scholarly journals Disease progression models of familial frontotemporal lobar degeneration and the temporal ordering of biomarker changes in an international cohort

2021 ◽  
Vol 17 (S5) ◽  
Author(s):  
Adam M. Staffaroni ◽  
Melanie Quintana ◽  
Barbara Wendelberger ◽  
Lucy L. Russell ◽  
Leonard Petrucelli ◽  
...  
Keyword(s):  
Disease Progression ◽  
Frontotemporal Lobar Degeneration ◽  
Temporal Ordering

IC-P2-116: Longitudinal atrophy rate as a biomarker of disease progression in frontotemporal lobar degeneration

2008 ◽  
Vol 4 ◽  
pp. T53-T54
Author(s):  
Elizabeth McNaught ◽  
Jonathan D. Rohrer ◽  
Rohani Omar ◽  
Laila Ahsan ◽  
Martin N. Rossor ◽  
...  
Keyword(s):  
Disease Progression ◽  
Frontotemporal Lobar Degeneration ◽  
Atrophy Rate

scholarly journals Peripheral inflammatory markers and clinical correlations in patients with frontotemporal lobar degeneration with and without the C9orf72 repeat expansion

2019 ◽  
Vol 267 (1) ◽  
pp. 76-86 ◽  
Author(s):  
Kasper Katisko ◽  
Eino Solje ◽  
Paula Korhonen ◽  
Olli Jääskeläinen ◽  
Sanna Loppi ◽  
...  
Keyword(s):  
Disease Progression ◽  
Frontotemporal Lobar Degeneration ◽  
Inflammatory Markers ◽  
Repeat Expansion ◽  
Rapid Disease Progression ◽  
Longitudinal Measurements ◽  
Inflammatory Profile ◽  
Hs Crp ◽  
And Gender ◽  
Inflammatory Changes

Abstract In this study, our aim was to evaluate potential peripheral inflammatory changes in frontotemporal lobar degeneration (FTLD) patients carrying or not the C9orf72 repeat expansion. To this end, levels of several inflammatory markers (MCP-1, RANTES, IL-10, IL-17A, IL-12p, IFN-γ, IL-1β, IL-8, and hs-CRP) and blood cells counts in plasma and/or serum of FTLD patients (N = 98) with or without the C9orf72 repeat expansion were analyzed. In addition, we evaluated whether the analyzed peripheral inflammatory markers correlated with disease progression or distinct clinical phenotypes under the heterogenous FTLD spectrum. Elevated levels of pro-inflammatory RANTES or MCP-1 and decreased levels of anti-inflammatory IL-10 were found to associate with Parkinsonism and a more rapid disease progression, indicated by longitudinal measurements of either MMSE or ADCS-ADL decline. These findings were observed in the total cohort in general, whereas the C9orf72 repeat expansion carriers showed only slight differences in IL-10 and hemoglobin levels compared to non-carriers. Furthermore, these C9orf72 repeat expansion-associated differences were observed mostly in male subjects. The females in general showed elevated levels of several pro-inflammatory markers compared to males regardless of the C9orf72 genotype. Our study suggests that pro-inflammatory changes observed in the early symptomatic phase of FTLD are associated with distinct clinical profiles and a more rapid disease progression, and that the C9orf72 repeat expansion and gender may also affect the inflammatory profile in FTLD.

scholarly journals Plasma Neurofilament Light for Prediction of Disease Progression in Familial Frontotemporal Lobar Degeneration

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000011848
Author(s):  
Julio C. Rojas ◽  
Ping Wang ◽  
Adam M. Staffaroni ◽  
Carolin Heller ◽  
Yann Cobigo ◽  
...  
Keyword(s):  
Disease Progression ◽  
Disease Severity ◽  
Frontotemporal Lobar Degeneration ◽  
Short Term ◽  
Neurofilament Light Chain ◽  
Mixed Effect ◽  
Neurofilament Light ◽  
Asymptomatic Carriers ◽  
Mutation Carriers ◽  
Risk Of Disease

