Detecting frontotemporal dysfunction in ALS: Utility of the ALS Cognitive Behavioral Screen (ALS-CBS™)

Woolley, Susan; York, Michele K.; Moore, Dan H.; Strutt, Adriana M.; Murphy, Jennifer; Schulz, Paul E.; Katz, Jonathan

doi:10.3109/17482961003727954

Cited by 177 publications

(157 citation statements)

References 27 publications

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“…16 The Delis-Kaplan Executive Function System was used to classify patients as having ALS with mild cognitive impairment (ALSci), and the Frontal Systems Behavioral Evaluation and UCLA Neuropsychiatric Index were used to classify patients as having ALS with mild behavioral impairment (ALSbi) using consensus criteria. 17 After 2010, patients were screened for cognitive deficits with the ALS Cognitive Behavioral Screen, 18 the Montreal Cognitive Assessment (www.mocatest.org), or the Mini-Mental State Examination.…”

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confidence: 99%

Impaired corticopontocerebellar tracts underlie pseudobulbar affect in motor neuron disorders

et al. 2014

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Objective: The objectives of the study were (1) to determine the prevalence and characteristics of pseudobulbar affect (PBA) in patients with primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) in an outpatient clinic population, and (2) to test the hypothesis that damage of inputs to the cerebellum, leading to cerebellar dysmodulation, is associated with PBA.Methods: Chart review of all patients with PLS and ALS seen between 2000 and 2013. The examining neurologist documented the presence or absence of PBA in 87 patients. Fortyseven patients also had diffusion tensor imaging (DTI) studies. Tract-based spatial statistics were used to compare DTI of patients with and without PBA to identify altered white matter tracts associated with PBA.Results: Thirty-one of 50 patients with PLS and 12 of 37 patients with ALS had PBA. Psychiatric/emotional assessment found congruence between mood and affect during episodes, but excessive magnitude of the response. DTI studies of 25 PLS and 22 ALS patient brains showed reduced fractional anisotropy of the corticospinal and callosal white matter tracts in all patients. Patients with PBA additionally had increased mean diffusivity of white matter tracts underlying the frontotemporal cortex, the transverse pontine fibers, and the middle cerebellar peduncle.Conclusions: PBA is common in PLS. Imaging findings showing disruption of corticopontocerebellar pathways support the hypothesis that PBA can be viewed as a "dysmetria" of emotional expression resulting from cerebellar dysmodulation. Neurology ® 2014;83:620-627 GLOSSARY ALS 5 amyotrophic lateral sclerosis; ALSbi 5 amyotrophic lateral sclerosis with mild behavioral impairment; ALSci 5 amyotrophic lateral sclerosis with mild cognitive impairment; ALSFRS-R 5 Amyotrophic Lateral Sclerosis Functional Rating Scale-revised; BDI-II 5 Beck Depression Inventory-II; DRS-2 5 Mattis Dementia Rating Scale-2; DTI 5 diffusion tensor imaging; FA 5 fractional anisotropy; FSL 5 FMRIB's Software Library; PBA 5 pseudobulbar affect; PLS 5 primary lateral sclerosis; TFCE 5 threshold-free cluster enhancement; UCLA 5 University of California Los Angeles.Pseudobulbar affect (PBA) is a syndrome of involuntary emotional expression dissociated from one's true emotional experience.1 It occurs in several neurologic disorders, including amyotrophic lateral sclerosis (ALS).2,3 In one retrospective series, PBA occurred in 50% of patients with ALS. 4 There have been case reports of PBA in patients with primary lateral sclerosis (PLS), a motor neuron disorder variant with relatively selective degeneration of upper motor neurons. However, the prevalence of PBA in PLS has not been evaluated in a large patient series. 2,6 To assess the occurrence of PBA in PLS, we reviewed the charts of all patients seen in an upper motor neuron disorder clinic.Two hypotheses have been proposed regarding the neural circuits that produce PBA. Wilson 7 proposed that brainstem centers controlling laughter and crying were separately controlled by volitional a...

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confidence: 99%

Impaired corticopontocerebellar tracts underlie pseudobulbar affect in motor neuron disorders

et al. 2014

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“…Most of these symptoms are overlapping and should be analysed carefully. For example, most behavioural changes seen in ALS patients are similar to those of patients diagnosed with behavioural variant fronto-temporal dementia (bvFTD) [221]. Woolley et al [221] found that unlike non-demented ALS patients, significant lack of insight was observed in patients diagnosed with both ALS and FTD [221].…”

Section: Amyotrophic Lateral Sclerosis and Related Psycho-behaviouralmentioning

confidence: 94%

Neurochemicals, Behaviours and Psychiatric Perspectives of Neurological Diseases

Choudhury¹,

Sahu²,

Ramanujam³

et al. 2018

Neuropsychiatry

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Structural changes in different regions of the brain have become clinically relevant and regarded as the signature phenomenon for neurological diseases. Morphological changes in brain are also associated with neuronal and neurochemical alterations. Studies have showed that minute changes in neurochemical levels may have marked impact on the psychobehaviour of the subject. Several neurological disease profiles have been reported with such specific psychobehavioural expression. However, application of behavioural abnormalities as possible disease progress markers has not been considered and emphasised much. Reports suggested that-the subjects, who have already entered into the terminal stage of the disease, used to show cardinal behavioural signs for a specific disease profile. However, psychological expressions are comparatively early expressive. As most of the neurodegenerative disorders are unidirectional and progressive by nature, therapeutic intervention at the right time is essential for attaining the desired outcome. Moreover, early diagnosis can aid in managing the disease progression also. Psychobehavioural analysis could meet the expected outcome of disease diagnosis if implemented properly and timely. In the present review, we have amalgamated the reported behavioural anomalies with the supportive background from neurochemical basis. Further, we have concluded that behaviour centric studies could be a potential diagnostic tool for the early diagnosis of major neurological diseases such as Alzheimer disease, Parkinson's disease, Amyotrophic lateral sclerosis (ALS), Bipolar disorder, Schizophrenia, Impulse control disorder (ICD) and Obsessive-compulsive disorder (OCD).

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“…5,6,18 The proportion of patients with mild behavioral changes as assessed with the ALS-FTD-Q (11%) is lower than that in other studies (17%-50%). 8,27,32,33 Earlier studies used instruments that have not been validated for the assessment of behavioral changes in ALS and contain items that have not been corrected for motor impairment. 8,11 In particular, apathy has been shown to be present in up to 50% of patients with ALS.…”

Section: Als-ftd-q and Comparable Scalesmentioning

confidence: 99%

“…9 -11 To our knowledge, 3 screening instruments for nonmotor involvement (focusing on cognitive functions) have been investigated in patients with ALS. [27][28][29] One screen contains 15 questions about behavior 27 ; 2 other screens included the FBI. 28,29 Compared with these screening instruments, the ALS-FTD-Q has 4 unique advantages.…”

Section: Als-ftd-q and Comparable Scalesmentioning

confidence: 99%

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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Detecting frontotemporal dysfunction in ALS: Utility of the ALS Cognitive Behavioral Screen (ALS-CBS™)

Cited by 177 publications

References 27 publications

Impaired corticopontocerebellar tracts underlie pseudobulbar affect in motor neuron disorders

Impaired corticopontocerebellar tracts underlie pseudobulbar affect in motor neuron disorders

Neurochemicals, Behaviours and Psychiatric Perspectives of Neurological Diseases

The ALS-FTD-Q

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