Article

AIM: To investigate if children with neurofibromatosis type 1 (NF1) have reduced
muscle strength compared with children with typical development. METHOD: Maximal
isometric strength of 15 upper and lower limb muscle groups was evaluated in 30
children with NF1 (16 males, 14 females; aged 4-16y) and 30 age-, sex-, height-,
and weight-matched controls using hand-held dynamometry by a single evaluator.
Both the left and right sides were assessed. RESULTS:
Children with NF1 were
significantly weaker than children with typical development across all 15 muscle
groups assessed (p<0.05). Apart from elbow flexion, there were no differences
between the left and right sides (p>0.05). Magnitude of differences between the
children with NF1 compared with the controls ranged from 3% to 43%. Males and
females were equally affected. INTERPRETATION:
This study shows that children
with NF1 have reduced muscle strength compared with children with typical
development. This muscle weakness is present from the earliest stages of the
disease assessed and persists throughout childhood with no sex difference. These
results support recent evidence from mouse studies that NF1 is associated with a
primary myopathy.
CI - (c) 2015 Mac Keith Press.

Langue : ANGLAIS