Examining the language and behavioural profile in FTD and ALS-FTD

Saxon, Jennifer A.; Thompson, James C.; Jones, Matthew; Harris, Jennifer; Richardson, Anna; Langheinrich, Tobias; Neary, David; Mann, David; Snowden, Julie S.

doi:10.1136/jnnp-2017-315667

Cited by 51 publications

(65 citation statements)

References 40 publications

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“…The amyotrophic lateral sclerosis (ALS) form of motor neuron disease is known to co-occur with FTD, the association being most common in bvFTD (Saxon et al., 2017). In line with those findings, ALS was present in 14 (20%) of bvFTD patients (10 with limb onset and four with bulbar onset) but none of the SD patients.…”

Section: Resultsmentioning

confidence: 99%

“…There are likely to be other contributory factors to within-group differences. Studies of ALS (Goldstein and Abrahams, 2013, Strong et al., 2017, Taylor et al., 2013) and FTD-ALS (Saxon et al., 2017) have highlighted the importance of language problems as a key part of patients’ cognitive disorder, raising the potential for differences in bvFTD patients with and without ALS. Nevertheless, in this study, the co-occurrence of ALS did not correlate with the magnitude of naming disorder.…”

Section: Discussionmentioning

confidence: 99%

“…There is wide clinical heterogeneity within FTD so that factors influencing naming performance may not pertain equally to all patients. Indeed, in a retrospective study of 185 bvFTD patients (Saxon et al., 2017) impairments in confrontation naming were identified in 54%, suggesting that not all patients are affected. Similarly, in the study of 24 bvFTD patients by Hardy et al.…”

Section: Introductionmentioning

confidence: 99%

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Naming and conceptual understanding in frontotemporal dementia

Snowden

Harris

Saxon

et al. 2019

Cortex

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Behavioural variant frontotemporal dementia (bvFTD) is characterised by behaviour change and impaired executive skills. There is growing evidence that naming difficulties may also be present but the basis for these is unclear. A primary semantic deficit has been proposed, although executive contributions to naming breakdown are also possible. The study aimed to improve understanding of the naming disorder in bvFTD through direct comparison with semantic dementia (SD), and examination of neural correlates. It aimed also to address current controversies about the role of the anterior temporal lobes in semantic memory. We studied 71 bvFTD and 32 SD patients. Naming data were elicited by two picture naming tests (one challenging and one less demanding) and word comprehension by word-picture matching. Structural magnetic resonance images were rated blind using a standardised visual rating scale. Around half of bvFTD patients showed impaired naming and 17% impaired word-picture matching. Deficits in bvFTD were less severe than in SD, but showed a similar pattern. There were strong inverse correlations between naming scores and atrophy in temporal structures, particularly temporal pole and fusiform gyrus. Word comprehension scores correlated more strongly with posterior than anterior temporal lobe atrophy in SD. Error analysis highlighted a significant relationship in both groups between associative-type responses and temporal pole atrophy. By contrast, ‘don't know’ responses, suggesting a loss of conceptual knowledge, correlated with more posterior temporal regions. There was some correlation in bvFTD between naming and executive test performance but not with frontal lobe atrophy. The findings support the view that naming problems can arise in bvFTD independently of patients' ‘frontal’ executive impairment and highlight clinical overlap between bvFTD and SD. We discuss the findings in relation to the hub and spoke model of semantic memory and argue against the notion of an anterior temporal lobe semantic hub.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Naming and conceptual understanding in frontotemporal dementia

Snowden

Harris

Saxon

et al. 2019

Cortex

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“…Another study considered the relative frequencies of FTD phenotypes (bvFTD, semantic dementia and progressive non-fluent aphasia) in a clinical consecutive cohort of patients diagnosed with FTD with or without associated ALS; they concluded that pure language phenotypes (semantic dementia and progressive non-fluent aphasia) occur infrequently in FTD cases with coexistent ALS (16). In another paper, the authors focused on the different symptoms’ profile of bvFTD as opposed to bvFTD with ALS (termed ALS-FTD), the same authors observed a comparatively greater degree of language impairment in the ALS-FTD group (17).…”

