echolalia (echo-like repeating of words and sounds) and pronoun inversion (children speak of themselves in the third person and only learn about themselves very late) To designate "I"). On the other hand, abnormalities in the speaking voice are often found in children with Asperger's Syndrome. Her voice often appears monotonous, tinny, monotonous and has little modulation.

The diagnosis of Asperger's Syndrome according to ICD-10 requires that individual words be spoken in the second year of life or earlier, and first sentences in the third year of life or earlier. The intelligence should be at least in the normal range or above. However, the milestones in motor development can be reached a little late. Motor clumsiness is a common trait, but not a necessary condition for diagnosis.

Limited interests and stereotypical behavior
Children with Asperger's syndrome show a variety of motor stereotypes and a movement pattern characterized by clumsiness, lack of coordination and appropriateness to the situation, which makes them appear awkward and clumsy in their environment. Their interests are often focused on specific topics and are unusual. They often show an apparently obsessive interest in areas such as mathematics, technology, scientific sub-areas or sub-areas of history or geography. Sometimes the special interests are simply exaggerations of common interests, such as Pokemon, dinosaurs or computers. However, special interests have a significant disruptive influence on other activities and significantly impair participation in everyday life. In addition, pronounced compulsions (for example, compulsively adhering to certain rituals in everyday life, such as times, processes) and fear of change (for example, only to walk known paths) are common behavioral problems.

etiology
Due to diverse research efforts, there is no longer any doubt about a biological pathogenesis of the autism spectrum disorders. The thesis, which was still held up into the 1960s, that autism arises due to the mother's emotional coldness (so-called “refrigerator mother”) is now considered to be refuted. In his first description in 1944, Hans Asperger (4) pointed out that the "autistic psychopathy" he described has a genetic background. Social and psychological factors have an influence on the course of the disorder, but are not to be regarded as causative. Although a large number of data suggest a biological pathogenesis, a conclusive model for the etiology and genesis of autistic disorders is still lacking. However, the results available to date suggest that the following factors are involved in the development of autism spectrum disorders: Genetic factors, damage or brain dysfunction, biochemical anomalies, associated physical illnesses, neuropsychological deficits, and the interaction of these factors (5–7).

While there are now a number of family and twin studies on early childhood autism, this is not the case with Asperger's syndrome. The data situation also varies greatly with regard to molecular genetic studies: at least 8 genome scans have so far been carried out with test subjects suffering from early childhood autism (10), with Asperger’s test subjects only one (11). It is now believed that up to 20 genes are involved in causing autism spectrum disorders. For the structural peculiarities of the brains of people with Asperger's syndrome, deviations in different brain regions could be demonstrated (including abnormalities of the cerebrum and the limbic system; abnormalities in the cerebellum and in the lower olive [12]). A model of insufficient neural networking of various cerebral areas is currently being discussed by many researchers (6). This means that autistic disorders are viewed as brain dysfunction.

In the area of ​​neuropsychological deficits, the following areas are examined as psychological correlates of autistic disorders:

- different intelligence structure
- Executive functions (these are the "higher" mental or cognitive processes that serve the self-regulation and targeted action control of the individual in his environment)
- "theory of mind" (ability to represent one's own and other people's psychological states in one's own cognitive system)
- poor central coherence. This means that less attention is paid to the context and the connections between objects and objects, but rather the perception is directed towards individual or isolated details.

In Graphic 1 (gifppt) an attempt is made to establish at least some connections, knowing full well that a uniform and comprehensive explanation is not yet possible.

Diagnosis
The history and observation of the child in different situations are the basis of the diagnosis. This requires a differentiated child and adolescent psychiatric examination according to the guidelines of the German Society for Child and Adolescent Psychiatry (15). It therefore makes sense to refer children with suspected Asperger's syndrome to a resident child and adolescent psychiatrist or to the responsible specialist clinic.

In addition to the psychiatric diagnosis according to ICD-10, the following additional areas should be diagnosed: comorbid psychopathology, assessment of the general level of development, cognitive abilities, adaptive behavior and neuropsychological functions. In addition, a physical / neurological examination must be carried out in each case.

Anamnestically, the entire symptoms for both current behavior and past behavior, especially early childhood, should be asked in order to assess whether the symptoms are to be viewed as a profound developmental disorder. This means that the conspicuous behavior is cross-situational and fundamental functional characteristic of the entire development and is not tied to certain situations (for example only outside the family) or was triggered by critical life events (for example separation of parents). From all three fault areas, abnormalities must be consistently and stringently found throughout the entire development. In Box 2 (gifppt) the symptom areas that are to be examined diagnostically are listed again.

