1,550 results match your criteria Dementia Frontotemporal Lobe


Frontal Pole Hypometabolism Linked to Reduced Prosocial Sexual Behaviors in Frontotemporal Dementia and Corticobasal Syndrome.

J Alzheimers Dis 2020 Jul 29. Epub 2020 Jul 29.

The Gertrude H. Sergievsky Center & Taub Institute for Research in Alzheimer's Disease and The Aging Brain, Department of Neurology, Columbia University Irving Medical Center, New York, NY, USA.

Background: Changes in sexual behaviors in frontotemporal dementia (FTD) are common and multifaceted, but not well characterized.

Objective: To characterize changes in sexual behaviors and intimacy in FTD compared to corticobasal syndrome (CBS) and normal controls (NC), and to evaluate the neuroanatomical associations of these changes.

Methods: Spouses of 30 FTD patients, 20 CBS patients, and 35 NC completed the Sexual Symptoms in Neurological Illness and Injury Questionnaire (SNIQ), which captures changes in sexual interest, inappropriate sexual behaviors, and prosocial sexual behaviors. Read More

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http://dx.doi.org/10.3233/JAD-200346DOI Listing

Recall and Recognition in Alzheimer's Disease and Frontotemporal Dementia.

J Alzheimers Dis 2020 Jul 25. Epub 2020 Jul 25.

Laboratory of Clinical and Behavioral Neurology, IRCCS Santa Lucia Foundation, Rome, Italy.

Background: It has long been debated whether performance on recall and recognition tests depends on the same or different memory systems and whether performance on these two tasks is dissociated in clinical populations. According to Dual process theories of recall, performance on recall and recognition tests dissociates in the relative reliance on frontal lobe related activities; in fact, the recall test requires more strategic retrieval of memoranda than the recognition task. By contrast, Dual process theories of recognition posit that performance on these tests differs in the relative contribution of recollection and familiarity memory processes in the two tasks: both recollection and familiarity contribute to recognition judgments, but only recollection supports recall performance. Read More

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http://dx.doi.org/10.3233/JAD-200126DOI Listing

Papez Circuit Gray Matter and Episodic Memory in Amyotrophic Lateral Sclerosis and Behavioural Variant Frontotemporal Dementia.

Brain Imaging Behav 2020 Jul 30. Epub 2020 Jul 30.

Center of Mathematics, Computing and Cognition, Universidade Federal do ABC, Santo André, Brazil.

Amyotrophic lateral sclerosis and behavioural variant frontotemporal dementia are two different diseases recognized to overlap at clinical, pathological and genetic characteristics. Both conditions are traditionally known for relative sparing of episodic memory. However, recent studies have disputed that with the report of patients presenting with marked episodic memory impairment. Read More

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http://dx.doi.org/10.1007/s11682-020-00307-5DOI Listing

PSP-FTD Complex: A Possible Variant of PSP.

Am J Alzheimers Dis Other Demen 2020 Jan-Dec;35:1533317520922383

Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Introduction: This study tried to find out type of lobar features found in patients with progressive supranuclear palsy (PSP) and whether they differ from those of frontotemporal dementia (FTD) as both of these are tauopathies.

Methods: We studied lobar functions of 45 patients with PSP.

Results: Five (11. Read More

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http://dx.doi.org/10.1177/1533317520922383DOI Listing

Effects of Palmitoylethanolamide Combined with Luteoline on Frontal Lobe Functions, High Frequency Oscillations, and GABAergic Transmission in Patients with Frontotemporal Dementia.

J Alzheimers Dis 2020 Jul 2. Epub 2020 Jul 2.

Santa Lucia Foundation, IRCCS, Rome, Italy.

Background: Frontotemporal dementia (FTD) is a presenile neurodegenerative disease for which there is no effective pharmacological treatment. Recently, a link has been proposed between neuroinflammation and FTD.

Objective: Here, we aim to investigate the effects of palmitoylethanolamide (PEA) combined with luteoline (PEA-LUT), an endocannabinoid with anti-inflammatory and neuroprotective effects, on behavior, cognition, and cortical activity in a sample of FTD patients. Read More

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http://dx.doi.org/10.3233/JAD-200426DOI Listing

High diagnostic value of plasma Niemann-Pick type C biomarkers in adults with selected neurological and/or psychiatric disorders.

J Neurol 2020 Jun 26. Epub 2020 Jun 26.

Neurology Department, Reference Center for Lysosomal Diseases, Neurogenetics and Metabolism Unit, Hôpital Pitié-Salpêtrière, 47-83 boulevard de l'Hôpital, 75013, Paris, France.

