Asperger syndrome (a specific autism spectrum disorder): Clinical features and diagnosis in children and adolescents
- L Erik von Hahn, MD
L Erik von Hahn, MD
- Associate Professor of Pediatrics
- Tufts University School of Medicine
- Section Editors
- Carolyn Bridgemohan, MD
Carolyn Bridgemohan, MD
- Section Editor — Developmental and Behavioral Pediatrics
- Assistant Professor of Pediatrics
- Harvard Medical School
- Marc C Patterson, MD, FRACP
Marc C Patterson, MD, FRACP
- Section Editor — Pediatric Neurology
- Professor of Neurology, Pediatrics, and Medical Genetics
- Chair, Division of Child and Adolescent Neurology
- Mayo Clinic College of Medicine
The American Psychiatric Association recognized Asperger disorder (also called Asperger syndrome) as a specific entity by publishing diagnostic criteria in the Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV) in 1994 . A modest literature on Asperger disorder has emerged since then, though there are no specific clinical features that establish Asperger disorder as distinct from the autism spectrum as a whole [2-4] and its status as a separate entity is uncertain. In the DSM-5, published in 2013, Asperger disorder is encompassed within the diagnosis of autism spectrum disorder . In contrast, Asperger syndrome remains a distinct entity in the International Classification of Diseases, 10th Revision (ICD-10) .
This topic review summarizes the modest literature that describes Asperger syndrome as a separate entity. The terms “Asperger syndrome” and “autism spectrum disorder” (ASD) will be used interchangeably to acknowledge the two classification systems. Most of the information in this topic review will apply to children with ASD of Level 1 severity who do not have intellectual impairment, but may have language impairment. This approach is consistent with research that shows that subtypes of autism differ primarily on the basis of IQ [7,8]. (See 'Terminology' below and "Autism spectrum disorder: Diagnosis", section on 'Diagnostic criteria'.)
The clinical features and diagnosis of Asperger syndrome will be reviewed here. The management and prognosis are discussed separately. (See "Asperger syndrome (a specific autism spectrum disorder): Management and prognosis in children and adolescents".)
Asperger syndrome is considered to be an "autism spectrum disorder" (ASD). ASD is a neurodevelopmental disorder that has diverse etiologies. It is characterized by a constellation of symptoms that includes deficits in reciprocal social interaction, social communication, and restricted and repetitive behavior (including stereotyped interests and activities) [5,6,9]. (See "Autism spectrum disorder: Diagnosis", section on 'Diagnostic criteria'.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders DSM-IV, 4th ed, American Psychiatric Association, Washington, DC 1994.
- Klin A, Volkmar FR. Asperger syndrome: diagnosis and external validity. Child Adolesc Psychiatr Clin N Am 2003; 12:1.
- Tantam D, Girgis S. Recognition and treatment of Asperger syndrome in the community. Br Med Bull 2009; 89:41.
- Macintosh KE, Dissanayake C. Annotation: The similarities and differences between autistic disorder and Asperger's disorder: a review of the empirical evidence. J Child Psychol Psychiatry 2004; 45:421.
- American Psychiatric Association. Autism spectrum disorder. In: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, American Psychiatric Association, Arlington, VA 2013. p.50.
- World Health Organization. The ICD-10 Classification of Mental and Behavioural Disorders. Clinical descriptions and diagnostic guidelines. www.who.int/classifications/icd/en/bluebook.pdf (Accessed on June 04, 2013).
- Witwer AN, Lecavalier L. Examining the validity of autism spectrum disorder subtypes. J Autism Dev Disord 2008; 38:1611.
- Sharma S, Woolfson LM, Hunter SC. Confusion and inconsistency in diagnosis of Asperger syndrome: a review of studies from 1981 to 2010. Autism 2012; 16:465.
- Johnson CP, Myers SM, American Academy of Pediatrics Council on Children With Disabilities. Identification and evaluation of children with autism spectrum disorders. Pediatrics 2007; 120:1183.
- American Psychiatric Association. Social (pragmatic) communication disorder. In: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, American Psychiatric Association, Arlington, VA 2013. p.47.
- World Health Organization. The ICD-10 Classification of Mental and Behavioural Disorders. Diagnostic criteria for research http://www.who.int/classifications/icd/en/GRNBOOK.pdf (Accessed on June 11, 2013).
- Woodbury-Smith MR, Volkmar FR. Asperger syndrome. Eur Child Adolesc Psychiatry 2009; 18:2.
