Table 1.
Research studies considering motor and bulbar skills-related outcome measures.
| Primary outcome measure | Reference number | Authors | Other outcome measures in the study | Type of study | Number of patients | Duration of follow-up |
|---|---|---|---|---|---|---|
| MOTOR SKILLS-RELATED OUTCOME MEASURES | ||||||
| 6MWT | (44) | Takeuci et al. | Modified Norris score, ALSFRS-R, grip strength | Observational, longitudinal study | 35 at baseline, 24 at follow-up | 12 months |
| (45) | Querin et al. | MMT, ALSFRS-R, FVC | Pilot, unblinded pharmacological trial (Clenbuterol) | 20 | 12 months | |
| AMAT | (46) | Harris-Love et al. | QMA, 2MWT, ADL assessment, SF-36v2 | Observational, cross-sectional study | 55 | / |
| (47) | Shrader et al. | QMA, STS test, Timed up and Go test, Balance tests, SF-36v2, Beck depression scale, serum CK, IGF-1 and testosterone | Randomized, evaluator-blinded pharmacological trial (Physical exercise) | 50 | 12 weeks | |
| SBMAFRS | (48) | Hashizume et al. | ALSFRS-R, Modified Norris Score | Observational, longitudinal study | 80 | 12 months |
| (49) | Querin et al. | MMT, 6MWT, ALSFRS-R | Observational, longitudinal study | 60 | 8 weeks | |
| 1234 scale | (50) | Lu et al. | ALSFRS-R | Observational, longitudinal study | 81 | 32 months |
| ALSFRS-R | (51) | Banno et al. | VF, MMT, FVC, serum CK, AST, ALT, Beck depression scale, 1C2-positive cells in scrotal skin biopsies | Randomized, double-blinded pharmacological trial (Leuprorelin) | 50 | 48+96 weeks |
| QMA | (52) | Fernández-Rhodes et al. | AMAT, MMT, 2MWT, SF-36v2, IIEF, MUNE, CMAP VF, FVC, serum CK and testosterone | Randomized, double-blinded pharmacological trial (Dutasteride) | 50 | 24 months |
| Hand grip strength | (53) | Hijikata et al. | Modified QMG score, ALSFRS-R, SBMAFRS, 15-foot timed-walk test, rise-from-bed test, swallowing questionnaires, FVC, Multidimensional Fatigue Inventory, urinary 8-OHdG | Randomized, double-blinded pharmacological trial (Creatine Monohydrate) | 45 | 8 weeks |
| BULBAR FUNCTION-RELATED OUTCOME MEASURES | ||||||
| Tongue pressure | (54) | Mano et al. | Modified Norris score, ALSFRS-R, QMA, grip strength, MMT, modified QMG score, VF, swallowing questionnaires, timed walk test | Observational, cross-sectional study (validity of tongue pressure as marker of dysphagia) | 47 | / |
| (55) | Mano et al. | VF, modified QMG score, ALSFRS-R, serum CK and testosterone | Non-randomized, interventional study (head lift exercises) | 6 | 12 weeks | |
| VF | (56) | Hashizume et al. | ALSFRS-R, SBMAFRS, swallowing questionnaires, Limbs Norris score, Bulbar Norris score | Observational, longitudinal study | 111 | 30 days |
| (57) | Katsuno et al. | ALSFRS-R, 6MWT, modified QMG score, 1C2-positive cells in scrotal skin biopsies, serum CK and testosterone, ALSAQ-5 score | Randomized, double-blinded pharmacological trial (Leuprorelin) | 204 | 12 months | |
| FEES | (21) | Warnecke et al. | MMT, modified Rankin scale | Observational, cross-sectional study | 10 | / |
| INSTRUMENTAL OUTCOME MEASURES | ||||||
| MUNE | (58) | Suzuki et al. | Limbs Norris score, Bulbar Norris score, ALSFRS-R, grip strength | Observational, longitudinal study | 52 | 12 months |
| (59) | Lehky et al. | CMAP, SMUP | Observational, cross-sectional study | 54 | / | |
| CMAP and SNAPs | (29) | Suzuki et al. | Limbs Norris score, Bulbar Norris score, ALSFRS-R, spinal cord tissue specimens | Observational, cross-sectional study | 106 | / |
| Muscle MRI | (60) | Hamano et al. | / | Observational, cross-sectional study | 3 | / |
ALSFRS-R, Amyotrophic Lateral Sclerosis functional rating scale-revised; MMT, manual muscle testing; FVC, forced vital capacity; QMA, quantitative muscle assessment, 2 or 6MWT, 2 or 6 minutes-walk-test; ADL, activity of daily living; DXA, Dual-energy X-ray absorptiometry, urinary 8-OHdG, 8-hydroxydeoxyguanosine; VF, videofluoroscopy; AMAT, adult myopathy assessment tool; IIEF, International Index of erectile function; MUNE, motor unit number estimate; CMAP, compound motor action potential; CK, creatine-kinase; QMC score, quantitative myasthenia gravis score; SMUP, single motor unit potential.