Temporal Variant Research Articles

Semantic and right temporal variant of FTD: Next generation sequencing genetic analysis on a single-center cohort.

Semantic and right temporal variant of frontotemporal dementia (svFTD and rtvFTD) are rare clinical phenotypes in which, in most cases, the underlying pathology is TDP-43 proteinopathy. They are usually sporadic disorders, but recent evidences suggest a higher frequency of genetic mutations for the right temporal versus the semantic variant. However, the genetic basis of these forms is not clear. In this study we performed a genetic screening of a single-center cohort of svFTD and rtvFTD patients, aiming at identifying the associated genetic variants. A panel of 73 dementia candidate genes has been analyzed by NGS target sequencing including both causal and risk/modifier genes in 23 patients (15 svFTD and 8 rtvFTD) and 73 healthy age-matched controls. We first performed a single variant analysis considering rare variants and then a gene-based aggregation analysis to evaluate the cumulative effects of multiple rare variants in a single gene. We found 12 variants in nearly 40% of patients (9/23), described as pathogenic or classified as VUS/likely pathogenic. The overall rate was higher in svFTD than in rtvFTD. Three mutations were located in MAPT gene and single mutations in the following genes: SQSTM1, VCP, PSEN1, TBK1, OPTN, CHCHD10, PRKN, DCTN1. Our study revealed the presence of variants in genes involved in pathways relevant for the pathology, especially autophagy and inflammation. We suggest that molecular analysis should be performed in all svFTD and rtvFTD patients, to better understand the genotype-phenotype correlation and the pathogenetic mechanisms that could drive the clinical phenotypes in FTD.

Significant contralaterality of temporal-predominant neuroastroglial tauopathy and FTLD-TDP type C presenting with the right temporal variant FTD.

Significant contralaterality of temporal-predominant neuroastroglial tauopathy and FTLD-TDP type C presenting with the right temporal variant FTD.

Stone Industry of the Bronze and Iron Ages Border (case study of the Oralovskoe Ozero II settlement on the Vishera River)

The authors publish the data on the stone assemblage of the Oralovskoe Ozero II settlement, 9th – 8th century B.C., the Kama River basin, Perm Krai, Russian Federation. Examination of the traces of processing on the flint artefacts made it possible to determine its basic characteristics. These are a stadial knapping; secondary bifacial thinning; heat treatment of the raw material to prepare it for knapping; leaf-shaped bifaces with a straight base – arrowheads and dart/spear points, decorated at the fnal stage with «serrated» retouching; unifacial scrapers. The search for assemblages with similar characteristics allowed to determine in the extreme north-east of Europe (the basins of the Pechora, Vychegda and Mezen rivers) a special tradition of the flint tools production of the Bronze and Iron Ages boundary. Reference stone assemblages accompany different types of pottery and their combinations: Korshak and Lebyazhskaya cultures, Ananyino cultural-historical community and ceramics with “cross-like” ornamentation. This indicates that mobile foragers established links with tundra and taiga cultures, uniting them into a single network of the Bronze and Iron Age boundary in the north-east of Europe. A detailed analysis of the identifed stone-working tradition, its naming, the search for origins and spatial and temporal variants is needed in the future.

Hereditary Succession in the DPRK: Temporal and Perceptional Variants in Regime Stability and their Foreign Policy Implications

This paper argues DPRK instability narratives that the Kim regime will collapse like the Soviet bloc are misguided and that US foreign policy founded upon them has at times led to a 'wait and see' approach sometimes referred to as strategic patience. One source of instability narratives, hereditary succession in North Korea, has occurred twice successfully, with leaders choosing their successor and being invested in their succession in a way that may not occur if the successor were not family. By comparing Kim Il Sung and Mao Ze Dong's succession attempts, this paper hypothesizes that the longer time a successor has to consolidate power prior to succession, herein referred to as the "power incubation period" (PIP), the more likely their regime's survival. This contributes to both the literature on authoritarian regime succession and foreign policy towards those regimes by demonstrating that the temporal aspect of regime consolidation takes place both before and after succession and that in cases of hereditary succession, the perceptional variant of family ties allows for a longer PIP prior to succession.

