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

ALSFRS-R Calculator

Speech

Salivation

Swallowing

Handwriting

Cutting Food / Handling Utensils

Dressing and Hygiene

Turning in Bed / Adjusting Bedclothes

Walking

Climbing Stairs

Dyspnea

Orthopnea

Respiratory Insufficiency

Total ALSFRS-R Score: –

Awaiting input

Default 4 (Normal) used for un-set items.

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

Why Use

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.

When to Use

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

Formula

Addition of the selected points: Variable Points Speech Normal 4 Detectable speech disturbance 3 Intelligible with repeating 2 Speech combined with nonvocal communications 1 Loss of useful speech 0 Salivation Normal 4 Slight but definite excess of saliva in mouth; may have nighttime drooling 3 Moderately excessive saliva; may have minimal drooling 2 Marked excess of saliva with some drooling 1 Marked drooling; requires constant tissue or handkerchief 0 Swallowing Normal eating habits 4 Early eating problems; occasional choking 3 Dietary consistency changes 2 Needs supplemental tube feedings 1 Nothing by mouth; exclusively parenteral or enteral feeding 0 Handwriting Normal 4 Slow or sloppy; all words are legible 3 Not all words are legible 2 Able to grip pen but unable to write 1 Unable to grip pen 0 Cutting food and handling utensils (patients without gastrostomy, or <50% of daily nutrition intake via G-tube) Normal 4 Somewhat slow and clumsy but no help needed 3 Can cut most foods although clumsy and slow; some help needed 2 Food must be cut by someone but can still feed slowly 1 Needs to be fed 0 Cutting food and handling utensils (patients with gastrostomy and >50% daily nutrition intake via G-tube) Normal 4 Clumsy but able to perform all manipulations independently 3 Some help needed with closures and fasteners 2 Provides minimal assistance to caregiver 1 Unable to perform any aspect of task 0 Dressing and hygiene Normal function 4 Independent and complete self-care with effort or decreased efficiency 3 Intermittent assistance or substitute methods 2 Needs attendant for self-care 1 Total dependence 0 Turning in bed and adjusting bed clothes Normal 4 Somewhat slow and clumsy but no help needed 3 Can turn alone or adjust sheets but with great difficulty 2 Can initiate but not turn or adjust sheets alone 1 Helpless 0 Walking Normal 4 Early ambulation difficulties 3 Walks with assistance 2 Nonambulatory functional movement 1 No purposeful leg movement 0 Climbing stairs Normal 4 Slow 3 Mild unsteadiness or fatigue 2 Needs assistance 1 Cannot do 0 Dyspnea None 4 Occurs when walking 3 Occurs with one or more of the following: eating, bathing, dressing 2 Occurs at rest, difficulty breathing when either sitting or lying 1 Significant difficulty, considering using mechanical respiratory support 0 Orthopnea None 4 Some difficulty sleeping at night due to shortness of breath; does not routinely use >2 pillows 3 Needs extra pillows in order to sleep (>2) 2 Can only sleep sitting up 1 Unable to sleep 0 Respiratory insufficiency None 4 Intermittent use of BiPAP 3 Continuous use of BiPAP during the night 2 Continuous use of BiPAP during the night and day 1 Invasive mechanical ventilation by intubation or tracheostomy 0

Pearls / Pitfalls

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.

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.

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.

More Information

Interpretation: ALSFRS-R 9-month survival ≤15 ≤25% 16-20 ~25-40% 21-25 ~40-60% 26-30 ~60-70% 31-35 ~70-80% 36-40 ~80-90% ≥41 >90% Figures are approximate and estimated from Cedarbaum 1999 .