Late-onset Niemann-Pick type C (NP-C) is a rare, underdiagnosed lysosomal disease with neurological manifestations. A specific treatment, miglustat, can stabilize the disease if given early. Recently, three plasma screening biomarkers (PSBs) were developed [cholestane3β,5α,6βtriol (C-triol), 7-ketocholesterol (7-KC), and lysosphingomyelin-509 (LSM-509)], allowing a simpler and quite robust screening of patients suitable for genetic testing. Read More

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http://dx.doi.org/10.1007/s00415-020-10020-4DOI Listing

Longitudinal structural and metabolic changes in frontotemporal dementia.

Neurology 2020 Jul 26;95(2):e140-e154. Epub 2020 Jun 26.

From the Memory and Aging Center, Department of Neurology (A.B., G.T., G.M., Y.C., L.I., J.K., A.M.S., M.G.-T., B.L.M., A.L.B., H.J.R., G.D.R.), and Department of Radiology and Biomedical Imaging (G.D.R.), University of California San Francisco; Frontotemporal Disorders Unit (B.C.D.), Department of Neurology, Massachusetts General Hospital, Boston; and Harvard Medical School, Charleston; Department of Neurology (B.F.B., D.S.K.), Mayo Clinic, Rochester, MN; Molecular Biophysics and Integrated Bioimaging Division (W.J.J., G.D.R.), Lawrence Berkeley National Laboratory, CA; and Helen Wills Neuroscience Institute (G.D.R.), University of California Berkeley.

Objective: To compare the sensitivity of structural MRI and F-fludeoxyglucose PET (FDG-PET) to detect longitudinal changes in frontotemporal dementia (FTD).

Methods: Thirty patients with behavioral variant FTD (bvFTD), 7 with nonfluent/agrammatic variant primary progressive aphasia (nfvPPA), 16 with semantic variant primary progressive aphasia (svPPA), and 43 cognitively normal controls underwent 2-4 MRI and FDG-PET scans (total scans/visit = 270) as part of the Frontotemporal Lobar Degeneration Neuroimaging Initiative study. Linear mixed-effects models were carried out voxel-wise and in regions of interest to identify areas showing decreased volume or metabolism over time in patients as compared to controls. Read More

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http://dx.doi.org/10.1212/WNL.0000000000009760DOI Listing

The effect of semantic memory degeneration on creative thinking: A voxel-based morphometry analysis.

Neuroimage 2020 Jun 20;220:117073. Epub 2020 Jun 20.

The University of Sydney, School of Psychology and Brain and Mind Centre, Camperdown, NSW, 2050, Australia. Electronic address:

Increasing attention is being directed towards explicating the neurocognitive mechanisms of divergent thinking. While neuroimaging studies have tended to dominate the contemporary creativity literature, lesion studies provide important converging evidence by revealing the regions that are not only implicated in, but essential for, task performance. Here we explored the capacity for divergent thinking in semantic dementia (SD), a neurodegenerative disorder characterised by the progressive degeneration of the conceptual knowledge base. Read More

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http://dx.doi.org/10.1016/j.neuroimage.2020.117073DOI Listing

DSC Brain Perfusion Using Advanced Deconvolution Models in the Diagnostic Work-up of Dementia and Mild Cognitive Impairment: A Semiquantitative Comparison with HMPAO-SPECT-Brain Perfusion.

J Clin Med 2020 06 9;9(6). Epub 2020 Jun 9.

Departments of Neuroradiology, Friedrich-Alexander-University Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.

Background: SPECT (single-photon emission-computed tomography) is used for the detection of hypoperfusion in cognitive impairment and dementia but is not widely available and related to radiation dose exposure. We compared the performance of DSC (dynamic susceptibility contrast) perfusion using semi- and fully adaptive deconvolution models to HMPAO-SPECT (99mTc-hexamethylpropyleneamine oxime-SPECT).

Material And Methods: Twenty-seven patients with dementia of different subtypes including frontotemporal dementia (FTD) and mild cognitive impairment (MCI) received a multimodal diagnostic work-up including DSC perfusion at a clinical 3T high-field scanner and HMPAO-SPECT. Read More

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http://dx.doi.org/10.3390/jcm9061800DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7356248PMC

[Evaluation of Semantic Dementia Patient].

Authors:
Kenjiro Komori

Brain Nerve 2020 Jun;72(6):593-610

Room of Psychology, Juzen-Yurinoki Hospital.

Semantic dementia (SD) is a clinical syndrome characterized by selective and progressive semantic memory impairment due to frontotemporal lobar degeneration (FTLD). Semantic memory disorders appear in every cognitive fields, be it language or recognition of familiar people and objects. Left-right asymmetry produces a distinctive clinical symptom of anterior temporal lobe atrophy. Read More

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http://dx.doi.org/10.11477/mf.1416201570DOI Listing

[Symptomatology of Behavioral Variant of Frontotemporal Dementia].