Range in the Use and Realization of BIN in African American English.

This paper jointly considers syntactic, semantic, and phonological/phonetic factors in approaching an understanding of BIN, a remote past marker in African American English that has been described as "stressed." It brings together data from the Corpus of Regional African American Language (CORAAL) and a production study in a small African American English-speaking community in southwest Louisiana to investigate the use and phonetic realization of BIN constructions. Only 20 instances of BIN constructions were found in CORAAL. This sparsity was not simply due to a dearth of semantic contexts for BIN in the interviews, since 122 instances of semantically equivalent been + temporal adverbial variants were also found. These results raise questions about the extent to which BIN constructions and been + temporal adverbial variants are used in different pragmatic and discourse contexts as well as in different speech styles. The production study elicited BIN and past participle been constructions in controlled syntactic and semantic environments. The phonetic realization of BIN was found to be distributed over the entire utterance rather than localized to BIN. BIN utterances were distinguished from past participle been utterances by having higher ratios of fundamental frequency (F0), intensity, and duration in BIN/been relative to preceding and following material in the utterance. In both studies, BIN utterances were generally realized with a high F0 peak on BIN and a reduced F0 range in the post-BIN region, with variability in the presence and kinds of F0 movements utterance-initially and utterance-finally, as well as in F0 downtrends in the post-BIN region.

Clinical, radiologic, and pathologic features of the globular glial tauopathy subtype of frontotemporal lobar degeneration in right temporal variant frontotemporal dementia with salient features of Geschwind syndrome

ABSTRACT Globular Glial Tauopathy (GGT) is a rare form of Frontotemporal Lobar Degeneration (FTLD) consisting of 4-repeat tau globular inclusions in astrocytes and oligodendrocytes. We present the pathological findings of GGT in a previously published case of a 73-year-old woman with behavioral symptoms concerning for right temporal variant frontotemporal dementia with initial and salient features of Geschwind syndrome. Clinically, she lacked motor abnormalities otherwise common in previously published GGT cases. Brain MRI showed focal right anterior temporal atrophy (indistinguishable from five FTLD-TDP cases) and subtle ipsilateral white matter signal abnormalities. Brain autopsy showed GGT type III and Alzheimer’s neuropathologic changes. .

Right Temporal Lobe Variant of Frontotemporal Dementia: Systematic Review.

Frontotemporal dementia corresponds to a heterogenous group of syndromes characterized by progressive changes in behavior and/or language. Approximately 30% of patients with primary progressive aphasia, semantic variant (semantic dementia), present with atrophy in the right cerebral hemisphere, in a rare clinical condition called right temporal variant of frontotemporal dementia (rtvFTD). The objective of the study is to present the main demographic, clinical, neuropsychological, neuroimaging, and pathologic characteristics of rtvFTD patients. A systematic review of the literature was carried out in the PubMed, LILACS, and SCIELO databases between January and March 2022. After the evaluation process, 41 articles were selected, published between 1993 and 2021. We found that rtvFTD presents with severe and progressive prosopagnosia (related to anterior temporal lobe injury) associated with behavioral symptoms-desinibition (51%), apathy (39%), obsessive-compulsive symptoms (37%), changes in eating habits (33%), and depression (28%), which is different from semantic dementia. The most common pathologic pattern is TDP-43, type C. This field of knowledge has few studies (mainly reports and case series) and heterogenous nomenclature, which is a limitation. A multinational longitudinal registry of people with rtvFTD, with standardized assessment and description of symptoms, is necessary to elucidate the characteristics of this entity.

