Frontal‐lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis

Witgert, Mariana E.; Salamone, Alicia R.; Strutt, Adriana M.; Massman, Paul J.; Bradshaw, Major; Mosnik, Diane; Appel, Stanley H.; Schulz, Paul E.

doi:10.1111/j.1468-1331.2009.02801.x

Cited by 105 publications

(81 citation statements)

References 29 publications

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“…The intermediate correlation between fluency and the ALS-FTD-Q in our study is comparable to findings by others and shows that the questionnaire measures a construct (frontal-mediated behavior) that is related to fluency, supporting the construct validity of the ALS-FTD-Q. 26 In addition, clinical validity was shown because the ALS-FTD-Q discriminated between patients with a known difference in the presence of frontal behavioral disturbances. These good clinimetric properties and the easy way of administering the ALS-FTD-Q make it a feasible screening instrument in clinical practice as well as for research projects.…”

Section: Als-ftd-q and Comparable Scalessupporting

confidence: 76%

“…8,11 In particular, apathy has been shown to be present in up to 50% of patients with ALS. 11,26,34 However, apathy was studied with the 14-item FrSBe apathy subscale, of which 7 items are directly related to speaking and moving, which may have led to overestimating motor-related mild behavioral changes, e.g., apathy. 11,26,34 The mild and severe behavioral changes in ALS in the present study have to be interpreted in relation to the cutoffs, which have to be further validated, and in relation to the study population.…”

Section: Als-ftd-q and Comparable Scalesmentioning

confidence: 99%

“…11,26,34 However, apathy was studied with the 14-item FrSBe apathy subscale, of which 7 items are directly related to speaking and moving, which may have led to overestimating motor-related mild behavioral changes, e.g., apathy. 11,26,34 The mild and severe behavioral changes in ALS in the present study have to be interpreted in relation to the cutoffs, which have to be further validated, and in relation to the study population. We did not perform a receiver operating characteristic analysis to define the cutoffs of the ALS-FTD-Q because, for mild behavioral disturbances, a gold standard in ALS does not exist (the FrSBe could not be used for this purpose as it contains motor-and speech-related items).…”

Section: Als-ftd-q and Comparable Scalesmentioning

confidence: 99%

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The ALS-FTD-Q

et al. 2012

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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.

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Section: Als-ftd-q and Comparable Scalessupporting

confidence: 76%

Section: Als-ftd-q and Comparable Scalesmentioning

confidence: 99%

Section: Als-ftd-q and Comparable Scalesmentioning

confidence: 99%

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The ALS-FTD-Q

et al. 2012

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“…35 The finding that apathy is one of the first neuropsychiatric symptoms in patients who have the dual diagnosis of ALSFTD is not surprising, given the pervasive nature of apathy in ALS. 29,36 Interestingly, the presence of early apathy may be a discriminating feature between ALSFTD and pure bvFTD in which disinhibition and stereotypical behavior are very common as initial features. 37 Brief screening tools cannot replace the comprehensive multidisciplinary assessment that would be needed to diagnose patients with ALS and, importantly, to identify those with concomitant FTD.…”

Section: Discussionmentioning

confidence: 99%

Cognitive and Behavioral Symptoms in ALSFTD

Hsieh

Caga²,

Leslie

et al. 2015

J Geriatr Psychiatry Neurol

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Brief screening tools that detect and differentiate patients with amyotrophic lateral sclerosis and frontotemporal dementia (ALSFTD) from those more subtle cognitive or behavioral symptoms (ALS plus) and motor symptoms only (ALS pure) is pertinent in a clinical setting. The utility of 2 validated and data-driven tests (Mini-Addenbrooke's Cognitive Examination [M-ACE] and Motor Neuron Disease Behavioral Scale [MiND-B]) was investigated in 70 ALS patients (24 ALSFTD, 19 ALS plus, and 27 ALS pure). More than 90% of patients with ALSFTD scored at or below the cutoff on the M-ACE, whereas this was seen in only about 20% of ALS patients without dementia. The MiND-B differentiated between ALS pure and ALS plus diagnostic categories. Rasch modeling of M-ACE and MiND-B items revealed early cognitive (fluency, memory recall) and behavioral (apathy) symptoms in ALSFTD. The combined use of the M-ACE and MiND-B detects patients with ALSFTD, differentiates along the ALS continuum, and offers insight into the progression of nonmotor symptomatology in ALSFTD.

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“…Another pathophysiological pathway discussed in the pathogenesis of FTD is the progranulin pathway (Sleegers et al, 2010). Cognitive impairment and frontal lobe dysfunction is seen in about 40-60% of ALS patients (Abrahams et al, 1996;Evdokimidis et al, 2002;Witgert et al, 2009). …”

Section: Dementiamentioning

confidence: 99%

Amyotrophic Lateral Sclerosis: An Introduction to Treatment and Trials

Maurer¹

2012

Amyotrophic Lateral Sclerosis

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Frontal‐lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis

Cited by 105 publications

References 29 publications

The ALS-FTD-Q

The ALS-FTD-Q

Cognitive and Behavioral Symptoms in ALSFTD

Amyotrophic Lateral Sclerosis: An Introduction to Treatment and Trials

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