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Results for “Muscular Atrophy, Spinal”

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PubMed2006

Valproate may improve strength and function in patients with type III/IV spinal muscle atrophy.

Spinal muscular atrophy (SMA) is a recessively inherited motor neuron disease caused by deficient survival motor neuron (SMN) protein. Valproate increases SMN protein in vitro by increasing transcription of SMN2 genes. The authors treated seven adult patients with SMA type III/IV with valproate for a mean duration of 8 months. The treated patients with SMA had increased quantitative muscle strength and subjective function. Further trials of valproate treatment for SMA type III/IV are warranted.

Adolescent↗
PubMed1992

Upper extremity strength and function in children with spinal muscular atrophy type II.

Upper extremity strength and function in a sample of ten children with spinal atrophy type II was determined using manual muscle testing, a sphygmomanometer, and a pinchometer. Functional abilities were recorded on self-report forms. Overall strength in this sample was determined to be 35% to 40% of normal. Repeated-measures analysis of covariance revealed no change with time in proximal or distal strength. Six children were capable of at least 1/2 pound of force in pinch or gross grasp vs four children with zero force in one of these measures. Ability to produce a measurable force in pinch or gross grasp was found to be associated with independence in mobility, hand function, and ADL by Fisher exact test (p = .02). These data document the weak, but stable, muscle force present in these children and relate the strength of function.

Activities of Daily Living↗