Postural control in the congenital and childhood forms of myotonic dystrophy type 1

Abstract

Aims: To increase knowledge regarding postural control in congenital (CDM1) and childhood (ChDM1) forms of myotonic dystrophy type 1 and to analyze whether variations can be explained by age, joint motion, muscle strength and molecular findings.

Methodology: In a cross-sectional study, postural control was measured with the Bruininks-Oseretsky test, sub-test balance, range of motion (ROM) in ankle dorsiflexion and muscle strength in ankle dorsiflexors. Simple linear regression analysis was used to investigate the association between postural control, molecular findings, muscle strength, age and ROM.

Major findings: Forty-four individuals participated in the study. All individuals with CDM1 and 80% with ChDM1 had reduced postural control. Simple linear regression analysis shows a negative association between z-BOT2 and CTG repeat expansion (R² = .342, p = .000), a negative association with age (R² = .148, p = .006), a positive association with muscle strength in ankle dorsiflexors (R² = .205, p = .001) and a positive association with ROM in ankle dorsiflexion (R² = .078, p = .037).

Principal conclusion: Reduced postural control is common in CDM1 and ChDM1. Size of CTG repeat expansion, muscle strength in ankle dorsiflexors, age and ROM in ankle dorsiflexion all contribute to variations in postural control. These findings provide a better understanding of the disease and contribute to improved health care for the patient group.

Keywords:

• Neuromuscular
• balance
• physiotherapy
• joint motion
• muscle strength

Acknowledgements

Special thanks to the individuals who participated in the study.

Disclosure statement

No potential conflict of interest was reported by the authors.