To assess the current impairment, exploration and behavioral observation of the child / adolescent should be carried out. Private video recordings from the family are also helpful here. The observation should take place in different situations (structured, unstructured, known and new). The focus should be on the ability to conversation, non-verbal and verbal communication (pragmatic aspects, language understanding, language abnormalities and others), playing behavior and social-emotional understanding. In spite of their good verbal skills, people with Asperger's syndrome have a significant impairment in prosody (metric-rhythmic aspects of language) and pragmatics of language (social use and understanding of language). The pragmatics of language regulates the communicative use of grammar and semantics in different contexts.

Only when these rules are understood and applied or broken within a culture can we understand that someone wants to tease, have ulterior motives, be polite, humorous, sarcastic and so on.

The following standardized procedures should be mentioned:

- "Marburg assessment scale for Asperger's Syndrome (MBAS)" as a screening questionnaire (14, 16)
- "Autism Diagnostic Interview-Revised" (ADI-R; Diagnostic Interview for Autism - Revised) (17)
- "Autism Diagnostic Observation Schedule-Generic" (ADOS-G; Diagnostic Observation Scale for Autistic Disorders) (18).

Differential diagnosis
First of all, for the diagnosis, it is important to differentiate Asperger's syndrome from other profound developmental disorders.

Children with early childhood autism are usually conspicuous from birth, often have multiple developmental disorders and are usually significantly limited in their cognitive functions.

If there is a clear general delay in spoken or receptive language or in cognitive development, this indicates the presence of early childhood autism.

In schizoid personality disorder, the characteristic symptoms of Asperger's syndrome are absent, especially the punched out special interests, the linguistic peculiarities and the obsessive-compulsive stereotypical behaviors. With schizophrenia there are different symptoms (formal thought disorders, delusion, hallucinations) and a different course: schizophrenic psychoses are often preceded by unspecific precursor symptoms, the prodromal symptoms only manifest for weeks or months. But they do not relate to the whole of early childhood development. The reactive attachment disorder also has a different course than Asperger's syndrome, and other causes are assumed for this disorder. Differentiating it from obsessive-compulsive disorder is sometimes difficult because obsessive-compulsive symptoms are also common in Asperger's syndrome. However, they do not represent the "core" of the disorder. The same applies to the demarcation from compulsive personality disorder.

Attention and hyperactivity disorder (ADHD) is a common misdiagnosis because this disorder also has significant, but secondary, contact difficulties. The cardinal symptoms of hyperkinetic disorder are impaired alertness, overactivity, and impulsiveness. Although these symptoms also lead to interaction disorders, the criteria for Asperger's syndrome are not fully met. For example, children with ADHD are usually capable of imaginative and creative play, there are no fundamental deficits in the area of ​​empathy, the non-verbal behavior is used communicatively or there are no or only minor fears of change, compulsions or other rigid behavior.

Comorbidity
In some cases, especially in times of crisis, it occurs either through external circumstances (moving, separation of parents, birth or death in the family) or through internal circumstances such as upcoming development tasks that have to be mastered (school enrollment, retraining, puberty, replacement from parental home) lead to additional mental disorders. On the one hand, existing symptoms can intensify, for example hyperactivity, autoaggressions or ritualized behavior; on the other hand, symptoms can develop that have their own disease value, such as affective disorders and obsessive-compulsive disorders.

There is also evidence that patients with Asperger's syndrome have a slightly increased risk of schizophrenic psychotic episodes (19, 20), but also of psychotic depression and bipolar disorder. Obsessive-compulsive disorder and Tourette's syndrome are common comorbid disorders in Asperger's syndrome. Another common disease is attention disorder, which goes beyond autistically impaired attention and leads to additional problems (21). In adolescents and adults with Asperger's syndrome, symptoms of depression can also appear in the course of the disease (22). Particularly in adolescence and early adulthood, depression is the most significant accompanying illness of Asperger's syndrome.

therapy
Proven therapeutic approaches for autism spectrum disorders
The therapy of Asperger's Syndrome is based on the current knowledge of the etiology, the symptoms and the empirical evidence of already tried and tested treatment methods. The etiological background has a significant influence on the treatment options and goals (Figure 1).