Brain Nerve 2020 Jun;72(6):585-592

Department of Psychiatry, The Jikei University School of Medicine.

In patients with the behavioral variant of frontotemporal dementia, the core clinical phenotype of frontotemporal lobar degeneration, various social behaviors such as disinhibition, apathy, lack of empathy, stereotypy, and changes in eating behavior occur from the onset of the disease, and progresses slowly with frontal lobe damage. Because there are no disease-specific biomarkers, the diagnosis of frontotemporal dementia is based on the evaluation of behavioral symptoms, with neuroimaging methods and cognitive tests as assisting methods. Although diagnostic criteria are useful, frontotemporal dementia may be difficult to differentiate from other conditions, including mental illnesses. Read More

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http://dx.doi.org/10.11477/mf.1416201569DOI Listing

Tau PET Imaging with [18F]PM-PBB3 in Frontotemporal Dementia with MAPT Mutation.

J Alzheimers Dis 2020 ;76(1):149-157

Department of Neurology, National Clinical Research Centre for Aging and Medicine, Huashan Hospital, State Key Laboratory of Medical Neurobiology, Fudan University, Shanghai, China.

Background: Flortaucipir (AV-1451) and pyridinyl-butadienyl-benzothiazole 3 (PBB3) are newly developed and commonly used positron emission tomography (PET) tracers to detect tau deposition in tauopathies, including frontotemporal dementia (FTD). [18F]PM-PBB3, as a second-generation compound, has not been described in FTD so far.

Objective: We aim to explore the in vivo performance of [18F]PM-PBB3 tau PET in an FTD case caused by microtubule-associated protein tau (MAPT) mutation and compare the binding to different tau strains between AV-1451 and PBB3. Read More

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http://dx.doi.org/10.3233/JAD-200287DOI Listing
January 2020

A snake that bites its own tail. Acquisition and loss of concepts in children and semantic dementia patients through the analysis of drawings.

Cortex 2020 Jul 2;128:162-173. Epub 2020 Apr 2.

Neurology Service and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), 'Marqués de Valdecilla' University Hospital, University of Cantabria, Institute for Research 'Marqués de Valdecilla' (IDIVAL), Santander, Spain.

Objective: The structure of the semantic network is constructed and organized during childhood development. Previous publications have hypothesized that neurodegenerative diseases would lead to a disruption of this network reversing the steps acquired in childhood. Semantic Dementia (SD) is a subtype of frontotemporal lobe degeneration in which the main symptom is a specific loss of semantic memory. Read More

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http://dx.doi.org/10.1016/j.cortex.2020.03.007DOI Listing

Psychotic symptoms in frontotemporal dementia with right frontotemporal atrophy.

Asian J Psychiatr 2020 Apr 18;52:102040. Epub 2020 Apr 18.

Geriatric Clinic & Services, Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bangalore, 560029, India. Electronic address:

Frontotemporal dementia (FTD) often mimics a primary psychiatric disorder. A subset of patients with FTD presents with psychotic symptoms either during the course of illness and less often prior to the onset of cognitive decline. This leads to delay in diagnosis and inappropriate exposure to high dose antipsychotic medication. Read More

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http://dx.doi.org/10.1016/j.ajp.2020.102040DOI Listing

Crossing Borders Between Frontotemporal Dementia and Psychiatric Disorders: An Updated Overview.

J Alzheimers Dis 2020 ;75(2):661-673

Department of Mental Health, Department of Biomedical and Clinical Sciences Luigi Sacco, University of Milan, Milan, Italy.

Frontotemporal dementia (FTD) includes a group of neurocognitive syndromes, clinically characterized by altered behaviors, impairment of language proficiency, and altered executive functioning. FTD is one of the most frequently observed forms of dementia in the elderly population and the most common in presenile age. As for other subtypes of dementia, FTD incidence is constantly on the rise due to the steadily increasing age of the population, and its recognition is now becoming a determinant for clinicians. Read More

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http://dx.doi.org/10.3233/JAD-191333DOI Listing
January 2020

Structural Brain Changes in Pre-Clinical FTD MAPT Mutation Carriers.

J Alzheimers Dis 2020 ;75(2):595-606

Department of Neurology, Columbia University, Cognitive Neuroscience Division of the Taub Institute, G.H. Sergievsky Center, New York, NY, USA.

Background: Frontotemporal dementia (FTD) is the second most common cause of early-onset neurodegenerative dementia. Several studies have focused on early imaging changes in FTD patients, but once subjects meet full criteria for FTD diagnosis, structural changes are generally widespread.

Objective: This study aims to determine the earliest structural brain changes in asymptomatic MAPT MUTATION carriers. Read More

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http://dx.doi.org/10.3233/JAD-190820DOI Listing
January 2020

Temporal variant of frontotemporal dementia in C9orf72 repeat expansion carriers: two case studies.

