scholarly journals Cerebrospinal fluid biomarkers and cognitive status in differential diagnosis of frontotemporal dementia and Alzheimer’s disease

2019 ◽  
Vol 47 (10) ◽  
pp. 4968-4980 ◽  
Author(s):  
Tiziana Casoli ◽  
Susy Paolini ◽  
Paolo Fabbietti ◽  
Patrizia Fattoretti ◽  
Lucia Paciaroni ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cerebrospinal Fluid ◽  
Frontotemporal Dementia ◽  
Primary Progressive Aphasia ◽  
Csf Biomarkers ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
T Tau ◽  
Domain Scores

Objective This study aimed to determine the most appropriate cognitive and cerebrospinal fluid (CSF) biomarker setting to distinguish frontotemporal dementia (FTD) from Alzheimer’s disease (AD). Method Patients with FTD, those with AD, and those without dementia were enrolled in this study. CSF amyloid-ß 42 (Aß42), total (t)-tau, and phosphorylated (p)-tau concentrations were determined by enzyme-linked immunosorbent assays. Cognition was evaluated by the Mini-Mental State Examination (MMSE) and its domain scores. The associations of CSF biomarkers with cognitive measures were examined using regression models and the diagnostic value of CSF biomarkers was determined by receiver operating characteristics curves. Results CSF Aß42 levels were lower, whereas t-tau/Aß42 and p-tau/Aß42 ratios were higher in patients with AD compared with those with FTD. Some MMSE domain scores were different in FTD and AD, but they did not improve the ability to distinguish between the two pathologies. Poor temporal orientation scores were associated with low Aß42 levels only in patients with FTD. The p-tau/Aß42 ratio reached sufficient levels of sensitivity and specificity to discriminate FTD with primary progressive aphasia from AD. Conclusions The ratio of CSF p-tau/Aß42 is a sensitive and specific biomarker for discriminating patients with primary progressive aphasia from those with AD.

scholarly journals Parietal Involvement in the Semantic Variant of Primary Progressive Aphasia with Alzheimer’s Disease Cerebrospinal Fluid Profile

2018 ◽  
Vol 66 (1) ◽  
pp. 271-280 ◽  
Author(s):  
Géraldine Bera ◽  
Raffaella Migliaccio ◽  
Thibaut Michelin ◽  
Foudil Lamari ◽  
Sophie Ferrieux ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cerebrospinal Fluid ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

scholarly journals Laughter as a Paradigm of Socio-emotional Signal Processing in Dementia

2020 ◽  
Author(s):  
Harri Sivasathiaseelan ◽  
Charles R Marshall ◽  
Elia Benhamou ◽  
Janneke EP van Leeuwen ◽  
Rebecca L Bond ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Signal Processing ◽  
Frontotemporal Dementia ◽  
Primary Progressive Aphasia ◽  
Identification Accuracy ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Emotional Dysfunction ◽  
Semantic Variant

Abstract Background: Laughter is a fundamental communicative signal in our relations with other people and is used to convey a diverse repertoire of social and emotional information. It is therefore potentially a useful probe of impaired socio-emotional signal processing in neurodegenerative diseases. Here we investigated the cognitive and affective processing of laughter systematically in patients representing all major syndromes of frontotemporal dementia, a disease spectrum characterised by severe socio-emotional dysfunction, as well as typical amnestic Alzheimer’s disease in relation to healthy age-matched individuals. Methods: We assessed cognitive labelling and valence rating of samples of spontaneous (mirthful and hostile) and volitional (posed) laughter versus two auditory control conditions (a synthetic laughter-like stimulus and spoken numbers) in 47 patients with frontotemporal dementia (22 with behavioural variant frontotemporal dementia, 12 with semantic variant primary progressive aphasia and 13 with nonfluent-agrammatic primary progressive aphasia), 15 patients with typical amnestic Alzheimer’s disease and 20 healthy age-matched individuals. Neuroanatomical associations of laughter processing were assessed using voxel-based morphometry of patients’ brain MR images. Results: While all dementia syndromes were associated with impaired identification of laughter subtypes relative to healthy controls, this was significantly more severe overall in frontotemporal dementia than in Alzheimer’s disease and particularly in the behavioural and semantic variants, which also showed abnormal affective evaluation of laughter. Certain striking syndromic signatures emerged, including enhanced liking for hostile laughter in behavioural variant frontotemporal dementia, impaired processing of synthetic laughter in the nonfluent-agrammatic variant (consistent with a generic complex auditory perceptual deficit) and ‘numerophilia’ in the semantic variant. Across the patient cohort, overall laughter identification accuracy correlated with regional grey matter in a core network encompassing inferior frontal and cingulo-insular cortices; and more specific correlates of laughter identification accuracy were delineated in cortical regions mediating affective disambiguation (identification of hostile and posed laughter in orbitofrontal cortex) and authenticity (social intent) decoding (identification of mirthful and posed laughter in anteromedial prefrontal cortex).Conclusions: These findings reveal a rich diversity of cognitive and affective laughter phenotypes in canonical dementia syndromes and suggest that laughter is an informative probe of neural mechanisms underpinning socio-emotional dysfunction in neurodegenerative disease.

