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. 2019 Mar 19;10:229. doi: 10.3389/fneur.2019.00229
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.