Multistage Vertex Cover

The NP-complete Vertex Cover problem asks to cover all edges of a graph by a small (given) number of vertices. It is among the most prominent graph-algorithmic problems. Following a recent trend in studying temporal graphs (a sequence of graphs, so-called layers, over the same vertex set but, over time, changing edge sets), we initiate the study of Multistage Vertex Cover. Herein, given a temporal graph, the goal is to find for each layer of the temporal graph a small vertex cover and to guarantee that two vertex cover sets of every two consecutive layers differ not too much (specified by a given parameter). We show that, different from classic Vertex Cover and some other dynamic or temporal variants of it, Multistage Vertex Cover is computationally hard even in fairly restricted settings. On the positive side, however, we also spot several fixed-parameter tractability results based on some of themost natural parameterizations.

A presumptive association between obsessive compulsions and asymmetric temporal lobe atrophy: a case report

BackgroundThe relatively isolated atrophy of the temporal lobes leads to a clinical radiological pattern, referred to as the temporal variant of frontotemporal dementia. While semantic dementia and behavioral variant frontotemporal dementia are classically related to this syndrome, the logopenic variant of primary progressive aphasia has been less commonly reported. This case report aims to give a pictorial description of a case in which a patient with asymmetric temporal lobe atrophy presented with the logopenic variant of primary progressive aphasia and complex rituals of cleanliness.Case presentationWe report on the case of a 68-year-old, right-handed White woman with complex rituals and progressive speech impairment. The obsessive–compulsive rituals represented an exacerbation of lifelong preoccupations with cleanliness and orderliness that were praised by her relatives. Neuropsychological assessment revealed a striking impairment of language and memory, with relative sparing of tool-use praxis and visuospatial skills. Magnetic resonance imaging and 18fluorodeoxyglucose-positron emission tomography scans showed bilateral asymmetrical temporal lobe atrophy and hypometabolism. A year later, she was still able to entertain conversation for a short while, but her vocabulary and fluency had further declined. Praxis and visuospatial skills remained intact. She did not experience pathological elation, delusions, or hallucinations. The disease followed a relentless progression into a partial Klüver–Bucy syndrome, abulia, and terminal dementia. She died from acute myocardial infarction 8 years after the onset of aphasia. The symptoms and their temporal course supported a diagnosis of logopenic variant of primary progressive aphasia due to asymmetric temporal variant frontotemporal lobar degeneration.ConclusionsThis report gives a pictorial description of a temporal variant of frontotemporal dementia in a patient who presented with worsening of a lifelong obsessive–compulsive disorder and logopenic variant of primary progressive aphasia.

Chapter 24 - Frontotemporal dementia: A unique window on the functional role of the temporal lobes

Frontotemporal dementia (FTD) is an umbrella term covering a plethora of progressive changes in executive functions, motor abilities, behavior, and/or language. Different clinical syndromes have been described in relation to localized atrophy, informing on the functional networks that underlie these specific cognitive, emotional, and behavioral processes. These functional declines are linked with the underlying neurodegeneration of frontal and/or temporal lobes due to diverse molecular pathologies. Initially, the accumulation of misfolded proteins targets specifically susceptible cell assemblies, leading to relatively focal neurodegeneration that later spreads throughout large-scale cortical networks. Here, we discuss the most recent clinical, neuropathological, imaging, and genetics findings in FTD-spectrum syndromes affecting the temporal lobe. We focus on the semantic variant of primary progressive aphasia and its mirror image, the right temporal variant of FTD. Incipient focal atrophy of the left anterior temporal lobe (ATL) manifests with predominant naming, word comprehension, reading, and object semantic deficits, while cases of predominantly right ATL atrophy present with impairments of socioemotional, nonverbal semantic, and person-specific knowledge. Overall, the observations in FTD allow for crucial clinical-anatomic inferences, shedding light on the role of the temporal lobes in both cognition and complex behaviors. The concerted activity of both ATLs is critical to ensure that percepts are translated into concepts, yet important hemispheric differences should be acknowledged. On one hand, the left ATL attributes meaning to linguistic, external stimuli, thus supporting goal-oriented, action-related behaviors (e.g., integrating sounds and letters into words). On the other hand, the right ATL assigns meaning to emotional, visceral stimuli, thus guiding socially relevant behaviors (e.g., integrating body sensations into feelings of familiarity).

