1,692 results match your criteria Dementia Frontotemporal Lobe

Right temporal lobe and socioemotional semantics: semantic behavioural variant frontotemporal dementia.

Brain 2022 Jun 22. Epub 2022 Jun 22.

Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA 94158, USA.

Focal anterior temporal lobe (ATL) degeneration often preferentially affects the left or right hemisphere. While patients with left-predominant ATL (lATL) atrophy show severe anomia and verbal semantic deficits and meet criteria for semantic variant primary progressive aphasia (svPPA) and semantic dementia, patients with early right ATL (rATL) atrophy are more difficult to diagnose as their symptoms are less well understood. Focal rATL atrophy is associated with prominent emotional and behavioral changes, and patients often meet, or go on to meet, criteria for behavioral variant frontotemporal dementia (bvFTD). Read More

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[A case of amyotrophic lateral sclerosis presenting with rapid progression of respiratory deterioration due to severe obesity].

Rinsho Shinkeigaku 2022 May 26. Epub 2022 May 26.

Department of Primary Care and Emergency Medicine, Graduate School of Medicine, Kyoto University.

A 55-year-old woman with extreme obesity presenting with limb weakness since 1 year was diagnosed with amyotrophic lateral sclerosis (ALS) based on clinical findings and needle electromyography. She had a habit of overeating, and her body mass index (BMI) was 38.2. Read More

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Heteroplasmic mitochondrial DNA mutations in frontotemporal lobar degeneration.

Acta Neuropathol 2022 Jun 30;143(6):687-695. Epub 2022 Apr 30.

Department of Clinical Neurosciences, School of Clinical Medicine, University of Cambridge, Cambridge Biomedical Campus, Cambridge, UK.

Frontotemporal lobar degeneration (FTLD) is a common cause of young onset dementia and is characterised by focal neuropathology. The reasons for the regional neuronal vulnerability are not known. Mitochondrial mechanisms have been implicated in the pathogenesis of FTLD, raising the possibility that frontotemporal regional mutations of mitochondrial DNA (mtDNA) are contributory causes. Read More

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Oxidized phospholipids as novel mediators of neurodegeneration.

Trends Neurosci 2022 Jun 4;45(6):419-429. Epub 2022 Apr 4.

Hotchkiss Brain Institute and the Department of Clinical Neuroscience, University of Calgary, Calgary, Alberta, Canada. Electronic address:

Neurodegeneration drives the progression of many neurological diseases. Inflammation and oxidative stress occurring in the CNS promote lipid peroxidation, leading to the generation of oxidized phospholipids such as oxidized phosphatidylcholines (OxPCs). OxPCs have been proposed as biomarkers of oxidative stress, where their detection in lesions in multiple sclerosis (MS), frontotemporal lobe dementia, spinal cord injury, and amyotrophic lateral sclerosis (ALS) implies that oxidative insult had occurred. Read More

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Anomia is present pre-symptomatically in frontotemporal dementia due to MAPT mutations.

J Neurol 2022 Mar 29. Epub 2022 Mar 29.

Paris Brain Institute - Institut du Cerveau - ICM, Inserm U1127, CNRS UMR 7225, Sorbonne Université, AP-HP - Hôpital Pitié-Salpêtrière, Paris, France.

Introduction: A third of frontotemporal dementia (FTD) is caused by an autosomal-dominant genetic mutation in one of three genes: microtubule-associated protein tau (MAPT), chromosome 9 open reading frame 72 (C9orf72) and progranulin (GRN). Prior studies of prodromal FTD have identified impaired executive function and social cognition early in the disease but few have studied naming in detail.

Methods: We investigated performance on the Boston Naming Test (BNT) in the GENetic Frontotemporal dementia Initiative cohort of 499 mutation carriers and 248 mutation-negative controls divided across three genetic groups: C9orf72, MAPT and GRN. Read More

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Examining empathy deficits across familial forms of frontotemporal dementia within the GENFI cohort.