scholarly journals A clinical-radiological framework of the right temporal variant of frontotemporal dementia

Brain ◽  
2020 ◽  
Vol 143 (9) ◽  
pp. 2831-2843 ◽  
Author(s):  
Hulya Ulugut Erkoyun ◽  
Colin Groot ◽  
Ronja Heilbron ◽  
Anne Nelissen ◽  
Jonathan van Rossum ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Diagnostic Criteria ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Behavioural Changes ◽  
The Right ◽  
Mental Slowness

Abstract The concept of the right temporal variant of frontotemporal dementia (rtvFTD) is still equivocal. The syndrome accompanying predominant right anterior temporal atrophy has previously been described as memory loss, prosopagnosia, getting lost and behavioural changes. Accurate detection is challenging, as the clinical syndrome might be confused with either behavioural variant FTD (bvFTD) or Alzheimer’s disease. Furthermore, based on neuroimaging features, the syndrome has been considered a right-sided variant of semantic variant primary progressive aphasia (svPPA). Therefore, we aimed to demarcate the clinical and neuropsychological characteristics of rtvFTD versus svPPA, bvFTD and Alzheimer’s disease. Moreover, we aimed to compare its neuroimaging profile against svPPA, which is associated with predominant left anterior temporal atrophy. Of 619 subjects with a clinical diagnosis of frontotemporal dementia or primary progressive aphasia, we included 70 subjects with a negative amyloid status in whom predominant right temporal lobar atrophy was identified based on blinded visual assessment of their initial brain MRI scans. Clinical symptoms were assessed retrospectively and compared with age- and sex-matched patients with svPPA (n = 70), bvFTD (n = 70) and Alzheimer’s disease (n = 70). Prosopagnosia, episodic memory impairment and behavioural changes such as disinhibition, apathy, compulsiveness and loss of empathy were the most common initial symptoms, whereas during the disease course, patients developed language problems such as word-finding difficulties and anomia. Distinctive symptoms of rtvFTD compared to the other groups included depression, somatic complaints, and motor/mental slowness. Aside from right temporal atrophy, the imaging pattern showed volume loss of the right ventral frontal area and the left temporal lobe, which represented a close mirror image of svPPA. Atrophy of the bilateral temporal poles and the fusiform gyrus were associated with prosopagnosia in rtvFTD. Our results highlight that rtvFTD has a unique clinical presentation. Since current diagnostic criteria do not cover specific symptoms of the rtvFTD, we propose a diagnostic tree to be used to define diagnostic criteria and call for an international validation.

Clinical Characteristic in Primary Progressive Aphasia in Relation to Alzheimer’s Disease Biomarkers

2021 ◽  
pp. 1-13
Author(s):  
Sung Hoon Kang ◽  
Hanna Cho ◽  
Jiho Shin ◽  
Hang-Rai Kim ◽  
Young Noh ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Language Impairment ◽  
Amyloid Β ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Clinical Feature ◽  
Progressive Aphasia ◽  
Disease Biomarkers ◽  
Primary Progressive

Background: Primary progressive aphasia (PPA) is associated with amyloid-β (Aβ) pathology. However, clinical feature of PPA based on Aβ positivity remains unclear. Objective: We aimed to assess the prevalence of Aβ positivity in patients with PPA and compare the clinical characteristics of patients with Aβ-positive (A+) and Aβ-negative (A–) PPA. Further, we applied Aβ and tau classification system (AT system) in patients with PPA for whom additional information of in vivo tau biomarker was available. Methods: We recruited 110 patients with PPA (41 semantic [svPPA], 27 non-fluent [nfvPPA], 32 logopenic [lvPPA], and 10 unclassified [ucPPA]) who underwent Aβ-PET imaging at multi centers. The extent of language impairment and cortical atrophy were compared between the A+ and A–PPA subgroups using general linear models. Results: The prevalence of Aβ positivity was highest in patients with lvPPA (81.3%), followed by ucPPA (60.0%), nfvPPA (18.5%), and svPPA (9.8%). The A+ PPA subgroup manifested cortical atrophy mainly in the left superior temporal/inferior parietal regions and had lower repetition scores compared to the A–PPA subgroup. Further, we observed that more than 90%(13/14) of the patients with A+ PPA had tau deposition. Conclusion: Our findings will help clinicians understand the patterns of language impairment and cortical atrophy in patients with PPA based on Aβ deposition. Considering that most of the A+ PPA patents are tau positive, understanding the influence of Alzheimer’s disease biomarkers on PPA might provide an opportunity for these patients to participate in clinical trials aimed for treating atypical Alzheimer’s disease.

O2-14-01: HETEROGENEOUS LANGUAGE PROFILES IN PATIENTS WITH PRIMARY PROGRESSIVE APHASIA DUE TO ALZHEIMER'S DISEASE

2014 ◽  
Vol 10 ◽  
pp. P196-P197
Author(s):  
Antoinette M. Keulen ◽  
Eva Louwersheimer ◽  
L.C. Jiskoot ◽  
John C. van Swieten ◽  
Yolande A.L. Pijnenburg
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Profiles
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