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Hammarén E.1,2, Kjellby-Wendt G.1,2, Lindberg C.2,3
1Sahlgrenska University Hospital, Physiotherapy Dpt, Göteborg, Sweden, 2Sahlgrenska Academy at University of Gothenburg, Dpt of Clinical Neuroscience and Rehabilitation, Institute of Neuroscience and Physiology, Göteborg, Sweden, 3Sahlgrenska University Hospital, Clinical Genetics, Göteborg, Sweden
Background: Myotonic dystrophy type 1 (DM1) is a dominantly inherited neuromuscular disorder with a slowly progressive weakness. It is a rare disease, the prevalence is 3-15/100000 in Europe, but much more common than other muscular dystrophies. Functional disturbances in different organs are common as DM1 is also a multisystem disorder (Gagnon, 2007). Presently, there is no cure for patients with DM1, but the research is on-going. The Neuromuscular Centre, Sahlgrenska University Hospital, in Sweden is a centre where physiotherapist and other health professionals, on a yearly basis, follow these persons.
The needs for care and exercise are great. Falling and activity restriction due to fear of falls seem to be a common problem.
For persons with DM1 the reliability of measurement methods, the natural course of the muscle force decrease and functional balance impairment and the factors behind functional balance skills and falls have not been investigated.
The needs for care and exercise are great. Falling and activity restriction due to fear of falls seem to be a common problem.
For persons with DM1 the reliability of measurement methods, the natural course of the muscle force decrease and functional balance impairment and the factors behind functional balance skills and falls have not been investigated.
Purpose: The aims were, in adults with DM1, to investigate: the natural course of leg muscle force and functional balance impairments; factors of importance for functional balance skills and falls; the reliability of functional balance measurement methods.
Methods: All studies were quantitative with continuous and categorical data.
All genetically proven 20-60 years old persons with DM1 in the western part of Sweden, able to perform Timed Up&Go (TUG), were invited to participate.
The measurements were performed at the clinic at baseline and after five years.
The isometric muscle force was measured with a handheld gauge-meter.
The TUG was performed in a self-selected safe pace.
10-meter walk (10MW) was performed in a long corridor, with standing start and flying finish in both self-selected and maximal speed.
Step test (STEP) was performed as described by Hill, 1996.
Number of falls was asked for by recall.
The Activities-specific Balance Confidence (ABC) scale measured fear of falls.
The reliability study was performed with only 10 persons, but they were tested at three sessions one week apart to examine any systematic difference/learning effect.
Results: N=43, 18 men/25 women were followed during five years. A force decrease was shown in examined leg muscles (mean change -8 to -15 N, p =0.017 to 0.001), but the men had a worse natural course and had all fallen the year prior to the five-year-assessment.
All functional measures showed deteriorations (p 0.001).
Factors of importance for falls showed to be the balance confidence (ABC) score and the isometric ankle dorsiflexor force.
All the functional balance tests had reliable repeatability, ICC2.1 0.83-0.94 and satisfying (low) variability.
Conclusion(s): Dynamic functional balance tests are more consistent than static tests and should be preferred.
Both isometric leg muscle force and functional balance measures deteriorated significantly after five years, but the course was worse in the men. An increasing proportion of the persons with DM1 avoided activities due to fear of falling.
Implications: This patient group should be
-regularly followed
-asked about falls
-encouraged to wear orthoses, use walking aids, and (if needed)
-provided with electric wheelchair for continued participation in different activities
Funding acknowledgements: Trust fund of Norrbacka-Eugenia; Promobilia; The Local Research and Development Board for Gothenburg/Bohuslän; Muskelfonden; Greta and Einar Asker foundation.
Topic: Professional practice: other
Ethics approval: The study was approved by the Regional Ethical Review Board in Gothenburg, Sweden, dnr 248-06.
All authors, affiliations and abstracts have been published as submitted.
