ALS Functional Rating Scale–Revised (ALSFRS-R)

1 ALS Functional Rating Scale Revised (ALSFRS-R). Measuring progression of disability in people with ALS1. 1991 1999 2010 Today ALSFRS development begins ALSFRS revised to ALSFRS-R Proposed clinical relevance ALSFRS-R widely accepted due to need for easily administered to balance respiratory with limb to be a 20% to 25% change in measure of ALS progression clinical Rating instrument2 and bulbar categories1,2 decline of the ALSFRS-R scale 2,* by clinical trialists and regulatory bodies3. * As rated by members of the Northeast ALS Consortium (NEALS). The ALSFRS-R is a simple, validated, and reliable tool for evaluating decline in function. Scores decline with disease 1 ~. progression at a rate that is generally consistent across clinical ,2. 1 month 1 point drop Individual patient rates of decline may See the full ALSFRS-R questionnaire on reverse.

2 ALS Functional Rating Scale Revised (ALSFRS-R)5. BULBAR FINE MOTOR GROSS MOTOR RESPIRATORY. Speech Handwriting Turning in Bed Dyspnea 4 Normal 4 Normal 4 Normal 4 None 3 Detectable speech disturbance 3 S. low or sloppy; all words 3 S. omewhat slow and clumsy, 3 Occurs when walking 2 Intelligible with repeating are legible but no help needed 2 O. ccurs with one or more of the 1 S. peech combined with nonvocal 2 Not all words are legible 2 C. an turn alone or adjust sheets, following: eating, bathing, dressing communication 1 Able to grip pen but unable to write but with great difficulty (ADL). 0 Loss of useful speech 0 Unable to grip pen 1 C. an initiate, but not turn or adjust 1 O. ccurs at rest, difficulty breathing sheets alone when either sitting or lying Salivation Cutting Food* 0 Helpless 0 S. ignificant difficulty, considering 4 Normal 4 Normal using mechanical respiratory Walking support 3 S.

3 Light but definite excess of saliva 3 S. omewhat slow and clumsy, in mouth; may have nighttime but no help needed 4 Normal drooling 3 Early ambulation difficulties Orthopnea 2 C. an cut most foods, although 4 None 2 M. oderately excessive saliva; clumsy and slow; some help needed 2 Walks with assistance may have minimal drooling 3 S. ome difficulty sleeping at night due 1 F. ood must be cut by someone, 1 N. on-ambulatory Functional to shortness of breath. Does not 1 M. arked excess of saliva with but can still feed slowly movement only routinely use more than two pillows some drooling 0 Needs to be fed 0 No purposeful leg movement 2 N. eeds extra pillow in order to 0 M. arked drooling; requires constant sleep (more than two). tissue or handkerchief Dressing and Hygiene Climbing Stairs 1 Can only sleep sitting up 4 Normal 4 Normal Swallowing 0 Unable to sleep 3 I ndependent and complete 3 Slow 4 Normal self-care with effort or decreased 2 Mild unsteadiness or fatigue 3 E.

4 Arly eating problems efficiency Respiratory Insufficiency 1 Needs assistance occasional choking 4 None 2 I ntermittent assistance or 0 Cannot do 2 Dietary consistency changes substitute methods 3 Intermittent use of BiPAP. 1 N. eeds supplemental tube feeding 1 Needs attendant for self-care 2 Continuous use of BiPAP. 0 N. PO (exclusively parenteral or 0 Total dependence 1 C. ontinuous use of BiPAP during enteral feeding) the night and day * There are different assessments for cutting 0 I nvasive mechanical ventilation food with gastrostomy. by intubation or tracheostomy References: 1. Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS-R: a revised ALS Functional Rating scale that incorporates assessments of respiratory function. J Neurol Sci. 1999;169(1-2):13 21. 2. Castrillo-Viguera C, Grasso DL, Simpson E, et al. Clinical significance in the change of decline in ALSFRS-R.

5 Amyotroph Lateral Scler. 2010;11(1-2):178 180. 3. Berry JD, Cudkowicz ME. New considerations in the design of clinical trials for amyotrophic lateral sclerosis. Clin Investig (Lond). 2011;1(10):1375 1389. 4. Simon GN, Turner MR, Vucic S, et al. Quantifying disease progression in amyotrophic lateral sclerosis. Ann Neurol. 2014;76(5):643 657. 5. The ALS Program. Center for Outcomes Research, University of Massachusetts Medical School. Accessed November 28, 2017. 2017 Mitsubishi Tanabe Pharma America, Inc. All rights reserved. CNP-NON-US-0011 12/17.