|1.||Frontotemporal Lobar Degeneration (Semantic Dementia)
|2.||Alzheimer Disease (Alzheimer's Disease)
|3.||Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
|4.||Neurodegenerative Diseases (Neurodegenerative Disease)
|1.||Trojanowski, John Q: 80 articles (01/2016 - 05/2005)|
|2.||Dickson, Dennis W: 66 articles (10/2015 - 05/2005)|
|3.||Lee, Virginia M-Y: 55 articles (01/2016 - 06/2006)|
|4.||Rademakers, Rosa: 54 articles (10/2015 - 03/2006)|
|5.||Grossman, Murray: 45 articles (01/2016 - 12/2005)|
|6.||Neumann, Manuela: 43 articles (07/2015 - 06/2005)|
|7.||Hasegawa, Masato: 40 articles (08/2015 - 12/2006)|
|8.||Miller, Bruce L: 38 articles (02/2016 - 03/2004)|
|9.||Arai, Tetsuaki: 37 articles (02/2015 - 12/2006)|
|10.||Akiyama, Haruhiko: 37 articles (02/2015 - 12/2006)|
|1.||protein TDP-43 (TDP-43)IBA
07/01/2007 - "Furthermore, the discovery that TDP-43 is a component of the neuronal inclusions seen in the most common neuropathological subtype has also helped expand the biochemical pathways that are the focus of much FTLD research. "
11/01/2015 - "Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43."
11/01/2007 - "Immunohistochemical and biochemical studies of TDP-43 have helped to clarify the relationship between different sub-types of FTLD-U and related conditions. "
04/01/2015 - "The goals of these efforts are to enhance power for clinical trials focused on slowing or preventing progression of spread of tau, TDP-43 and other FTLD-associated pathologies and work toward the goal of defining clinical endophenotypes of FTD."
09/01/2014 - "With this study, we provide further evidence for a putative role of rare mutations in SQSTM1 in the genetic etiology of FTLD and showed that, comparable to other FTLD/ALS genes, SQSTM1 mutations are associated with TDP-43 pathology. "
02/01/2007 - "The purpose of this study was to determine whether there were distinctive clinical and neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with PGRN mutations. "
11/07/2014 - "In conclusion, our LC-MS/MS method may facilitate ubiquitin determination to a broader community and might help to discriminate AD, CJD, and FTLD patients. "
12/15/2013 - "Only one out of nine FTLD-FUS cases showed pathology that corresponds to atypical FTLD with ubiquitin-positive inclusions (aFTLD-U). "
01/01/2013 - "However, test measures did not discriminate between FTLD-tau and FTLD-ubiquitin. "
06/26/2012 - "Mutations in the progranulin gene (GRN) have recently been identified as a major cause of FTLD with ubiquitin positive inclusions (FTLD-U). "
|3.||DNA-Binding Proteins (DNA Binding Protein)IBA
06/01/2013 - "Laminar distribution of the pathological changes in sporadic frontotemporal lobar degeneration with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy: a quantitative study using polynomial curve fitting."
07/26/2011 - "The aim of this study was to compare clinical features and perfusion patterns on SPECT of patients with familial FTLD-TAR DNA binding protein 43 kDa (TDP) and MAPT mutations. "
04/01/2008 - "TAR DNA-binding protein 43 immunohistochemistry reveals extensive neuritic pathology in FTLD-U: a midwest-southwest consortium for FTLD study."
06/01/2015 - "All 4 had unclassifiable FTLD with TAR DNA-binding protein inclusions, with characteristics of both type A and type B. "
01/01/2015 - "A high-ranking candidate to become a diagnostic marker for a major pathological subtype of FTLD is the transactive response DNA binding protein of 43 kDa (TDP-43). "
|4.||Biological Markers (Surrogate Marker)IBA
05/01/2010 - "The clinical and pathological heterogeneity of FTLD poses a significant diagnostic challenge, and in vivo prediction of underlying histopathology can be significantly improved by supplementing the clinical evaluation with genetic tests and emerging biological markers. "
04/01/2012 - "This reviews discusses comprehensively the state-of-the-art of neuroimaging in the study of FTLD spectrum of disorders, and attempts to envisage which will be new neuroimaging biomarkers that could serve as surrogate measures of the underlying pathology. "
01/01/2010 - "These biomarkers may support the accurate diagnosis of the specific diseases causing FTLD, can be useful in assessing efficacy during pharmacological trials, and may help in identifying new molecular targets for treatment approaches. "
11/15/2015 - "Diagnostic accuracy between FTLD and AD was still sub-optimal, with a significant percentage (23%) of FTLD patients, in particularly women, carrying an ApoE-ε4 allele, showing a CSF-AD biomarkers profile. "
07/01/2015 - "Cerebrospinal fluid proteomics and protein biomarkers in frontotemporal lobar degeneration: Current status and future perspectives."
