Autistic behavior in children with fragile X syndrome: Prevalence, stability, and the impact of FMRP

We examined autistic behavior in a cross‐sectional sample of 179 children with fragile X syndrome (FXS) and a longitudinal subset of 116 children using the Childhood Autism Rating Scale (CARS) to (a) determine a prevalence of autistic behavior in FXS, (b) examine the stability of autistic ratings over time, and (c) assess the association between the fragile X mental retardation protein (FMRP) and autistic behavior. Approximately 21% of the sample of 129 children (25.9% of boys) scored at or above the cutoff for autism. CARS scores increased slowly, yet significantly, over time, and low levels of FMRP were associated with higher mean levels of autistic behavior as measured by the CARS. © 2006 Wiley‐Liss, Inc.

[1]  S. Hepburn,et al.  Linguistic and cognitive functioning and autism symptoms in young children with fragile X syndrome. , 2004, American journal of mental retardation : AJMR.

[2]  L. Frank The Society for Research in Child Development , 1935 .

[3]  A. Wijeratne,et al.  Autistic-like behaviour profile and psychiatric morbidity in Fragile X Syndrome , 2003, European child & adolescent psychiatry.

[4]  J. Singer,et al.  Applied Longitudinal Data Analysis , 2003 .

[5]  W. Kaufmann,et al.  Social behavior profile in young males with fragile X syndrome: Characteristics and specificity , 2004, American journal of medical genetics. Part A.

[6]  Deborah D. Hatton,et al.  Early Development, Temperament, and Functional Impairment in Autism and Fragile X Syndrome , 2000, Journal of autism and developmental disorders.

[7]  Silverman,et al.  Fragile X Syndrome Diagnosis Treatment And Research , 2016 .

[8]  Deborah D. Hatton,et al.  Cardiovascular indices of physiological arousal in boys with fragile X syndrome. , 2001, Developmental psychobiology.

[9]  Deborah D. Hatton,et al.  Mapping nonverbal IQ in young boys with fragile X syndrome , 2005, American journal of medical genetics. Part A.

[10]  J. Fryns,et al.  Fragile (X) syndrome: a study of the psychological profile in 23 prepubertal patients , 1987, Clinical genetics.

[11]  Deborah D. Hatton,et al.  Adaptive behavior in children with fragile X syndrome. , 2003, American journal of mental retardation : AJMR.

[12]  Anthony S. Bryk,et al.  Hierarchical Linear Models: Applications and Data Analysis Methods , 1992 .

[13]  W. Kaufmann,et al.  Autism spectrum disorder in fragile X syndrome: Communication, social interaction, and specific behaviors , 2004, American journal of medical genetics. Part A.

[14]  N Holmes,et al.  The Disability Assessment Schedule: a brief screening device for use with the mentally retarded , 1982, Psychological Medicine.

[15]  A. Reiss,et al.  Autism: The Point of View from Fragile X Studies , 1998, Journal of autism and developmental disorders.

[16]  P. Graham,et al.  Fragile X Syndrome, Autism and Autistic Features , 1997 .

[17]  L. Wing,et al.  The MRC Handicaps, Behaviour & Skills (HBS) schedule , 1980 .

[18]  J. Holden,et al.  Behavioral relationship between autism and fragile x syndrome. , 2003, American journal of mental retardation : AJMR.

[19]  Deborah D. Hatton,et al.  Autistic Behavior in Young Boys with Fragile X Syndrome , 1998, Journal of autism and developmental disorders.

[20]  T. Achenbach Manual for the child behavior checklist/4-18 and 1991 profile , 1991 .

[21]  S. Hepburn,et al.  Imitation performance in toddlers with autism and those with other developmental disorders. , 2003, Journal of child psychology and psychiatry, and allied disciplines.

[22]  A L Reiss,et al.  Fragile X syndrome, DSM-III-R, and autism. , 1990, Journal of the American Academy of Child and Adolescent Psychiatry.

