Fixed dynamometry is more sensitive than vital capacity or ALS rating scale

Abstract

ATLIS was more sensitive to change than ALSFRS-R or VC and could decrease sample size requirements by approximately one-third. The ability of ATLIS to detect prefunctional change has potential value in early trials. Muscle Nerve 56: 710-715, 2017.

Motor unit number index and neurophysiological index as candidate biomarkers of presymptomatic motor neuron loss in amyotrophic lateral sclerosis

“…The APB had greater mean‐MUNIX decline than the ADM, following the split‐hand phenomenon, and the HHD measures had a slight decline. This later finding of insidious loss of strength in the intrinsic hand muscles and finger flexors not captured by the fine motor functional subscore of the ALSFRS‐R or manual muscle testing supports the use of HHD as an objective and sensitive strength measure and is in line with prior studies …”

“…This later finding of insidious loss of strength in the intrinsic hand muscles and finger flexors not captured by the fine motor functional subscore of the ALSFRS-R or manual muscle testing supports the use of HHD as an objective and sensitive strength measure and is in line with prior studies. 29,30 As has been demonstrated in this study and in prior studies, 15,29 clinical examination and ALSFRS-R subscores may erroneously display disease stability in the early phase of ALS because muscle strength can be maintained by compensatory mechanisms until more than 70% of the motor units are lost. 17,31 Moreover, the duration of clinical measure stability may exceed the duration of a phase 2 study and is likely even longer in patients with slowly progressive disease (up to 15 months in our cohort).…”

“…As has been demonstrated in this study and in prior studies, clinical examination and ALSFRS‐R subscores may erroneously display disease stability in the early phase of ALS because muscle strength can be maintained by compensatory mechanisms until more than 70% of the motor units are lost . Moreover, the duration of clinical measure stability may exceed the duration of a phase 2 study and is likely even longer in patients with slowly progressive disease (up to 15 months in our cohort).…”

CDC Grand Rounds: National Amyotrophic Lateral Sclerosis (ALS) Registry Impact, Challenges, and Future Directions

“…Researchers can measure and monitor ALS progression and the effectiveness of drugs in clinical trials using self-rating of function with the ALS Functional Rating Scale or quantitative measures of muscle power, including pulmonary function tests (e.g., percentage of forced vital capacity, maximum inspiratory pressure, sniff nasal pressure), measurement of walking speed, and isometric muscle power ( 16 ). However, disease progression varies widely among patients.…”

Portable fixed dynamometry to quantify ankle dorsiflexion force

“…HHDs are inexpensive, relatively easy to use, and with proper technique they are reliable and validated against IKDs . PFD, which is used increasingly in clinical studies, represents a compromise between the strengths of IKD and practicality of MMT.…”