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

Lulé, Dorothée; Aho-Özhan, Helena E. A.; Vázquez, Cynthia; Weiland, Ulrike; Weishaupt, Jochen H.; Otto, Markus; Anderl‐Straub, Sarah; Semler, Elisa; Uttner, Ingo; Ludolph, Albert C.

doi:10.1136/jnnp-2018-318800

Cited by 27 publications

(25 citation statements)

References 15 publications

(21 reference statements)

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“…For example, the relevance given to apathy in the diagnosis of ALSbi could indeed reduce the specificity of the criteria, considering the complexity of the theoretical construct of this particular behaviour23 and of its neuroanatomical and cognitive substrates 24. However, it should be noted that in a study based on ECAS, apathy was the most common behavioural symptoms detected in patients with ALS compared with patients with FTD, in whom disinhibition predominated 25. Longitudinal studies evaluating the progression over time of such patients are necessary to rule out this possibility.…”

Section: Discussionmentioning

confidence: 99%

Validation of the revised classification of cognitive and behavioural impairment in ALS

Iazzolino

Pain

Peotta

et al. 2019

J Neurol Neurosurg Psychiatry

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ObjectiveIn 2017, the diagnostic criteria for cognitive and behavioural impairment in amyotrophic lateral sclerosis (ALS) with frontotemporal dementia (ALSFTD-1) have been modified (ALSFTD-2) with the inclusion of a novel category (ALS with combined cognitive and behavioural impairment, ALScbi) and with changes of operational criteria of the other categories (ALS with cognitive impairment (ALSci), ALS with behavioural impairment (ALSbi) and ALS with frontotemporal dementia (ALS-FTD)). We compared the two sets of criteria to assess the effect of the revised criteria on the cognitive classification of patients with ALS.MethodsTwo cohorts of patients with ALS were included in this study: a population-based cohort including patients identified through the Piemonte/Valle d’Aosta register for ALS in the 2014–2017 period (n=321), and a referral cohort recruited at the Turin ALS centre and at the ALS centre of the Maugeri Institute in Milan in the same period (n=205). Cognitive function was classified in blind by two neuropsychologists expert in ALS.ResultsALSFTD-2 criteria determined a shift of about 15% of patients from their original category to a new one. In both cohorts, about 9% of patients were reclassified to the novel category ALScbi. Among patients previously classified as cognitively normal, 14 (4.3%, population-based cohort) and 19 (9.3%, referral cohort) were reclassified as ALSbi or ALSci. The median survival of the different categories was significantly different with both with sets of criteria.ConclusionsThe new ALSFTD-2 criteria, compared with the old ones, have positive effects on the clinical practice being more sensitive to the early cognitive impairment and having a better prognostic yield.

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Section: Discussionmentioning

confidence: 99%

Validation of the revised classification of cognitive and behavioural impairment in ALS

Iazzolino

Pain

Peotta

et al. 2019

J Neurol Neurosurg Psychiatry

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“…[6][7][8][9] Such convergent evidence supports the notion of a spectrum or continuum of disease. 3 10 On the other hand, some authors 11 have reported distinct cognitive profiles in ALS and ALS-FTD, and explicitly argue against the notion of a continuum. Moreover, while FTD-ALS is pathologically homogenous, invariably being associated with TDP-43 pathology, 5 half of FTD cases without ALS have alternative pathologies: tau or fused-in-sarcoma.…”

Section: Introductionmentioning

confidence: 99%

Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis

Saxon

Thompson

Harris

et al. 2020

J Neurol Neurosurg Psychiatry

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ObjectiveThe precise relationship between frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) is incompletely understood. The association has been described as a continuum, yet data suggest that this may be an oversimplification. Direct comparisons between patients who have behavioural variant FTD (bvFTD) with and without ALS are rare. This prospective comparative study aimed to determine whether there are phenotypic differences in cognition and behaviour between patients with FTD-ALS and bvFTD alone.MethodsPatients with bvFTD or FTD-ALS and healthy controls underwent neuropsychological testing, focusing on language, executive functions and social cognition. Behavioural change was measured through caregiver interview. Blood samples were screened for known FTD genes.Results23 bvFTD, 20 FTD-ALS and 30 controls participated. On cognitive tests, highly significant differences were elicited between patients and controls, confirming the tests’ sensitivities to FTD. bvFTD and FTD-ALS groups performed similarly, although with slightly greater difficulty in patients with ALS-FTD on category fluency and a sentence-ordering task that assesses grammar production. Patients with bvFTD demonstrated more widespread behavioural change, with more frequent disinhibition, impulsivity, loss of empathy and repetitive behaviours. Behaviour in FTD-ALS was dominated by apathy. The C9ORF72 repeat expansion was associated with poorer performance on language-related tasks.ConclusionsDifferences were elicited in cognition and behaviour between bvFTD and FTD-ALS, and patients carrying the C9ORF72 repeat expansion. The findings, which raise the possibility of phenotypic variation between bvFTD and FTD-ALS, have clinical implications for early detection of FTD-ALS and theoretical implications for the nature of the relationship between FTD and ALS.

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“…Cognitive function may be impaired in up to 50% of ALS patients in various degrees up to full blown (mostly behavioural variant) frontotemporal dementia (5%–15% of cases); in the former patients, about half of them show deficits in one cognitive domain only, for example, in executive, language or fluency function 3. The pattern of neurocognitive changes in ALS has been described consistently in several studies but it is poorly understood how these cognitive changes are associated with brain pathology in ALS.…”

Section: The Thought-provoking Hypothesis Has Been Empirically Testedmentioning

confidence: 99%

In vivo tracking of TDP43 in ALS: cognition as a new biomarker for brain pathology

Lulé

Ludolph

2019

J Neurol Neurosurg Psychiatry

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

Cited by 27 publications

References 15 publications

Validation of the revised classification of cognitive and behavioural impairment in ALS

Validation of the revised classification of cognitive and behavioural impairment in ALS

Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis

In vivo tracking of TDP43 in ALS: cognition as a new biomarker for brain pathology

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