- Gillberg IC, Gillberg C. Asperger syndrome--some epidemiological considerations: a research note. J Child Psychol Psychiatry 1989; 30:631.
- Gillberg C. Asperger syndrome in 23 Swedish children. Dev Med Child Neurol 1989; 31:520.
- Ehlers S, Gillberg C. The epidemiology of Asperger syndrome. A total population study. J Child Psychol Psychiatry 1993; 34:1327.
- Ellefsen A, Kampmann H, Billstedt E, et al. Autism in the Faroe Islands: an epidemiological study. J Autism Dev Disord 2007; 37:437.
- Fombonne E, Tidmarsh L. Epidemiologic data on Asperger disorder. Child Adolesc Psychiatr Clin N Am 2003; 12:15.
- Gillberg C, Wing L. Autism: not an extremely rare disorder. Acta Psychiatr Scand 1999; 99:399.
- Kopra K, von Wendt L, Nieminen-von Wendt T, Paavonen EJ. Comparison of diagnostic methods for asperger syndrome. J Autism Dev Disord 2008; 38:1567.
- Fombonne E. Epidemiology of pervasive developmental disorders. Pediatr Res 2009; 65:591.
- Fombonne, E. The Changing Epidemiology of Autism. J Appl Res Intellect Disabil 2005; 18:281.
- Ghaziuddin M. A family history study of Asperger syndrome. J Autism Dev Disord 2005; 35:177.
- Susceptibility to Asperger syndrome. In: Online Mendelian Inheritance in Man. Available at: www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=608638. (Accessed on August 16, 2009).
- Rinehart NJ, Bradshaw JL, Brereton AV, Tonge BJ. A clinical and neurobehavioural review of high-functioning autism and Asperger's disorder. Aust N Z J Psychiatry 2002; 36:762.
- Howlin P, Asgharian A. The diagnosis of autism and Asperger syndrome: findings from a survey of 770 families. Dev Med Child Neurol 1999; 41:834.
- American Psychiatric Association. Asperger's disorder. In: Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR®), American Psychiatric Association, Arlington, VA 2000. p.80.
- McConachie H, Le Couteur A, Honey E. Can a diagnosis of Asperger syndrome be made in very young children with suspected autism spectrum disorder? J Autism Dev Disord 2005; 35:167.
- Volkmar FR, Klin A. Diagnostic issues in Asperger syndrome. In: Asperger syndrome, The Guilford Press, New York 2000. p.25.
- Johnson CP, Myers SM. Autism spectrum disorders. In: Developmental-Behavioral Pediatrics: Evidence and Practice, Wolraich ML, Drotar DD, Dworkin PH, Perrin EC (Eds), Mosby Elsevier, Philadelphia 2008. p.519.
- Baron-Cohen S, Leslie AM, Frith U. Does the autistic child have a "theory of mind"? Cognition 1985; 21:37.
- Hill EL, Bird CM. Executive processes in Asperger syndrome: patterns of performance in a multiple case series. Neuropsychologia 2006; 44:2822.
- Wing L. Asperger's syndrome: a clinical account. Psychol Med 1981; 11:115.
- Gjevik E, Eldevik S, Fjæran-Granum T, Sponheim E. Kiddie-SADS reveals high rates of DSM-IV disorders in children and adolescents with autism spectrum disorders. J Autism Dev Disord 2011; 41:761.
- Leyfer OT, Folstein SE, Bacalman S, et al. Comorbid psychiatric disorders in children with autism: interview development and rates of disorders. J Autism Dev Disord 2006; 36:849.
- Gillberg IC, Helles A, Billstedt E, Gillberg C. Boys with Asperger Syndrome Grow Up: Psychiatric and Neurodevelopmental Disorders 20 Years After Initial Diagnosis. J Autism Dev Disord 2016; 46:74.
- Ghaziuddin M, Weidmer-Mikhail E, Ghaziuddin N. Comorbidity of Asperger syndrome: a preliminary report. J Intellect Disabil Res 1998; 42 ( Pt 4):279.
- Yoshida Y, Uchiyama T. The clinical necessity for assessing Attention Deficit/Hyperactivity Disorder (AD/HD) symptoms in children with high-functioning Pervasive Developmental Disorder (PDD). Eur Child Adolesc Psychiatry 2004; 13:307.
- Lee DO, Ousley OY. Attention-deficit hyperactivity disorder symptoms in a clinic sample of children and adolescents with pervasive developmental disorders. J Child Adolesc Psychopharmacol 2006; 16:737.
