Development and validation of a muscular dystrophy-specific functional rating scale

Abstract

The MDFRS is a reliable and valid disease-specific measure of functional status for patients with muscular dystrophy.

Medida da função motora: versão da escala para o português e estudo de confiabilidade

“…Th e methods of clinical evaluation in the diagnosis phase of the various neuromuscular diseases encompass simple observation of the pacient 1 in search of indicative phenotypes, qualitative exam of muscle tonus 6,7 , quantitative study of muscle strength 8 , motor skills in activities of daily living (Barthel Index) 9 , use of functional scales 10,11 and quality of life 12 . Functional scales have been designed for some of the most prevalent neuromuscular diseases.…”

Strength and Functional Measurement for Patients with Muscular Dystrophy

“…Four domains are included in MDFRS. It was developed in many stages: the preliminary pool of items, the admission of various types of muscular dystrophies and the reliability, validity and responsiveness studies (Lue et al 2006). The results showed the MDFRS is a reliable and valid disease-specific measure of functional status for patients with muscular dystrophy.…”

“…The scale offers much important information of muscular dystrophy such as the mobility ability, dependence of daily living, the arm function, and many impairment conditions. The arm function part of the MDFRS effectively conquers the disadvantages of the Brooke scale (Lue et al 2006;Lue 2010).…”

Development and preliminary evidence of the psychometric properties of the GNE myopathy functional activity scale

“…Instruments considered for use in GNEM clinical trials included the inclusion body myositis function rating scale [5], the muscular dystrophy functional rating scale [6], the NorthStar Ambulatory Assessment [7], the Amyotrophic Lateral Sclerosis functional rating scale [8], the Outpatient Physical Therapy Improvement in Movement Assessment Log [9] and the WHO Disability Assessment Schedule [10].…”

“…Finally, having been developed for use in other conditions, these existing measures lacked adequate content validity in the specific 'context of use' of GNEM studies. As such, none of the measures were deemed adequately specific to GNEM and the unique functional limitations experienced in these patients as a result of their distinct pattern of weakness [5][6][7][8][9][10][11] and thus, the need to develop an instrument specific to functional impairment in GNEM was identified. The GNEM functional activity scale (GNEM-FAS) was therefore developed.…”