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Table 1 ALSFRS-R versus ALSFRS-R-SE after consensus process

From: ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale

No/Pts

ALSFRS-R item

ALSFRS-R-SE item

ALSFRS-R-SE item additional explanation

Type of adaption of ALSFRS-R-SE

Deviation from ALSFRS-R

1

Speech

Speech

   

1.4

Normal speech process

Normal

Speaking and/or articulation is the same as it was before the onset of initial ALS symptoms

Harmonization

Minor

1.3

Detectable speech disturbance

Detectable speech disturbance

Speech, articulation or phonation have changed, as perceived either by the affected themselves or by their immediate contacts. This may present as slurred speech or hoarseness of voice

None

 

1.2

Intelligible with repeating

Intelligible speech with repetition

Frequent repetition of single words or parts of a sentence are required to convey meaning

Linguistic adaption

Minor

1.1

Speech combined with non-vocal communication

Speech combined with non-vocal communication

Writing things down, use of communication aids, and similar methods are needed to convey meaning

None

 

1.0

Loss of useful speech

Loss of useful speech

Communication aids or similar methods are always required

None

 

2

Salivation

Salivation

   

2.4

Normal

Normal

No excess saliva accumulates in the mouth

None

 

2.3

Slight but definite excess of saliva in mouth; may have night-time drooling

Slight but definite excess of saliva in mouth; night-time drooling may take place

Increased accumulation of saliva in the mouth; however, subjectively not an impairment or impediment and no loss of saliva during the day

Linguistic adaption

Minor

2.2

Moderately excessive saliva; may have minimal drooling

Moderately excessive saliva; may experience minimal drooling

During the day, a tissue is occasionally used to dab the edges of the mouth

Linguistic adaption

Minor

2.1

Marked excess of saliva with some drooling

Marked excess of saliva with some drooling

Regular loss of saliva, a tissue is used often but not constantly

None

 

2.0

Marked drooling

Marked drooling

Permanent use of tissues or a suction device is required

None

 

3

Swallowing

Swallowing

   

3.4

Normal eating habits

Normal

Swallowing any type of food or liquid is unproblematic

Harmonization

Minor

3.3

Early eating problems-occasional choking

Minor swallowing problems-occasional choking

Food intake takes longer; food must be cut into smaller bites and swallowed with care. Occasionally, choking on food or higher frequency of coughing is observed

Linguistic adaption

Minor

3.2

Dietary consistency changes

Dietary consistency changes

Difficulty swallowing (dysphagia) and trouble with certain consistencies of food and beverages results in the avoidance of some types of food consistencies (e.g., meat, dry biscuits, nuts). Dietary supplements or thickeners may be used due to difficulty in swallowing

None

 

3.1

Needs supplemental tube feeding

Supplemental tube feeding

Due to dysphagia, food intake has become so difficult that an enteral feeding tube (PEG) must be fitted or is highly recommended by the physician to supplement caloric intake and/or prevent choking on food

Harmonization

Minor

3.0

NPO (exclusively parenteral or enteral feeding)

Exclusively enteral tube feeding

Food and liquid intake happen exclusively via a feeding tube; oral food intake is impossible due to high-grade dysphagia

Linguistic adaption and harmonization

Moderate

4

Handwriting

Handwriting

The subject of this assessment is writing with one’s dominant hand (writing hand) in the usual posture

Addition

Moderate

4.4

Normal

Normal

Writing with the dominant writing hand causes no problems

None

 

4.3

Slow or sloppy: all words are legible

Slow or sloppy, but all words are legible

Writing is more difficult, or alternately, the appearance of a person's written text has changed even though the words remain legible

Harmonization

Minor

4.2

Not all words are legible

Not all words are legible

Some written words are illegible. Writing aids are used to promote legibility

None

 

4.1

Able to grip pen but unable to write

Able to grip pen but unable to write

Holding a pen is possible; however, anything beyond signing or writing one's own name is not

None

 

4.0

Unable to grip pen

Unable to grip pen

Holding a pen is impossible

None

 

5a

Cutting food and handling utensils

Cutting food and handling utensils

Pertaining to persons not regularly using an enteral feeding tube for caloric intake

Addition

Moderate

5a.4

Normal

Normal

The use of cutlery is not problematic. Problems would be, for example, the use of knives and forks instead of chopsticks or the inclination to use a spoon more often

None

 

5a.3

Somewhat slow and clumsy, but no help needed

Somewhat slow and clumsy, but no help needed

Eating takes more time due to impairment of the hands. Method of handing cutlery has changed, but its use is still possible without assistance

None

 

5a.2

Can cut most foods, although clumsy and slow; some help needed

Can cut most foods, although slowly and clumsily; some help is needed

Assistance is needed on occasion when cutting certain types of foods; alternately, eating aids such as special cutlery are in use

Linguistic adaption

Minor

5a.1

Food must be cut by someone, but can still feed slowly

Food must be cut by someone else, but can still feed themself slowly

Assistance is needed to cut solid food and on most other occasions. However, eating on one's own is still possible (e.g., using a fork or a spoon)

