_id

683a1637c782e11e38d1b28d

id

10166

complaint

[ "Dyspnea", "Shortness of Breath", "Weakness" ]

departments

[ "Neurologic" ]

description

The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) stratifies severity of ALS, including respiratory function.

diseases

[ "Amyotrophic Lateral Sclerosis", "Motor Neuron Disease" ]

evidence

The ALSFRS-R was created several years after the ALSFRS (Cedarbaum 1997) due to the acknowledgment that respiratory function (and deterioration) is a critical prognostic factor. Thus, the original 10-item scale was expanded to the current 12-items.

The revised score was developed whilst undertaking the Phase II-III brain-derived neurotrophic factor (BDNF) study (Cedarbaum 1999), using the 387 placebo-treated patients included in that trial. Patients were assessed monthly for 9 months, including ALSFRS, ALSFRS-R, forced vital capacity, “Sickness Impact Profile”, and record of any respiratory complications (including need for ventilation).

The ALSFRS-R was shown to be a more reliable predictor of prognosis compared to the original ALSFRS (likelihood ratio chi-square 5.3, compared to 4.0 for the original).

Several studies have validated the use of ALSFRS-R as a marker of disease severity, measurement of disease progression, and early-disease prognostication. Kaufmann et al (2005) found that the baseline ALSFRS-R in an ALS clinic predicted survival over 2 years of follow up. Further data from a Japanese cohort showed that the rate-of-change of ALSFRS-R added further prognostic information and improved evaluation of the disease process (Kimura 2006).

formula

Addition of the selected points:

full_title

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

information

Interpretation:

Figures are approximate and estimated from Cedarbaum 1999.

input_schema

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occasional choking", "value": 3 }, { "label": "Dietary consistency changes", "value": 2 }, { "label": "Needs supplemental tube feedings", "value": 1 }, { "label": "Nothing by mouth; exclusively parenteral or enteral feeding", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Handwriting", "name": "handwrite", "optional": false, "options": [ { "label": "Normal", "value": 4 }, { "label": "Slow or sloppy; all words are legible", "value": 3 }, { "label": "Not all words are legible", "value": 2 }, { "label": "Able to grip pen but unable to write", "value": 1 }, { "label": "Unable to grip pen", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Patients with gastrostomy and >50% daily nutrition intake via G-tube", "name": "gastro", "optional": false, "options": [ { "label": "No", "value": 0 }, { "label": "Yes", "value": 1 } ], "show_points": false, "tips_en": "", "type": "toggle" }, { "conditionality": "gastro == 0", "default": 0, "label_en": "Cutting food and handling utensils", "name": "cutting1", "optional": false, "options": [ { "label": "Normal", "value": 4 }, { "label": "Somewhat slow and clumsy but no help needed", "value": 3 }, { "label": "Can cut most foods although clumsy and slow; some help needed", "value": 2 }, { "label": "Food must be cut by someone but can still feed slowly", "value": 1 }, { "label": "Needs to be fed", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "gastro == 1", "default": 0, "label_en": "Cutting food and handling utensils", "name": "cutting2", "optional": false, "options": [ { "label": "Normal", "value": 4 }, { "label": "Clumsy but able to perform all manipulations independently", "value": 3 }, { "label": "Some help needed with closures and fasteners", "value": 2 }, { "label": "Provides minimal assistance to caregiver", "value": 1 }, { "label": "Unable to perform any aspect of task", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Dressing and hygiene", "name": "dress", "optional": false, "options": [ { "label": "Normal function", "value": 4 }, { "label": "Independent and complete self-care with effort or decreased efficiency", "value": 3 }, { "label": "Intermittent assistance or substitute methods", "value": 2 }, { "label": "Needs attendant for self-care", "value": 1 }, { "label": "Total dependence", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Turning in bed and adjusting bed clothes", "name": "bed", "optional": false, "options": [ { "label": "Normal", "value": 4 }, { "label": "Somewhat slow and clumsy but no help needed", "value": 3 }, { "label": "Can turn alone or adjust sheets but with great difficulty", "value": 2 }, { "label": "Can initiate but not turn or adjust sheets alone", "value": 1 }, { "label": "Helpless", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Walking", "name": "walk", "optional": false, "options": [ { "label": "Normal", "value": 4 }, { "label": "Early ambulation difficulties", "value": 3 }, { "label": "Walks with assistance", "value": 2 }, { "label": "Nonambulatory functional movement", "value": 1 }, { "label": "No purposeful leg movement", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Climbing stairs", "name": "climb", "optional": false, "options": [ { "label": "Normal", "value": 4 }, { "label": "Slow", "value": 3 }, { "label": "Mild unsteadiness or fatigue", "value": 2 }, { "label": "Needs assistance", "value": 1 }, { "label": "Cannot do", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Dyspnea", "name": "dys", "optional": false, "options": [ { "label": "None", "value": 4 }, { "label": "Occurs when walking", "value": 3 }, { "label": "Occurs with one or more of the following: eating, bathing, dressing", "value": 2 }, { "label": "Occurs at rest, difficulty breathing when either sitting or lying", "value": 1 }, { "label": "Significant difficulty, considering using mechanical respiratory support", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Orthopnea", "name": "ortho", "optional": false, "options": [ { "label": "None", "value": 4 }, { "label": "Some difficulty sleeping at night due to shortness of breath; does not routinely use >2 pillows", "value": 3 }, { "label": "Needs extra pillows in order to sleep (>2)", "value": 2 }, { "label": "Can only sleep sitting up", "value": 1 }, { "label": "Unable to sleep", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" }, { "conditionality": "", "default": 0, "label_en": "Respiratory insufficiency", "name": "resp", "optional": false, "options": [ { "label": "None", "value": 4 }, { "label": "Intermittent use of BiPAP", "value": 3 }, { "label": "Continuous use of BiPAP during the night", "value": 2 }, { "label": "Continuous use of BiPAP during the night and day", "value": 1 }, { "label": "Invasive mechanical ventilation by intubation or tracheostomy", "value": 0 } ], "show_points": true, "tips_en": "", "type": "radio" } ]

