Medical decisions are independent of cognitive impairment in amyotrophic lateral sclerosis

Böhm, Sarah; Aho-Özhan, Helena E. A.; Keller, Jürgen; Dorst, Johannes; Uttner, Ingo; Ludolph, Albert C.; Lulé, Dorothée

doi:10.1212/wnl.0000000000003232

Cited by 23 publications

(29 citation statements)

References 5 publications

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“…Thus, ALS and FTD do not represent one entity in the sense that a single patient, for example, with ALS necessarily changes into a patient with FTD and vice versa, which is in line with the fact that cognitive performance does not necessarily decline in the course of ALS 14. This has major clinical implications as these minor cognitive impairments in ALS do not interfere with medical decision-making6 and thus have mostly no impact on the daily living of patients with ALS, whereas for patients with ALS with full-blown behavioural phenotype of bvFTD (ALS-bvFTD), the interference can be expected.…”

Section: Discussionmentioning

confidence: 92%

“…However, clinical experience and previous studies suggest only very mild cognitive and behavioural alterations in patients with ALS1 with also distinct differences to those seen in dementia. Furthermore, there were no associations of these impairments seen to clinical decision-making 6. Furthermore, neuropathological data provide evidence for distinct entities with two different neuropathological spreading patterns of TDP43 pathology for ALS and FTD 7 8.…”

Section: Introductionmentioning

confidence: 88%

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Story of the ALS-FTD continuum retold: rather two distinct entities

Lulé

Aho-Özhan

Vázquez

et al. 2018

J Neurol Neurosurg Psychiatry

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ObjectiveTo determine the evolution and profile of cognitive and behavioural deficits in amyotrophic lateral sclerosis (ALS) and behavioural variant frontotemporal dementia (bvFTD) to disentangle the development of FTD in ALS and vice versa.MethodsIn a prospective design, cognitive and behavioural profiles of 762 patients with motor predominant ALS (flail arm/leg syndrome, primary lateral sclerosis, pseudobulbar palsy, ALS) and behavioural predominant FTD (bvFTD, ALS-FTD) were determined and caregivers of patients with ALS were asked on the evolution of behavioural symptoms. Data were compared with 49 healthy controls. Cognition was measured with the Edinburgh Cognitive and Behavioral ALS Screen.ResultsEvolution and features of cognitive profile of patients with motor predominant ALS were distinctly different from patients with behavioural FTD with regard to number and degree of affected cognitive domains. Also, in ALS mostly minus symptoms evolved after physical symptom onset whereas in ALS-FTD plus and minus symptoms were reported with an onset before physical degradation.ConclusionEvolution of cognitive and behavioural profile in patients with motor predominant ALS is distinctly different from those psychocognitive findings in patients with behavioural variant dementia. This may support the hypothesis that (possibly genetic) triggers decide in the preclinical phase on either motor or psychocognitive phenotypes.

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Section: Discussionmentioning

confidence: 92%

Section: Introductionmentioning

confidence: 88%

Story of the ALS-FTD continuum retold: rather two distinct entities

Lulé

Aho-Özhan

Vázquez

et al. 2018

J Neurol Neurosurg Psychiatry

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“…In a large study of 169 patients from a specialized German ALS clinic, analysis found no association with patients' decisions regarding the use or decline of percutaneous endoscopic gastrostomy (PEG), non-invasive ventilation (NIV) and invasive ventilation or hypothetical hastened death, and cognitive impairment. 31 Contrastingly, two qualitative studies interviewing carers and health care professionals both reported cognitive impairment impacted patients' health care decision making. 12,32 Carers reported it was more challenging to advocate for patients, and they needed to provide additional emotional support to facilitate patient decision making, when cognitive changes had occurred.…”

Section: Resultsmentioning

confidence: 99%

“…The issue of cognitive impairment and its impact on decision making is discussed in three studies. In a large study of 169 patients from a specialized German ALS clinic, analysis found no association with patients' decisions regarding the use or decline of percutaneous endoscopic gastrostomy (PEG), non‐invasive ventilation (NIV) and invasive ventilation or hypothetical hastened death, and cognitive impairment . Contrastingly, two qualitative studies interviewing carers and health care professionals both reported cognitive impairment impacted patients' health care decision making .…”

Section: Resultsmentioning

confidence: 99%

“…This review provides further evidence that studies addressing the association between, or the impact of, cognitive impairment and decision making are scarce and currently undetermined . However, the need to screen for cognitive changes and the impact of cognitive changes on decision making, as well the impact for carers, come through strongly in the studies included in this review.…”

Section: Discussionmentioning

confidence: 99%

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Communication and cognitive impairments and health care decision making in MND: A narrative review

Paynter

Cruice

Mathers

et al. 2019

Evaluation Clinical Practice

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Rationale Motor neurone disease (MND) is a neurodegenerative disease presenting with progressive weakness of voluntary muscles. For any condition, person‐centred health care relies on the sharing of information and a mutual understanding of the person's needs and preferences. Decision making in MND becomes more complex as there is no cure and a high prevalence of co‐morbid communication and/or cognitive difficulties. Objective To identify the reported impact of communication and/or cognitive impairment on patient and carer involvement in health care decision making in MND. Methods A review and synthesis of studies addressing issues of communication impairment and/or cognitive impairment in relation to decision making focussed on MND was conducted. Articles were excluded if they were reviews, case studies, conference papers, or commentaries. To be included studies needed to address issues of communication impairment or cognitive impairment specifically in relation to decision making. Relevant data were extracted verbatim and subjected to content analysis to support the narrative summary. Results Seventy‐six articles were identified, and 35 articles screened. Six articles met inclusion criteria each describing examples of decision making in MND. There was limited data related to communication and/or cognitive impairment, and the impact these impairments may have on decision making despite recognition that many people with MND may lose verbal communication or develop subtle cognitive impairments. The literature is primarily from the perspective of others. Conclusion This review highlights that the current body of literature exploring decision making within the MND population presents us with extremely limited insights into the impact of communication and/or cognitive impairments on health care decision making. Extant literature focuses on interventions (namely, ventilation and gastrostomy), the broad process of decision making, or cognitive assessment of decision‐making ability. Whilst most studies acknowledge that deficits in communication or cognition impact the decision‐making process, this issue is not the focus of any study.

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Amyotrophe Lateralsklerose (ALS)

Ludolph¹

2018

Klinische Neurologie

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Medical decisions are independent of cognitive impairment in amyotrophic lateral sclerosis

Cited by 23 publications

References 5 publications

Story of the ALS-FTD continuum retold: rather two distinct entities

Story of the ALS-FTD continuum retold: rather two distinct entities

Communication and cognitive impairments and health care decision making in MND: A narrative review

Amyotrophe Lateralsklerose (ALS)

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