Excellent inter‐rater, intra‐rater, and telephone‐administered reliability of the ALSFRS‐R in a multicenter clinical trial

Abstract

We wished to determine whether the Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (ALSFRS-R) is reliable when used as primary outcome measure in a multicenter clinical trial. To establish inter-rater reliability, we randomly assigned 19 primary raters and 11 back-up raters to score nine amyotrophic lateral sclerosis (ALS) patients using the ALSFRS-R. To assess intra-rater reliability and reliability of telephone administration, we randomly assigned consecutive participants of the Clinical Trial of High Dose Coenzyme Q10 in ALS (QALS) to have in-person ALSFRS-R interviews at both screening and baseline visits (n=41 patients) or to have the ALSFRS-R interview by telephone at screening and in person at the baseline visit (n=27). An intraclass correlation coefficient (ICC) of reliability was calculated using a one-way random effects analysis of variance model. In the inter-rater reliability assessment, the primary raters performed 54 ratings on nine patients with ICC=0.93 (95% CI 0.84-0.98). For back-up raters, 32 ratings on nine patients resulted in ICC=0.93 (95% CI 0.82-0.98). The intra-rater reliability for in-person interviews was ICC = 0.95 (95% CI 0.92-0.98). The reliability of telephone administration compared to in-person interviews was ICC=0.97 (95% CI 0.93-0.98). We conclude that the ALSFRS-R shows excellent inter- and intra-rater reliability, and reliability of telephone administration when used as primary outcome measure in a multicenter ALS trial.

Motor unit number index (MUNIX): principle, method, and findings in healthy subjects and in patients with motor neuron disease

“…As an example, in Figure 1, the MUNIX would be 262 if we had used an SIP area of 10 in eq. (2). That would make the numerical data similar to other MUNE methods.…”

“…Clinically, amyotrophic lateral sclerosis (ALS) can be monitored with different scoring methods, such as the ALS Functional Rating Scale (ALSFRS-R), which also includes respiratory status. [1][2][3] The clinical assessment provides the functional output as a combination of central factors, motor neuron degeneration, and compensatory sprouting. A progressive loss of motor neurons may be clinically masked by the reinnervation process.…”

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

“…The validity of the scale has been shown in several studies to be high, in its being relatively sensitive to disease progression and being reliable [5][6][7]. The rate of deterioration also correlates with survival [8].…”

“…The rate of deterioration also correlates with survival [8]. An obvious advantage of such a measure is that it is very quick to perform and can even be completed over the telephone or online, making it simple to obtain regular data, even if the patient is unable to return to the clinic for evaluation [5]. It also ensures that all realms of potential disease progression are being captured.…”

Cross-cultural adaptation and validation of als Functional Rating Scale-Revised in Portuguese language

“…Within clinical evaluations that detail mortality, pulmonary function, muscular strength and incapacity, there is the Amyotrophic Lateral Sclerosis Functional Rating Sca le -ALSFRS [5][6][7] .…”