Linguistic adaption

Minor

5a.0

Needs to be fed

Total dependence

The affected person is unable to use cutlery (e.g., a fork or a spoon) on their own, and can only eat when fed

Harmonization

Moderate

5b

Cutting food and handling utensils

Cutting food and handling utensils

For persons regularly in need of a feeding tube for caloric intake. The subject of this assessment is manual dexterity

addition

Moderate

5b.4

Normal

Normal

The tube can be handled independently, and locks and packets can be opened and closed without assistance

None

 

5b.3

Clumsy but able to perform all manipulations independently

Clumsy but able to perform all manipulations independently

No assistance is needed when handling the feeding tube, however use is somewhat difficult

None

 

5b.2

Some help needed with closures and fasteners

Some help needed with closures and fasteners

Handling the feeding tube is done more or less independently. Assistance is needed when opening locks and fasteners

None

 

5b.1

Provides minimal assistance to caregiver

Provides minimal assistance to caregiver

Another person mostly handles the feeding tube. The affected can only carry out minimal actions themselves

None

 

5b.0

Unable to perform any aspect of a given task

Total dependence

Handling the feeding tube is done entirely by another person. No actions can be executed by the affected

Harmonization

Moderate

6

Dressing and hygiene

Dressing and hygiene

   

6.4

Normal function

Normal

Getting dressed and tending to personal hygiene are unproblematic

Harmonization

 

6.3

Independent and complete self-care with effort or decreased efficiency

Independent and complete self-care requires effort and is less efficient

Getting (un)dressed and tending to personal hygiene are executed more slowly than before but are performed autonomously and require neither aids nor assistance from another person

Linguistic adaption

Minor

6.2

Intermittent assistance or substitute methods

Intermittent assistance or substitute methods

At times, another person is called upon to assist, or strategies are developed to counteract impairment (e.g., wearing clothes that are easy to put on or take off, getting (un)dressed or showering while sitting down, use of aids)

None

 

6.1

Attendant needed to assist with self-care

Attendant needed to assist with self-care

Another person is required on a regular basis to (un)dress and attend to the affected person’s personal hygiene

None

 

6.0

Total dependence

Total dependence

Dressing, undressing and personal hygiene must be entirely performed by another person

None

 

7

Turning in bed and adjusting bed clothes

Turning in bed and adjusting bed clothes

   

7.4

Normal

Normal

Turning in bed and handling blankets do not cause problems

None

 

7.3

Somewhat slow and clumsy, but no help needed

Somewhat slow and clumsy, but no help needed

Turning in bed or handling blankets is difficult

None

 

7.2

Can turn alone or adjust sheets, but with great difficulty

Can turn on their own or adjust sheets, but with great difficulty

Turning in bed and handling blankets is possible but requires great effort. Either action may require support, or a grip may be used when turning in bed

Linguistic adaption

Minor

7.1

Can initiate, but not turn or adjust sheets alone

Can initiate action, but not turn or adjust sheets without assistance

The actions of turning in bed and handling blankets can be initiated, however another person's assistance is required to complete these actions

Linguistic adaption

Minor

7.0

Helpless

Total dependence

Assistance is consistently required when turning in bed or handling blankets

Harmonization

Moderate

8

Walking

Walking

   

8.4

Normal

Normal

No change in walking ability

None

 

8.3

Early ambulatory difficulties

Minor ambulatory difficulties

Changes, such as walking more slowly, stumbling, or a loss of stability, are apparent, although the affected does not require outside assistance on a regular basis, either in the form of another person, a walking aid (e.g., foot lifter, cane, walkers) or holding on to a stable object

Linguisitic adaption

 

8.2

Walks with assistance

Walks with assistance

The affected regularly requires assistance when walking-either in the form of holding on to something, or, outside the home, use of a foot lifter, walking aids or help from another person

None

 

8.1

Nonambulatory functional movement

Nonambulatory functional movement

Targeted leg movements are still possible. Standing with support, e.g., for transfer, can be possible. The affected has no ambulatory capacity, not even with the assistance of another person

None

 

8.0

No purposeful leg movement

No purposeful leg movement

The legs cannot support the weight of the body (e.g., for transfer), no purposeful movements can be executed, such as helping with care activities

None

 

9

Climbing stairs

Climbing stairs

   

9.4

Normal

Normal

No change is observed when climbing the stairs

None

 

9.3

Slow

Slow

Climbing the stairs without taking a break or feeling unstable is possible if done slowly

None

 

9.2

Mild unsteadiness or fatigue

Mild unsteadiness or fatigue

Climbing the stairs is accompanied by a feeling of instability, and breaks might be necessary. Use of a handrail or assistance from another person are not absolutely necessary

None

 

9.1

Needs assistance

Needs assistance

Climbing the stairs cannot be executed without use of a handrail or assistance from another person

None

 

9.0

Cannot do

Cannot do

Stairs cannot be climbed, even with assistance or support

None

 

10

Dyspnea

Dyspnea and shortness of breath

 

