Table 2.
Average UE outcome measures scores for healthy subjects and DMD patients in different Brooke scales
| Healthy | DMD Brooke 1 | DMD Brooke 2 | DMD Brooke 3 | DMD Brooke 4 | DMD Brooke 5 | |||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) | P-value healthy/patient | P-value Brooke scale | |
| Functional UE scales | ||||||||||||||||||||
| Performance of Upper Limb Scale | ||||||||||||||||||||
| PUL shoulder | 20 | 16 | (.;.) | 5 | 13 | (10;15) | 8 | 6 | (3;10) | 4 | 0 | (.;.) | 3 | 0 | (.;.) | 2 | 0 | (.;.) | 0,000 | 0,002 |
| PUL elbow | 20 | 32 | (.;.) | 5 | 32 | (31;32) | 8 | 27 | (22;32) | 4 | 15 | (6;24) | 3 | 10 | (4;15) | 2 | 5 | (−15;24) | 0,000 | 0,002 |
| PUL wrist | 20 | 24 | (.;.) | 5 | 24 | (23;24) | 8 | 22 | (21;23) | 4 | 22 | (21;23) | 3 | 20 | (15;26) | 2 | 20 | (.;.) | 0,000 | 0,014 |
| Sum scorea | 20 | 78 | (.;.) | 5 | 74 | (71;76) | 8 | 60 | (52;68) | 4 | 41 | (31;50) | 3 | 32 | (21;43) | 2 | 26 | (6;45) | 0,000 | 0,001 |
| Physiologic UE outcome measures | ||||||||||||||||||||
| Maximal muscle force (N) | ||||||||||||||||||||
| Trapezius | 20 | 476 | (344;609) | 5 | 147 | (62;233) | 8 | 109 | (85;134) | 3 | 138 | (−4;280) | 3 | 59 | (29;90) | 3 | 40 | (−6;85) | 0,000 | 0,012 |
| Biceps | 20 | 178 | (139;217) | 5 | 37 | (27;47) | 8 | 27 | (19;34) | 3 | 21 | (17;24) | 3 | 7 | (−3;17) | 3 | 5 | (−2;12) | 0,000 | 0,003 |
| Triceps | 20 | 152 | (121;182) | 5 | 27 | (21;33) | 8 | 17 | (11;23) | 3 | 13 | (4;21) | 3 | 8 | (2;14) | 2 | 7 | (−40;53) | 0,000 | 0,011 |
| Deltoid | 20 | 83 | (64;102) | 5 | 32 | (24;40) | 8 | 21 | (14;27) | 3 | 21 | (17;26) | 2 | 15 | (3;26) | 2 | 7 | (−21;35) | 0,000 | 0,020 |
| Pectoralis major | 20 | 199 | (156;242) | 5 | 69 | (49;89) | 8 | 45 | (30;60) | 3 | 40 | (21;58) | 3 | 14 | (−6;34) | 3 | 15 | (7;23) | 0,000 | 0,003 |
| Wrist flexors | 20 | 127 | (102;152) | 5 | 29 | (14;44) | 7 | 27 | (11;42) | 3 | 26 | (−38;90) | 3 | 19 | (−9;47) | 2 | 14 | (−32;61) | 0,000 | 0,386 |
| Wrist extensors | 20 | 120 | (94;146) | 5 | 32 | (13;51) | 7 | 30 | (14;45) | 3 | 30 | (−20;80) | 3 | 8 | (−8;24) | 2 | 14 | (−19;47) | 0,000 | 0,061 |
| Sum score | 20 | 1334 | (1038;1631) | 5 | 373 | (271;474) | 7 | 278 | (212;344) | 3 | 288 | (16;561) | 2 | 147 | (−81;374) | 2 | 110 | (−192;412) | 0,000 | 0,024 |
| Maximal muscle torque (Nm) | ||||||||||||||||||||
| Trapezius | 20 | 86,4 | (57,3;115,5) | 5 | 24,6 | (11,5;37,7) | 8 | 16,2 | (11,9;20,5) | 3 | 24,8 | (−6,0;55,5) | 3 | 8,5 | (2,4;14,6) | 3 | 7,2 | (−2,6;16,9) | 0,000 | 0.016 |