Cortex 2022 05 9;150:12-28. Epub 2022 Feb 9.

Univ Lille, France; Inserm 1172, Lille, France; CHU, CNR-MAJ, Labex Distalz, LiCEND Lille, France.

Background: Reduced empathy is a common symptom in frontotemporal dementia (FTD). Although empathy deficits have been extensively researched in sporadic cases, few studies have explored the differences in familial forms of FTD.

Methods: Empathy was examined using a modified version of the Interpersonal Reactivity Index (mIRI) in 676 participants from the Genetic FTD Initiative: 216 mutation-negative controls, 192 C9orf72 expansion carriers, 193 GRN mutation carriers and 75 MAPT mutation carriers. Read More

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A Novel Variant (Lys694del) Presenting With Corticobasal Syndrome in a Family With FTD-ALS Spectrum Diseases: Case Report.

Front Neurol 2022 4;13:826676. Epub 2022 Mar 4.

Department of Neurology, Center for Comprehensive Care and Research on Memory Disorders, University of Chicago, Chicago, IL, United States.

Several variants of the TANK-Binding Kinase 1 () gene have been associated with frontotemporal dementia - amyotrophic lateral sclerosis (FTD-ALS) spectrum diseases. Corticobasal syndrome (CBS) is characterized by asymmetric limb rigidity, dystonia or myoclonus, in association with speech or limb apraxia, cortical sensory deficit, and/or alien limb. It can result from a variety of underlying pathologies and although typically sporadic, it has been occasionally associated with and variants. Read More

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TMEM106B deficiency impairs cerebellar myelination and synaptic integrity with Purkinje cell loss.

Acta Neuropathol Commun 2022 03 14;10(1):33. Epub 2022 Mar 14.

Department of Molecular Biology and Genetics, Weill Institute for Cell and Molecular Biology, Cornell University, 345 Weill Hall, Ithaca, NY, 14853, USA.

TMEM106B, a type II lysosomal transmembrane protein, has recently been associated with brain aging, hypomyelinating leukodystrophy, frontotemporal lobar degeneration (FTLD) and several other brain disorders. TMEM106B is critical for proper lysosomal function and TMEM106B deficiency leads to myelination defects, FTLD related pathology, and motor coordination deficits in mice. However, the physiological and pathological functions of TMEM106B in the brain are still not well understood. Read More

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[Frontotemporal dementia with cortico-basal syndrome].

Zh Nevrol Psikhiatr Im S S Korsakova 2022 ;122(2):107-114

Republican Center for Movement Disorders and Botulinum Therapy, Kazan, Russia.

FTD is a group of neurodegenerative diseases with progressive deterioration of behavioral and speech disorders, morphologically associated with pathology of the frontal or temporal lobes. International clinical trials have made it possible to define modern diagnostic criteria for various subtypes of clinically «possible/probable» FTD. Our article is devoted to one of the rare subtypes of frontotemporal dementia (FTD), corticobasal syndrome (CBD), in which we presented a review of current data with a demonstration of clinical observation. Read More

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Quantified Brain Magnetic Resonance Imaging Volumes Differentiate Behavioral Variant Frontotemporal Dementia from Early-Onset Alzheimer's Disease.

J Alzheimers Dis 2022 ;87(1):453-461

Department of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.

Background: The differentiation of behavioral variant frontotemporal dementia (bvFTD) from early-onset Alzheimer's disease (EOAD) by clinical criteria can be inaccurate. The volumetric quantification of clinically available magnetic resonance (MR) brain scans may facilitate early diagnosis of these neurodegenerative dementias.

Objective: To determine if volumetric quantification of brain MR imaging can identify persons with bvFTD from EOAD. Read More

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January 2022

Cortical and subcortical changes in resting-state neuronal activity and connectivity in early symptomatic ALS and advanced frontotemporal dementia.

Neuroimage Clin 2022 12;34:102965. Epub 2022 Feb 12.