Objective:We tested the hypothesis that plasma neurofilament light chain (NfL) identifies asymptomatic carriers of familial frontotemporal lobar degeneration (FTLD)-causing mutations at risk of disease progression.Methods:Baseline plasma NfL concentrations were measured with Simoa in original (n = 277) and validation (n = 297) cohorts. C9orf72, GRN and MAPT mutation carriers and non-carriers from the same families were classified by disease severity [asymptomatic, prodromal and full phenotype] using the CDR® Dementia Staging Instrument plus behavior and language domains from the National Alzheimer’s Disease Coordinating Center FTLD module (CDR®+NACC-FTLD). Linear mixed effect models related NfL to clinical variables.Results:In both cohorts, baseline NfL was higher in asymptomatic mutation carriers who showed phenoconversion or disease progression compared to non-progressors (original: 11.4 ± 7 pg/mL vs. 6.7 ± 5 pg/mL, p = 0.002; validation: 14.1 ± 12 pg/mL vs. 8.7 ± 6 pg/mL, p = 0.035). Plasma NfL discriminated symptomatic from asymptomatic mutation carriers or prodromal disease (original cutoff: 13.6 pg/mL, 87.5% sensitivity, 82.7% specificity; validation cutoff: 19.8 pg/mL, 87.4% sensitivity, 84.3% specificity). Higher baseline NfL correlated with worse longitudinal CDR®+NACC-FTLD sum of boxes scores, neuropsychological function and atrophy, regardless of genotype or disease severity, including asymptomatic mutation carriers.Conclusions:Plasma NfL identifies asymptomatic carriers of FTLD-causing mutations at short-term risk of disease progression, and is a potential tool to select participants for prevention clinical trials.Classification of evidence:This study provides Class I evidence that in carriers of FTLD-causing mutations, elevation of plasma NfL predicts short-term risk of clinical progression.

scholarly journals Measuring disease progression in frontotemporal lobar degeneration: A clinical and MRI study

Neurology ◽  
2010 ◽  
Vol 74 (8) ◽  
pp. 666-673 ◽  
Author(s):  
E. Gordon ◽  
J. D. Rohrer ◽  
L. G. Kim ◽  
R. Omar ◽  
M. N. Rossor ◽  
...  
Keyword(s):  
Disease Progression ◽  
Frontotemporal Lobar Degeneration ◽  
Mri Study

scholarly journals TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates

2018 ◽  
Vol 22 (1) ◽  
pp. 65-77 ◽  
Author(s):  
Florent Laferrière ◽  
Zuzanna Maniecka ◽  
Manuela Pérez-Berlanga ◽  
Marian Hruska-Plochan ◽  
Larissa Gilhespy ◽  
...  
Keyword(s):  
Disease Progression ◽  
Frontotemporal Lobar Degeneration ◽  
Neurotoxic Effects

P2-189: Longitudinal atrophy rate as a biomarker of disease progression in frontotemporal lobar degeneration

2008 ◽  
Vol 4 ◽  
pp. T426-T426
Author(s):  
Elizabeth McNaught ◽  
Jonathan D. Rohrer ◽  
Rohani Omar ◽  
Laila Ahsan ◽  
Martin N. Rossor ◽  
...  
Keyword(s):  
Disease Progression ◽  
Frontotemporal Lobar Degeneration ◽  
Atrophy Rate

Frontotemporal Lobar Degeneration: Characterizing Semantic Binding and Abstracted Meaning Abilities

2009 ◽  
Vol 19 (4) ◽  
pp. 117-125 ◽  
Author(s):  
Raksha Anand ◽  
John Hart ◽  
Patricia S. Moore ◽  
Sandra B. Chapman
Keyword(s):  
Frontotemporal Lobar Degeneration ◽  
Assessment Tools ◽  
Progressive Decline ◽  
Cognitively Normal ◽  
Nonfluent Aphasia ◽  
Traditional Assessment ◽  
Language Measures ◽  
Semantic Object ◽  
Semantic Problem ◽  
Progressive Nonfluent Aphasia

Abstract Purpose: Frontotemporal lobar degeneration (FTLD) encompasses a group of neurodegenerative disorders characterized by gradual and progressive decline in behavior and/or language. Identifying the subtypes of FTLD can be challenging with traditional assessment tools. Growing empirical evidence suggests that language measures might be useful in differentiating FTLD subtypes. Method: In this paper, we examined the performance of five individuals with FTLD (two with frontotemporal dementia, two with semantic dementia, and one with progressive nonfluent aphasia) and 10 cognitively normal older adults on measures of semantic binding (Semantic Object Retrieval Test and semantic problem solving) and abstracted meaning (generation of interpretive statement and proverb interpretation). Results and Conclusion: A differential profile of impairment was observed in the three FTLD subtypes on these four measures. Further examination of these measures in larger groups will establish their clinical utility in differentiating the FTLD subtypes.

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