| Biceps | 20 | 45,8 | (33,8;57,7) | 5 | 7,2 | (4,8;9,6) | 8 | 5,9 | (4,1;7,6) | 3 | 5,0 | (2,8;7,2) | 3 | 1,7 | (−1,2;4,5) | 3 | 1,3 | (−0,7;3,3) | 0,000 | 0.010 |
| Triceps | 20 | 38,2 | (28,9;47,5) | 5 | 5,4 | (4,0;6,8) | 8 | 3,8 | (2,3;5,4) | 3 | 3,2 | (1,2;5,2) | 3 | 1,8 | (−0,4;4,0) | 2 | 1,6 | (−11,1;14,3) | 0,000 | 0.024 |
| Deltoid | 20 | 41,4 | (29,8;52,9) | 5 | 11,1 | (8,4;13,7) | 8 | 8,6 | (5,5;11,6) | 3 | 9,5 | (7,9;11,2) | 2 | 5,6 | (4,9;6,2) | 2 | 2,7 | (−10,0;15,4) | 0,000 | 0.071 |
| Pectoralis major | 20 | 55,2 | (41,2;69,3) | 5 | 13,6 | (9,4;17,7) | 8 | 10,2 | (6,9;13,6) | 3 | 10,3 | (7,0;13,5) | 3 | 3,4 | (−3,0;9,8) | 3 | 3,8 | (1,4;6,2) | 0,000 | 0.010 |
| Wrist flexors | 20 | 8,4 | (6,4;10,4) | 5 | 2,9 | (−0,5;6,2) | 7 | 2,0 | (0,5;3,4) | 3 | 1,5 | (−1,9;4,9) | 3 | 1,1 | (−0,9;3,1) | 2 | 0,8 | (−1,2;2,7) | 0,000 | 0.311 |
| Wrist extensors | 20 | 7,9 | (5,8;10,0) | 5 | 3,2 | (−0,8;7,2) | 7 | 2,1 | (0,7;3,6) | 3 | 1,8 | (−0,7;4,3) | 3 | 0,4 | (−0,3;1,1) | 2 | 0,8 | (−1,2;2,7) | 0,000 | 0.027 |
| Sum score | 20 | 283,2 | (207,5;358,9) | 5 | 68,0 | (48,1;87,8) | 7 | 48,1 | (34,0;62,2) | 3 | 56,1 | (19,0;93,2) | 2 | 25,5 | (−35,5;86,5) | 2 | 19,5 | (−65,0;103,9) | 0,000 | 0.026 |
| Maximal sEMG amplitude (mV) | ||||||||||||||||||||
| Trapezius | 20 | 0,38 | (0,27;0,50) | 5 | 0,19 | (0,03;0,35) | 8 | 0,16 | (0,09;0,24) | 4 | 0,09 | (0,05;0,13) | 3 | 0,07 | (−0,04;0,19) | 3 | 0,02 | (0,00;0,03) | 0,000 | 0,032 |
| Biceps | 20 | 0,89 | (0,73;1,05) | 5 | 0,19 | (0,01;0,37) | 8 | 0,15 | (0,09;0,21) | 4 | 0,11 | (−0,06;0,28) | 3 | 0,07 | (−0,01;0,15) | 2 | 0,02 | (−0,18;0,21) | 0,000 | 0,096 |
| Triceps | 20 | 0,61 | (0,48;0,74) | 5 | 0,10 | (0,03;0,16) | 8 | 0,07 | (0,05;0,09) | 4 | 0,03 | (0,02;0,03) | 3 | 0,04 | (−0,02;0,09) | 3 | 0,02 | (−0,02;0,05) | 0,000 | 0,004 |
| Deltoid | 20 | 0,61 | (0,50;0,72) | 5 | 0,17 | (0,12;0,22) | 8 | 0,20 | (0,10;0,30) | 3 | 0,07 | (.;.) | 2 | 0,06 | (−0,14;0,25) | 3 | 0,06 | (−0,06;0,19) | 0,000 | 0,023 |
| Pectoralis major | 20 | 0,57 | (0,42;0,73) | 5 | 0,12 | (0,04;0,20) | 8 | 0,08 | (0,04;0,11) | 4 | 0,03 | (0,00;0,05) | 3 | 0,05 | (−0,04;0,14) | 3 | 0,01 | (0,00;0,03) | 0,000 | 0,007 |
| Wrist flexors | 20 | 0,26 | (0,19;0,32) | 5 | 0,06 | (0,04;0,09) | 8 | 0,07 | (0,04;0,11) | 4 | 0,05 | (0,02;0,07) | 3 | 0,03 | (0,01;0,05) | 3 | 0,04 | (0,02;0,06) | 0,000 | 0,056 |
