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Updated: Aug 31, 2025

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Reliability of functional outcome measures in adults with neurofibromatosis 2
Rebecca Louise Mullin1,2, Rebecca Smith1,2, Susan Wood1
1National Centre for Neurofibromatosis, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Objective:
To determine inter- and intra-rater reliability of functional performance outcome measures in people with neurofibromatosis 2. To ascertain how closely objective and subjective measures align.
Methods:
Twenty-nine people with neurofibromatosis 2 were recorded performing the modified clinical test of sensory integration and balance, four square step test and modified nine-hole peg tests. Three raters scored each measure to determine inter-rater reliability. One rater scored the measures a second time to determine intra-rater reliability. Participants also completed a disease-specific quality of life questionnaire and dynamic visual acuity testing.
Results:
Inter-rater and intra-rater reliability scores (intra-class correlation coefficient) were excellent for all tests (intra-class correlation coefficient r ⩾ 0.9). The four square step test correlated with perceived walking challenges and modified clinical test of sensory integration and balance correlated with perceived balance challenges in a neurofibromatosis 2 quality of life patient report outcome measure.
Conclusion:
The modified clinical test of sensory integration and balance, four square step test and modified nine-hole peg tests are potentially useful measures for monitoring neurofibromatosis 2.

