Variation factors of stereotypical hand movements in subjects with Rett syndrome

ABSTRACT

Background: Stereotypical hand movements have been observed in most individuals diagnosed with Rett syndrome. Objectives: To investigate factors that increase or decrease hand stereotypies in individuals with Rett syndrome. Methods: A questionnaire regarding hand stereotypies or purposeful hand behaviours was sent to 1016 schools for special needs education and 204 facilities in Japan. Results: Information was acquired from 216 cases (3–53 years old) with Rett syndrome; 81.9% and 87.6% of individuals had factors that increased and decreased stereotypical hand movements, respectively. Stereotypies were mainly increased by displeasure (63.8%) or pleasure (48.5%), and decreased by somnolence (43.5%), pleasure (30.0%), or food (24.1%). Conclusion: Emotion was the main factor increasing stereotypical hand movements, whereas there were a large number of factors that decreased these movements. The factors that decrease stereotypies could be useful to prevent the skin problems or joint contracture observed in patients with Rett syndrome.

KEYWORDS:

• Hand
• RTT
• SMID
• stereotypy
• upper extremity

Acknowledgments

We thank all participants who answered the questionnaire in this study, which has provided us with invaluable information. The present study was supported by JSPS KAKENHI Grant Number JP16K17472.

Disclosure statement

The authors report no conflicts of interest for the submission of this manuscript.

Additional information

Funding

The present study was supported by JSPS KAKENHI Grant Number [JP16K17472].