Brain Imaging Behav 2020 Apr;14(2):336-345

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, IRCCS Ospedale San Raffaele, Via Olgettina, 60, 20132, Milan, Italy.

The temporal variant of frontotemporal dementia (tv-FTD) is a progressive neurodegenerative disease with a complex clinical picture mainly characterized by behavioral and language disorders. In this work, we describe clinical, genetic, neuroanatomical and neuropathological (only in one case) features of two patients with tv-FTD carrying C9orf72 repeat expansion. The first patient (AB) presented with a 1-year disease duration showing focal right anterior temporal lobe (ATL) atrophy on magnetic resonance imaging (MRI). Read More

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http://dx.doi.org/10.1007/s11682-019-00253-xDOI Listing

Anti-Cholinergic Derangement of Cortical Metabolism on 18F-FDG PET in a Patient with Frontotemporal Lobar Degeneration Dementia: A Case of the TREDEM Registry.

J Alzheimers Dis 2020 ;74(4):1107-1117

Department of Neuroscience Imaging and Clinical Sciences and CESI, University G D'Annunzio of Chieti-Pescara, Chieti, Italy.

We present the case of a patient with an atypical course of frontotemporal lobar degeneration (FTLD) complicated by the use of an anticholinergic drug. A 70-year-old patient, followed by psychiatrists for depression and behavioral disorders, received a diagnosis of dementia with Lewy bodies (DLB) at another Center due to auditory hallucinations, gait impairment, and tendency to fall. He was then admitted to our Memory Clinic Unit for behavioral disturbances, such as delusional thinking, auditory hallucinations, and memory complaints. Read More

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http://dx.doi.org/10.3233/JAD-191290DOI Listing
January 2020

Expression of mutant CHMP2B linked to neurodegeneration in humans disrupts circadian rhythms in .

FASEB Bioadv 2019 Aug 11;1(8):511-520. Epub 2019 Jul 11.

Department of Biology Colby College Waterville Maine.

Mutations in CHMP2B, an ESCRT-III (endosomal sorting complexes required for transport) component, are associated with frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Neurodegenerative disorders including FTD are also associated with a disruption in circadian rhythms, but the mechanism underlying this defect is not well understood. Here, we ectopically expressed the human CHMP2B variant associated with FTD (CHMP2B) in flies using the -GAL4 driver (>CHMP2B) and analyzed their circadian rhythms at behavioral, cellular, and biochemical level. Read More

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http://dx.doi.org/10.1096/fba.2019-00042DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6996329PMC

Progressive supranuclear palsy presenting with hyperkinetic movement disorder and hemiplegic dystonia: a case report.

Int J Neurosci 2020 Mar 5:1-4. Epub 2020 Mar 5.

Department of Neurobiology, Neurology and Geriatrics, Xuanwu Hospital of Capital Medical University, Beijing Institute of Geriatrics, Beijing, China.

Progressive supranuclear palsy (PSP) is a progressive neurodegenerative brain disease which has been rarely described in association with hyperkinetic symptoms. Here, we report a case of PSP that was presented with hyperkinetic movement disorder, hemiplegic dystonia, and other clinical features that overlap with behavioral variant frontotemporal dementia (bvFTD) and corticobasal syndrome (CBS). A 63-year-old female presented to our hospital with a history of frontal lobe symptoms, impaired cognition, hyperkinetic movement disorders, dystonia, and frequent falls. Read More

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http://dx.doi.org/10.1080/00207454.2020.1732965DOI Listing

Novel tau filament fold in corticobasal degeneration.

Nature 2020 04 12;580(7802):283-287. Epub 2020 Feb 12.

MRC Laboratory of Molecular Biology, Cambridge, UK.

Corticobasal degeneration (CBD) is a neurodegenerative tauopathy-a class of disorders in which the tau protein forms insoluble inclusions in the brain-that is characterized by motor and cognitive disturbances. The H1 haplotype of MAPT (the tau gene) is present in cases of CBD at a higher frequency than in controls, and genome-wide association studies have identified additional risk factors. By histology, astrocytic plaques are diagnostic of CBD; by SDS-PAGE, so too are detergent-insoluble, 37 kDa fragments of tau. Read More

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http://dx.doi.org/10.1038/s41586-020-2043-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7148158PMC

Behavioural disturbances in patients with frontotemporal lobe degeneration focusing on caregiver burden at home and in nursing homes.

J Clin Nurs 2020 May 27;29(9-10):1733-1743. Epub 2020 Feb 27.

Department of Nursing and Health Promotion, Faculty of Health Sciences, Oslo Metropolitan University, Oslo, Norway.

Aim And Objective: To explore the challenges faced by family caregivers of people with frontotemporal dementia and other forms of dementia affecting the frontal and temporal lobes causing behavioural disturbances through a qualitative approach with in-depth interviews.

