Author(s) and year publication | Follow-up interval (months) | Number of patients/Number of controls | Clinical assessment batteries/Functional rating scales | Summary of findings |
CLINICAL STUDIES | ||||
ALSFRS-R | ||||
Thakore et al. (70) | NA | 3367/0 | ALSFRS-R, ALSFRS, bloods- creatinine, uric acid, CK, albumin, sodium bicarbonate, hematocrit, TWBC | - ALSFRS-R progression detected, pre-slope and post-slope have effects on survival |
Rooney et al. (71) | NA | 407/0 | ALSFRS-R | - progression detected in ALSFRS-R subscores |
*ACTS trial. (72) | NA | 75/NA | ALSFRS | progression detected (ALSFRS-R), associated with motor and pulmonary function |
Cognitive and behavior assessments | ||||
Floeter et al. (73) | 18 | NA | ALSFRS-R, letter fluency, FBI | - progression detected (ALSFRS-R, FBI, letter fluency) |
Elamin et al. (74) | NA | 186/NA | cognitive testing | - progression detected (cognitive function) |
Roberts-South et al. (75) | 24 | 16/12 | neuropsychology, language, discourse sampling, perfusion computerized transaxial tomography, pulmonary, clinical | - progression detected (cognitive language deficits) |
*Duning et al. (76) | 3 | 10/32 | ALSFRS, clinical neuropsychological battery, imaging | - progression detected (DTI) |
Poletti et al. (77) | 24 | 168/0 | ECAS | - no progression detected, ECAS scores improved over time |
Xu et al. (78) | 6 | 108/60 | ACE-3, FAB, ECAS executive, MoCA, ALSFRS-R, ALS-FTD-Q, MiND-B | - no progression detected |
Gillingham et al. (79) | 9 | 20/36 | ALS-CFB, ALSFRS-R | - no progression reported |
Mioshi et al. (80) | 6 | 79/53 | MiND-B- apathy, disinhibition, stereotypical behavior, ACE-R, ALSFRS-R | - no progression reported |
Quality of life assessments | ||||
Jakobsson Larsson et al. (81) | 24 | 36/0 | SEIQoL-DW, ALSFRS-R, HADS | - anxiety decreased over time, depression correlated to QOL, QOL remained stable despite physical deterioration |
BMI and other clinical assessments | ||||
Beck et al. (82) | 6 | 78/39 | skin water loss | - progression detected (skin water loss) |
Garruto et al. (83) | NA | 31/66 | bone mass (wrist radiograph) | - progression detected (bone loss) |
Ioannides et al. (84) | 6 | 44/29 | FM-ADP, BMI, BAI, ALSFRS-R | - BMI and BAI not accurate measures of fat mass in ALS |
Peter et al. (85) | 3 | 393/791 | BMI, ALSFRS-R | - alterations in body weight present in ALS patients decades before manifestation of symptoms |
Nunes et al. (86) | 3 | 37/0 | BMI, serum albumin, transferrin, total cholesterol | - no progression reported |
Jablecki et al. (87) | NA | NA | clinical scores | - no progression reported |
Respiratory and muscle assessments | ||||
Andres et al. (88) | 4–21 | 100/0 | ATLIS, ALSFRS, VC | - ATLIS more sensitive to change than ALSFRS and VC |
de Bie et al. (89) | 12 | 10/0 | RSA, ALSFRS-R, FVC | - progression detected(RSA and ALSFRS-R) |
Shellikeri et al. (90) | NA | 33/13 | kinematic measures of tongue and jaw movement, speaking rate, intelligibility, ALSFRS-R | - progression detected (tongue movement size and speed) |
Londral et al. (91) | 2–20 | 19/26 | typing activity, ALSFRS-R | - progression detected (typing activity) |
Panitz et al. (92) | 12 | 51/0 | fatigue severity scale (FSS), CIS20-R- subjective fatigue experience, concentration, motivation, activity, ALSFRS-R, MRC, SVC | - progression detected (FSS, CIS20-R), correlated to ALSFRS-R, and ALSFRS-R progression |
Atassi et al. (93) | NA | 8635/0 | ALSFRS-R, VC | - PRO-ACT database- progression detected (ALSFRS-R and VC) |
Watanabe et al. (94) | 1.7 years | 451/0 | ALSFRS-R, MRC, MMT | -progression detected (ALSRS-R) |
Leonardis et al. (95) | every 3 months | NA/0 | ALSFRS-R, Norris-r, AGA, FVC, MIP, MEP, SNIP | - progression detected (respiratory measures) |
Mahajan et al. (96) | NA | 362/0 | VC | - progression detected (VC) |
Pinto et al. (97) | 4–6 | 49/0 | Diaphragm amplitude, ALSFRS-R, MIP, FVC, SNIP, SPO2 | - progression detected (Diaphragm amplitude, ALSFRS-R, respiratory measures) |
Montes et al. (98) | 6 | 31/0 | TUG, ALSFRS-R, FVC, MMT | - linear progression detected (TUG) - associated with ALSFRS-R, MMT |
Vender et al. (99) | NA | 139/0 | FVC | - progression detected (FVC) |
Wilson et al. (100) | NA | 55/NA | respiratory- FVC, FEV1, PEFT | - linear progression detected (PEFT) |
Poloni et al. (101) | NA | NA | VC, Motley index, FEV1 | - progression detected (respiratory measures) |
Andersen et al. (102) | 6–59 | 20/0 | respiratory- SVC, cough peak flow, max inspiratory muscle strength, SNIP, max insufflation capacity | - no progression reported |
Quaranta et al. (103) | NA | NA | respiratory function | - no progression reported |
Proudfoot et al. (104) | 24 | 61/39 | eye tracking- anti saccadic, trail making, visual search tasks, ALSFRS-R, ACE-R, UMN, imaging) | - no progression detected |
*Lenglet et al. (105) | 18 | 512/0 | ALSFRS-R, MMT, SVC | - clinical trial |
Yamauchi et al. (106) | Every 6 months | 43/30 | ALSFRS-R, phrenic nerve conduction study (DCMAP), respiratory function tests (SNIP, FVC), nocturnal pulsed oximetry, MMT | - no progression reported |
Mendoza et al. (107) | NA | 161/0 | MIP, FVC | - no progression reported |
Marti-Fabregas et al. (108) | NA | NA | FVC | - no progression detected |
Palmowski et al. (109) | NA | NA | electro-oculography | - not well-defined progression |
Studies detecting progressive changes are listed first followed by studies not capturing longitudinal changes.
indicates clinical trial.