Amsterdam University Medical Centers, Vrije Universiteit Amsterdam, Department of Clinical Neurophysiology, Magnetoencephalography Centre, Amsterdam Neuroscience, Amsterdam, the Netherlands.

The objective of this study was to examine if patterns of resting-state brain activity and functional connectivity in cortical and subcortical regions in patients with early symptomatic amyotrophic lateral sclerosis (ALS) resemble those of behavioural variant frontotemporal dementia (bvFTD). In a cross-sectional design, eyes-closed resting-state magnetoencephalography (MEG) data of 34 ALS patients, 18 bvFTD patients and 18 age- and gender-matched healthy controls (HCs) were projected to source-space using an atlas-based beamformer. Group differences in peak frequency, band-specific oscillatory activity and functional connectivity (corrected amplitude envelope correlation) in 78 cortical regions and 12 subcortical regions were determined. Read More

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February 2022

TDP-43-associated atrophy in brains with and without frontotemporal lobar degeneration.

Neuroimage Clin 2022 4;34:102954. Epub 2022 Feb 4.

Department of Neurology, Mayo Clinic, 200 1(st) Street NW, Rochester, MN 55905, USA. Electronic address:

Transactive response DNA-binding protein of ∼43 kDa (TDP-43), a primary pathologic substrate in tau-negative frontotemporal lobar degeneration (FTLD), is also often found in the brains of elderly individuals without FTLD and is a key player in the process of neurodegeneration in brains with and without FTLD. It is unknown how rates and trajectories of TDP-43-associated brain atrophy compare between these two groups. Additionally, non-FTLD TDP-43 inclusions are not homogeneous and can be divided into two morphologic types: type-α and neurofibrillary tangle-associated type-β. Read More

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February 2022

Lesions in different prefrontal sectors are associated with different types of acquired personality disturbances.

Cortex 2022 02 31;147:169-184. Epub 2021 Dec 31.

Department of Neurology, Carver College of Medicine, Iowa City, IA, United States; Department of Psychiatry, Carver College of Medicine, Iowa City, IA, United States; Department of Pediatrics, Carver College of Medicine, Iowa City, IA, United States.

"Frontal lobe syndrome" is a term often used to describe a diverse array of personality disturbances following frontal lobe damage. This study's guiding premise was that greater neuroanatomical specificity could be achieved by evaluating specific types of personality disturbances following acquired frontal lobe lesions. We hypothesized that three acquired personality disturbances would be associated with lesion involvement of distinct sectors of the prefrontal cortex (PFC): 1) emotional-social disturbance and ventromedial PFC, 2) hypoemotional disturbance and dorsomedial PFC, and 3) dysexecutive and dorsolateral PFC. Read More

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February 2022

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

J Med Case Rep 2022 Jan 20;16(1):21. Epub 2022 Jan 20.

Cognitive and Behavioral Neuroscience Unit, D'Or Institute for Research & Education (IDOR), Rio de Janeiro, RJ, Brazil.

Background: The 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. Read More

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January 2022

Try to see it my way - Examining the relationship between visual perspective taking and theory of mind in frontotemporal dementia.

Brain Cogn 2022 03 7;157:105835. Epub 2022 Jan 7.

The University of Sydney, Brain and Mind Centre, Australia; The University of Sydney, School of Psychology, Australia. Electronic address:

The behavioural variant of frontotemporal dementia (bvFTD) is characterised by pronounced alterations in social functioning, including the understanding of others' thoughts and feelings via theory of mind. The emergence of such impairments in other social disorders such as autism and schizophrenia is suggested to reflect an inability to imagine the other person's visual perspective of the world. To our knowledge, relationships between visual perspective taking and theory of mind have not previously been explored in bvFTD. Read More

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Proton magnetic resonance spectroscopy in frontotemporal lobar degeneration-related syndromes.

Neurobiol Aging 2022 03 29;111:64-70. Epub 2021 Oct 29.

Department of Clinical Neurosciences, University of Cambridge, UK; Cambridge University Hospitals NHS Foundation Trust, UK; MRC Cognition and Brain, Sciences Unit, University of Cambridge, UK.