[23]  P. Mirrett,et al.  Receptive and expressive communication development of young males with fragile X syndrome. , 2001, American journal of mental retardation : AJMR.

[24]  Deborah D. Hatton,et al.  Academic skills of boys with fragile X syndrome: profiles and predictors. , 2005, American journal of mental retardation : AJMR.

[25]  Deborah D. Hatton,et al.  Problem behavior in boys with fragile X syndrome. , 2002, American journal of medical genetics.

[26]  A. Couteur,et al.  Autism Diagnostic Interview-Revised: A revised version of a diagnostic interview for caregivers of individuals with possible pervasive developmental disorders , 1994, Journal of autism and developmental disorders.

[27]  J. Leckman,et al.  The trajectory of cognitive development in males with fragile X syndrome. , 1989, Journal of the American Academy of Child and Adolescent Psychiatry.

[28]  J. Opitz,et al.  An analysis of autism in fifty males with the fragile X syndrome. , 1986, American journal of medical genetics.

[29]  S. Hepburn,et al.  Parent Reports of Sensory Symptoms in Toddlers with Autism and Those with Other Developmental Disorders , 2003, Journal of autism and developmental disorders.

[30]  F. Montecchi,et al.  Childhood Autism Rating Scale (CARS) and Autism Behavior Checklist (ABC) Correspondence and Conflicts with DSM-IV Criteria in Diagnosis of Autism , 2004, Journal of autism and developmental disorders.

[31]  Deborah D. Hatton,et al.  Autistic Behavior, FMR1 Protein, and Developmental Trajectories in Young Males with Fragile X Syndrome , 2001, Journal of autism and developmental disorders.

[32]  B. Leventhal,et al.  The Autism Diagnostic Observation Schedule—Generic: A Standard Measure of Social and Communication Deficits Associated with the Spectrum of Autism , 2000, Journal of autism and developmental disorders.

[33]  A. Reiss,et al.  Autistic Behaviors Among Girls with Fragile X Syndrome , 1997, Journal of autism and developmental disorders.

[34]  P. Hagerman,et al.  The fragile-X premutation: a maturing perspective. , 2004, American journal of human genetics.

[35]  F. Tassone,et al.  Autistic Spectrum Disorder and the Fragile X Premutation , 2004, Journal of developmental and behavioral pediatrics : JDBP.

[36]  A. Reiss,et al.  Behavioral phenotype of fragile X syndrome: DSM-III-R autistic behavior in male children. , 1992, American journal of medical genetics.

[37]  D. McIntosh,et al.  Electrodermal responses to sensory stimuli in individuals with fragile X syndrome: a preliminary report. , 1999, American journal of medical genetics.

[38]  M. Appelbaum,et al.  Estimating Individual Developmental Functions: Methods and Their Assumptions , 1991 .

[39]  A. Reiss,et al.  Social behavior and cortisol reactivity in children with fragile X syndrome. , 2006, Journal of child psychology and psychiatry, and allied disciplines.

[40]  Deborah D. Hatton,et al.  Variability in FMRP and early development in males with fragile X syndrome. , 2001, American journal of mental retardation : AJMR.

[41]  S. Rogers,et al.  The Behavioral Phenotype in Fragile X: Symptoms of Autism in Very Young Children with Fragile X Syndrome, Idiopathic Autism, and Other Developmental Disorders , 2001, Journal of developmental and behavioral pediatrics : JDBP.

[42]  D. Hay Does IQ decline with age in fragile-X? A methodological critique. , 1994, American journal of medical genetics.

[43]  G. Baranek,et al.  Nonverbal Assessment of IQ, Attention, and Memory Abilities in Children with Fragile-X Syndrome Using the Leiter-R , 2000 .

[44]  A. Reiss,et al.  The influence of environmental and genetic factors on behavior problems and autistic symptoms in boys and girls with fragile X syndrome. , 2001, Pediatrics.

[45]  T. Hastie,et al.  Cortisol and behavior in fragile X syndrome , 2002, Psychoneuroendocrinology.