A modified Camel and Cactus Test detects presymptomatic semantic impairment in genetic frontotemporal dementia within the GENFI cohort

Moore, Katrina; Convery, Rhian S.; Bocchetta, Martina; Neason, Mollie; Cash, David M.; Greaves, Caroline; Russell, Lucy L.; Clarke, Mica T. M.; Peakman, Georgia; Swieten, John van; Jiskoot, Lize C.; Moreno, Fermín; Barandiarán, Myriam; Sánchez‐Valle, Raquel; Borroni, Barbara; Laforce, Robert; Doré, Marie-Claire; Masellis, Mario; Tartaglia, Maria Carmela; Graff, Caroline; Galimberti, Daniela; Rowe, James B.; Finger, Elizabeth; Synofzik, Matthis; Karnath, Hans‐Otto; Vandenberghe, Rik; Maruta, Carolina; Tagliavini, Fabrizio; Santana, Isabel; Ducharme, Simon; Butler, Chris; Gerhard, Alex; Xiong, Chengjie; Danek, Adrian; Otto, Markus; Warren, Jason D.; Rohrer, Jonathan D.; Initiative, Genfi Genetic Ftd

doi:10.1080/23279095.2020.1716357

Cited by 27 publications

(34 citation statements)

References 24 publications

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“…An example of specific C9orf72-related impairment is the presence, in pre-symptomatic carriers, of deficits in cognitive inhibition (i.e., the ability to resist to interference from irrelevant stimuli) which seems to become more evident in individuals who are closer to symptom onset [40]. Some other studies have shown the presence of deficits in semantic access [41] as well as early involvement of praxis, which has been observed already in subjects younger than 40 years of age, supporting the hypothesis that frontal and prefrontal structures are early involved in degeneration [42]. Deficits in verbal fluency are also well recognized.…”

Section: Clinical Spectrum Of C9orf72-related Als-ftd and Perspective...mentioning

confidence: 99%

Biomarkers for C9orf7-ALS in Symptomatic and Pre-symptomatic Patients: State-of-the-art in the New Era of Clinical Trials

Querin

Biferi

2022

JND

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The development of new possible treatments for C9orf72-related ALS and the possibility of early identification of subjects genetically at risk of developing the disease is creating a critical need for biomarkers to track neurodegeneration that could be used as outcome measures in clinical trials. Current candidate biomarkers in C9orf72-ALS include neuropsychology tests, imaging, electrophysiology as well as different circulating biomarkers. Neuropsychology tests show early executive and verbal function involvement both in symptomatic and asymptomatic mutation carriers. At brain MRI, C9orf72-ALS patients present diffuse white and grey matter degeneration, which are already identified up to 20 years before symptom onset and that seem to be slowly progressive over time, while regions of altered connectivity at fMRI and of hypometabolism at [18F]FDG PET have been described as well. At the same time, spinal cord MRI has also shown progressive decrease of FA in the cortico-spinal tract over time. On the side of wet biomarkers, neurofilament proteins are increased both in the CSF and serum just before symptom onset and tend to slowly increase over time, while poly(GP) protein can be detected in the CSF and probably used as target engagement marker in clinical trials.

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Section: Clinical Spectrum Of C9orf72-related Als-ftd and Perspective...mentioning

confidence: 99%

Biomarkers for C9orf7-ALS in Symptomatic and Pre-symptomatic Patients: State-of-the-art in the New Era of Clinical Trials

Querin

Biferi

2022

JND

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“…It is therefore likely that the more extensive linguistic de cits seen in this group relate to the wider involvement of key linguistic regions in the brain as the disease progresses in addition to a contribution from executive dysfunction. As mentioned above, this latter cognitive de cit is likely to be a factor in a number of apparent language problems [32], including the decreased score in the mCCT, which has previously been shown to be related to the executive control of semantic knowledge [20].…”

Section: Discussionmentioning

confidence: 88%

Language Impairment in the Genetic Forms of Behavioural Variant Frontotemporal Dementia

