title: Acquired autistic behaviors in children with mucopolysaccharidosis type IIIA.
abstract: OBJECTIVE
  To assess autism spectrum disorder (ASD) behaviors in children with mucopolysaccharidosis type IIIA (MPS IIIA) using a standard measure, understand the behavioral evolution of the disease, and provide specific guidelines for diagnosis.
  
  
  METHODS
  Children (n = 21) with documented enzyme deficiency and SGSH gene mutations, cognitive age-equivalent >12 months, and early onset were administered the Autism Diagnostic Observation Schedule (ADOS) (module 1) and Bayley Scales of Infant Development-Third Edition. ADOS Social Affect and Restricted Repetitive Behavior total scores, as well as Bayley Scales of Infant Development-Third Edition cognitive age-equivalent scores, are reported using descriptive statistics and graphic presentations.
  
  
  RESULTS
  Thirteen of the 21 children evaluated met the ADOS criteria for ASD/autism. ADOS score was strongly associated with age; all 11 children aged >46 months met the criteria, compared with only 2 of 10 aged <46 months. Social and affective abnormalities were most frequent; restricted interests and repetitive behaviors were largely absent. Lack of cognitive growth paralleled ADOS score.
  
  
  CONCLUSIONS
  An increased incidence of ASD-like social behaviors was seen at age 3-4 years in children with early-onset MPS IIIA. Although more frequent in the severely impaired children, ASD-like behaviors were observed across the entire range of cognitive impairment. Clinicians must be aware that when a child acquires ASD-like behaviors, MPS IIIA should be included in the differential diagnosis.
authors.affiliation: Department of Pediatrics, University of Minnesota, Minneapolis, MN. Electronic address: rumse002@umn.edu.
authors.forename: Robin K
authors.initials: RK
authors.lastname: Rumsey
authors.affiliation: Division of Biostatistics, University of Minnesota, Minneapolis, MN.
authors.forename: Kyle
authors.initials: K
authors.lastname: Rudser
authors.affiliation: Department of Pediatrics, University of Minnesota, Minneapolis, MN.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Delaney
authors.affiliation: Department of Pediatrics, University of Minnesota, Minneapolis, MN.
authors.forename: Michael
authors.initials: M
authors.lastname: Potegal
authors.affiliation: Department of Pediatrics, University of Minnesota, Minneapolis, MN.
authors.forename: Chester B
authors.initials: CB
authors.lastname: Whitley
authors.affiliation: Department of Pediatrics, University of Minnesota, Minneapolis, MN.
authors.forename: Elsa
authors.initials: E
authors.lastname: Shapiro
mesh_terms: D000293:Adolescent; D000367:Age Factors; D017668:Age of Onset; D002648:Child; D002659:Child Development Disorders, Pervasive / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D009084:Mucopolysaccharidosis III / Q000150:complications*; D011581:Psychological Tests