01/01/2013 - "The present study compares the severity of pathological lesions identified with pTDP43 and iTDP43 in a cohort of 14 FTLD-TDP cases, and assesses the accuracy and inter-observer reliability found with either of these antibodies. "
01/01/2013 - "To date, no studies have performed direct comparisons between these TDP43 antibodies to determine if they identify the same FTLD-TDP subtypes. "
10/01/2015 - "Using these antibodies, we have shown that C9orf72 may be involved in nucleocytoplasmic shuttling and this may have relevance to pathophysiology of ALS/FTLD. "
01/01/2013 - "Two commercially available TDP43 antibodies (phosphorylated or pTDP43, non-phosphorylated or iTDP43) are currently in use for the neuropathological classification of FTLD-TDP cases into pathological subtypes. "
01/01/2013 - "Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43."
|6.||Protein Isoforms (Isoforms)IBA
04/01/2006 - "We also observed two patients with sporadic FTLD, but without Pick bodies, in whom the tau pathology comprised only of 4R-tau isoforms. "
04/01/2006 - "Patients with familial FTLD associated with exon 10 N279K, N296H or +16 splice site mutations showed tau pathology characterised by neuronal neurofibrillary tangles (NFT) and glial cell tangles that contained only 4R-tau isoforms, as did the NFT in P301L MAPT mutation. "
04/01/2006 - "Only 3R-tau isoforms were present in Pick bodies in those patients with familial FTLD associated with L266V, Q336R, E342V, K369I or G389R MAPT mutations. "
04/01/2006 - "In all 12 cases of sporadic FTLD where Pick bodies were present, these contained only 3R-tau isoforms. "
02/01/2004 - "However, 33 (60%) of 55 FTLD cases showed no tau pathology in the brain, except for the rare NFTs, composed of a mix of 3-R and 4-R isoforms, in some of the more elderly cases. "
|7.||Fluorodeoxyglucose F18 (Fludeoxyglucose F 18)FDA Link
08/01/2010 - "All affected members of the 3 families developed FTLD after the onset of ALS, confirmed by neuropsychological testing and hypometabolism in frontal associative areas assessed with fludeoxyglucose F 18 positron emission tomography and computed tomography. "
06/30/2013 - "The application of support vector machine classification (SVM) to combined information from magnetic resonance imaging (MRI) and [F18]fluorodeoxyglucose positron emission tomography (FDG-PET) has been shown to improve detection and differentiation of Alzheimer's disease dementia (AD) and frontotemporal lobar degeneration. "
01/01/2013 - "Positron emission tomography with [18F] fluorodeoxyglucose (FDG-PET) plays a well-established role in assisting early detection of frontotemporal lobar degeneration (FTLD). "
03/01/2011 - "To evaluate the cause of diagnostic errors in the visual interpretation of positron emission tomographic scans with fludeoxyglucose F 18 (FDG-PET) in patients with frontotemporal lobar degeneration (FTLD) and patients with Alzheimer disease (AD). "
01/01/2011 - "Patients with clinically diagnosed Alzheimer's disease (AD: n = 21), with frontotemporal lobar degeneration (FTLD: n = 14) and control subjects (n = 13) underwent both [F18]fluorodeoxyglucose positron emission tomography (FDG-PET) scanning and magnetic resonance imaging (MRI), together with clinical and behavioral assessment. "
|8.||pemetrexed (MTA)FDA Link
03/01/2012 - "Adding the PA to the MTA scale improved discrimination of AD from FTLD, and early-onset AD from normal aging. "
03/01/2012 - "In this study, 2 visual rating scales measuring MTA and PA were used to compare atrophy patterns in 62 pathologically-confirmed AD and 40 FTLD patients. "
12/01/2012 - "Episodic memory impairment and MTA are also found in semantic dementia (SD) and in right temporal lobe atrophy (RTLA), the temporal variants of frontotemporal dementia, but their relationship is unclear. "
03/01/2012 - "Medial temporal lobe atrophy (MTA) is a recognized marker of Alzheimer's disease (AD), however, it can be prominent in frontotemporal lobar degeneration (FTLD). "
|9.||Fluvoxamine (Luvox)FDA LinkGeneric
05/01/2012 - "Our results provide further support to the notion that ATXN2 associated polyglutamine amplification is specific to the ALS-end of the FTLD-ALS disease spectrum."
