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Muscle weakness in children with neurofibromatosis type 1
Author(s) -
Cornett Kayla M D,
North Kathryn N,
Rose Kristy J,
Burns Joshua
Publication year - 2015
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.12777
Subject(s) - isometric exercise , neurofibromatosis , weakness , medicine , elbow , muscle weakness , muscle strength , physical therapy , pediatrics , physical medicine and rehabilitation , anatomy , pathology
Aim To investigate if children with neurofibromatosis type 1 ( NF 1) 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 NF 1 (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 NF 1 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 NF 1 compared with the controls ranged from 3% to 43%. Males and females were equally affected. Interpretation This study shows that children with NF 1 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 NF 1 is associated with a primary myopathy.