For Patients
 

Assessing ALS Function

Frequently monitoring your decline in function is important to understanding how ALS is affecting your body. The most widely used test in clinical trials to track ALS called the ALS Functional Rating Scale-Revised (ALSFRS-R). Tracking your ALSFRS-R score over time can help you better prepare for the road ahead. 1
 
ALS function

Assessing ALS Function

Frequently monitoring your decline in function is important to understanding how ALS is affecting your body. The most widely used test in clinical trials to track ALS called the ALS Functional Rating Scale-Revised (ALSFRS-R). Tracking your ALSFRS-R score over time can help you better prepare for the road ahead. 1
ALS function
The ALSFRS-R

What Is the ALSFRS-R?1-4

The ALSFRS-R is questionnaire-based scale that measures and tracks changes in a person's physical function over time.

Today, the ALSFRS-R is a widely used test in clinical trials to track ALS and is considered the gold standard measure of disability progression.

The ALSFRS was first developed and used in the 1990s, but since then has been revised and refined.

How Does It Work?2,4

The ALSFRS-R measures 12 aspects of physical function, ranging from one’s ability to swallow and use utensils to climbing stairs and breathing. Each function is scored from 4 (normal) to 0 (no ability), with a maximum total score of 48 and a minimum total score of 0.
Speech Salivation Swallowing
Handwriting Cutting food Climbing stairs
Turning in bed Walking Dressing and hygiene
Dypspnea
(difficulty breathing)
Orthopnea
(Shortness of breath while lying down)
Breathing
insufficiency
Speech
Handwriting
Turning in bed
Dypspnea (difficulty breathing)
Salivation
Cutting food
Walking
Orthopnea 
(Shortness of breath while lying down)
Swallowing
Climbing stairs
Dressing and hygiene
Breathing insufficiency
Patients with higher scores across the 12 domains have more physical function.

How Is the ALSFRS-R Administered?1,4

The ALSFRS-R is administered by a healthcare provider. The test is quick and easy to perform.

The questionnaire can be completed over the telephone or online, so you can still easily monitor ALS even if you have difficulty returning to the clinic for an evaluation.
Talk with your healthcare provider(s) about the ALSFRS-R 
scale and why it’s important.
Your Score Matters
 

Your ALSFRS-R Score Matters

Remember, the higher your ALSFRS-R score, the more physical function you retain.
Quality of Life
There is a strong connection between your physical function and quality of life. Generally, the better you can move and the longer you can preserve physical function, the more independence you can maintain over everyday activities.2
A change of just 1-2 points can mean a considerable 
reduction of physical function.2
Every Score is Unique
 

Every Score Is Personal5

No two people with ALS are alike, meaning every ALSFRS-R score is completely personal and unique.

In fact, even if two people have the exact same score, they could still be experiencing drastically different symptoms. This is because symptoms affect different regions of the body and can vary from person to person.

For example, both Steven and Mary have the same ALSFRS-R score; however, while Steven experiences symptoms in his head, throat, and upper body, Mary experiences symptoms in her legs and respiratory system.*†
how to test for ALS
ALS test example
*Steven and Mary are hypothetical patients.
Remember each function is scored from 4 (normal) to 0 (no ability), with a maximum total score of 48 and a minimal total score of 0.
Discussing Your Score
 

Discussing the ALSFRS-R With Your Healthcare Provider(s)

It can’t be overemphasized just how important it is to speak regularly with your healthcare provider(s) about your ALSFRS-R score.

Below are some important questions that can help initiate a productive conversation around the ALSFRS-R scale and help you obtain the information needed to better understand the impact your score can have on your diagnosis.
  • Do you use the ALS Function Rating Scale-Revised (ALSFRS-R) with your ALS patients?
  • Why is the ALSFRS-R important for measuring my ALS progression?
  • How often should the ALSFRS-R test be administered?
  • What is my ALSFRS-R score?
  • Which regions of my body is ALS primarily affecting?
  • What types of symptoms am I likely to experience?
  • How quickly is my ALS progressing?
  • (If your score has previously been measured) What was my score when I was first diagnosed compared to my score now? What does this mean in terms of disease progression?
  • Based on how my ALS is progressing, what are some appropriate interventions?
  • What are good ways to help manage my symptoms and slow the progression of ALS?
References: 1. Berry JD, Cudkowicz ME. New considerations in the design of clinical trials for amyotrophic lateral sclerosis. Clin Investig (Lond). 2011;1(10):1375-1389. 2. Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. J Neurol Sci. 1999;169(1-2):13-21. 3. Eisen A. Motor neurone disease. In Turner MR, Kiernan MC, eds. Landmark Papers in Neurology. 1st ed. Oxford, United Kingdom: Oxford University Press; 2015:264. 4. Rutkove SB. Clinical measures of disease progression in amyotrophic lateral sclerosis. Neurotherapeutics. 2015;12(2):384-393. 5. Amyotrophic lateral sclerosis (ALS) fact sheet. National Institute of Neurological Disorders and Stroke website. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet. Published June 2013. Accessed April 13, 2017.