01/01/2006 - "We find no evidence to suggest that autosomal dominant FTLD-U with NII is a polyglutamine expansion disease."
01/01/2006 - "The genetic basis of familial FTLD-U is currently not known, however the presence of NII suggests that a subset of cases may represent a polyglutamine expansion disease. "
05/01/2012 - "In contrast, in a Flanders-Belgian series of 270 FTLD and 22 FTLD-ALS patients, we found no association with intermediate-length polyQ expansions nor did we observe patient-specific long expansions in agreement with the recent observation in a screening of a substantial sized cohort of patients with diverse neurodegenerative brain diseases. "
04/01/2011 - "Mutations in TLS are responsible for familial amyotrophic lateral sclerosis (ALS) type 6. Furthermore, TLS-containing intracellular inclusions are found in polyglutamine diseases, sporadic ALS, non-SOD1 familial ALS and a subset of frontotemporal lobar degeneration, indicating a pathological significance of TLS in a wide variety of neurodegenerative diseases. "
01/01/2013 - "Detecting an ongoing AD pathological process in FTD has several implications for defining distinctive treatment approaches, guiding genetic screening, and helping in patient selection in future clinical trials in both FTLD and AD therapeutics."
12/01/2015 - "Therefore, TAU58/2 mice recapitulate aspects of human FTLD-tau and AD pathology, and will become instrumental in studying disease mechanisms and therapeutics in the future."
01/01/2010 - "This review focuses on the current understanding of the molecular neuropathology of FTLD, and their relevance to the development of the therapeutics."
08/01/2008 - "It is anticipated that these discoveries and a revised nosology of FTLD will contribute toward an accurate diagnosis, and facilitate the development of new diagnostic tests and therapeutics."
08/01/2010 - "A report showed that mice expressing a mutant form of human TDP-43 develop a progressive and fatal neurodegenerative disease reminiscent of both ALS and frontotemporal lobar degeneration with ubiquitin aggregates (FTLD-U), providing a new animal model that may help to better understand the pathophysiology and test new therapeutics. "
01/01/2004 - "The behavioral symptoms, especially stereotyped behaviors of FTLD, significantly improved after treatment. "
02/01/2010 - "The patient with semantic dementia was able to relearn all the vocabulary she was shown, even though she lost everything she had acquired after treatment was interrupted. "
|3.||Activities of Daily Living (ADL)
01/01/2015 - "Relying on procedural memory to enhance independence in daily living activities: Smartphone use in a case of semantic dementia."
12/01/2007 - "Although PPA in its early stages can usually be differentiated from probable Alzheimer's disease (PRAD) and the behavioral variant of frontotemporal lobar degeneration by the absence of significant changes in memory and behavior, and the preservation of activities daily living, progression of the disease often leads to deficits more consistent with the latter. "
01/01/2013 - "The bibliographic search was conducted using the terms "frontotemporal dementia" and "frontotemporal lobar degeneration" in combination with "independence," "functionality," "basic activities of daily living," "disability," and scales that measure functional performance: "Disability Assessment for Dementia-DAD," "Functional Activities Questionnaire (FAQ)," "Direct Assessment of Functional Status (DAFS)." To be included in the review, the study had to mention the characterization of the functional status of patients with bvFTD in the objectives of the study, using a previously validated instrument of functional assessment. "
05/01/2014 - "In this study, we analysed three unrelated FTLD patients with low plasma progranulin levels but no point GRN mutations by multiplex ligation-dependent probe amplification (MLPA) and quantitative multiplex polymerase chain reaction of short fluorescent fragments (QMPSF). "
08/31/2007 - "To determine whether MAPT gene copy number variation is involved in FTLD, 70 patients with clinical diagnosis of FTLD and no MAPT mutation (including 12 patients with pathologically proven tau-positive FTLD) were screened by using multiplex ligation probe amplification (MLPA) with specific oligonucleotide probes. "