Restricted and Repetitive Behaviors in Males and Females with Fragile X Syndrome: Developmental Trajectories in Toddlers
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ORIGINAL PAPER
Restricted and Repetitive Behaviors in Males and Females with Fragile X Syndrome: Developmental Trajectories in Toddlers Through Young Adults Lauren J. Moskowitz1 · Elizabeth A. Will2 · Conner J. Black2 · Jane E. Roberts2
© Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract There is limited research on the trajectory of restricted and repetitive behaviors (RRBs) in fragile X syndrome (FXS), with previous studies only examining males and/or examining RRBs as a unitary construct rather than delineating subtypes of RRBs. Thus, we described the trajectory of five subtypes of RRBs in 153 males and females with FXS (aged 1–18 years) with repeated measurement over time (445 total assessments). Multilevel modeling was used to test age-related differences in RRB subtypes between males and females with FXS, controlling for nonverbal IQ. Results showed that lower-order SensoryMotor behaviors decreased over time for both males and females, while there was no significant change in the higher-order RRBs. The trajectory between males and females differed for Self-Injury. Keywords Fragile X syndrome · Repetitive behavior · Developmental trajectory · Sensory motor behaviors · Restricted interests · Self-injurious behavior
Developmental Trajectories of Restricted and Repetitive Behaviors in Males and Females with Fragile X Syndrome Fragile X syndrome (FXS) is the most common heritable cause of intellectual disability (ID), with an estimated prevalence of approximately 1 in 4000–5000 males and 1 in 6000–8000 females (Coffee et al. 2009; Hunter et al. 2014). FXS is caused by a cytosine-guanine-guanine (CGG) repeat expansion on the Fragile X Mental Retardation-1 (FMR1) gene on the X chromosome, resulting in a loss of production of Fragile X Mental Retardation Protein (FMRP), which is considered to be responsible for the physical and behavioral phenotype of FXS. Because FXS is an X-linked disorder, females are differentially affected, and often experience reduced symptomatology and lower rates of comorbid ID (Hagerman and Hagerman 2002; Reiss and Dant 2003). In * Lauren J. Moskowitz [email protected] 1
Department of Psychology, St. John’s University, 8000 Utopia Parkway, Queens, NY 11439, USA
Department of Psychology, University of South Carolina, Columbia, SC, USA
2
addition to ID, FXS is also associated with a complex phenotypic profile that includes impairments in language and communication (Abbeduto et al. 2007), elevated physiological arousal (Klusek et al. 2013), socially avoidant behavior and anxiety (Cordeiro et al. 2011; Ezell et al. 2019; Roberts et al. 2019a, b), aggressive and self-injurious behavior (SIB) (Hall et al. 2008; Hessl et al. 2008), and restricted and repetitive behaviors (RRBs) (Lachiewicz et al. 1994). While many aspects of the FXS behavioral phenotype have been relatively well characterized, patterns of RRBs across age and in the context of sex and cognitive ability remain poorly characterized. RRBs are highly prevalent in FXS, with elevated rates in FX
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