Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that typically affects adults around 54–67 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. 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. 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.
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, 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) to mitigate the floor effect and a version with explanatory notes, which is particularly suitable for self-assessment (ALSFRS-R-SE, self-explanatory) and has a strong agreement with the harmonized SOP for interview-based assessment.
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.
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) 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 while Milano-Torino Staging (MiToS) utilizes the subscores produced by the ALSFRS-R to define stages.
Questions The questions used to determine an individual's ALSFRS-R score are listed below. 1. Speech4Normal speech processes 3Detectable speech disturbance 2Intelligible with repeating 1Speech combined with nonvocal communication 0Loss of useful speech2. Salivation4Normal3Slight but definite excess of saliva in mouth; may have nighttime drooling2Moderately excessive saliva; may have minimal drooling1Marked excess of saliva with some drooling0Marked drooling; requires constant tissue or handkerchief3. Swallowing4Normal eating habits3Early eating problems — occasional choking2Dietary consistency changes1Needs supplemental tube feeding0NPO (exclusively parenteral or enteral feeding)4. Handwriting4Normal3Slow or sloppy: all words are legible2Not all words are legible1Able to grip pen but unable to write0Unable to grip pen5a. Cutting food and handling utensils (patients without gastrostomy)?4Normal3Somewhat slow and clumsy, but no help needed2Can cut most foods, although clumsy and slow; some help needed1Food must be cut by someone, but can still feed slowly0Needs to be fed5b. Cutting food and handling utensils (scale for patients with gastrostomy)?4Normal3Clumsy but able to perform all manipulations independently2Some help needed with closures and fasteners1Provides minimal assistance to caregiver0Unable to perform any aspect of task6. Dressing and hygiene4Normal function3Independent and complete self-care with effort or decreased efficiency2Intermittent assistance or substitute methods1Needs attendant for self-care0Total dependence7. Turning in bed and adjusting bed clothes4Normal3Somewhat slow and clumsy, but no help needed2Can turn alone or adjust sheets, but with great difficulty1Can initiate, but not turn or adjust sheets alone0Helpless8. Walking4Normal3Early ambulation difficulties2Walks with assistance1Nonambulatory functional movement0No purposeful leg movement9. Climbing stairs4Normal3Slow2Mild unsteadiness or fatigue1Needs assistance0Cannot do10. Dyspnea (new)4None3Occurs when walking2Occurs with one or more of the following: eating, bathing, dressing (ADL)1Occurs at rest, difficulty breathing when either sitting or lying0Significant difficulty, considering using mechanical respiratory support11. Orthopnea (new)4None3Some difficulty sleeping at night due to shortness of breathDoes not routinely use more than two pillows2Needs extra pillows in order to sleep (more than two)1Can only sleep sitting up0Unable to sleep12. Respiratory insufficiency (new)4None3Intermittent use of BiPAP2Continuous use of BiPAP during the night1Continuous use of BiPAP during the night and day0Invasive mechanical ventilation by intubation or tracheostomy
References
External links Free online ALSFRS-R calculator ALSFRS-R.net: Track ALSFRS-R scores over time