Table 1.
Patient and study characteristics
| Study characteristics | Patient characteristics | |||||||
|---|---|---|---|---|---|---|---|---|
| Author (date) | Country of research | Sample size n (male n) |
Design | Age in years | Time since onset (O)/ Time since diagnosis (D) |
ALSFRS | Diagnostic criteria |
Onset bulbar (%) |
| Bremer (2004)T0 [16] | U.S.A | 49 (29) | Longitudinal | 57.8 (13.0) | 34.9 (13.2) moO | 27.9 (6.3) | El Escorial | n.m |
| Chio (2004) [13] | Italy | 80 (49) | Cross sectional | 59.8 (12.6; 26–81) | 2.1 (1.7; 1–7.8) yrO | 26.6 (9.5; 3–38) | El Escorial | n.m. |
| Clarke (2001) [15] | Ireland | 26 (18) | Cross sectional | 63M (34–86) | 31.5 (4–156)M moD | 22.5M (11–36) | El Escorial | n.m |
| Dal Bello-Haas (2000) [47] | U.S.A | 60 (38) | Cross sectional | 56.2 (12.2) | n.m | n.m | El Escorial | n.m. |
| Ganzini (1999) [45] | U.S.A. | 100 (61) | Cross sectional | 54M (51.6–56.8) | 2.8 (2.0–3.6)M yrD | n.m | n.m | n.m |
| Gibbons (2013) [37] | U.K. | 147 (90) | Cross sectional | 61 (11; 35–81) | n.m | 22.3 (9.5; 4–48) | ‘confirmed diagnosis’ | n.m |
| Goldstein (2002) [38] | U.K | 31 (19) | Cross sectional | 64.0 (11.9) | 15.9 (5.2) moD | n.m | El Escorial | n.m |
| Ilse (2015) [10] | Germany | 49 (25) | Cross sectional | 63.8 (10.0) | 35.1 (36.3) moO | 32.6 (9.2) R | El Escorial | 33 % |
| Krampe (2008)T0 [27] | Germany | 31 (19) | Longitudinal | 60.3 (10.4; 32.9–79.7) | 96.3 (70.5; 22.4–330.4) wkO | 27.0 (6.6; 12–38) | El Escorial | 19 % |
| Lule (2008)T0 [28] | Germany | 39 (19) | Longitudinal | n.m | 43.9 (37.5; 0–170) moD | 19.9 (21.1; 0–39) | El Escorial | n.m |
| Matuz (2010) [36] | Germany | 27 (15) | Cross sectional | 55.3 (11.1; 35–73) | 36 (4–129) moD | 17.4 (9.8; 0–36) | ‘by a neurologist’ | 7 % |
| Matuz T0 (2015) [30] | Germany | 27 (15) | Longitudinal | 55.3 (11.1; 35–73) | 43.2 (30.5; 4–129) moD | 17.4 (9.8; 0–36) | El Escorial | 7 % |
| McCabe (2009) [19] | Spain | 120 (72) | Cross sectional | 63.2 (12.4) | 5.7 (5.8)O yr | n.m | n.m | n.m |
| Montel (2012) [46] | France | 49 (26) | Cross sectional | 63 (12) | 45 (28) moO | 28.2 (9.0)R | El Escorial | 22 % |
| Pagnini T0 (2015) [29] | Italy USA | 197 (115) | Longitudinal | a | a | 30.6(9.9)SA | ‘self-declared’ | n.m |
| Peric (2010) [40] | Serbia | 74 (45) | Cross sectional | 57 (11) | 29 (27) moO | 34 (8)R | El Escorial | n.m. |
| Pizzimenti (2013) [43] | Italy | 36 (22) | Cross sectional | 63.7 (10.9) | 22 (14) moO | 35.1 (8.7) | El Escorial | 22 % |
| Robbins (2001)T0 [31] | U.S.A | 60 (32) | Longitudinal | 58.5 (13.5; 27–83) | n.m | 28.1 (6.3; 12–39) | El Escorial | n.m |
| Simmons (2000) [14] | U.S.A | 96 (52) | Cross sectional | 57.8 (23–80) | 31.8 (2 mo-10 year) moO | 26.6 (9–39) | ‘met the criteria’ | n.m |
| Tramonti (2012) [42] | Italy | 40 (30) | Cross sectional | 59.1 (10.9; 34–84) | n.m | 20.8 (8.3; 7–36) | n.m | n.m |
| Vignola (2008)TD [41] | Italy | 29 (20) | Cross sectional | 63.6 (7.8; 44–78) | 11.7 (23.7; 2–43) moO | 33.1 (4.8; 22–39) | El Escorial | n.m |
| Winter (2010) [44] | Germany | 37 (21) | Cross sectional | 59.6 (11.0) | 2.3 (1.9) yrO | n.m | El Escorial | n.m |
Note. Numbers are presented as means (SD; range), unless stated otherwise
Abbreviations: n.m not mentioned, M median instead of mean, yr year, mo months, wk weeks, ALSFRS Amyotrophic Lateral Sclerosis Functional Rating Scale, R ALSFRS-Revised, SA self-administered ALSFRS, T0 data from baseline measurement, TD data from diagnostic phase, O onset, D diagnosis
a time since diagnosis and age were reported in categories