| Wrist extensors | 20 | 0,47 | (0,38;0,56) | 5 | 0,13 | (0,06;0,20) | 8 | 0,18 | (0,08;0,28) | 4 | 0,10 | (0,05;0,15) | 3 | 0,12 | (−0,10;0,34) | 3 | 0,05 | (−0,03;0,12) | 0,000 | 0,090 |
| Sum score | 20 | 3,79 | (3,28;4,30) | 5 | 0,96 | (0,57;1,34) | 8 | 0,92 | (0,71;1,13) | 3 | 0,39 | (0,32;0,46) | 2 | 0,51 | (−1,08;2,09) | 2 | 0,21 | (−0,62;1,03) | 0,000 | 0,011 |
| Z-scores Echogenicity (1/3 ROI) | ||||||||||||||||||||
| Trapezius | 16 | 0,37 | (−0,27;1,02) | 5 | 3,24 | (0,89;5,59) | 8 | 3,23 | (1,35;5,11) | 4 | 2,90 | (−0,60;6,40) | 3 | 6,47 | (5,02;7,91) | 2 | 3,19 | (2,61;3,76) | 0,093 | |
| Deltoid | 16 | 0,36 | (−0,21;0,94) | 5 | 5,07 | (4,17;5,96) | 8 | 5,48 | (4,03;6,92) | 4 | 4,53 | (0,39;8,67) | 3 | 9,30 | (8,39;10,21) | 3 | 7,64 | (5,18;10,10) | 0,016 | |
| Biceps | 16 | 0,14 | (−0,30;0,59) | 5 | 5,73 | (3,39;8,06) | 8 | 5,74 | (4,44;7,04) | 4 | 6,35 | (3,91;8,78) | 3 | 7,38 | (5,88;8,88) | 3 | 6,96 | (3,08;10,84) | 0,418 | |
| Triceps | 16 | 0,23 | (−0,43;0,88) | 5 | 4,92 | (3,47;6,37) | 8 | 7,20 | (5,58;8,82) | 4 | 7,51 | (5,19;9,83) | 3 | 6,73 | (4,44;9,03) | 3 | 7,39 | (4,61;10,17) | 0,114 | |
| Wrist flexors | 20 | 0,47 | (−0,04;0,97) | 5 | 3,21 | (2,57;3,84) | 8 | 3,63 | (2,48;4,78) | 4 | 4,32 | (3,20;5,44) | 3 | 5,19 | (2,77;7,60) | 2 | 5,02 | (0,12;9,91) | 0,065 | |
| Wrist extensors | 14 | 0,24 | (−0,07;0,55) | 5 | 2,91 | (1,31;4,52) | 8 | 3,03 | (1,56;4,50) | 4 | 2,97 | (1,59;4,36) | 3 | 4,57 | (4,12;5,03) | 2 | 4,68 | (3,79;5,57) | 0,372 | |
| Mean score | 14 | 0,34 | (0,01;0,68) | 5 | 4,18 | (3,08;5,28) | 8 | 4,72 | (3,66;5,77) | 4 | 4,77 | (2,83;6,70) | 3 | 6,61 | (5,79;7,43) | 2 | 5,79 | (2,61;8,97) | 0,053 | |
| Healthy | DMD Brooke 1 | DMD Brooke 2 | DMD Brooke 3 | DMD Brooke 4 | DMD Brooke 5 | |||||||||||||||
| N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) | N | Mean | (95% CI) |
P-value healthy/ patient |
P-value Brooke scale | |
| Z-scores Muscle Thickness | ||||||||||||||||||||
| Trapezius | 15 | −0,04 | (−0,83;0,74) | 4 | 0,54 | (−1,15;2,23) | 7 | 1,08 | (−0,50;2,65) | 2 | 1,53 | (−17,15;20,21) | 2 | −1,40 | (−14,49;11,69) | 2 | 0,29 | (−8,67;9,24) | 0,401 | |
| Deltoid | 16 | 0,02 | (−0,77;0,80) | 4 | 0,82 | (−1,05;2,70) | 4 | 0,88 | (−0,96;2,71) | 1 | 0,02 | (.;.) | 3 | −1,86 | (−5,64;1,91) | 3 | 1,47 | (−5,05;7,99) | 0,289 | |
| Biceps | 16 | 0,73 | (−0,67;2,14) | 5 | −0,80 | (−2,75;1,14) | 7 | −1,64 | (−3,06;−0,22) | 2 | −1,61 | (−35,98;32,77) | 1 | −0,26 | (.;.) | 2 | 1,14 | (−26,69;28,97) | 0,684 | |
