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Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire (ALS-FTD-Q)
Please visit this website for more information about the instrument: "Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire (ALS-FTD-Q) "
Measure is freely available for clinical and research use if permission and consent is requested via website.
Supplemental: Amyotrophic Lateral Sclerosis (ALS)
Short Description of Instrument
The Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire (ALS-FTD-Q) is a screening tool used to detect the behavioral variant of frontotemporal dementia (bvFTD) and mild behavioral disturbances in ALS (Raaphorst et al., 2012a). The 25-item questionnaire is an observer report scale aimed at the proxy (partner, family member, or friend) of a patient with ALS (Raaphorst et al., 2012a) and takes approximately 10 minutes to complete. The ALS-FTD-Q was originally developed to be used by physicians to gain insight into the extent of the behavioral changes, but can also be used for research purposes.
Comments/Special Instructions
The ALS-FTD-Q 25-item scale with two sections (A & B). Section A asks caregivers to compare patient's current behavior to their behavior three years ago, while section B asks about behavioral changes in the past month. The ALS-FTD-Q includes 3 cognitive items: memory, concentration, and orientation in time.
Items 1–13: Completely disagree = 0; Largely disagree = 1; Largely agree = 3; Completely agree = 4
Items 14, 15, 16, 18, 20, 22, 23 and 25: Never = 0; Sometimes = 1; Often = 3; Always = 4
Items 17, 19, 21 and 24: Never = 4; Sometimes = 3; Often = 1; Always = 0
Reverse Scoring: items 17, 19, 21 and 24 are reverse-scored items (i.e., 0=4; 1=3; 3=1; and 4=0).
Scoring: Item scores are summed to calculate the ALS-FTD-Q score. A higher score indicates more behavioral disturbances.
Tentative cut off-values, pending on future validation: Mild behavioral disturbances = 22
Severe behavioral disturbances = 29
Strengths/Weaknesses: Strengths: Behavioral items selected from a systematic review of 170 case descriptions of patients with ALS-bvFTD (Raaphorst et al., 2012b); motor and speech dysfunction were taken into account when phrasing items; the ALS-FTD-Q has good internal consistency, construct and clinical validity; and test scores were compared with both negative and positive control groups (Raaphorst et al., 2012a).
Weaknesses: Behavioral disturbances may be overestimated in ALS patients; scoring cut-offs require further validation; and the use of the ALS-FTD-Q is limited to patients with a proxy.
Psychometric Properties: The initial validation study included five groups of patients (103 ALS patients, 10 ALS-FTD patients, 25 patients with FTD without ALS, 39 patients with muscle diseases, and 31 subjects evaluated at the out-patient neurology clinic for other complaints). Patients with ALS-FTD and FTD served as positive controls (n=35); patients with muscle diseases and the other complaints served as negative controls (n=70) (Raaphorst et al., 2012).
Clinimetrics: ALS-FTD scores based on a study patients with ALS showed internal consistency (Cronbach a = 0.92). Twenty-three of the 25 items showed an item-total score correlation range of 0.31 – 0.78, while the 2 items (hypersexuality and euphoria) had an item-total score correlation of 0.20 – 0.26.
Construct validity was shown by high correlations between ALS-FTD-Q, Frontal Systems Behavior scale (FrSBe) and Frontal Behavioral Inventory (FBI); moderate correlations between ALS-FTD-Q and the Frontal Assessment Battery (FAB), fluency, and Mini-Mental State Examination (MMSE); and low correlations between the ALS- FTD-Q and the Hospital Anxiety and Depression Scale (HADS) and ALS-Functional Rating Scale-Revised (ALSFRS-R) (Raaphorst et al., 2012a). Measure authors suggest that the ALS-FTD-Q measures behavioral changes more precisely than the two measures, because the effect of motor and speech dysfunction were taken into account and are minimized in the ALS-FTD-Q.
Cultural considerations: The original ALS-FTD-Q was developed in Amsterdam and is currently used in several countries, for clinical and research purposes. Validation studies of the Spanish, Italian and Japanese version of the ALS-FTD-Q are in progress.
Raaphorst J, Beeldman E, Schmand B, Berkhout J, Linssen WH, van den Berg LH, Pijnenburg YA, Grupstra HF, Weikamp JG, Schelhaas HJ, Papma JM, van Swieten JC, de Visser M, de Haan RJ. The ALS-FTD-Q: a new screening tool for behavioral disturbances in ALS. Neurol. 2012a;79(13):1377–1383.
de Visser M, van den Berg L, van Swieten J, Scheltens P, Pijnenburg Y. (2012) ALS- FTD-Q. Accessed 5 October 2016:
Raaphorst J, Beeldman E, De Visser M, De Haan RJ, Schmand B. A systematic review of behavioural changes in motor neuron disease. Amyotroph Lateral Scler. 2012b;13(6):493–501.
Watanabe Y, Beeldman E, Raaphorst J, Izumi Y, Yoshino H, Masuda M, Atsuta N, Ito S, Adachi T, Adachi Y, Yokota O, Oda M, Hanashima R, Ogino M, Ichikawa H, Hasegawa K, Kimura H, Shimizu T, Aiba I, Yabe H, Kanba M, Kusumi K, Aoki T, Hiroe Y, Watanabe H, Nishiyama K, Nomoto M, Sobue G, Nakashima K; ALS-FTD-Q-J Research Group. Japanese version of the ALS-FTD-Questionnaire (ALS-FTD-Q-J). J Neurol Sci. 2016;367:51–55.