Section: Introductionmentioning

confidence: 99%

Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates

Vinceti

Olney

Mandelli

et al. 2019

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Objective: Behavioral variant frontotemporal dementia (bvFTD), is commonly considered the cognitive presentation of the frontotemporal dementia-motor neuron disease (FTD-MND) spectrum disorder. We evaluated the prevalence of primary progressive aphasia in a series of pathologically confirmed cases of FTD-MND spectrum. Methods: Pathologically confirmed cases of frontotemporal lobar degeneration-motor neuron disease (FTLD-MND) were obtained from the UCSF brain bank. Cases were analyzed for presence of language impairment via retrospective chart review of research visits that include neurologic exam, in-depth cognitive testing and magnetic resonance imaging (MRI) imaging. Forty one cases were included. Thirty two were diagnosed with FTD-MND, while nine cases were diagnosed as MND-only from clinical evaluation. Results: Ten FTLD-MND cases (31%) presented with prominent or isolated language involvement consistent with a diagnosis of primary progressive aphasia (PPA), which we called progressive aphasia with motor neuron disease (PA-MND). Of these, three cases that mirrored the non-fluent variant of PPA (nfvPPA) were named nfvPA-MND. The imaging pattern of these nfvPA-MND showed atrophy strictly confined to the frontal and anterior temporal language cortical areas. Another group of seven cases that resembled patients with the semantic variant PPA (svPPA) were named svPA-MND. The group of svPPA-MND on imaging analysis showed selective atrophy of the temporal lobe and orbitofrontal cortex. Conclusions: Language impairment was a frequent phenotype of FTD-MND associated with focal atrophy patterns within the language networks. This data suggest patients with FTD-MND can present quite often with language phenotype of nfvPPA and svPPA, as opposed to exclusive bvFTD symptoms.

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“…However, many FTD patients do initially present with complaints of memory loss and often meet AD consensus criteria (Khachaturian, 2011; Fernandez-Matarrubia et al, 2017). Apart from AD, there is also substantial symptomatic overlap between FTD and MND, with co-occurrence of MND in 12.5–15% of FTD patients (Burrell et al, 2011; Saxon et al, 2017).…”

Section: Clinical Diagnostic Criteria For Ftd: Strengths and Limitationsmentioning

confidence: 99%

The Use of Biomarkers and Genetic Screening to Diagnose Frontotemporal Dementia: Evidence and Clinical Implications

2019

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Within the wide range of neurodegenerative brain diseases, the differential diagnosis of frontotemporal dementia (FTD) frequently poses a challenge. Often, signs and symptoms are not characteristic of the disease and may instead reflect atypical presentations. Consequently, the use of disease biomarkers is of importance to correctly identify the patients. Here, we describe how neuropsychological characteristics, neuroimaging and neurochemical biomarkers and screening for causal gene mutations can be used to differentiate FTD from other neurodegenerative diseases as well as to distinguish between FTD subtypes. Summarizing current evidence, we propose a stepwise approach in the diagnostic evaluation. Clinical consensus criteria that take into account a full neuropsychological examination have relatively good accuracy (sensitivity [se] 75–95%, specificity [sp] 82–95%) to diagnose FTD, although misdiagnosis (mostly AD) is common. Structural brain MRI (se 70–94%, sp 89–99%) and FDG PET (se 47–90%, sp 68–98%) or SPECT (se 36–100%, sp 41–100%) brain scans greatly increase diagnostic accuracy, showing greater involvement of frontal and anterior temporal lobes, with sparing of hippocampi and medial temporal lobes. If these results are inconclusive, we suggest detecting amyloid and tau cerebrospinal fluid (CSF) biomarkers that can indicate the presence of AD with good accuracy (se 74–100%, sp 82–97%). The use of P-tau 181 and the Aβ 1 – 42 /Aβ 1 – 40 ratio significantly increases the accuracy of correctly identifying FTD vs. AD. Alternatively, an amyloid brain PET scan can be performed to differentiate FTD from AD. When autosomal dominant inheritance is suspected, or in early onset dementia, mutation screening of causal genes is indicated and may also be offered to at-risk family members. We have summarized genotype–phenotype correlations for several genes that are known to cause familial frontotemporal lobar degeneration, which is the neuropathological substrate of FTD. The genes most commonly associated with this disease ( C9orf72, MAPT, GRN, TBK1 ) are discussed, as well as some less frequent ones ( CHMP2B, VCP ). Several other techniques, such as diffusion tensor imaging, tau PET imaging and measuring serum neurofilament levels, show promise for future implementation as diagnostic biomarkers.

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Examining the language and behavioural profile in FTD and ALS-FTD

Cited by 51 publications

References 40 publications

Naming and conceptual understanding in frontotemporal dementia

Naming and conceptual understanding in frontotemporal dementia

Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates

The Use of Biomarkers and Genetic Screening to Diagnose Frontotemporal Dementia: Evidence and Clinical Implications

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