Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R)

Patients with ALS, both to establish baseline severity at diagnosis and to assess disease progression over time.

• The ALSFRS-R provides more weight to respiratory symptoms (compared to the original ALSFRS), and is quick and easy to score.

• Validated specifically in the ALS variant of motor neuron disease and cannot necessarily be extrapolated to other variants (e.g. progressive muscular atrophy, primary lateral sclerosis).

• The questions relate only to a change from baseline relating to motor neuron disease, so premorbid disabilities from other causes should be excluded from scoring (e.g. if a patient had prior hemiparesis that limited ability to write, only the change from baseline attributable to ALS should be scored).

• For patients who are quadriplegic and ventilated, there is a floor effect that limits detection of further disease progression near the terminal phase of the disease.

• Some patients with frontotemporal dementia phenotypes may also under-recognize deficits. Care should be taken when scoring any items based on the patient’s account of function. Consider asking the patient to demonstrate function rather than relying exclusively on the history.

• Motor neuron disease is a heterogeneous group of conditions, with ALS being the most common variant seen in clinical practice.

• Even within ALS, severity at diagnosis and progression over time is highly variable among individuals. Being able to objectively grade severity, both at diagnosis and then through the disease course, allows better prognostication for patients.

• This scale is also used for measurement of outcomes in clinical trials.