instructions

ismed

true

keywords

Als, revised als scale, als scale, revised alsfrs, ALSFRS-R, Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, Amyotrophic Lateral Sclerosis, als severity

measurements

[]

med_description

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

next_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.

next_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.

next_management

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

pearls

• 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.

published

purpose

[ "Diagnosis" ]

reasons

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.

refrences

{ "Clinical Practice Guidelines": [ { "href": "", "text": "" } ], "Manufacturer Website": [ { "href": "", "text": "" } ], "Original/Primary Reference": [ { "href": "https://www.ncbi.nlm.nih.gov/pubmed/10540002", "text": "Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci. 1999;169(1-2):13-21." } ], "Other References": [ { "href": "https://www.ncbi.nlm.nih.gov/pubmed/9419047", "text": "Cedarbaum JM, Stambler N. Performance of the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) in multicenter clinical trials. J Neurol Sci. 1997;152 Suppl 1:S1-9." } ], "Outcomes": [ { "href": "", "text": "" } ], "Validation": [ { "href": "https://www.ncbi.nlm.nih.gov/pubmed/15642901", "text": "Kaufmann P, Levy G, Thompson JL, et al. The ALSFRSr predicts survival time in an ALS clinic population. Neurology. 2005;64(1):38-43." }, { "href": "https://www.ncbi.nlm.nih.gov/pubmed/16434671", "text": "Kimura F, Fujimura C, Ishida S, et al. Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology. 2006;66(2):265-7." } ], "Validations": [] }

related

[ { "calcId": 10567, "short_title_en": "Berg Balance Scale (BBS)", "slug": "berg-balance-scale-bbs" }, { "calcId": 4058, "short_title_en": "MADRS", "slug": "montgomery-asberg-depression-rating-scale-madrs" }, { "calcId": 64, "short_title_en": "Glasgow Coma Scale (GCS)", "slug": "glasgow-coma-scale-score-gcs" } ]

search_terms

[ "Als", "revised als scale", "als scale", "revised alsfrs", "ALSFRS-R", "Revised Amyotrophic Lateral Sclerosis Functional Rating Scale", "Amyotrophic Lateral Sclerosis", "als severity", "motor neuron disease" ]

section

[ "whenToUseViewed", "pearlsPitfallsViewed", "whyUseViewed", "nextStepsViewed", "evidenceViewed" ]

seo

{ "keywords_en": "Als, revised als scale, als scale, revised alsfrs, ALSFRS-R, Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, Amyotrophic Lateral Sclerosis, als severity", "meta_description_en": "The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) stratifies severity of ALS, including respiratory function." }

short_description

short_title

ALSFRS-R for Amyotrophic Lateral Sclerosis

slug

revised-amyotrophic-lateral-sclerosis-functional-rating-scale-alsfrs-r

specialty

[ "Hospitalist Medicine", "Internal Medicine", "Neurology", "Neurosurgery" ]

tags

[]

title

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

usecase

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

version_number

versions

[]

cleaned_departments

[ "neurology" ]

cleaned_use

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

pub

false