There is an urgent need for a better understanding of the pathophysiology of cognitive impairment in syndromes associated with frontotemporal lobar degeneration. Here, we used magnetic resonance spectroscopy to quantify metabolite deficits in sixty patients with a clinical syndrome associated with frontotemporal lobar degeneration (behavioral variant frontotemporal dementia n = 11, progressive supranuclear palsy n = 26, corticobasal syndrome n = 11, primary progressive aphasias n = 12), and 38 age- and sex-matched healthy controls. We measured nine metabolites in the right inferior frontal gyrus, superior temporal gyrus and right primary visual cortex. Read More

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Cognitive and Neural Mechanisms of Social Communication Dysfunction in Primary Progressive Aphasia.

Brain Sci 2021 Dec 1;11(12). Epub 2021 Dec 1.

Brain and Mind Centre, The University of Sydney, Sydney, NSW 2050, Australia.

Mounting evidence suggests that, in parallel with well-defined changes in language, primary progressive aphasia (PPA) syndromes display co-occurring social cognitive impairments. Here, we explored multidimensional profiles of carer-rated social communication using the La Trobe Communication Questionnaire (LCQ) in 11 semantic dementia (SD), 12 logopenic progressive aphasia (LPA) and 9 progressive non-fluent aphasia (PNFA) cases and contrasted their performance with 19 Alzheimer's disease (AD) cases, 26 behavioural variant frontotemporal dementia (bvFTD) cases and 31 healthy older controls. Relative to the controls, the majority of patient groups displayed significant overall social communication difficulties, with common and unique profiles of impairment evident on the LCQ subscales. Read More

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December 2021

Degeneration of basal and limbic networks is a core feature of behavioural variant frontotemporal dementia.

Brain Commun 2021 21;3(4):fcab241. Epub 2021 Oct 21.

School of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen AB25 2ZD, UK.

The behavioural variant of frontotemporal dementia is a clinical syndrome characterized by changes in behaviour, cognition and functional ability. Although atrophy in frontal and temporal regions would appear to be a defining feature, neuroimaging studies have identified volumetric differences distributed across large parts of the cortex, giving rise to a classification into distinct neuroanatomical subtypes. Here, we extended these neuroimaging studies to examine how distributed patterns of cortical atrophy map onto brain network hubs. Read More

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October 2021

Mapping cortical disease-burden at individual-level in frontotemporal dementia: implications for clinical care and pharmacological trials.

Brain Imaging Behav 2022 Jun 9;16(3):1196-1207. Epub 2021 Dec 9.

Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Dublin, Ireland.

Imaging studies of FTD typically present group-level statistics between large cohorts of genetically, molecularly or clinically stratified patients. Group-level statistics are indispensable to appraise unifying radiological traits and describe genotype-associated signatures in academic studies. However, in a clinical setting, the primary objective is the meaningful interpretation of imaging data from individual patients to assist diagnostic classification, inform prognosis, and enable the assessment of progressive changes compared to baseline scans. Read More

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Corpus Callosum Atrophy in Detection of Mild and Moderate Alzheimer's Disease Using Brain Magnetic Resonance Image Processing and Machine Learning Techniques.

J Alzheimers Dis Rep 2021 25;5(1):771-788. Epub 2021 Oct 25.

Structural Biology and Bioinformatics Division, Council for Scientific and Industrial Research (CSIR) - Indian Institute of Chemical Biology (IICB), Kolkata, West Bengal, India.

Background: The total number of people with dementia is projected to reach 82 million in 2030 and 152 in 2050. Early and accurate identification of the underlying causes of dementia, such as Alzheimer's disease (AD) is of utmost importance. A large body of research has shown that imaging techniques are most promising technologies to improve subclinical and early diagnosis of dementia. Read More

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October 2021

Cortical network modularity changes along the course of frontotemporal and Alzheimer's dementing diseases.

