MDCalc

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

Stratifies severity of amyotrophic lateral sclerosis (ALS), including respiratory function.

Speech
Salivation
Swallowing
Handwriting
Patients with gastrostomy and >50% daily nutrition intake via G-tube
Cutting food and handling utensils
Dressing and hygiene
Turning in bed and adjusting bed clothes
Walking
Climbing stairs
Dyspnea
Orthopnea
Respiratory insufficiency

Result:

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Advice

More severe scores at diagnosis (i.e., lower ALSFRS-R) may necessitate earlier discussions around respiratory support (e.g. non-invasive ventilation), supplemental feeding (PEG), and end-of-life care.

Management

Consider referral to a multidisciplinary motor neuron disease clinic, particularly for those more severely affected.

Critical Actions

Overall, while baseline ALSFRS-R and rate-of-change can be used in the clinic setting to provide some prognostic information, it is important to emphasize that the disease remains heterogeneous and the total score needs careful interpretation in the context of disease duration and an individual patient’s disease course.