PrecipLSTM: A Meteorological Spatiotemporal LSTM for Precipitation Nowcasting

Accurate and timely nowcasting precipitation has huge social and economic benefits. However, the changes of clouds including expansion, dissipation, and distortion are extremely complex, which exacerbates the difficulty of forecasting. Fortunately, it still follows certain meteorological laws, which can be explored based on spatiotemporal information but are not fully considered by previous models. In this paper, we design two modules to focus on these messages based on atmospheric characteristics. Specifically, the SLAM (Spatial Local Attention Memory) module combines local attention and memory mechanism to capture the meteorological spatial relationship, while the TDM (Time Difference Memory) module combines differential technology and memory mechanism to capture the meteorological temporal variants. We combine these two modules with PredRNN and propose PrecipLSTM to sufficiently capture the spatiotemporal dependencies of radar data. We do exhaustive experiments with five baselines on four radar datasets. It is verified that PrecipLSTM achieves state-of-the-art results with fewer parameters than the previous state-of-the-art method.

Is it a new phenotype? Right temporal variant frontotemporal dementia

AbstractBackgroundFrontotemporal dementia (FTD) is heterogeneous and presents with behavioral or language symptoms with frontal and/or temporal atrophy. A variant presenting with anomia and word comprehension deficits, is termed semantic dementia (SD) and is associated with predominant left anterior temporal atrophy. Although the clinical presentation is different on the right side, no unique syndrome to identify right temporal variant FTD (rtvFTD) has been established.MethodsWe retrospectively collected 70 rtvFTD patients from our cohort based on their initial MRI and identified the clinical characteristics based on the case notes and compared them with those reported in Alzheimer’s disease (n=70), behavioral FTD (n=70), and SD (n=70). In a subset of cases we reported the genetic (n=6) and pathological (n=5) features along with a review of cases in the literature (n=49).ResultsBased on our single‐center study, rtvFTD presented a unique combination of cognitive (face recognition and memory impairments), behavioral/psychiatric (disinhibition, apathy, depression, and bizarre preoccupations), and language problems (word‐finding difficulties and anomia). It's genetic/pathological background was heterogeneous, in contrast to SD. The most common genetic mutation was MAPT, followed by GRN and TARDBP. The most common pathological diagnoses were FTLD‐TDP type C and FTLD‐tau, followed by FTLD‐TDP type A and B. Moreover, pyramidal degeneration was observed as a co‐pathology.ConclusionThe definition of a syndrome associated with right temporal atrophy could promote its recognition as a variant of FTD, which is crucial for individual patient management. A multicenter study will shed light on the many unanswered questions about this equivocal syndrome.

Differences in sex distribution between genetic and sporadic FTD

AbstractBackgroundThe reported sex distribution differs between frontotemporal dementia (FTD) cohorts. Possible explanations are the evolving clinical criteria of FTD and its subtypes and the discovery of FTD causal genetic mutations that have resulted in variable findings. Our aim was to determine the sex distribution in a large international retrospective cohort of sporadic and genetic FTD.MethodWe included patients with probable and definite behavioural variant frontotemporal dementia (bvFTD), non‐fluent variant primary progressive aphasia (nfvPPA), semantic variant primary progressive aphasia (svPPA) and right temporal variant frontotemporal dementia (rtvFTD) from the Amsterdam Dementia Cohort, the Montreal Neurological Institute Cohort, the University of Ulm and Technical University of Munich Cohort (part of the German Consortium of Frontotemporal Lobal Degeneration), the Policlinico Milan Cohort and the Sydney FRONTIER Cohort. We compared sex distribution between genetic and sporadic FTD using χ2 tests.ResultA total of 910 subjects were included (56.3% male), of whom 654 had bvFTD, 99 nfvPPA, 117 svPPA and 40 rtvFTD. Of these, 215 had genetic FTD and the sex distribution was equal (51.2% male), which did not differ significantly from sporadic FTD (57.8% male, χ2 p=0.081). In the sporadic bvFTD subgroup, we found a male predominance (61.6% males compared to 52.9% males in the bvFTD genetic group, χ 2 p=0.04). No sex distribution differences between sporadic and genetic cases were found in the other clinical FTD subgroups (all p>0.05).ConclusionDifferences in sex distribution between genetic and sporadic behavioural variant of FTD may provide important clues for its differential pathogenesis and warrants further research.

