Table 2.
Summary of participant characteristics.
| Study | Sample size (baseline), n | Controls, n | Sex, n | Age (y), mean (SD) | Disease phenotype, n | Symptom duration at baseline (mo), median and range | ALSFRS-Ra total score at baseline, mean (SD) | |||||||
|
|
Male | Female |
|
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| van Eijk et al [24] | MNDb: 42 | —c | 31 | 11 | 60 (12) | MND subtypes: ALSd: 39; progressive muscular atrophy: 3; and primary lateral sclerosis: 0 | 25 (7-218) | 36 (8) | ||||||
| Garcia-Gancedo et al [20] | ALS: 25 | — | 21 | 4 | 53.1 (9.93) | Phenotype at onset: upper limb: 15; lower limb: 6; upper limb and lower limb: 2; and bulbar=2 | Reported in 22 participants as <18 months | 41.6 (4.98) | ||||||
| Kelly et al [22] | ALS: 25 | — | 21 | 4 | 53.1 (9.93) | Phenotype at onset: upper limb: 15; lower limb: 6; upper limb and lower limb: 2; and bulbar: 2 | <3 months: n=3; 3-6 months: n=8; 6-12 months: n=9; 1 year-18 months: n=2; and missing data: n=3 | 41.6 (4.98) | ||||||
| Rutkove et al [21] | ALS: 75 (111 consented, and 75 began contributing data) | 25 (30 consented and 25 began contributing data) | Baseline characteristicse MND: 65 and controls: 9 | Baseline characteristicse MNDb: 42 and controls: 20 | 60 (30-80)f and controls: 51 (27-79)f | — | — | 34 (9-43)f | ||||||
| Rutkove et al [23] | ALS: 72 (113 enrolled and 72 provided data at least once) | — | 50 | 22 | 60.1 (9.9) | Not disclosed | Not disclosed | 36.1 (no SD reported) | ||||||
| Karas et al [25] | ALS=45 | — | 29 | 16 | 60.1 (10.7) | Symptom onset site: nonbulbar: 31; bulbar: 8; and unknown or not reported: 6 | 50 (93-281) and unknown or not reported: n=6 | 36.0 (6.2) | ||||||
| Holdom et al [26] | MND wrist: 97 and hipg: 42 | 58 | MND wrist: 75; MND hipg: 31; and controls wrist: 29 | MND wrist: 22, MND hipg: 11; and controls wrist: 29 | MND wrist: 60.69 (IQR 12.55); MND hipg: 61.28 (IQR 15.74); and controls wrist: 55.33 (IQR 16.11) | MND subtype: wrist: ALS: 87; progressive muscular atrophy: 1; primary lateral sclerosis: 6; and hipg: ALS: 39, progressive muscular atrophy: 3, and primary lateral sclerosis: 0 | MND wrist: 21.31 (IQR 13.27) and MND hipg: 24.92 (IQR 21.39) | Wrist: 38 (IQR 9) and hipg: 38 (IQR 12) | ||||||
| Johnson et al [27] | ALS: 46 enrolled, 40 met the analysis sample criteria | — | Total: 25; wrist cohort: 12; and ankle cohort: 13 | Total: 15; wrist cohort: 8; and ankle cohort: 7 | Total: 61.8 (12.0); wrist cohort: 62.9 (13.4); and ankle cohort: 60.6 (10.7) | — | — | Total: 31.4 (8.1); wrist cohort: 31.4 (8.6); and ankle cohort: 31.4 (7.9) | ||||||
| Gupta et al [28] | ALS: 376 | 26 | ALS: 247 and controls: 14 | ALS: 129 and controls: 12 | ALS: 57 (21-79)e and 33 (20-67)f | First symptoms include: upper limb: 159; lower limb: 164; bulbar symptoms: 75; and respiratory symptoms: 9 | 22.8 (0-246.3) | 41 (14-48)f | ||||||
| van Unnik et al [29] | ALS: 97 (2 cohorts); first cohortg: 42) and second cohort: 55 | — | Both cohorts: 68; first cohortg: 31 and second cohort: 37 | Both cohorts: 29; first cohortg: 11 and second cohort: 18 | Both cohorts: 60.5 (11.1); first cohortg:59.9 (11.6); and second cohort: 61 (10.7) | Both cohorts: ALS: 94; progressive muscular atrophy: 3; first cohortg: ALS: 39; progressive muscular atrophy: 3; and second cohort ALS: 55 | Both cohorts: 22.1 (2.2-217.9; First cohortg: 24.9 (6.9-217.9) and second cohort: 18.5 (2.2-93.9) | Both cohorts: 37.9 (6.8); first cohortg: 36.3 (8.1); and second cohort: 39.1 (5.3) | ||||||
| Beswick et al [30] | MNDb: 10 | — | 8 8 | 2 | 62 (12) | ALS: 5; primary lateral sclerosis: 2; and not disclosed: 3 | Survival length: long survivor (>8 years): n=2 | 40 (6) | ||||||
| Straczkiewicz et al [31] | ALS: 20h | — | 12h | 8i | 61.4 (10.6)h | Not disclosedc | Not disclosedc | No baseline reported-estimated baseline total score: 34.4 (30.4-38.3)h | ||||||
aALSFRS-R: Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised.
bMND: motor neuron disease.
cNot available.
dALS: amyotrophic lateral sclerosis.
eData taken from Rutkove et al [21] baseline data for the entire group of individuals enrolled.
fData reported as median and range.
gAs van Eijk et al [24].
hAs Johnson et al [27] wrist cohort.