| Triceps | 16 | −0,22 | (−0,85;0,41) | 5 | 0,28 | (−1,40;1,96) | 5 | −1,01 | (−2,27;0,25) | 0 | . | (.;.) | 2 | −1,72 | (−5,08;1,65) | 0 | . | (.;.) | 0,087 | |
| Wrist flexors | 16 | −0,31 | (−0,96;0,33) | 5 | −1,08 | (−1,94;−0,23) | 7 | −1,10 | (−1,87;−0,33) | 2 | −1,86 | (−11,70;7,99) | 2 | −1,46 | (−9,97;7,05) | 1 | 0,83 | (.;.) | 0,466 | |
| Wrist extensors | 14 | 0,39 | (−0,31;1,08) | 5 | −0,04 | (−0,70;0,61) | 8 | 0,88 | (−0,34;2,10) | 4 | 0,76 | (−0,49;2,00) | 3 | −0,64 | (−5,03;3,75) | 1 | 0,97 | (.;.) | 0,494 | |
| Mean score | 13 | 0,13 | (−0,69;0,96) | 3 | −0,17 | (−1,13;0,79) | 4 | −0,48 | (−1,41;0,45) | 0 | . | (.;.) | 1 | −0,41 | (.;.) | 0 | . | (.;.) | 0,757 | |
| Passive maximal joint angles (°) | ||||||||||||||||||||
| Shoulder abduction | 20 | 149 | (142;155) | 5 | 155 | (145;164) | 8 | 151 | (136;166) | 4 | 151 | (134;168) | 3 | 128 | (97;160) | 2 | 114 | (63;165) | 0,420 | 0,046 |
| Elbow flexion | 20 | 145 | (143;148) | 5 | 133 | (126;139) | 8 | 138 | (131;145) | 4 | 134 | (116;151) | 3 | 135 | (114;155) | 3 | 129 | (123;134) | 0,000 | 0,595 |
| Elbow extension | 20 | 4 | (−1;9) | 5 | 4 | (−7;15) | 8 | 23 | (15;32) | 4 | 35 | (−11;80) | 3 | 50 | (−45;146) | 3 | 58 | (18;98) | 0,001 | 0,031 |
| Pronation | 20 | 94 | (85;103) | 5 | 70 | (37;103) | 8 | 56 | (33;78) | 4 | 73 | (16;129) | 3 | 76 | (32;119) | 3 | 57 | (19;94) | 0,001 | 0,665 |
| Supination | 20 | −59 | (−68;−50) | 5 | −56 | (−79;−34) | 8 | −50 | (−73;−27) | 4 | −7 | (−64;50) | 3 | −35 | (−152;83) | 3 | −9 | (−75;56) | 0,072 | 0,097 |
| Wrist flexion | 20 | −76 | (−82;−71) | 5 | −65 | (−88;−42) | 8 | −63 | (−83;−44) | 4 | −60 | (−78;−42) | 3 | −32 | (−127;63) | 3 | −43 | (−73;−12) | 0,001 | 0,187 |
| Wrist extension | 20 | 81 | (72;91) | 5 | 65 | (54;76) | 8 | 84 | (74;95) | 4 | 55 | (−6;116) | 3 | 65 | (25;106) | 3 | 29 | (−41;99) | 0,019 | 0,047 |
| Radial deviation | 20 | −34 | (−38;−30) | 5 | −37 | (−54;−21) | 8 | −41 | (−53;−30) | 4 | −29 | (−52;−5) | 3 | −19 | (−97;59) | 3 | −16 | (−34;3) | 0,893 | 0,188 |
| Ulnar deviation | 20 | 31 | (28;34) | 5 | 25 | (9;42) | 8 | 21 | (0;43) | 4 | 19 | (13;25) | 3 | 30 | (−29;89) | 3 | 20 | (−13;52) | 0,047 | 0,854 |
| Sum score | 20 | 666 | (643;690) | 5 | 603 | (525;680) | 8 | 581 | (519;644) | 4 | 492 | (265;720) | 3 | 470 | (248;691) | 2 | 398 | (−381;1177) | 0,000 | 0,109 |
| Active maximal joint angles (°) | ||||||||||||||||||||
