Abstract:
: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.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Merlini L,Bertini E,Minetti C,Mongini T,Morandi L,Angelini C,Vita Gdoi
10.1002/mus.20018subject
Has Abstractpub_date
2004-04-01 00:00:00pages
548-52issue
4eissn
0148-639Xissn
1097-4598journal_volume
29pub_type
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