Multidimensional Development and Adaptive Behavioral Functioning in Younger and Older Children With Rett Syndrome

Shih-Ya Wang; Wang-Tso Lee; Jeng-Yi Shieh; Yen-Hsun Huang; Lee-Chin Wong; Chih-Hsuan Tsao; Yi-Lun Chiu; Yen-Tzu Wu

doi:10.1093/ptj/pzab297

Multidimensional Development and Adaptive Behavioral Functioning in Younger and Older Children With Rett Syndrome

Phys Ther. 2022 Apr 1;102(4):pzab297. doi: 10.1093/ptj/pzab297.

Authors

Shih-Ya Wang^{1

2}, Wang-Tso Lee^{3

4

5}, Jeng-Yi Shieh⁶, Yen-Hsun Huang^{7

8}, Lee-Chin Wong^{3

9}, Chih-Hsuan Tsao^{10

11}, Yi-Lun Chiu¹, Yen-Tzu Wu^{1

12}

Affiliations

¹ School and Graduate Institute of Physical Therapy, College of Medicine, National Taiwan University, Taipei, Taiwan.
² Department of Physical Medicine and Rehabilitation, Da Chien Health Medical System, Miaoli County, Taiwan.
³ Department of Pediatric Neurology, National Taiwan University Children's Hospital, Taipei, Taiwan.
⁴ Department of Pediatrics, College of Medicine, National Taiwan University, Taipei, Taiwan.
⁵ Graduate Institute of Brain and Mind Sciences, College of Medicine, National Taiwan University, Taipei, Taiwan.
⁶ Department of Physical Medicine and Rehabilitation, National Taiwan University, Taipei, Taiwan.
⁷ Being Alive Clinic, Taipei, Taiwan.
⁸ Department of Clinical Psychology, Fu Jen Catholic University, New Taipei City, Taiwan.
⁹ Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan.
¹⁰ Department of Psychology, National Taiwan University, Taipei, Taiwan.
¹¹ Department of Foreign Languages and Literatures, National Taiwan University, Taipei, Taiwan.
¹² Physical Therapy Center, National Taiwan University Hospital, Taipei, Taiwan.

PMID: 35023562
DOI: 10.1093/ptj/pzab297

Abstract

Objective: The purpose of this study was to examine clinical severity, multidimensional development, and adaptive behavioral functioning in younger and older children with Rett syndrome (RTT) in the pseudostationary stage (stage III).

Methods: Fourteen younger (≤10 years of age) and 15 older (11-18 years of age) children with confirmed stage III RTT (assigned to young-RTT and old-RTT groups, respectively) participated in this study. Clinical severity was determined using the Clinical Severity Score (CSS) scale for RTT. The children's cognitive, language, motor, and sociocommunicative development was assessed using the Mullen Scales of Early Learning (MSEL) and the Early Social Communication Scale (ESCS). Their adaptive behavioral and daily functional skills were assessed using the Vineland Adaptive Behavior Scales-Chinese version (VABS-C) and Pediatric Evaluation of Disability Inventory-Chinese version (PEDI-C).

Results: Compared with the young-RTT group, the old-RTT group had higher severity of scoliosis on the CSS scale, poorer fine motor scores on the MSEL, reduced eye contact, reduced alternating eye gaze, and reduced turn-taking during social interaction on the ESCS. However, none of the VABS-C or PEDI-C subscale scores differed significantly between the groups. Higher CSSs were significantly correlated with lower scores in several subscales of MSEL, ESCS, VABS-C, and PEDI-C, especially for gross motor, mobility, and socialization functioning in all children with RTT.

Conclusion: Age-related differences in fine motor and sociocommunicative skills were observed between the young-RTT and old-RTT group, as measured using standardized assessments. Greater severity of RTT was correlated with poor motor, sociocommunicative, adaptive behavioral, and daily functional skills in stage III RTT.

Impact: Practitioners should be aware of clinical severity and the differences of developmental and adaptive behavioral functioning between younger and older children in the pseudostationary stage of RTT to provide specific age-related treatments.

Lay summary: With an understanding of severity and differences of developmental and adaptive behavioral functioning between younger and older children, clinical professionals can provide specific age-related treatments.

Keywords: Children; Development; Pseudostationary; Rett Syndrome; Severity.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Child
Humans
Language
Learning
Rett Syndrome*