Ulla Werlauff Methods to assess physical functioning - Danske ...

upper limb scale -0.627). There was no difference in FVC% mean value between younger patients
and older patients with SMA II.
In study III, FVC% was evaluated over a period of 15 years (13-16) in ten patients with SMA II and
five patients SMA III. Two of the SMA II patients were ventilated via tracheotomy at the last
assessment. Consequently, FVC% from the previous assessment (two years before) was used for
calculations in these two patients. There was no difference between first and last assessments for
patients with SMA II (p = 0.184) nor for patients with SMA III (p = 0.188).
Figure 5 FVC% over time in SMA II patients (n = 10/blue) and SMA III patients (n = 5/red).
with tracheostomy at last assessment. Change over time (p = 0.184, p = 0.188)
= patients
Assessments at activity level
Brooke upper limb scale (studies I, II, III)
Brooke score correlated highly with a total MRC% score (-0.885), and MRC% score of the upper
limbs (0.887). Correlation with age was moderate (0.452); nevertheless younger patients had a
higher level of upper limb function (lower Brooke score) compared to older patients (p = 0.003).
Brooke score in individual patients changed significantly over time (p < 0.0001). Approximately
20% of the patients in studies I and II were categorized at Brooke level 6 corresponding to no useful
function of hands. This indicates a floor effect in patients with SMA II, which was supported by the
fact that several of these patients possessed upper limb capabilities, which could be measured by
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