Background: Studies of different forms of dementia involving degeneration of the frontal and temporal lobes have mainly focused on the neurophysiology and physiology of the disease and on caregivers' health. Few studies have described the challenges and burdens connected with everyday life and in relation to suitable nursing home placement that are faced by family caregivers. Read More

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http://dx.doi.org/10.1111/jocn.15208DOI Listing

A modified Camel and Cactus Test detects presymptomatic semantic impairment in genetic frontotemporal dementia within the GENFI cohort.

Appl Neuropsychol Adult 2020 Feb 5:1-8. Epub 2020 Feb 5.

Department of Neurodegenerative Disease, Dementia Research Centre, UCL Institute of Neurology, London, UK.

Impaired semantic knowledge is a characteristic feature of some forms of frontotemporal dementia (FTD), particularly the sporadic disorder semantic dementia. Less is known about semantic cognition in the genetic forms of FTD caused by mutations in the genes , , and . We developed a modified version of the Camel and Cactus Test (mCCT) to investigate the presence of semantic difficulties in a large genetic FTD cohort from the Genetic FTD Initiative (GENFI) study. Read More

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http://dx.doi.org/10.1080/23279095.2020.1716357DOI Listing
February 2020

Speech and Language Presentations of FTLD-TDP Type B Neuropathology.

J Neuropathol Exp Neurol 2020 03;79(3):277-283

From the Mesulam Center for Cognitive Neurology and Alzheimer's Disease.

Four right-handed patients who presented with an isolated impairment of speech or language had transactive response DNA-binding protein of 43 kDa (TDP-43) type B pathology. Comportment and pyramidal motor function were preserved at presentation. Three of the cases developed axial rigidity and oculomotor findings late in their course with no additional pyramidal or lower motor neuron impairments. Read More

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http://dx.doi.org/10.1093/jnen/nlz132DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7036652PMC

Plasma glial fibrillary acidic protein is raised in progranulin-associated frontotemporal dementia.

J Neurol Neurosurg Psychiatry 2020 Mar 14;91(3):263-270. Epub 2020 Jan 14.

Dementia Research Centre, UCL Queen Square Institute of Neurology, London, UK

Background: There are few validated fluid biomarkers in frontotemporal dementia (FTD). Glial fibrillary acidic protein (GFAP) is a measure of astrogliosis, a known pathological process of FTD, but has yet to be explored as potential biomarker.

Methods: Plasma GFAP and neurofilament light chain (NfL) concentration were measured in 469 individuals enrolled in the Genetic FTD Initiative: 114 expansion carriers (74 presymptomatic, 40 symptomatic), 119 mutation carriers (88 presymptomatic, 31 symptomatic), 53 mutation carriers (34 presymptomatic, 19 symptomatic) and 183 non-carrier controls. Read More

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http://dx.doi.org/10.1136/jnnp-2019-321954DOI Listing
March 2020
6.807 Impact Factor

Corticobasal degeneration with deep white matter lesion diagnosed by brain biopsy.

Neuropathology 2020 Jun 10;40(3):287-294. Epub 2020 Jan 10.

Department of Neurology, Tokyo Teishin Hospital, Tokyo, Japan.

Corticobasal degeneration (CBD) is a rare progressive neurodegenerative disorder characterized by asymmetric presentation of cerebral cortex signs, cortical sensory disturbance and extrapyramidal signs. Herein, we report a case of a 66-year-old Japanese woman who presented with apraxia of the right hand. She subsequently developed postural instability and cognitive impairments that rapidly worsened. Read More

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http://dx.doi.org/10.1111/neup.12638DOI Listing

Trajectory of lobar atrophy in asymptomatic and symptomatic GRN mutation carriers: a longitudinal MRI study.

Neurobiol Aging 2020 04 12;88:42-50. Epub 2019 Dec 12.

Department of Radiology, Mayo Clinic, Rochester, MN, USA; Alzheimer's Disease Research Center, Mayo Clinic, Rochester, MN, USA. Electronic address:

Loss-of-function mutations in the progranulin gene (GRN) are one of the major causes of familial frontotemporal lobar degeneration. Our objective was to determine the rates and trajectories of lobar cortical atrophy from longitudinal structural magnetic resonance imaging in both asymptomatic and symptomatic GRN mutation carriers. Individuals in this study were from the ADRC and LEFFTDS studies at the Mayo Clinic. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2019.12.004DOI Listing

Scene construction impairments in frontotemporal dementia: Evidence for a primary hippocampal contribution.

Neuropsychologia 2020 02 27;137:107327. Epub 2019 Dec 27.

