Motor function-muscle strength relationship in spinal muscular atrophy

Luciano Merlini, Enrico Bertini, Carlo Minetti, Tiziana Mongini, Lucia Morandi, Corrado Angelini, Giuseppe Vita

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)548-552
Number of pages5
JournalMuscle and Nerve
Volume29
Issue number4
DOIs
Publication statusPublished - Apr 2004

Keywords

  • Forced vital capacity
  • Motor function
  • Muscle strength
  • Spinal muscular atrophy
  • Timed tests

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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