Differences in Sex Distribution Between Genetic and Sporadic Frontotemporal Dementia.

Background:Reported sex distributions differ between frontotemporal dementia (FTD) cohorts. Possible explanations are the evolving clinical criteria of FTD and its subtypes and the discovery of FTD causal genetic mutations that has resulted in varying demographics.Objective:Our aim was to determine the sex distribution of sporadic and genetic FTD cases and its subtypes in an international cohort.Methods:We included 910 patients with behavioral variant frontotemporal dementia (bvFTD; n = 654), non-fluent variant primary progressive aphasia (nfvPPA; n = 99), semantic variant primary progressive aphasia (svPPA; n = 117), and right temporal variant frontotemporal dementia (rtvFTD; n = 40). We compared sex distribution between genetic and sporadic FTD using χ2-tests.Results:The genetic FTD group consisted of 51.2% males, which did not differ from sporadic FTD (57.8% male, p = 0.08). In the sporadic bvFTD subgroup, males were predominant in contrast to genetic bvFTD (61.6% versus 52.9% males, p = 0.04). In the other clinical FTD subgroups, genetic cases were underrepresented and within the sporadic cases the sex distribution was somewhat equal.Conclusion:The higher male prevalence in sporadic bvFTD may provide important clues for its differential pathogenesis and warrants further research.

Frontotemporal right predominant hypometabolism in bvFTD is not frequent and associated with more severe temporal atrophy

Prosopagnosia, memory deficits and behavioural changes has recently proposed as the core symptoms of a further variant of frontotemporal dementia, associated with right temporal atrophy or hypometabolism and named right temporal variant (rtvFTD). Aim of the study was to evaluate frequency and clinical characteristics of patients with prevalent fronto and/or temporal right atrophy/hypometabolism.

Frontotemporal Dementia - Current Concepts.

Frontotemporal dementia (FTD) is an entity that includes a group of neurodegenerative disease with symptoms predominantly pertaining to deficits in behavior, executive function (or) language. FTD is one of the most common type of dementia before 65 years of age and is one of the most underdiagnosed dementia as most often the symptoms overlap with psychiatric manifestations. Based on the clinical features, FTD is further subdivided into behavioral variant FTD (Bv-FTD) and primary progressive dementia (PPA). We searched PubMed, MEDLINE, and Google Scholar for articles about FTD disease published in English between January 1, 1975 till 2018. We used the search terms "frontotemporal dementia," "Fronto temporal dementia-motor neuron disease," "dementia," "cognition," "behavioral variant," and "primary progressive aphasia.