Samra

MacDougall

Bouzigues

et al. 2022

Preprint

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Background Behavioural variant frontotemporal dementia (bvFTD) is characterised by a progressive change in personality in association with atrophy of the frontal and temporal lobes. Whilst language impairment has been described in people with bvFTD, little is currently known about the extent or type of linguistic difficulties that occur, particularly in the genetic forms. Methods Participants with genetic bvFTD along with healthy controls were recruited from the international multicentre Genetic FTD Initiative (GENFI) study. Linguistic symptoms were assessed using items from the Progressive Aphasia Severity Scale (PASS). Additionally, participants undertook the Boston Naming Test (BNT), modified Camel and Cactus Test (mCCT) and a test of category fluency. Participants also underwent a 3T volumetric T1-weighted MRI, with language network regional brain volumes measured and compared between the genetic groups and controls. Results 76% of the genetic bvFTD cohort had impairment in at least one language symptom: 83% C9orf72, 80% MAPT and 56% GRN mutation carriers. All three genetic groups had significantly impaired functional communication, decreased fluency, and impaired sentence comprehension. C9orf72 mutation carriers also had significantly impaired articulation and word retrieval as well as dysgraphia whilst the MAPT mutation group also had impaired word retrieval and single word comprehension. All three groups had difficulties with naming, semantic knowledge and verbal fluency. Atrophy in key left perisylvian language regions differed between the groups, with more generalized involvement in the C9orf72 group and more focal temporal and insula involvement in the other groups. Correlates of language symptoms and test scores also differed between the groups. Conclusions Language deficits exist in a substantial proportion of people with familial bvFTD across all three genetic groups. Significant atrophy is seen in the dominant perisylvian language areas and correlates with language impairments within each of the genetic groups. Improved understanding of the language phenotype in the main genetic bvFTD subtypes will be helpful in future studies, particularly in clinical trials where accurate stratification and monitoring of disease progression is required.

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“…When developing semantic tasks for this study, we aimed to advance the currently available clinical tasks by using standardized photographs (BOSS). A similar development has recently been made to the Camel and Cactus Test (modified CCT; Moore et al, 2020) and the Nombela 2.0 semantic battery (Moreno-Mart ınez & Rodr ıguez-Rojo, 2015). To further develop these semantic tasks for clinical use, the psychometric properties of the task battery should be studied (e.g.…”

Section: Discussionmentioning

confidence: 96%

“…face validity, inter-rater reliability, test-retest reliability, internal consistency). Additionally, as suggested by Moore et al (2020) and Ohman et al (2020), the number of tasks and the number of items in each task should be reviewed in order to find the most effective ways to assess semantic functions in a clinical setting.…”

Section: Discussionmentioning

confidence: 99%

Factor structure and clinical applicability of new semantic tasks in Alzheimer’s disease and aphasia

Luotonen

Karrasch

Korpilahti

et al. 2021

Applied Neuropsychology: Adult

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Semantic tasks are frequently used when examining language functions in patients with acquired disorders such as Alzheimer's disease (AD) and aphasia. Little is known about the possible covariation between different types of tasks or their factor structure in healthy adults. Additionally, few studies have examined semantic task performances in different patient groups. The aims of this data-driven study were to examine the factor structure in a wide range of semantic tasks in healthy older adults, the possible differences in factor variables between healthy controls, patients with AD and patients with stroke aphasia, as well as the clinical applicability of tasks in differentiating the two patient groups from controls. Participants included 59 healthy older adults, 13 patients with AD and 14 patients with aphasia. The results indicated a four-factor solution for the semantic task variables: (1) the Semantic association factor, (2) the Time factor, (3) the Verbal factor and ( 4) the Synonym factor. The Verbal factor was the only distinguishing factor between the two patient groups. Three factors reliably discriminated between the controls and the AD patients, and the Verbal factor reliably discriminated between the controls and the aphasia patients. In addition, a few single task variables showed outstanding discrimination for both patient groups. This study supports the notions of semantic tasks tapping into more than one cognitive subcomponent and a more general semantic impairment in AD than in aphasia. In clinical assessment, choosing appropriate semantic tasks is crucial in order to reliably detect the characteristics of the impairment.

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A modified Camel and Cactus Test detects presymptomatic semantic impairment in genetic frontotemporal dementia within the GENFI cohort

Cited by 27 publications

References 24 publications

Biomarkers for C9orf7-ALS in Symptomatic and Pre-symptomatic Patients: State-of-the-art in the New Era of Clinical Trials

Biomarkers for C9orf7-ALS in Symptomatic and Pre-symptomatic Patients: State-of-the-art in the New Era of Clinical Trials

Language Impairment in the Genetic Forms of Behavioural Variant Frontotemporal Dementia

Factor structure and clinical applicability of new semantic tasks in Alzheimer’s disease and aphasia

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