The ALS-FTD-Q

Abstract

Objective: The assessment of behavioral disturbances in amyotrophic lateral sclerosis (ALS) is important because of the overlap with the behavioral variant of frontotemporal dementia (ALS-bvFTD). Motor symptoms and dysarthria are not taken into account in currently used behavioral questionnaires. We examined the clinimetric properties of a new behavioral questionnaire for patients with ALS (Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire [ALS-FTD-Q]). Methods:In addition to other clinimetric properties, we examined reliability, clinical validity, and construct validity of the ALS-FTD-Q, using data from patients with ALS (n ϭ 103), ALS-bvFTD (n ϭ 10), bvFTD (n ϭ 25), muscle disease control subjects (n ϭ 39), and control subjects (n ϭ 31). Construct validity of the ALS-FTD-Q was assessed using the Frontal Systems Behavior scale (FrSBe), Frontal Behavioral Inventory (FBI), Hospital Anxiety and Depression Scale, ALS Functional Rating ScaleϪRevised, Frontal Assessment Battery, Mini-Mental State Examination, and a fluency index. In addition, the point prevalence of behavioral disturbances according to the ALS-FTD-Q was compared with those obtained with the FrSBe and FBI. Results:The internal consistency of the ALS-FTD-Q was good (Cronbach ␣ ϭ 0.92). The ALS-FTD-Q showed construct validity because it correlated highly with other behavioral measures (r ϭ 0.80 and 0.79), moderately with measures of frontal functions and global cognitive functioning (r ϭ 0.37; r ϭ 0.32), and poorly with anxiety/depression and motor impairment (r ϭ 0.18 for both). The ALS-FTD-Q discriminated between patients with ALS-bvFTD, patients with ALS, and control subjects. The point prevalence of behavioral disturbances in patients with ALS measured with the ALS-FTD-Q was lower than that for the FrSBe and FBI.

Motor function and behaviour across the ALS-FTD spectrum

“…ALS specific assessments, such as the MiND-B, (26) have been developed recently to document behavioural symptoms. (26,27) However, one advantage of the FTDFRS is that it also includes functional items, addressing disease severity from both functional and behavioural perspectives. Given that ALSFRS-R follow-up data was insufficient, this finding may simply reflect small sample size, although differences in patterns of disease progression cannot be excluded.…”

The Behavioral/Cognitive Status of Motor Neuron Disease Patients Should Be Assessed With Disease-Specific Tools

“…The development of disease-specific, comprehensive rating scales for the assessment of behavioral manifestations in MND is extremely important for clinical practice and research purposes, and the contribution of Raaphorst and colleagues represents a significant advancement in this area [10]. The main strength of the behavioral questionnaire lies in the selection of the items, which is based on a careful analysis of the pertinent literature, rather than on a priori definition of expected consequences of frontal lobe involvement.…”

Measuring the progression of upper motor neuron disease using diffusion MRI and its correlation with Clinical phenotype

“…Disease progression rate = (48 -ALSFRS score)/disease duration (Hsieh, Schubert et al 2013), the Frontal Assessment Battery (FAB) (Dubois, Slachevsky et al 2000), and the ALS-FTD-Questionnaire (ALS-FTD-Q) (Raaphorst, Beeldman et al 2012), which have been introduced in Chapter 1.…”