The University of Sydney, School of Psychology and Brain and Mind Centre, Sydney, New South Wales, Australia; Australian Research Council Centre of Excellence in Cognition and Its Disorders, Sydney, New South Wales, Australia. Electronic address:

The capacity to generate naturalistic three-dimensional and spatially coherent representations of the world, i.e., scene construction, is posited to lie at the heart of a wide range of complex cognitive endeavours. Read More

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http://dx.doi.org/10.1016/j.neuropsychologia.2019.107327DOI Listing
February 2020

Two pathologically confirmed cases of novel mutations in the MAPT gene causing frontotemporal dementia.

Neurobiol Aging 2020 03 20;87:141.e15-141.e20. Epub 2019 Nov 20.

Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, London, UK. Electronic address:

MAPT mutations were the first discovered genetic cause of frontotemporal dementia (FTD) in 1998. Since that time, over 60 MAPT mutations have been identified, usually causing behavioral variant FTD and/or parkinsonism clinically. We describe 2 novel MAPT mutations, D252V and G389_I392del, each presenting in a patient with behavioral variant FTD and associated language and cognitive deficits. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2019.11.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7082764PMC

Binding of [F]AV1451 in post mortem brain slices of semantic variant primary progressive aphasia patients.

Eur J Nucl Med Mol Imaging 2020 Jul 18;47(8):1949-1960. Epub 2019 Dec 18.

Laboratory for Cognitive Neurology, Department of Neurosciences, KU Leuven, Herestraat 49, 3000, Leuven, Belgium.

Purpose: In vivo tau-PET tracer retention in the anterior temporal lobe of patients with semantic variant primary progressive aphasia (SV PPA) has consistently been reported. This is unexpected as the majority of these patients have frontotemporal lobar degeneration TDP (FTLD-TDP).

Methods: We conducted an in vitro [F]AV1451 autoradiography binding study in five cases with a clinical diagnosis of SV PPA constituting the range of pathologies (i. Read More

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http://dx.doi.org/10.1007/s00259-019-04631-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7300115PMC
July 2020
5.383 Impact Factor

The behavioral dyscontrol scale in the differential diagnosis of behavioral variant of frontotemporal dementia and Alzheimer disease.

Clin Neuropsychol 2019 Dec 16:1-10. Epub 2019 Dec 16.

Department of Psychiatry and Human Behavior, Brown University, Providence, RI, USA.

The Behavioral Dyscontrol Scale (BDS) is a brief measure of frontal systems that is adopted from Luria's syndrome analysis approach. The aim of this study was to evaluate the diagnostic utility of the BDS as an objective measure of self-regulation in behavioral variant of frontotemporal dementia (bvFTD) and Alzheimer's disease (AD). Two patient groups matched in education and dementia severity (n = 21 bvFTD and 21 AD) recruited from a memory clinic and a matched normal control (NC) group (n = 21) were administered a battery of neuropsychological tests including the BDS. Read More

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http://dx.doi.org/10.1080/13854046.2019.1701709DOI Listing
December 2019

Frontotemporal dementia: an unusual cause.

Int J Neurosci 2020 Jul 20;130(7):736-738. Epub 2019 Dec 20.

Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.

Intracerebral pneumocephalus is commonly associated with head and facial trauma, ear infection, tumors and surgical interventions. Osteomas are relatively common, benign tumors that occur mainly in the paranasal sinuses, the frontal sinus in particular. Pneumocephalus has been commonly reported with frontal osteoma but isolated presentation as frontotemporal dementia is uncommon. Read More

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http://dx.doi.org/10.1080/00207454.2019.1702538DOI Listing

Incidence of frontotemporal disorders in Olmsted County: A population-based study.

Alzheimers Dement 2020 03 4;16(3):482-490. Epub 2020 Jan 4.

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Introduction: Frontotemporal dementia disorders (FTDs) are heterogeneous phenotypical behavioral and language disorders usually associated with frontal and/or temporal lobe degeneration. We investigated their incidence in a population-based cohort.

Methods: Using a records-linkage system, we identified all patients with a diagnostic code for dementia in Olmsted County, MN, 1995-2010, and confirmed the diagnosis of FTD. Read More

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http://dx.doi.org/10.1016/j.jalz.2019.08.199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7067641PMC

Testing the therapeutic effects of transcranial direct current stimulation (tDCS) in semantic dementia: a double blind, sham controlled, randomized clinical trial.

Trials 2019 Nov 20;20(1):632. Epub 2019 Nov 20.

Institut du Cerveau et de la Moelle Epinière, ICM, INSERM U 1127, CNRS UMR 7225, Sorbonne Université, Frontlab team, Paris, France.

