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Motor function–muscle strength relationship in spinal muscular atrophy
Author(s) -
Merlini Luciano,
Bertini Enrico,
Minetti Carlo,
Mongini Tiziana,
Morandi Lucia,
Angelini Corrado,
Vita Giuseppe
Publication year - 2004
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20018
Subject(s) - spinal muscular atrophy , motor function , physical medicine and rehabilitation , medicine , atrophy , muscle strength , electromyography , physical therapy , disease
The relationship between motor function and muscle strength in patients with spinal muscular atrophy (SMA) is still controversial. In 120 genetically proven SMA patients, aged 5 years or older, we measured muscle strength in the arms and legs by a hand‐held dynamometer, forced vital capacity by a spirometer, and the time needed to walk 10 m, arise from the floor, and climb steps. SMA patients had markedly reduced muscle strength, approximating 20% of that predicted from age‐ and gender‐matched normative data. Knee extensors were the weakest muscles in SMA patients. The young ambulant SMA patients performed better than adults in all the timed tests and had greater muscle strength on knee extension. This study shows a good relationship between motor ability and muscle strength in SMA and confirms that age‐related loss of function in SMA is due to loss of muscle strength. Muscle Nerve 29: 548–552, 2004