Right temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review

Although the right temporal variant frontotemporal dementia (rtvFTD) is characterised by distinct clinical and radiological features, its underlying histopathology remains elusive. Being considered a right-sided variant of semantic variant primary progressive aphasia (svPPA), TDP-43 type C pathology has been linked to the syndrome, but this has not been studied in detail in large cohorts. In this case report and systematic review, we report the autopsy results of five subjects diagnosed with rtvFTD from our cohort and 44 single rtvFTD subjects from the literature. Macroscopic pathological evaluation of the combined results revealed that rtvFTD demonstrated either a frontotemporal or temporal evolution, even if the degeneration started in the right temporal lobe initially. FTLD-TDP type C was the most common underlying pathology in rtvFTD, however, in 64% of rtvFTD, other underlying pathologies than FTLD-TDP type C were present, such as Tau-MAPT and FTLD-TDP type A and B. Additionally, accompanying motor neuron or corticospinal tract degeneration was observed in 28% of rtvFTD patients. Our results show that in contrast to the general assumption, rtvFTD might not be a pure FTLD-TDP type C disorder, unlike its left temporal counterpart svPPA. Large sample size pathological studies are warranted to understand the diverse pathologies of the right and left temporal variants of frontotemporal dementia.

The characteristics of spatial expansion and driving forces of land urbanization in counties in central China: A case study of Feixi county in Hefei city.

Urbanization in Central and Western China has attracted increasing attention in the advent of new-type urbanization in China and the age of 'Global Urbanism'. Although land urbanization is at the epicenter of Chinese urbanization, its process and driving forces in counties beyond the Eastern coastal areas are less known. This paper therefore seeks to investigate the spatial expansion and driving forces of land urbanization in Feixi county, a relatively advanced county neighboring Hefei city proper in Anhui province in Central China. Based on the land-use change survey data, remote sensing interpretation data, and statistical yearbook data of Feixi county from 2002 to 2016, it is revealed that the overall scale of urban land in Feixi county increased remarkably, though with obvious temporal and geographical variants. The year 2009 appears to be the cutting line between quantity-based land urbanization and quality-based land urbanization. Land urbanization rate is sensitive to both geographical distance and traffic accessibility to Hefei city proper. Driving forces of land urbanization in Feixi county are summarized as: (1) central city expansion, (2) government-led industrial park construction, and (3) large industrial projects along traffic corridors. A better understanding of urbanization in Feixi county cannot be fulfilled without taking into account the wider spatial process in Hefei city and the Yangtze River Delta city-region.

Empirical Insights from a Study on Outlier Preserving Value Generalization in Animated Choropleth Maps

Time series animation of choropleth maps easily exceeds our perceptual limits. In this empirical research, we investigate the effect of local outlier preserving value generalization of animated choropleth maps on the ability to detect general trends and local deviations thereof. Comparing generalization in space, in time, and in a combination of both dimensions, value smoothing based on a first order spatial neighborhood facilitated the detection of local outliers best, followed by the spatiotemporal and temporal generalization variants. We did not find any evidence that value generalization helps in detecting global trends.

The Right Temporal Variant of Frontotemporal Dementia Is Not Genetically Sporadic: A Case Series.

Background:Right temporal variant frontotemporal dementia (rtvFTD) has been generally considered as a right sided variant of semantic variant primary progressive aphasia (svPPA), which is a genetically sporadic disorder. Recently, we have shown that rtvFTD has a unique clinical syndrome compared to svPPA and behavioral variant frontotemporal dementia.Objective:We challenge the assumption that rtvFTD is a sporadic, non-familial variant of FTD by identifying potential autosomal dominant inheritance and related genes in rtvFTD.Methods:We collected all subjects with a diagnosis of FTD or primary progressive aphasia who had undergone genetic screening (n = 284) and subsequently who had a genetic variant (n = 48) with a diagnosis of rtvFTD (n = 6) in 2 specialized memory clinics.Results:Genetic variants in FTD related genes were found in 33% of genetically screened rtvFTD cases; including MAPT (n = 4), GRN (n = 1), and TARDBP (n = 1) genes, whereas only one svPPA case had a genetic variant in our combined cohorts. Additionally, 4 out of 6 rtvFTD subjects had a strong family history for dementia.Conclusion:Our results demonstrate that rtvFTD, unlike svPPA, is not a pure sporadic, but a heterogeneous potential genetic variant of FTD, and screening for genetic causes for FTD should be performed in patients with rtvFTD.