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

Author:

Wang Shih-Ya12,Lee Wang-Tso3ORCID,Shieh Jeng-Yi4,Huang Yen-Hsun5,Wong Lee-Chin36,Tsao Chih-Hsuan78,Chiu Yi-Lun1,Wu Yen-Tzu19ORCID

Affiliation:

1. School and Graduate Institute of Physical Therapy, College of Medicine, National Taiwan University, Taipei, Taiwan

2. Da Chien Health Medical System, Miaoli County, Taiwan

3. Department of Pediatrics, National Taiwan University Hospital, Taiwa

4. Department of Physical Medicine and Rehabilitation, National Taiwan University Hospital, Taiwan

5. Division of Child and Adolescent Psychiatry, Taipei City Psychiatric Center, Taipei City Hospital, Taipei, Taiwan

6. Department of Pediatrics, Cathay General Hospital, Taipei, Taiwan

7. Department of Psychology, National Taiwan University, Taipei, Taiwan

8. Department of Foreign Languages and Literatures, National Taiwan University, Taipei, Taiwan

9. Physical Therapy Center, National Taiwan University Hospital, Taipei, Taiwan

Abstract

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. Conclusions Age-related difference on fine motor and sociocommunicative skills were observed between younger and older children with RTT, 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.

Publisher

Oxford University Press (OUP)

Subject

Physical Therapy, Sports Therapy and Rehabilitation

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