- Gadow KD, Devincent CJ, Drabick DA. Oppositional defiant disorder as a clinical phenotype in children with autism spectrum disorder. J Autism Dev Disord 2008; 38:1302.
- Mattila ML, Hurtig T, Haapsamo H, et al. Comorbid psychiatric disorders associated with Asperger syndrome/high-functioning autism: a community- and clinic-based study. J Autism Dev Disord 2010; 40:1080.
- White SW, Oswald D, Ollendick T, Scahill L. Anxiety in children and adolescents with autism spectrum disorders. Clin Psychol Rev 2009; 29:216.
- Kuusikko S, Pollock-Wurman R, Jussila K, et al. Social anxiety in high-functioning children and adolescents with Autism and Asperger syndrome. J Autism Dev Disord 2008; 38:1697.
- Weisbrot DM, Gadow KD, DeVincent CJ, Pomeroy J. The presentation of anxiety in children with pervasive developmental disorders. J Child Adolesc Psychopharmacol 2005; 15:477.
- Matson JL, Nebel-Schwalm MS. Comorbid psychopathology with autism spectrum disorder in children: an overview. Res Dev Disabil 2007; 28:341.
- Kim JA, Szatmari P, Bryson SE, et al. The prevalence of anxiety and mood problems among children with autism and Asperger syndrome. Autism 2000; 4:17.
- Kerns CM, Kendal PC. The presentation and classification of anxiety in autism spectrum disorder. Clinical Psychology: Science and Practice 2012; 19.
- Mazefsky CA, Herrington J, Siegel M, et al. The role of emotion regulation in autism spectrum disorder. J Am Acad Child Adolesc Psychiatry 2013; 52:679.
- Simonoff E, Pickles A, Charman T, et al. Psychiatric disorders in children with autism spectrum disorders: prevalence, comorbidity, and associated factors in a population-derived sample. J Am Acad Child Adolesc Psychiatry 2008; 47:921.
- Gargaro BA, Rinehart NJ, Bradshaw JL, et al. Autism and ADHD: how far have we come in the comorbidity debate? Neurosci Biobehav Rev 2011; 35:1081.
- Richdale AL, Schreck KA. Sleep problems in autism spectrum disorders: prevalence, nature, & possible biopsychosocial aetiologies. Sleep Med Rev 2009; 13:403.
- Danelle Hodge D, Parnell AM, Hoffman CD, Sweeney DP6. Methods for assessing sleep in children with autism spectrum disorders: A review. Research in Autism Spectrum Disorders 2012; 6:1337.
- Kotagal S, Broomall E. Sleep in children with autism spectrum disorder. Pediatr Neurol 2012; 47:242.
- Ozonoff S, Goodlin-Jones BL, Solomon M. Evidence-based assessment of autism spectrum disorders in children and adolescents. J Clin Child Adolesc Psychol 2005; 34:523.
- Saulnier CA, Volkmar FR. Asperger syndrome. In: The Zuckerman Parker Handbook of Developmental and Behavioral Pediatrics for Primary Care, 3rd ed, Augustyn M, Zuckerman B, Caronna EB (Eds), Lippincott Williams & Wilkins, Philadelphia 2011. p.120.
- Campbell JM. Diagnostic assessment of Asperger's disorder: a review of five third-party rating scales. J Autism Dev Disord 2005; 35:25.
- Szatmari P, Bremner R, Nagy J. Asperger's syndrome: a review of clinical features. Can J Psychiatry 1989; 34:554.
- Hartley SL, Sikora DM. Which DSM-IV-TR criteria best differentiate high-functioning autism spectrum disorder from ADHD and anxiety disorders in older children? Autism 2009; 13:485.
- First MB. Mutually Exclusive versus Co-Occurring Diagnostic Categories: The Challenge of Diagnostic Comorbidity. In: Clinical Psychiatry, Columbia University College of Physicians and Surgeons, New York 2005.
- Clark T, Feehan C, Tinline C, Vostanis P. Autistic symptoms in children with attention deficit-hyperactivity disorder. Eur Child Adolesc Psychiatry 1999; 8:50.
- Ivarsson T, Melin K. Autism spectrum traits in children and adolescents with obsessive-compulsive disorder (OCD). J Anxiety Disord 2008; 22:969.
- CLINICAL FEATURES
- Early features
- Core features
- - Social skills
- Language skills
- - Behavioral features
- Other features
- - Clumsiness
- - School performance
- Associated conditions
- DIAGNOSTIC APPROACH
- Role of the primary care provider
- Multidisciplinary evaluation
- DIFFERENTIAL DIAGNOSIS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS