scholarly journals Neurofilaments as Emerging Biomarkers of Neuroaxonal Damage to Differentiate Behavioral Frontotemporal Dementia from Primary Psychiatric Disorders: A Systematic Review

Diagnostics ◽  
2021 ◽  
Vol 11 (5) ◽  
pp. 754
Author(s):  
Vincent Davy ◽  
Julien Dumurgier ◽  
Aurore Fayosse ◽  
Claire Paquet ◽  
Emmanuel Cognat
Keyword(s):  
Psychiatric Disorders ◽  
Frontotemporal Dementia ◽  
Frontotemporal Lobar Degeneration ◽  
Social Cognitive ◽  
Functional Neuroimaging ◽  
Short Review ◽  
Clinical Syndrome ◽  
Light Chains ◽  
Neurofilament Light ◽  
Personality Changes

The behavioral variant of frontotemporal dementia (bvFTD) is a clinical syndrome resulting from various causes of neuronal demises associated with frontotemporal lobar degeneration. Symptoms include behavioral and personality changes, social cognitive impairment, and executive function deficits. There is a significant clinical overlap between this syndrome and various primary psychiatric disorders (PPD). Structural and functional neuroimaging are considered helpful to support the diagnosis of bvFTD, but their sensitivity and specificity remain imperfect. There is growing evidence concerning the potential of neurofilaments as biomarkers reflecting axonal and neuronal lesions. Ultrasensitive analytic platforms have recently enabled neurofilament light chains’ (NfL) detection not only from cerebrospinal fluid but also from peripheral blood samples in FTD patients. In this short review, we present recent advances and perspectives for the use of NfL assessments as biomarkers of neuroaxonal damage to differentiate bvFTD from primary psychiatric disorders.

Delusions and Hallucinations as First Symptoms of Frontotemporal Dementia: A Case Report of a 48-Year Old Male

2009 ◽  
Vol 24 (S1) ◽  
pp. 1-1
Author(s):  
R. Malta ◽  
C. Torres ◽  
T. Faria ◽  
S. Almeida
Keyword(s):  
Differential Diagnosis ◽  
Frontotemporal Dementia ◽  
Frontotemporal Lobar Degeneration ◽  
Psychotic Disorders ◽  
Functional Neuroimaging ◽  
Neuropsychiatric Symptoms ◽  
Psychotic Symptoms ◽  
Psychiatric Ward ◽  
Personality Changes ◽  
Behavior Impairment

Frontotemporal dementia (FTD) generally has a presenile onset, behavioral manifestations dominate the clinical picture during which cognitive functions are still relatively intact. Therefore, particularly in the early stages of FTD it is difficult to differentiate this type of dementia from other types of dementia and psychiatric disorders.Although most patients with frontotemporal dementia (FTD) present with neuropsychiatric symptoms, the frequency of psychotic symptoms, assumed to be rare, is still unclear possibly due to limited temporal-limbic involvement in this disorder. We report the case of a 48-year old man admitted in a psychiatric ward with delusions and hallucinations.Significant behavior impairment was present related to social isolation and personality changes. Structural and functional neuroimaging methods disclosed asymmetric frontal and bitemporal damage, mainly on the left. Differential diagnosis consisted of psychotic disorders as well as one of the subtypes of the frontotemporal lobar degeneration group.

scholarly journals Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration

Neurology ◽  
2018 ◽  
Vol 90 (12) ◽  
pp. e1047-e1056 ◽  
Author(s):  
Sang Won Seo ◽  
Marie-Pierre Thibodeau ◽  
David C. Perry ◽  
Alice Hua ◽  
Manu Sidhu ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Symptom Onset ◽  
Frontotemporal Lobar Degeneration ◽  
Age At Onset ◽  
Dna Binding Protein ◽  
Clinical Syndrome ◽  
Early Age ◽  
Consecutive Series ◽  
Argyrophilic Grain Disease ◽  
Argyrophilic Grain

ObjectiveTo examine clinicopathologic correlations in early vs late age at onset frontotemporal dementia (FTD) and frontotemporal lobar degeneration (FTLD).MethodsAll patients were clinically evaluated and prospectively diagnosed at the UCSF Memory and Aging Center. Two consecutive series were included: (1) patients with a clinically diagnosed FTD syndrome who underwent autopsy (cohort 1) and (2) patients with a primary pathologic diagnosis of FTLD, regardless of the clinical syndrome (cohort 2). These series were divided by age at symptom onset (cutoff 65 years).ResultsIn cohort 1, 48 (25.3%) were 65 years or older at symptom onset. Pathologic causes of behavioral variant FTD (bvFTD) were similar in the early age at onset (EO) and late age at onset (LO) bvFTD groups. In corticobasal syndrome (CBS), however, the most common pathologic substrate differed according to age at onset: progressive supranuclear palsy (42.9%) in LO-CBS and Alzheimer disease (AD; 40.7%) in EO-CBS. In cohort 2, 57 (28.4%) were classified as LO-FTLD. Regarding FTLD major molecular classes, FTLD with transactive response DNA-binding protein of 43 kDa was most common in EO-FTLD (44.4%), whereas FTLD-tau (58.3%) was most common in LO-FTLD. Antemortem diagnosis of a non-FTD syndrome, usually AD-type dementia, was more frequent in LO-FTLD than EO-FTLD (19.3% vs 7.7%, p = 0.017). LO-FTLD was also associated with more prevalent comorbid pathologic changes. Of these, moderate to severe AD neuropathologic change and argyrophilic grain disease were overrepresented among patients who received an antemortem diagnosis of AD-type dementia.ConclusionPatients with FTD and FTLD often develop symptoms after age 65, and age at onset represents an important consideration when making antemortem neuropathologic predictions.

Neurofilament light chain as a blood biomarker to differentiate psychiatric disorders from behavioural variant frontotemporal dementia

2019 ◽  
Vol 113 ◽  
pp. 137-140 ◽  
Author(s):  
MHD Rami Al Shweiki ◽  
Petra Steinacker ◽  
Patrick Oeckl ◽  
Bastian Hengerer ◽  
Adrian Danek ◽  
...  
Keyword(s):  
Psychiatric Disorders ◽  
Frontotemporal Dementia ◽  
Light Chain ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Blood Biomarker

scholarly journals Serum neurofilament light chain is a discriminative biomarker between frontotemporal lobar degeneration and primary psychiatric disorders

2019 ◽  
Vol 267 (1) ◽  
pp. 162-167 ◽  
Author(s):  
Kasper Katisko ◽  
Antti Cajanus ◽  
Olli Jääskeläinen ◽  
Aleksi Kontkanen ◽  
Päivi Hartikainen ◽  
...  
Keyword(s):  
Psychiatric Disorders ◽  
Single Molecule ◽  
Light Chain ◽  
Frontotemporal Lobar Degeneration ◽  
Psychotic Disorders ◽  
Late Onset ◽  
Single Subject ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Csf Levels

Abstract Due to the significant clinical overlap between frontotemporal lobar degeneration (FTLD) spectrum disorders and late-onset primary psychiatric disorders (PPD), diagnostic biomarkers reflecting the different underlying pathophysiologies are urgently needed. Thus far, elevated cerebrospinal fluid (CSF) levels of neurofilament light chain (NfL) have been reported in various neurological conditions. Furthermore, recent advancements in ultrasensitive analytical methods (e.g., single molecule array, Simoa) have enabled sensitive and less invasive NfL detection also from blood samples. In this study, we evaluated the potential of serum NfL (sNfL) as a diagnostic tool between FTLD and PPD. We analyzed sNfL levels with Simoa from 125 participants including patients from FTLD (n = 91) and PPD (n = 34) spectra. Our results show that sNfL levels are higher in the FTLD group compared to the PPD group as well as in separate clinical subtypes of FTLD compared to different psychiatric manifestations (i.e., mood or psychotic disorders). At single-subject level, discrimination between FTLD and PPD was possible with 80% sensitivity and 85% specificity (AUC = 0.850, 95% CI 0.776–0.923), and between behavioral variant frontotemporal dementia (bvFTD) and PPD with 79% sensitivity and 85% specificity (AUC = 0.830, 95% CI 0.732–0.908). These findings highlight the potential of sNfL as a discriminating biomarker for FTLD over PPD in patients with wide-ranging behavioral, psychiatric and cognitive symptoms.

scholarly journals The Revised Self-Monitoring Scale detects early impairment of social cognition in genetic frontotemporal dementia within the GENFI cohort

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Hannah D. Franklin ◽  
Lucy L. Russell ◽  
Georgia Peakman ◽  
Caroline V. Greaves ◽  
Martina Bocchetta ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Medial Temporal Lobe ◽  
Social Cognitive ◽  
Early Stage ◽  
Self Monitoring ◽  
Self Presentation ◽  
Genetic Groups ◽  
Mutation Carriers ◽  
Large Patient ◽  
Weighted Magnetic Resonance Imaging

Abstract Background Although social cognitive dysfunction is a major feature of frontotemporal dementia (FTD), it has been poorly studied in familial forms. A key goal of studies is to detect early cognitive impairment using validated measures in large patient cohorts. Methods We used the Revised Self-Monitoring Scale (RSMS) as a measure of socioemotional sensitivity in 730 participants from the genetic FTD initiative (GENFI) observational study: 269 mutation-negative healthy controls, 193 C9orf72 expansion carriers, 193 GRN mutation carriers and 75 MAPT mutation carriers. All participants underwent the standardised GENFI clinical assessment including the ‘CDR® plus NACC FTLD’ scale and RSMS. The RSMS total score and its two subscores, socioemotional expressiveness (EX score) and modification of self-presentation (SP score) were measured. Volumetric T1-weighted magnetic resonance imaging was available from 377 mutation carriers for voxel-based morphometry (VBM) analysis. Results The RSMS was decreased in symptomatic mutation carriers in all genetic groups but at a prodromal stage only in the C9orf72 (for the total score and both subscores) and GRN (for the modification of self-presentation subscore) groups. RSMS score correlated with disease severity in all groups. The VBM analysis implicated an overlapping network of regions including the orbitofrontal cortex, insula, temporal pole, medial temporal lobe and striatum. Conclusions The RSMS indexes socioemotional impairment at an early stage of genetic FTD and may be a suitable outcome measure in forthcoming trials.

scholarly journals ATN incorporating cerebrospinal fluid neurofilament light chain detects frontotemporal lobar degeneration

2020 ◽  
Author(s):  
Katheryn A.Q. Cousins ◽  
Jeffrey S. Phillips ◽  
David J. Irwin ◽  
Edward B. Lee ◽  
David A. Wolk ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Light Chain ◽  
Frontotemporal Lobar Degeneration ◽  
Neurofilament Light Chain ◽  
Neurofilament Light

scholarly journals Functional neuroimaging in psychiatry

1999 ◽  
Vol 354 (1387) ◽  
pp. 1359-1370 ◽  
Author(s):  
Cynthia H. Y. Fu ◽  
Philip K. McGuire
Keyword(s):  
Psychiatric Disorders ◽  
Functional Neuroimaging ◽  
Psychiatric Research ◽  
Potential Contribution ◽  
Powerful Means ◽  
Methodological Approaches

Functional neuroimaging is one of the most powerful means available for investigating the pathophysiology of psychiatric disorders. In this review, we shall focus on the different ways that it can be employed to this end, describing the major findings in the field in the context of different methodological approaches. We will also discuss practical issues that are particular to studying psychiatric disorders and the potential contribution of functional neuroimaging to future psychiatric research.

Apraxia screening predicts Alzheimer pathology in frontotemporal dementia

2018 ◽  
Vol 90 (5) ◽  
pp. 562-569 ◽  
Author(s):  
Matthias Pawlowski ◽  
Viktoria Joksch ◽  
Heinz Wiendl ◽  
Sven G Meuth ◽  
Thomas Duning ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Clinical Presentation ◽  
Neuropsychological Testing ◽  
High Sensitivity ◽  
Clinical Syndrome ◽  
Test Results ◽  
Discriminative Power ◽  
Bedside Test ◽  
Neurodegenerative Dementias ◽  
Alzheimer Pathology

ObjectivesFrontotemporal dementia (FTD) is a heterogeneous clinical syndrome linked to diverse types of underlying neuropathology. Diagnosis is mainly based on clinical presentation and accurate prediction of underlying neuropathology remains difficult.MethodsWe present a large cohort of patients with FTD spectrum diseases (n=84). All patients were thoroughly characterised by cerebrospinal fluid (CSF) Alzheimer’s disease (AD) biomarkers, neuroimaging, neuropsychological testing and standardised apraxia screening.ResultsA potential AD pathology was found in 43% of patients with FTD. CSF AD biomarker levels positively correlated with AD-typical apraxia scores in patients with FTD. The discriminative power of apraxia test results indicative of AD pathology was high (sensitivity: 90%, specificity: 66%).ConclusionsApraxia is common in neurodegenerative dementias but under-represented in clinical workup and diagnostic criteria. Standardised apraxia screening may serve as bedside test to objectify an AD-typical apraxia profile as an early and robust sign of AD pathology in patients with FTD.

scholarly journals Very late-onset behavioral variant frontotemporal dementia

2013 ◽  
Vol 7 (1) ◽  
pp. 136-139
Author(s):  
Henrique Cerqueira Guimarães ◽  
Tatiana de Carvalho Espindola
Keyword(s):  
Case Report ◽  
Frontotemporal Dementia ◽  
Frontotemporal Lobar Degeneration ◽  
Clinical Presentation ◽  
Late Onset ◽  
Ground Level ◽  
Dementia Diagnosis ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Genetically Determined ◽  
Late Onset Dementia

ABSTRACT Current concepts regarding frontotemporal lobar degeneration (FTLD) have evolved rapidly in recent years. Genetically determined FTLD cohorts have broadened our knowledge pertaining to its clinical presentation, neuroimaging findings and demographics. In this study we present a case report of a patient diagnosed with behavioral variant frontotemporal dementia diagnosis in her nineties during hospital admission for a ground-level fall. We believe this case reinforces the pervasive nature of this clinical entity, and may contribute to an increased awareness of this diagnostic possibility in late-onset dementia.

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