Neurobiol Aging 2022 02 1;110:37-46. Epub 2021 Nov 1.

Department of Medicine and Aging Sciences, University G. d'Annunzio of Chieti-Pescara, Chieti, Italy. Electronic address:

Cortical network modularity underpins cognitive functions, so we hypothesized its progressive derangement along the course of frontotemporal (FTD) and Alzheimer's (AD) dementing diseases. EEG was recorded in 18 FTD, 18 AD, and 20 healthy controls (HC). In the FTD and AD patients, the EEG recordings were performed at the prodromal stage of dementia, at the onset of dementia, and three years after the onset of dementia. Read More

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February 2022

Effect of Multiple Medicines on Dementia Initial Treatment: Experience and Thinking.

Am J Alzheimers Dis Other Demen 2021 Jan-Dec;36:15333175211053134

Department of Neurology, 105738Beijing Tiantan Hospital Affiliated to Capital Medical University, Beijing, China.

Little is known about multiple medicines and initial therapy among people with dementia. To examine the effect of multiple medicines on the initiation of anti-dementia therapy in patients diagnosed with cognitive impairment (CI), a retrospective study with 2742 CI patients was conducted based on the outpatients' medical records. The dementias receiving 1-2 drugs were more likely to be prescribed with anti-dementia (one drug: OR = 1. Read More

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Analysis of Genotype-Phenotype Correlations in Patients With Degenerative Dementia Through the Whole Exome Sequencing.

Front Aging Neurosci 2021 14;13:745407. Epub 2021 Oct 14.

Alzheimer's Disease and Related Disorders Center, Shanghai Mental Health Center, Department of Geriatric Psychiatry, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Sporadic dementias generally occur in older age and are highly polygenic, which indicates some patients transmitted in a poly-genes hereditary fashion. Our study aimed to analyze the correlations of genetic features with clinical symptoms in patients with degenerative dementia. We recruited a group of 84 dementia patients and conducted the whole exome sequencing (WES). Read More

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October 2021

Primary progressive aphasias associated with C9orf72 expansions: Another side of the story.

Cortex 2021 12 1;145:145-159. Epub 2021 Oct 1.

Sorbonne Université, Paris Brain Institute - Institut Du Cerveau - ICM, Inserm U1127, CNRS UMR 7225, AP-HP - Hôpital Pitié-Salpêtrière, Paris, France; Reference Centre for Rare or Early-Onset Dementias, IM2A, Département de Neurologie, AP-HP - Hôpital Pitié-Salpêtrière, Paris, France; Paris Brain Institute - Institut Du Cerveau (ICM), FrontLab, Paris, France. Electronic address:

C9orf72 repeat expansions are rarely associated with primary progressive aphasias (PPA). In-depth characterization of the linguistic deficits, and the underlying patterns of grey-matter atrophy in PPA associated with the C9orf72 expansions (PPA-C9orf72) are currently lacking. In this study, we comprehensively analyzed a unique series of 16 patients affected by PPA-C9orf72. Read More

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December 2021

Early-Onset Frontotemporal Dementia-Related Semantic Variant of Primary Progressive Aphasia: Multimodal Evaluation With Brain Perfusion SPECT, SPECT/MRI Coregistration, and MRI Volumetry.

Clin Nucl Med 2022 Mar;47(3):260-264

From the Nuclear Medicine Department.

Abstract: Frontotemporal dementia (FTD) is a neurodegenerative disorder characterized by cortical and subcortical atrophies, with early involvement of the hippocampus and amygdala. A 58-year-old man with clinical presentation of primary progressive aphasia-particularly its svPPA (semantic variant)-and bilateral asymmetric (left-predominant) anterior temporal lobe atrophy on MRI was referred for brain perfusion SPECT. This revealed bilateral hypoperfusion of the anterior temporal lobe (sustained by software-fused SPECT/MRI), pointing toward FTD rather than Alzheimer disease. Read More

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Characteristics of behavioral symptoms in right-sided predominant semantic dementia and their impact on caregiver burden: a cross-sectional study.

