Calc Function

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    Patent Pending

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

    Stratifies severity of amyotrophic lateral sclerosis (ALS), including respiratory function.
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    When to Use
    Pearls/Pitfalls
    Why Use

    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.

    Normal
    +4
    Detectable speech disturbance
    +3
    Intelligible with repeating
    +2
    Speech combined with nonvocal communications
    +1
    Loss of useful speech
    0
    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
    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
    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
    No
    Yes
    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
    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
    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
    Normal
    +4
    Early ambulation difficulties
    +3
    Walks with assistance
    +2
    Nonambulatory functional movement
    +1
    No purposeful leg movement
    0
    Normal
    +4
    Slow
    +3
    Mild unsteadiness or fatigue
    +2
    Needs assistance
    +1
    Cannot do
    0
    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
    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
    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

    Result:

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    Next Steps
    Evidence
    Creator Insights

    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.

    Content Contributors
    • Antony Winkel, MBBS, FRACP
    About the Creator
    Dr. Jesse M. Cedarbaum
    Are you Dr. Jesse M. Cedarbaum?
    Content Contributors
    • Antony Winkel, MBBS, FRACP