Amyotrophic Lateral Sclerosis- Frontotemporal Dementia - Questionnaire (ALS-FTD-Q)

The behaviour of your partner, family member or friend will be evaluated inthe following questionnaire. Wherever, “him” or “his” is stated, it can be replaced by “her”. Completing the questionnaire should take approximately 10 minutes and ideally should be done in a room separated from the patient.To answer a question, check the box with the most appropriate answer. The questionnaire consists of two parts; A and B.

Date . . - . . - . . . . Filled out by (e.g. partner, sibling, child) ......

Name of the patient ......

Date of birth of the patient . . - . . - . . . . Gender of the patient M / F

Highest level of education completed bythe patient ......

Part AThe following 13 statements compare the present behaviour of your partner to his behaviour three years ago.

Possible answers are the following:

- completely disagree

- largely disagree

- largely agree

- completely agree

1.Your partner is less interested in his surroundings

2.Your partner pays less attention to his personal hygiene

3.Your partner puts himself firstmore often

4.Your partner becomesirritated or angry more easily

5.Your partner’s ability to concentrate has decreased

6.Your partner’s behaviour is more restless

7.Your partner displays more withdrawn behaviour

8.Your partner seems to undertake more aimless activities

9.Your partner has more problems with memory

10.Your partner contacts strangers more frequently

11.Your partner has an increased urge for sex

(If not applicable; leave unanswered)

Some ALS patients experience compulsive laughter or crying:laughing or

crying without a logical reason.

The following two statements DO NOT describe this phenomenon;

they describe changes in your partner’s emotions in general.

12.Your partner is emotionally less stable

13.Your partner is more often extremely cheerful

Part B The following 12 statements are about the behaviour of your partner during the past month. Please note: some statements describe normal behaviour, while others describe abnormal behaviour. Therefore, please read the statement carefully prior to answering.

Possible answers are the following:

- Never

- Sometimes

- Often

- Always

14.Your partner is suspicious

15.Your partner repeatedly uses the same gesturesor sentences

16.Your partner is shameless

17.Your partner is aware of his whereabouts

18.Your partner displays offensive behaviour

19.Your partner is able to assess situations well

20.Your partner hoards food or is preoccupied by food

21.Your partner understands what his disease is about

22.Your partner sees or hears things that are not there

23.Your partner displays childish behaviour

24.Your partner knows which part of the day it is

25.Your partner imitates you or others

Scoring of the ALS-FTD-Q

Items 1-13:

Value = 0 1 3 4

Items14, 15, 16, 18, 20, 22, 23 and 25:

Value = 0 1 3 4

Items 17, 19, 21 and 24:

Value = 4 3 1 0

ALS-FTD-Q score: item scores are summed to calculate the ALS-FTD-Q score.

Cut-off values: mild behavioral disturbances ≥ 22

severe behavioral disturbances (in the bvFTD range) ≥ 29

These are tentative cut-off values, pending future validation