ALS Functional Rating Scale - Revised
{{Short description|Human disease}}
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that typically affects adults around 54–67{{Cite journal|last1=Chiò|first1=A.|last2=Logroscino|first2=G.|last3=Traynor|first3=B.J.|last4=Collins|first4=J.|last5=Simeone|first5=J.C.|last6=Goldstein|first6=L.A.|last7=White|first7=L.A.|date=2013|title=Global Epidemiology of Amyotrophic Lateral Sclerosis: A Systematic Review of the Published Literature|journal=Neuroepidemiology|language=english|volume=41|issue=2|pages=118–130|doi=10.1159/000351153|pmid=23860588|issn=0251-5350|pmc=4049265}} years of age, although anyone can be diagnosed with the disease. People diagnosed with ALS live on average 2–4 years after diagnosis due to the quick progression of the disease.{{Cite journal|last1=Hobson|first1=Esther V.|last2=McDermott|first2=Christopher J.|title=Supportive and symptomatic management of amyotrophic lateral sclerosis|journal=Nature Reviews Neurology|volume=12|issue=9|pages=526–538|doi=10.1038/nrneurol.2016.111|pmid=27514291|year=2016|s2cid=8547381 |url=http://eprints.whiterose.ac.uk/104445/1/16.pdf}}{{Cite news|url=https://www.als.ca/about-als/|title=About ALS - ALS Society of Canada|work=ALS Society of Canada|access-date=2017-10-31|language=en-US}} The progression and severity of ALS is rated by doctors on the ALS Functional Rating Scale, which has been revised and is referred to as ALSFRS-R.
Criteria
ALSFRS-R includes 12 questions that can have a score of 0 to 4. A score of 0 on a question would indicate no function while a score of 4 would indicate full function.{{Cite journal|last1=Martin|first1=Sarah|last2=Al Khleifat|first2=Ahmad|last3=Al-Chalabi|first3=Ammar|date=2017-03-28|title=What causes amyotrophic lateral sclerosis?|journal=F1000Research|volume=6|pages=371|doi=10.12688/f1000research.10476.1|issn=2046-1402|pmc=5373425|pmid=28408982 |doi-access=free }}{{Cite journal|last1=Cedarbaum|first1=Jesse M.|last2=Stambler|first2=Nancy|last3=Malta|first3=Errol|last4=Fuller|first4=Cynthia|last5=Hilt|first5=Dana|last6=Thurmond|first6=Barbara|last7=Nakanishi|first7=Arline|title=The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function|journal=Journal of the Neurological Sciences|volume=169|issue=1–2|pages=13–21|doi=10.1016/s0022-510x(99)00210-5|pmid=10540002|date=1999-10-31|s2cid=7057926 }} This scale has been useful for doctors in diagnosing patients, measuring disease progression and also for researchers when selecting patients for a study and measuring the potential effects of a clinical trial.{{Cite journal|last=Mora|first=Jesús S|date=2017-10-01|title=Edaravone for treatment of early-stage ALS|journal=The Lancet Neurology|volume=16|issue=10|pages=772|doi=10.1016/S1474-4422(17)30289-2|pmid=28920880|s2cid=2292915 |doi-access=}}
The ALSFRS-R scale has some limitations though since it is not useful to compare scores of people who present with different onset. In ALS the main type of onset is bulbar followed by limb-onset ALS which describes the region of motor neurons first affected. Individuals may also present with respiratory-onset ALS,{{Cite journal|last1=Rooney|first1=James|last2=Burke|first2=Tom|last3=Vajda|first3=Alice|last4=Heverin|first4=Mark|last5=Hardiman|first5=Orla|date=2017-05-01|title=What does the ALSFRS-R really measure? A longitudinal and survival analysis of functional dimension subscores in amyotrophic lateral sclerosis|url=http://jnnp.bmj.com/content/88/5/381|journal=J Neurol Neurosurg Psychiatry|language=en|volume=88|issue=5|pages=381–385|doi=10.1136/jnnp-2016-314661|issn=0022-3050|pmid=27888187|hdl=2262/100143 |s2cid=24719932 |hdl-access=free}} but this occurs very rarely. Since there are three different types of ALS, ALSFRS-R scores are often grouped in categories depending on type of onset.