Background: Semantic dementia is a neurodegenerative disease that primarily affects the left anterior temporal lobe, resulting in a gradual loss of conceptual knowledge. There is currently no validated treatment. Transcranial stimulation has provided evidence for long-lasting language effects presumably linked to stimulation-induced neuroplasticity in post-stroke aphasia. Read More

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http://dx.doi.org/10.1186/s13063-019-3613-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6868701PMC
November 2019

A case of TDP-43 type C pathology presenting as nonfluent variant primary progressive aphasia.

Neurocase 2020 02 21;26(1):1-6. Epub 2019 Nov 21.

Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK.

We report a case of rapidly progressive nonfluent variant PPA (nfvPPA), age at onset 77 years old and disease duration 3.3 years, who came to post mortem and was found to have TDP-43 type C pathology, an unusual finding for nfvPPA. All prior TDP-43 type C cases from the UCL FTD cohort (n=25) had a semantic variant PPA (svPPA) phenotype, with all having a younger age at onset and longer disease duration than the nfvPPA case. Read More

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http://dx.doi.org/10.1080/13554794.2019.1690665DOI Listing
February 2020

Temporal Variant Frontotemporal Dementia.

Authors:
Kyum-Yil Kwon

Neurol India 2019 Sep-Oct;67(5):1395

Department of Neurology, Soonchunhyang University School of Medicine, Soonchunhyang University Seoul Hospital, Seoul, Korea.

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http://dx.doi.org/10.4103/0028-3886.271255DOI Listing

Hoarding and obsessive-compulsive behaviours in frontotemporal dementia: Clinical and neuroanatomic associations.

Cortex 2019 12 15;121:443-453. Epub 2019 Oct 15.

Department of Clinical Neurological Sciences, Schulich School of Medicine and Dentistry, University of Western Ontario, Canada; Robarts Research Institute, Canada; Lawson Health Research Institute, Canada; Parkwood Institute, St. Josephs Health Care, Canada. Electronic address:

Background: Hoarding and obsessive-compulsive behaviours (OCB) are well documented symptoms in frontotemporal dementia (FTD). While contemporary models consider hoarding and obsessive-compulsive disorder distinct, the related behaviours have not been separately examined in patients with FTD, and the neuroanatomical correlates of hoarding in patients with FTD have not been previously examined (American Psychiatric Association, 2013; Grisham and Baldwin, 2015; Mataix-Cols et al., 2010). Read More

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http://dx.doi.org/10.1016/j.cortex.2019.09.012DOI Listing
December 2019

Visual Search in Behavioral Variant Frontotemporal Dementia.

J Alzheimers Dis 2019 ;72(4):1303-1312

Department of Optometry and Vision Sciences, The University of Melbourne, Melbourne, Australia.

Background: Changes to visual search have shown specific patterns in a number of dementia subtypes. The cortical regions involved in the control of visual search overlap with the regions affected in behavioral variant frontotemporal dementia (bvFTD). Previous literature has examined visual search in bvFTD with smaller array sizes. Read More

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http://dx.doi.org/10.3233/JAD-190981DOI Listing
January 2019

Naming and Knowing Revisited: Eyetracking Correlates of Anomia in Progressive Aphasia.

Front Hum Neurosci 2019 11;13:354. Epub 2019 Oct 11.

Eleanor M. Saffran Center for Cognitive Neuroscience, Temple University, Philadelphia, PA, United States.

Progressive naming impairment (i.e., anomia) is a core diagnostic symptom of numerous pathologies that impact anterior and inferior portions of the temporal lobe. Read More

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http://dx.doi.org/10.3389/fnhum.2019.00354DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6797589PMC
October 2019
1 Read

Establishing links between abnormal eating behaviours and semantic deficits in dementia.

J Neuropsychol 2019 Oct 16. Epub 2019 Oct 16.

Neuroscience Area, SISSA, Trieste, Italy.

The hypothesis that semantic deficits in dementia may contribute in producing changes in eating preferences has never been experimentally investigated despite this association has been clinically observed. We administered tasks assessing semantic memory and the Appetite and Eating Habits Questionnaire (APEHQ) to 23 patients with dementia (behavioural frontotemporal dementia, primary progressive aphasia, and Alzheimer's disease) and to 21 healthy controls. We used voxel-based morphometry and diffusion tensor imaging to identify regions and white matter tracts of significant atrophy associated with the performance at the semantic tasks and the pathological scores at the APEHQ. Read More

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http://dx.doi.org/10.1111/jnp.12195DOI Listing
October 2019
2 Reads

Corticospinal tract degeneration and temporal lobe atrophy in frontotemporal lobar degeneration TDP-43 type C pathology.

Neuropathol Appl Neurobiol 2020 Apr 30;46(3):296-299. Epub 2019 Oct 30.

Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, UK.

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http://dx.doi.org/10.1111/nan.12582DOI Listing
April 2020
5 Reads

Inherited and Sporadic Amyotrophic Lateral Sclerosis and Fronto-Temporal Lobar Degenerations arising from Pathological Condensates of Phase Separating Proteins.