| Shoulder flexion | 20 | 140 | (134;145) | 5 | 142 | (129;154) | 8 | 126 | (104;148) | 4 | 36 | (−3;75) | 0 | . | (.;.) | 0 | . | (.;.) | 0,003 | 0,001 |
| Shoulder abduction | 20 | 141 | (134;148) | 5 | 142 | (121;163) | 8 | 125 | (101;149) | 4 | 36 | (−2;73) | 0 | . | (.;.) | 0 | . | (.;.) | 0,002 | 0,001 |
| Shoulder adductionb | 20 | 129 | (124;135) | 5 | 128 | (120;137) | 8 | 93 | (70;116) | 4 | 19 | (−41;78) | 0 | . | (.;.) | 0 | . | (.;.) | 0,000 | 0,001 |
| Elbow flexion | 20 | 138 | (135;141) | 5 | 122 | (110;133) | 8 | 130 | (123;137) | 4 | 117 | (95;139) | 3 | 110 | (16;204) | 0 | . | (.;.) | 0,000 | 0,035 |
| Elbow extension | 20 | 7 | (2;12) | 5 | 9 | (1;17) | 8 | 29 | (17;41) | 4 | 49 | (12;86) | 3 | 40 | (−24;104) | 0 | . | (.;.) | 0,007 | 0,006 |
| Pronation | 20 | 80 | (73;87) | 5 | 59 | (34;84) | 8 | 62 | (51;73) | 4 | 67 | (0;134) | 3 | 50 | (2;99) | 3 | 13 | (−44;71) | 0,000 | 0,148 |
| Supination | 20 | −46 | (−52;−41) | 5 | −39 | (−54;−24) | 8 | −33 | (−52;−14) | 4 | 9 | (−47;65) | 3 | −10 | (−90;71) | 3 | −5 | (−28;18) | 0,001 | 0,066 |
| Wrist flexion | 20 | −67 | (−72;−61) | 4 | −57 | (−76;−37) | 8 | −57 | (−76;−38) | 4 | −57 | (−63;−52) | 3 | −16 | (−124;92) | 3 | −36 | (−59;−12) | 0,003 | 0,114 |
| Wrist extension | 20 | 80 | (73;86) | 4 | 78 | (48;108) | 8 | 77 | (67;86) | 4 | 46 | (−28;119) | 3 | 66 | (35;96) | 3 | 20 | (−49;90) | 0,027 | 0,063 |
| Radial deviation | 20 | −30 | (−35;−24) | 4 | −39 | (−53;−25) | 8 | −36 | (−48;−24) | 4 | −29 | (−43;−14) | 3 | −25 | (−97;47) | 3 | −14 | (−23;−6) | 0,365 | 0,177 |
| Ulnar deviation | 20 | 31 | (29;33) | 4 | 29 | (8;50) | 8 | 26 | (19;34) | 4 | 19 | (9;30) | 3 | 23 | (−15;61) | 3 | 14 | (−6;33) | 0,007 | 0,334 |
| Sum score | 20 | 875 | (846;903) | 4 | 838 | (782;894) | 8 | 736 | (658;814) | 4 | 367 | (176;557) | 3 | 260 | (151;369) | 3 | 102 | (−60;265) | 0,000 | 0,001 |
aPUL sum scores is calculated including the score of the entry item (maximal score = 78)
bShoulder adduction in the horizontal plane (Fig. 2.C.)
P-values healthy/patient show the differences between healthy subjects and patients. P-values Brooke scale show the differences between DMD patients in different Brooke scales. P-values > 0.05 indicate a statistical significant difference and are displayed bold. P-values healthy/patient are not shown for echogenicity and muscle thickness z-scores, as the z-scores already indicate the difference with a healthy reference population