There are a number of behavior therapy programs for autism spectrum disorders (Lovaas, TEACCH, language structure, alternative communication systems [13]), for which first comparative studies have also shown a positive effect (23) (Box 3gifppt). Studies on depth psychological treatment that meet the methodological requirements of controlled studies do not yet exist. For other alternative therapeutic approaches, however, only subjective experience reports are available. Therapy success for these could not be proven by scientific studies. Unfortunately, there is still a lack of verified standards in the treatment of autism spectrum disorders. Furthermore, it is left to parents to choose from the multitude of very different autism therapies for their child that seems to be the most suitable method for their child in terms of requirements, effectiveness, efficiency and ethical harmlessness. Box 3 lists the common intervention techniques with regard to their empirically determined effectiveness (24, 25, 26).

Specific procedures for Asperger's syndrome
Due to the wide range of symptoms associated with Asperger's syndrome, comprehensive treatment approaches that are based on behavioral principles and always focus on promoting several functional areas are useful (see guidelines of the German Society for Child and Adolescent Psychiatry and Psychotherapy) (15). The therapy of choice is behavioral therapy in the developmental context, that is, it is based on the current level of development of the child / adolescent and adjusts the measures accordingly. In the holistic approach, besides the behavior to be modified, the cognitive and affective experiences of people with Asperger's syndrome, which need to be expanded, are of particular importance. The skills in this area should be promoted in a development-oriented manner and the existing deficits bridged by compensation.

The aim of the interventions can only be to alleviate the symptoms and to develop and expand skills in order to help the patient to lead a largely independent life. For this purpose, various intervention methods are used in a multimodal therapy plan to form a holistic treatment approach (Graphic 2gifppt) individually combined with each other.

The “desired” behaviors are initially divided into small learning steps and help (“prompts”) are given, which are then gradually withdrawn (“fading”). The reinforcers used can be very "unusual", such as being allowed to briefly pursue stereotypes or special interests. In addition to behavioral therapy measures, the repertoire also includes educational programs, early intervention, drug therapy (Table 3gifppt) as well as other behavioral methods (e.g. occupational therapy). In order to continuously practice the newly learned skills and abilities and, above all, to enable a transfer to real situations, the parents as co-therapists are indispensable for a successful therapy. Self-help organizations and parents' associations support parents in this complex and demanding task.

A therapy for Asperger's syndrome as well as for other autism spectrum disorders is always a long-term therapy, because the development of basic skills such as the "theory of mind" - which in healthy children develops rather intuitively and "incidentally" - requires long and patient explicit guidance in people with Asperger's Syndrome. Basic contact and behavior training is the focus of the therapy. Due to the inability to generalize, these behaviors must be practiced in many different real-world situations. Another important point in the treatment is the gradual expansion of the areas of interest towards more realistic activities or tasks. Starting the therapeutic steps as early as possible is of the utmost importance for their chances of success (27). The interventions should always be highly structured as well as directive and concrete.

The treatment of comorbid disorders, for example hyperactive and depressive disorders, anxiety and obsessive-compulsive disorders, should not be neglected. This may also make pharmacological treatment necessary (28) (Table 3). Regarding the evidence base in pharmacological therapy, it can be said that research has come to contradicting results and therapeutic effectiveness of drugs can only ever be observed in a subpopulation (“responders”). In addition, pharmacological treatment is useful for extremely rigid and compulsive behaviors, when aggressive reactions occur that cannot be treated otherwise (for example with risperidone).

Course and prognosis
The core symptoms of Asperger's syndrome show developmental variability, but remain as persistent and profound symptoms into adulthood (29). It is true that the majority of those affected gradually improve their contact and social behavior when compared with the related symptoms in childhood and adolescence. Certain routines in everyday life are also coped better, but the basic communication disorder, in many cases also stereotypes, the restricted interests and the restricted ability to make contact with other people remain. Overall, the course is very variable. Although the prognosis for Asperger's syndrome is better than for early childhood autism, the course does not only depend on good cognitive and language skills. The occurrence of comorbid illnesses significantly impairs further development opportunities and the prognosis.

Conflict of interest
The authors declare that there is no conflict of interest within the meaning of the guidelines of the International Committee of Medical Journal Editors.

Manuscript dates
submitted: August 1, 2006, revised version accepted: January 11, 2007

Updated by the authors: April 28, 2009


Address for the authors
Prof. Dr. med. Dr. phil. Helmut Remschmidt
Clinic and Polyclinic for Child and Adolescent Psychiatry
of the Philipps University of Marburg
Hans-Sachs-Strasse 6
35039 Marburg
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Clinic for Child and Adolescent Psychiatry and Psychotherapy at the Philipps University of Marburg: Prof. Dr. med. Dr. phil. Remschmidt, Dr. phil. Kamp-Becker