Sonja Mensch

35 A systematic review of the literature Table 4. (continued) Instrument Study Study population Psychometrics Methodological quality Diagnoses N Age (Y) COSMIN property Results Rating COSMIN boxes* 1 Rating of results* 2 Modified Hammersmith Functional Motor Scale (MHFMS) (Glanzman et al., 2011) SMA Type II and III 70 4.6-15.7 Construct validity Correlation between HFMSE and GMFM r= .98, CI .97-.99, p<.0001 Fair + 56 Correlation between HFMSE and forced vital capacity r= .87, CI .78-.92, p<.0001 Fair + 60 Correlation between HFMSE and Knee Extension Myometry r= .74, CI .60-.84, p<.0001 Fair + SMA Type II and III 58/ 12 HFMSE scores higher in subjects needed <8 hours bi-level positive airway per day (median = 23, IQR =9-50) –vs at least 8 hours per day (median =3, IQR = 0-7.5) Poor ? SMA Type II and III 25/ 44 HFMSE scores higher in ambulatory subjects (median=52, IQR = 48- 57, p<.0001) vs non-ambulatory (median=8, IQR 2-18.5, p<.0001) Poor ? SMA Type II and III 33/ 37 HFMSE scores higher in SMA type III (median 49, IQR 29-55, p<.0001) vs SMA type II (median=8, IQR=2-17, p<.0001) Poor ? (O’Hagen et al., 2007) SMA type II and III 38/ 17 Intra-rater reliability Combined group SMA type II and III/ SMA Type III HFMS ICC=.98 / .97 HFMSE ICC=.99/ .99 Poor +