author={Paul H. Gordon and Robert G. Miller and D. Moore},
  journal={Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders},
  pages={90 - 93}
The ALSFRS‐R is an attractive primary outcome measure in clinical trials of ALS because it is validated, easy to administer, minimizes dropout, reduces cost, and correlates with survival. Unlike the other standard outcome measures currently employed, the ALSFRS‐R is also a measure of global function. 

Using the ALSFRS-R in multicentre clinical trials for amyotrophic lateral sclerosis: potential limitations in current standard operating procedures

Sudden 5-point or more increases in ALSFRS-R total scores between two consecutive visits are relatively common, but these sudden increases were not found to occur with equal frequency in trial centers, which underscores the need for amending existing standard operating procedures toward a universal version.

Clinical and neurophysiological biomarkers of disease progression in amyotrophic lateral sclerosis

Rate of disease progression, measured as change in the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS‐R) and body mass index (BMI), and the utility of these clinical biomarkers along with neurophysiological measures, such as the split hand index, in monitoring disease progression are assessed.

Primary lateral sclerosis (PLS) functional rating scale: PLS‐specific clinimetric scale

Our research aim was to develop a novel clinimetric scale sensitive enough to detect disease progression in primary lateral sclerosis (PLS).

Assessing upper limb function with ALSFRS-R in amyotrophic lateral sclerosis patients

ALSFRS-R is an important tool to assess functional UL involvement in ALS, however, it can mislead clinicians when evaluating right-handed patients with initial LUL onset form.

ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale

An adapted and annotated version of the ALSFRS-R scale was developed that closely adheres to the well-established standardized English version, and includes brief explanations of specific scale items and reply options to facilitate ALSF RS-R-SE assessments by both healthcare professionals and patients.

Patient reported outcome measures (PROMs) in amyotrophic lateral sclerosis

Collection of PROMs is feasible in the context of routine ALS care and PROM scores are highly correlated with validated ALS outcome measures.

Validation of the Italian version of the Rasch-Built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) administered to patients and their caregivers

The Italian version of the ROADS scale is a valid and reliable tool to monitor disease burden, showing a high level of agreement between the responses given by patients and caregivers.

Validation of the revised Amyotrophic Lateral Sclerosis Functional Rating Scale in Poland and its reliability in conditions of the medical experiment

Validation of the Polish version of the ALSFRS-R supports the reliability and internal consistency of scale and proved also to be a proper tool for monitoring the course of the experimental medical therapy for patients with ALS.

Clinical Measures of Bulbar Dysfunction in ALS

A comprehensive appraisal of the most commonly utilized clinical tools for assessing and monitoring bulbar dysfunction in ALS based on the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) evaluation framework is provided.

Regional spreading of symptoms at diagnosis as a prognostic marker in amyotrophic lateral sclerosis: a population-based study

The evaluation of number of body regions involved (NBRI) at diagnosis, with the inclusion of initial respiratory/thoracic involvement and cognition, can be useful in many research fields, improving the stratification of patients.



The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function

Performance of the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) in multicenter clinical trials

Amyotrophic lateral sclerosis severity scale.

The amyotrophic lateral sclerosis severity scale has been shown to have an average estimated reliability coefficient of 0.95 between examiners and speech ratings were correlated greater than 0.80 for objective speech measures.

Unified Huntington's disease rating scale: A follow up

The authors suggest performing a total UHDRS evaluation every second year for every HD patient as part of the routine longitudinal evaluation.

Response of patients with Alzheimer disease to rivastigmine treatment is predicted by the rate of disease progression.

A more rapid disease progression rate while receiving placebo treatment was predictive of a significantly stronger patient response to rivastigmine therapy, and this relation was observed with the other 3 outcome measures and was still apparent when accounting for disease severity.

Phase II/II/III randomized trial of gabapentin in patients with amyotrophic lateral sclerosis

  • Neurology
  • 2001

ACTS) Group

  • A double-blind, placebo controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis. Neurology
  • 1996

Proton magnetic resonance imaging reveals in vivo evidence of neurodegeneration and elevated glutamate in the medulla of patients with ALS

  • Neurology
  • 1998

ALSFRS as a measure of disease progression and a predictor of survival

  • In preparation,
  • 2003