Addition

Minor

10.4

None

None

No dyspnea or shortness of breath when performing daily routines at normal intensity

None

 

10.3

Occurs when walking

Occurs when walking

Dyspnea or shortness of breath may occur when walking at a normal pace or performing activities at moderate intensity

None

 

10.2

Occurs during one or more of the following: eating, bathing, dressing (ADL)

Occurs during one or more of the following: eating, bathing, dressing (ADL)

Dyspnea or shortness of breath may occur when performing activities at low intensity or when talking for longer periods of time

None

 

10.1

Occurs at rest, difficulty breathing when either sitting or lying

Difficulty breathing when at rest, including sitting or lying down

Dyspnea or shortness of breath in the absence of any physical strain when either sitting and/or lying down

Linguistic adaption

Minor

10.0

Significant difficulty, considering using mechanical respiratory support

Significant difficulty breathing, mechanical respiratory support may be needed

Significant dyspnea or shortness of breath is present when at rest; mask ventilation (non-invasive ventilation) or ventilation via tracheostomy must be applied to alleviate dyspnea and shortness of breath

Linguistic adaption

Moderate

11

Orthopnea

Sleep disturbance due to breathing problems

If mechanical ventilation is usually provided during the night, but sleep is possible without it, nighttime breathing should be assessed without the use of ventilation

Harmonization and Addition

Substantial

11.4

None

None

Falling asleep and sleeping through the night are unimpaired by dyspnea or shortness of breath

None

 

11.3

Some difficulty sleeping at night due to shortness of breath, does not routinely use more than two pillows

Some difficulty sleeping at night due to shortness of breath, more than two pillows are not routinely used

Dyspnea and shortness of breath are present at night and when lying down. Breathing may be improved by sleeping on one side. To support the torso, a maximum of two pillows are used or the head section of the bed may be elevated by no more than 30 degrees

Linguistic adaption

Minor

11.2

Needs extra pillows in order to sleep (more than two)

More than two pillows are needed in order to sleep

When lying down flat on one's back, breathing is noticeably bothersome, which in turn disturbs the process of falling asleep and sleeping through the night. To support the torso, three or more pillows are used or the head section of the bed is elevated by more than 30 degrees

Linguistic adaption

Minor

11.1

Can only sleep sitting up

Can only sleep sitting up

A seated position must be assumed, either in bed or on a chair, to sleep

None

 

11.0

Unable to sleep

Unable to sleep

Due to dyspnea or shortness of breath, sleep is impossible without mask ventilation (non-invasive ventilation) or ventilation via tracheostomy. Mechanical ventilation is in regular use to alleviate symptoms

None

 

12

Respiratory insufficiency

Mechanical ventilation

 

Linguistic adaption

Moderate

12.4

None

None

Breathing is always an autonomous action, not requiring use of mechanical ventilation. Nocturnal air pressure support (i.e., CPAP therapy to treat sleep apnea syndrome) does not constitute mechanical ventilation

None

 

12.3

Intermittent use of BiPAP

Intermittent use of non-invasive ventilation

Mask ventilation (non-invasive ventilation, e.g., BiPAP) is in use at irregular intervals or for a shorter period of time than the normal nocturnal sleep cycle

Harmonization

Minor

12.2

Continuous use of BiPAP during the night

Continuous use of non-invasive ventilation during the night

Mask ventilation (non-invasive ventilation) is in regular use at night and possibly on an hourly basis during the day (a total of 8 to 22 h in any 24-h cycle)

Harmonization

Minor

12.1

Continuous use of BiPAP during the night and day

Continuous use of non-invasive ventilation during the night and day

Mask ventilation (non-invasive ventilation) is in use almost all of the time (more than 22 h per in any 24-h cycle)

Harmonization

Minor

12.0

Invasive mechanical ventilation by intubation or tracheostomy

Invasive mechanical ventilation by intubation or tracheostomy

Continuous mechanical ventilation via a ventilation tube (intubation) or tracheostomy

None

 
  1. Introduction: The self-explanatory Amyotrophic Lateral Sclerosis Functional Rating Scale – Revised (ALSFRS-R-SE) is comprised of various motor functioning items that typically have limiting characteristics in ALS. The ALSFRS-R-SE assessment can be performed by patients themselves as well as by others (e.g., an attending physician, a relative, a healthcare professional) following an interview with the affected. Please carefully read the explanations and options and provide an assessment of functionality, and, respectively, relevant ALS-related limitations that reflect actual capacities at the time of filling in the questionnaire
  2. If the cause of a limitation in any functional area is attributable to a medical condition other than ALS, or if a limitation was already present before the onset of ALS (e.g., gait impairment following hip replacement surgery) the respective item can be assessed as “normal” (4 score points). Functionality should always be assessed relative to one’s status before the onset of initial ALS symptoms. The affected may deviate from this recommendation if they are experiencing additional limitations that are likely to be due to ALS. Once an approach is chosen, please be consistent in following it when answering all questions on this and on future ALSFRS-R-SE assessments