Alzheimers Res Ther 2021 10 9;13(1):166. Epub 2021 Oct 9.

Department of Psychiatry, Osaka University Graduate School of Medicine, D3, 2-2 Yamadaoka,, Suita City,, Osaka, 565-0871, Japan.

Background: This study aimed to clarify the neuropsychiatric symptoms of right-sided predominant semantic dementia (SD-R) by comparing them with those of behavioral variant frontotemporal dementia (bvFTD), left-sided predominant SD (SD-L), and Alzheimer's disease (AD). This study also aimed to identify clinical factors related to caregiver burden for bvFTD, SD-R, and SD-L.

Methods: The neuropsychiatric symptoms of 28 patients with bvFTD, 14 patients with SD-R, 24 patients with SD-L, and 43 patients with AD were evaluated using the Neuropsychiatric Inventory (NPI) and the Stereotypy Rating Inventory (SRI). Read More

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October 2021

Considering Hemispheric Specialization in Emotional Face Processing: An Eye Tracking Study in Left- and Right-Lateralised Semantic Dementia.

Brain Sci 2021 Sep 10;11(9). Epub 2021 Sep 10.

Brain & Mind Centre, School of Psychology, The University of Sydney, Sydney, NSW 2050, Australia.

Face processing relies on a network of occipito-temporal and frontal brain regions. Temporal regions are heavily involved in looking at and processing emotional faces; however, the contribution of each hemisphere to this process remains under debate. Semantic dementia (SD) is a rare neurodegenerative brain condition characterized by anterior temporal lobe atrophy, which is either predominantly left- (left-SD) or right-lateralised (right-SD). Read More

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September 2021

Design and Verbal Fluency in Alzheimer's Disease and Frontotemporal Dementia: Clinical and Metabolic Correlates.

J Int Neuropsychol Soc 2021 Sep 27:1-16. Epub 2021 Sep 27.

Department of Neurology, Hospital Clinico San Carlos, San Carlos Institute for Health Research (IdiSSC), Universidad Complutense, Madrid, Spain.

Objective: Cognitive processes underlying verbal and design fluency, and their neural correlates in patients with Alzheimer's disease (AD) and behavioural variant Frontotemporal Dementia (bvFTD) remain unclear. We hypothesised that verbal and design fluency may be associated with distinct neuropsychological processes in AD and FTD, showing different patterns of impairment and neural basis.

Methods: We enrolled 142 participants including patients with AD (n = 80, mean age = 74. Read More

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September 2021

Distinct phosphorylation profiles of tau in brains of patients with different tauopathies.

Neurobiol Aging 2021 12 21;108:72-79. Epub 2021 Aug 21.

Department of Biological Sciences, Graduate School of Science, Tokyo Metropolitan University, Hachioji, Tokyo, Japan; Department of Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science, Setagaya, Tokyo, Japan. Electronic address:

Tauopathies are neurodegenerative diseases that are characterized by pathological accumulation of tau protein. Tau is hyperphosphorylated in the brain of tauopathy patients, and this phosphorylation is proposed to play a role in disease development. However, it has been unclear whether phosphorylation is different among different tauopathies. Read More

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December 2021

Common genetic variation is associated with longitudinal decline and network features in behavioral variant frontotemporal degeneration.

Neurobiol Aging 2021 12 3;108:16-23. Epub 2021 Aug 3.

Frontotemporal Degeneration Center, Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. Electronic address:

The T allele in rs1768208 located in or near the myelin oligodendrocyte basic protein gene (MOBP) is a risk factor for frontotemporal degeneration pathology. We evaluated the hypothesis that the presence of a T allele in rs1768208 will be associated with rate of cognitive decline in behavioral variant frontotemporal degeneration (bvFTD) related to compromised frontal networks. We studied 81 individuals clinically diagnosed with bvFTD who were genotyped for rs1768208 and coded using a dominant model reflecting the presence (i. Read More

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December 2021