Since there are three main pathways of progression, the questions are also divided in relation to the types of onset. Questions 1 to 3 are related to bulbar onset, questions 4 to 9 are related to limb onset and questions 10 to 12 are related to respiratory onset. Further developments of the ALSFRS-R include an extended version (ALSFRS-EX){{Cite journal|last1=Wicks|first1=P.|last2=Massagli|first2=M.P.|last3=Wolf|first3=C. |last4= Heywood|first4=J.|date=May 2009|title= Measuring function in advanced ALS: validation of ALSFRS-EX extension items.|journal=European Journal of Neurology|volume=16|issue=3 | pages=353–9|doi=10.1111/j.1468-1331.2008.02434.x|pmid=19364363|s2cid=27696665 }} to mitigate the floor effect and a version with explanatory notes, which is particularly suitable for self-assessment (ALSFRS-R-SE, self-explanatory).{{Cite journal|last1=Maier|first1=André|last2=Boentert|first2=Matthias|last3=Reilich|first3=Peter|last4=Witzel|first4=Simon|last5=Petri|first5=Susanne|last6=Großkreutz|first6=Julian|last7=Metelmann|first7=Moritz|last8=Lingor|first8=Paul|last9=Cordts|first9=Isabell|last10=Dorst|first10=Johannes|last11=Zeller|first11=Daniel|last12=Günther|first12=René|last13=Hagenacker|first13=Tim|last14=Grehl|first14=Torsten|last15=Spittel|first15=Susanne|last16=Schuster|first16=Joachim|last17=Ludolph|first17=Albert|last18=Meyer|first18=Thomas|date=December 2022|title=ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale|journal=Neurological Research and Practice|volume=4|issue=1|pages=60|doi=10.1186/s42466-022-00224-6|issn=2524-3489|pmc=9753252|pmid=36522775 |doi-access=free }}
Progression
ALSFRS-R scores calculated at diagnosis can be compared to scores throughout time to determine the speed of progression. The rate of change, called the ALSFRS-R slope can be used as a prognostic indicator.{{Cite journal|last1=Kimura|first1=F.|last2=Fujimura|first2=C.|last3=Ishida|first3=S.|last4=Nakajima|first4=H.|last5=Furutama|first5=D.|last6=Uehara|first6=H.|last7=Shinoda|first7=K.|last8=Sugino|first8=M.|last9=Hanafusa|first9=T.|date=2006-01-24|title=Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS|journal=Neurology|language=en|volume=66|issue=2|pages=265–267|doi=10.1212/01.wnl.0000194316.91908.8a|issn=0028-3878|pmid=16434671|s2cid=31365609 }}{{Cite journal|last1=Elamin|first1=Marwa|last2=Bede|first2=Peter|last3=Montuschi|first3=Anna|last4=Pender|first4=Niall|last5=Chio|first5=Adriano|last6=Hardiman|first6=Orla|date=2015-06-01|title=Predicting prognosis in amyotrophic lateral sclerosis: a simple algorithm|journal=Journal of Neurology|language=en|volume=262|issue=6|pages=1447–1454|doi=10.1007/s00415-015-7731-6|pmid=25860344|issn=0340-5354|pmc=4469087}}
Although the ALSFRS-R score is a recognized prognostic indicator, it is more useful to compare various indicators including vital capacity (FVC%) and the Sickness Impact Profile (SIP){{Cite journal|last1=Bergner|first1=M.|last2=Bobbitt|first2=R. A.|last3=Carter|first3=W. B.|last4=Gilson|first4=B. S.|date=August 1981|title=The Sickness Impact Profile: development and final revision of a health status measure|journal=Medical Care|volume=19|issue=8|pages=787–805|issn=0025-7079|pmid=7278416|doi=10.1097/00005650-198108000-00001|s2cid=20325580 }} to increase the accuracy of a given prognosis.
Relating the ALSFRS-R score to staging criteria is also useful in determining prognosis. King's system relies on the clinical spread of disease as a measure of progression{{Cite journal|last1=Roche|first1=Jose C.|last2=Rojas-Garcia|first2=Ricardo|last3=Scott|first3=Kirsten M.|last4=Scotton|first4=William|last5=Ellis|first5=Catherine E.|last6=Burman|first6=Rachel|last7=Wijesekera|first7=Lokesh|last8=Turner|first8=Martin R.|last9=Leigh|first9=P. Nigel|date=March 2012|title=A proposed staging system for amyotrophic lateral sclerosis|journal=Brain: A Journal of Neurology|volume=135|issue=Pt 3|pages=847–852|doi=10.1093/brain/awr351|issn=1460-2156|pmc=3286327|pmid=22271664}} while Milano-Torino Staging (MiToS) utilizes the subscores produced by the ALSFRS-R to define stages.{{Cite journal|last1=Chiò|first1=Adriano|last2=Hammond|first2=Edward R.|last3=Mora|first3=Gabriele|last4=Bonito|first4=Virginio|last5=Filippini|first5=Graziella|date=January 2015|title=Development and evaluation of a clinical staging system for amyotrophic lateral sclerosis|journal=Journal of Neurology, Neurosurgery, and Psychiatry|volume=86|issue=1|pages=38–44|doi=10.1136/jnnp-2013-306589|issn=1468-330X|pmid=24336810|hdl=2318/153858 |s2cid=10365806 |hdl-access=free}}
Questions
The questions used to determine an individual's ALSFRS-R score are listed below.
