Harmonized standard operating procedures for administering the ALS functional rating scale-revised

Figures & data

Table 1. Summary of changes: NEALS, TRICALS and harmonized ALSFRS-R guidance.

	NEALS ALSFRS-R Guidance For Scoring Level	TRICALS ALSFRS-R Guidance For Scoring Level	Harmonized ALSFRS-R Guidance For Scoring Level
1. Speech	2 - Some repetition required to understand speech in daily conversations	2 - “Intelligible with repeating” means that >25% of the time, repeating is necessary for comprehension.	2 - Significant repetition is required for normal conversation.
2. Salivation	3 - Swallowing more frequently. 2 - Occasionally dabbing at at the mouth with a tissue.	3 - “Slight saliva” with or without night time drooling, means that there is an excess, but there is usually no need to mop up the saliva with a tissue. 2 - “Moderately excessive saliva” means that a tissue needs to be used, but <25% of the time.	3 - Some excess saliva, but no need to mop up. 2 - Reports occasionally dabbing the mouth with a tissue during the day.
3. Swallowing	3 - Careful with foods because they get caught in throat; can eat all foods of choice but with occasional choking. 2 - Avoids certain foods or requires that consistency of foods be changed. 1 - Needs to have a gastrostomy to rate as 1.	3 - “Early eating problems” means that occasionally food will stick, or cause coughing or choking. Food may need to be cut up small, but is not mashed or liquidized. 2 - “Dietary consistency changes” means that food needs to be mashed or liquidized, drinks may need thickener, or some foods such as steak, dry biscuits or cornflakes are avoided in favor of yoghurts, casseroles or porridge. 1 -“Needs supplemental tube feeding” means that oral intake of food is so difficult that significant weight loss (>10%) has occurred and gastrostomy is required to supplement caloric intake regardless of whether one is fitted or not.	3 - There are early eating problems; occasionally food may stick in the mouth or throat, or cause coughing or choking. 2 - Dietary consistency changes are required. Dietary consistency changes are defined as: Food needs to be mashed, liquidized, or cut into smaller pieces to allow swallowing, drinks need thickener, or some foods are avoided in favor of others that are easier to swallow. 1 - The participant cannot maintain weight by oral intake due to swallowing issues, whether or not a feeding tube is in place.
4. Handwriting	Consistent guidance		No change
5a. Cutting food and handling utensils	3 - Some difficulty cutting or handling utensils by methods used prior to disease onset but continues to do so independently. Does not use altered utensils. 2 - If assistance is required, but still tries to cut some foods, and still does >50% of the task successfully. Adaptive utensils/tableware is rated as 2. 1 - Cannot cut foods by methods used prior to disease onset, but still tries to feed themselves and succeed at least occasionally.	3 -“Somewhat slow and clumsy, but no help needed” means that there is some difficulty either cutting food or holding utensils, but the patient is able to do this independently. Use of large handled cutlery to achieve the task counts as slow and clumsy. 2-“Can cut most foods although slow and clumsy; some help needed” means that occasionally assistance is needed, but the patient is independent for the task otherwise. 1 - Assistance is needed at least half the time for cutting but not for feeding. 1-“Food must be cut by someone but can still feed slowly” means that assistance is required at least half the time for cutting but not for feeding. For example, if food must be cut but the patient can feed themselves otherwise, score 1.	3 - There is some difficulty either cutting food or holding utensils, but the participant is able to do this independently. 2- If occasional assistance is needed for cutting food, but the participant is independent for the task otherwise. Use of altered utensils constitutes assistance and is rated 2. 1 - Assistance is required at least half the time for cutting but not for feeding.
5b. CUTTING FOOD AND HANDLING UTENSILS:	Consistent guidance		No change
6. Dressing and hygiene	2 - Methods used now are different than those used prior to disease onset. Substitute methods can include: sitting to get dressed, use of shower chair, sitting on a stool to shave and/or brush teeth, using built up toothbrush, Velcro clothes, pull-on clothes, adaptive clothes, not wearing pants anymore because skirts are easier. Caregiver assistance is not required. 1 - Needs daily caregiver for assistance with dressing but patient has some level of function.	2 - “Intermittent assistance or substitute methods” means that some help is needed either from a caregiver or by use of devices such as button hooks or self-tying laces, but the patient is otherwise independent. If the patient has changed the clothing they normally wear such as having zipped clothing instead of buttons, score as substitute method. 1 -“Needs attendant for self-care” means that all aspects of the task require assistance, but the patient is able to assist the caregiver for much of it	2 - Methods used now are different than those used prior to disease onset. Minimal help is needed either from a caregiver or by use of devices such as button hooks or self-tying laces, or clothing used has changed for reasons of ease of use, but the participant is otherwise independent. 1 - Both dressing and hygiene activities require significant level of caregiver assistance, but the participant is able to assist the caregiver.
7. Turning and adjusting bedclothes	Consistent guidance		No change
8. Walking	Consistent guidance		No change
9. Climbing stairs	Consistent guidance		No change
10. Dyspnea	If a participant is using noninvasive ventilation (NIV), score according to when NIV is not being used.	If someone is using noninvasive ventilation at night or in the day for ALS, score 0.	If a participant is using noninvasive ventilation (NIV), score according to when NIV is not being used.
11. Orthopnea	Rate 0 if using nocturnal BiPAP and patient NEVER sleeps without device. If patient uses BiPAP, but sometimes sleeps without it, select the number that best describes the patient’s orthopnea when sleeping without device 3 –I f a patient can sleep flat on their side but has avoided sleeping on their back since symptom onset, rate as 3 0 - If patient is using NIV at night and cannot sleep without it	Score based on difficulty regardless of the apparent underlying cause (so for example, needing to sleep sitting up because of excessive saliva scores 1). Treat a hospital style bed in which the back can be raised independently as if pillows were in place of the raised section. 3 - If there is difficulty falling asleep or if the patient wakes because of breathlessness but they do not use more than two pillows, score 3. 0 - If noninvasive ventilation is used most or all of the night, score 0. If NIV is used for an hour or so only, score as if not used.	Score based on difficulty regardless of the apparent underlying cause (for example, needing to sleep sitting up because of excessive saliva scores 1). Treat a hospital style bed in which the back can be raised independently as if pillows were in place of the raised section. 3 - if there is difficulty falling asleep, or if the participant wakes because of breathlessness but they do not use more than two pillows, or if they have changed their sleeping position (i.e. from supine to the side, prone to side, etc.). 0 - If using nocturnal BiPAP or any form of NIV and the participant NEVER sleeps without device. If the participant uses BiPAP or other form of NIV, but sometimes sleeps without it, select the number that best describes the participant’s orthopnea when sleeping without device.
12. Respiratory insufficiency	Consistent guidance		No change