Apathy is associated with poor prognosis in amyotrophic lateral sclerosis

Caga, Jashelle; Turner, Martin R; Hsieh, Sharpley; Ahmed, Rebekah M.; Devenney, Emma; Ramsey, Eleanor; Zoing, Margaret; Mioshi, Eneida; Kiernan, Matthew C.

doi:10.1111/ene.12959

Cited by 57 publications

(46 citation statements)

References 40 publications

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“…In our sample, only two ALS cases presented apathy (FBI apathy item score ≥1) as the only behavioural disturbance, and apathetic symptoms tended to be associated with inattentive and hypocritical behaviours. For clinical purposes, apathetic symptoms should be taken into account, since severe apathy is linked to poorer prognosis in ALS 37. Overall, our findings proved that the bvFTD behavioural profiles partially overlapped those of non-demented patients with ALS patients.…”

Section: Discussionsupporting

confidence: 57%

Cortical correlates of behavioural change in amyotrophic lateral sclerosis

Consonni

Cappa

Bella

et al. 2018

J Neurol Neurosurg Psychiatry

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BackgroundBehavioural changes in amyotrophic lateral sclerosis (ALS) are heterogeneous. The study aim was to identify the behavioural profiles of non-demented patients with ALS and their neuroimaging correlates and to elucidate if they are comparable to those reported in studies of the behavioural-variant of frontotemporal dementia (bvFTD).MethodsBehavioural changes of 102 non-demented patients with ALS were assessed through the Frontal Behavioural Inventory (FBI), a 24-item scale assessing different behavioural modifications, mainly chosen from the core clinical features of FTD. Principal component analysis (PCA) was used to detect distinct clusters of behavioural changes based on FBI subscores. The cortical thinning related to each behavioural profile was analysed in 29 patients with ALS. Cronbach’s α was used to test the reliability of bvFTD-related FBI clustering in our cohort.ResultsSixty patients with ALS had FBI score≥1. PCA identified three phenotypic clusters loading on disinhibited/hostile, dysexecutive and apathetic FBI subscores. Imaging analyses revealed that the thinning of bilateral orbitofrontal cortex was related to apathy, the right frontotemporal and cingular cortex to the disinhibited/hostile profile and the left precuneus cortex to the dysexecutive behaviours. The bvFTD-associated aggressive profile reliably applied to our cohort.ConclusionsIn non-demented patients with ALS, different behavioural profiles could be identified. The right frontotemporal and cingular cortex thinning was the hallmark of the behavioural profile mostly overlapping that described in bvFTD. Our findings provide the unbiased identification of determinants relevant for a novel stratification of patients with ALS based on their behavioural impairment, which might be useful as proxy of cognitive decline.

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Section: Discussionsupporting

confidence: 57%

Cortical correlates of behavioural change in amyotrophic lateral sclerosis

Consonni

Cappa

Bella

et al. 2018

J Neurol Neurosurg Psychiatry

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“…Not only has the ECAS shown high sensitivity and specificity to cognitive impairment against a full neuropsychological battery (17)(18)(19)(20)(21), it has high clinical utility in describing the nature of these impairments. Monitoring progression of cognitive and behavioural symptoms may have important implications for patient management, treatment, prognosis, end-oflife decision making, and caregiver burden (22)(23)(24)(25)(26)(27)(28). The purpose of this study was to develop alternate forms of the ECAS to facilitate repeated assessment and longitudinal monitoring of cognition and behaviour in patients with ALS.…”

Section: Discussionmentioning

confidence: 99%

“…Cognitive dysfunction may have important implications for patient management, treatment fidelity, power of attorney, and endof-life decision making (22)(23)(24). Behaviour change has been linked to increased carer burden (25,26) and shortened survival (27,28). As such, the accurate assessment of cognition and behaviour over time is of vital importance to meeting the needs of patients and their families.…”

Section: Introductionmentioning

confidence: 99%

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen

Crockford

Kleynhans

Wilton

et al. 2017

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Background: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a short assessment by which neuropsychological symptoms can be detected and quantified in people with ALS. To avoid potential practice effects with repeated administration, here we present alternative versions of the ECAS suitable for measuring change over time. Objective: To develop two alternate versions of the ECAS: ECAS-B and ECAS-C. Method: One hundred and forty-nine healthy adult participants were recruited. Thirty participants completed a pilot study in developing the alternate versions. Two groups of 40 participants were administered the ECAS-B or ECAS-C and compared to published data of the original ECAS (ECAS-A) to determine equivalence. An additional 39 participants were administered the ECAS consecutively, either repeating the original version (ECAS-A-A-A) serially or the different versions (ECAS-A-B-C) to determine potential practice effects. Recordings of assessments were scored by a second researcher to determine inter-rater reliability. Results: No significant differences were found between versions (A, B, C) of the composite performance measures of ALS Specific, ALS Non-Specific, and ECAS Total scores. Repeated serial administration of ECAS-A (A-A-A) produced some practice effects for composite scores, whereas no such effects were found when alternate versions were administered serially (A-B-C). Exceptionally high intra-class correlations were found for all three versions of the ECAS suggesting a high degree of rater agreement. Conclusion: The newly developed alternate forms of the ECAS are both highly equitable to the original ECAS-A and enable avoidance of practice effects, thus supporting their use in measuring cognition and behaviour over time.

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“…In particular, more data should be obtained on compliance with NIV, the role of patients, proxies and physicians in the decision-making process, and on end of life practices in ALS patients with the frontotemporal syndrome [9, 21, 31]. In addition, a study in a larger cohort may elucidate the impact of different aspects of behavioral changes in ALS (i.e., apathetic vs disinhibited type) [7]. …”

Section: Discussionmentioning

confidence: 99%

The frontotemporal syndrome of ALS is associated with poor survival

et al. 2016

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Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the FS on survival and the start and duration of NIV in ALS. Behavioral changes were defined as >22 points on the ALS-Frontotemporal-Dementia-Questionnaire or ≥3 points on ≥2 items of the Neuropsychiatric Inventory. Cognitive impairment was defined as below the fifth percentile on ≥2 tests of executive function, memory or language. Classic ALS was defined as ALS without the frontotemporal syndrome. We performed survival analyses from symptom onset and time from NIV initiation, respectively, to death. The impact of the explanatory variables on survival and NIV initiation were examined using Cox proportional hazards models. We included 110 ALS patients (76 men) with a mean age of 62 years. Median survival time was 4.3 years (95 % CI 3.53–5.13). Forty-seven patients (43 %) had an FS. Factors associated with shorter survival were FS, bulbar onset, older age at onset, short time to diagnosis and a C9orf72 repeat expansion. The adjusted hazard ratio (HR) for the FS was 2.29 (95 % CI 1.44–3.65, p < 0.001) in a multivariate model. Patients with an FS had a shorter survival after NIV initiation (adjusted HR 2.70, 95 % CI 1.04–4.67, p = 0.04). In conclusion, there is an association between the frontotemporal syndrome and poor survival in ALS, which remains present after initiation of NIV.

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Apathy is associated with poor prognosis in amyotrophic lateral sclerosis

Abstract: The presence of severe apathy is an independent, negative prognostic factor in ALS.

Cited by 57 publications

References 40 publications

Cortical correlates of behavioural change in amyotrophic lateral sclerosis

Cortical correlates of behavioural change in amyotrophic lateral sclerosis

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen

The frontotemporal syndrome of ALS is associated with poor survival

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