| class="wikitable"
| colspan="2" |1. Speech |
| 4
|Normal speech processes |
| 3
|Detectable speech disturbance |
| 2
|Intelligible with repeating |
| 1
|Speech combined with nonvocal communication |
| 0
|Loss of useful speech |
| colspan="2" |2. Salivation |
| 4
|Normal |
| 3
|Slight but definite excess of saliva in mouth; may have nighttime drooling |
| 2
|Moderately excessive saliva; may have minimal drooling |
| 1
|Marked excess of saliva with some drooling |
| 0
|Marked drooling; requires constant tissue or handkerchief |
| colspan="2" |3. Swallowing |
| 4
|Normal eating habits |
| 3
|Early eating problems — occasional choking |
| 2
|Dietary consistency changes |
| 1
|Needs supplemental tube feeding |
| 0
|NPO (exclusively parenteral or enteral feeding) |
| colspan="2" |4. Handwriting |
| 4
|Normal |
| 3
|Slow or sloppy: all words are legible |
| 2
|Not all words are legible |
| 1
|Able to grip pen but unable to write |
| 0
|Unable to grip pen |
| colspan="2" |5a. Cutting food and handling utensils (patients without gastrostomy)? |
| 4
|Normal |
| 3
|Somewhat slow and clumsy, but no help needed |
| 2
|Can cut most foods, although clumsy and slow; some help needed |
| 1
|Food must be cut by someone, but can still feed slowly |
| 0
|Needs to be fed |
| colspan="2" |5b. Cutting food and handling utensils (scale for patients with gastrostomy)? |
| 4
|Normal |
| 3
|Clumsy but able to perform all manipulations independently |
| 2
|Some help needed with closures and fasteners |
| 1
|Provides minimal assistance to caregiver |
| 0
|Unable to perform any aspect of task |
| colspan="2" |6. Dressing and hygiene |
| 4
|Normal function |
| 3
|Independent and complete self-care with effort or decreased efficiency |
| 2
|Intermittent assistance or substitute methods |
| 1
|Needs attendant for self-care |
| 0
|Total dependence |
| colspan="2" |7. Turning in bed and adjusting bed clothes |
| 4
|Normal |
| 3
|Somewhat slow and clumsy, but no help needed |
| 2
|Can turn alone or adjust sheets, but with great difficulty |
| 1
|Can initiate, but not turn or adjust sheets alone |
| 0
|Helpless |
| colspan="2" |8. Walking |
| 4
|Normal |
| 3
|Early ambulation difficulties |
| 2
|Walks with assistance |
| 1
|Nonambulatory functional movement |
| 0
|No purposeful leg movement |
| colspan="2" |9. Climbing stairs |
| 4
|Normal |
| 3
|Slow |
| 2
|Mild unsteadiness or fatigue |
| 1
|Needs assistance |
| 0
|Cannot do |
| colspan="2" |10. Dyspnea (new) |
| 4
|None |
| 3
|Occurs when walking |
| 2
|Occurs with one or more of the following: eating, bathing, dressing (ADL) |
| 1
|Occurs at rest, difficulty breathing when either sitting or lying |
| 0
|Significant difficulty, considering using mechanical respiratory support |
| colspan="2" |11. Orthopnea (new) |
| 4
|None |
| 3
|Some difficulty sleeping at night due to shortness of breath |
| |Does not routinely use more than two pillows |
| 2
|Needs extra pillows in order to sleep (more than two) |
| 1
|Can only sleep sitting up |
| 0
|Unable to sleep |
| colspan="2" |12. Respiratory insufficiency (new) |
| 4
|None |
| 3
|Intermittent use of BiPAP |
| 2
|Continuous use of BiPAP during the night |
| 1
|Continuous use of BiPAP during the night and day |
| 0
|Invasive mechanical ventilation by intubation or tracheostomy |
References
{{reflist}}
External links
- [https://neurotoolkit.com/alsfrs-r/ Free online ALSFRS-R calculator]
Category:Neurology articles needing expert attention