Hum Mol Genet 2019 11;28(R2):R187-R196

Cambridge Institute for Medical Research, Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK, CB2 0XY.

Recent work on the biophysics of proteins with low complexity, intrinsically disordered domains that have the capacity to form biological condensates has profoundly altered the concepts about the pathogenesis of inherited and sporadic neurodegenerative disorders associated with pathological accumulation of these proteins. In the present review, we use the FUS, TDP-43 and A11 proteins as examples to illustrate how missense mutations and aberrant post-translational modifications of these proteins cause amyotrophic lateral sclerosis (ALS) and fronto-temporal lobar degeneration (FTLD). Read More

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http://dx.doi.org/10.1093/hmg/ddz162DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6872449PMC
November 2019
1 Read

Extensive transcriptomic study emphasizes importance of vesicular transport in C9orf72 expansion carriers.

Acta Neuropathol Commun 2019 10 8;7(1):150. Epub 2019 Oct 8.

Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.

The majority of the clinico-pathological variability observed in patients harboring a repeat expansion in the C9orf72-SMCR8 complex subunit (C9orf72) remains unexplained. This expansion, which represents the most common genetic cause of frontotemporal lobar degeneration (FTLD) and motor neuron disease (MND), results in a loss of C9orf72 expression and the generation of RNA foci and dipeptide repeat (DPR) proteins. The C9orf72 protein itself plays a role in vesicular transport, serving as a guanine nucleotide exchange factor that regulates GTPases. Read More

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https://actaneurocomms.biomedcentral.com/articles/10.1186/s4
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http://dx.doi.org/10.1186/s40478-019-0797-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6781370PMC
October 2019
7 Reads

Neurodegenerative disorders of the human frontal lobes.

Handb Clin Neurol 2019 ;163:391-410

Department of Neurology, UCSF Memory and Aging Center, UCSF School of Medicine, San Francisco, CA, United States. Electronic address:

The frontal lobes play an integral role in human socioemotional and cognitive function. Sense of self, moral decisions, empathy, and behavioral monitoring of goal-states all depend on key nodes within frontal cortex. While several neurodegenerative diseases can affect frontal function, frontotemporal dementia (FTD) has particularly serious and specific effects, which thus provide insights into the role of frontal circuits in homeostasis and adaptive behavior. Read More

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http://dx.doi.org/10.1016/B978-0-12-804281-6.00021-5DOI Listing
February 2020
1 Read

Frontal lobe syndromes.

Handb Clin Neurol 2019 ;163:147-164

Department of Neurology, University of Iowa College of Medicine, Iowa City, IA, United States. Electronic address:

The frontal lobes contain a complex set of diverse anatomic regions that form multiple distinct, complex networks with cortical and subcortical regions. Damage to these cortical-subcortical networks can have dramatic behavioral consequences, ranging from apathy to impairments in executive functioning. This chapter provides a brief overview of the common syndromes caused by damage to the mediodorsal and dorsolateral prefrontal circuits, followed by a more detailed review of the syndrome-sometimes referred to as pseudopsychopathy or acquired sociopathy-associated with damage to the ventromedial prefrontal circuit. Read More

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http://dx.doi.org/10.1016/B978-0-12-804281-6.00008-2DOI Listing
February 2020

Antemortem volume loss mirrors TDP-43 staging in older adults with non-frontotemporal lobar degeneration.

Brain 2019 11;142(11):3621-3635

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Over the past decade, the transactive response DNA-binding protein of 43 kDa (TDP-43) has been recognized as a major protein in normal and pathological ageing, increasing the risk of cognitive impairment and dementia. In conditions distinct from the frontotemporal lobar degenerations, TDP-43 appears to progress in a stereotypical pattern. In the present study, we aimed at providing a better understanding of the effects of TDP-43 and other age-related neuropathologies on cross-sectional grey matter volume in a cohort of non-FTLD subjects. Read More

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http://dx.doi.org/10.1093/brain/awz277DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6821218PMC
November 2019
3 Reads

A Brief Drawing Task for the Differential Diagnosis of Semantic Dementia.

J Alzheimers Dis 2019 ;72(1):151-160

Neurology Service and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), 'Marqués de Valdecilla' University Hospital, University of Cantabria, Institute for Research 'Marqués de Valdecilla' (IDIVAL), Santander, Spain.

Background: Semantic dementia (SD) is a subtype of frontotemporal lobe degeneration characterized by semantic loss, with other cognitive functions initially preserved. SD requires differential diagnosis with Alzheimer's disease (AD) and behavioral variant frontotemporal dementia (bvFTD). Semantic knowledge can be evaluated through different tests; however, most of them depend on language. Read More

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http://dx.doi.org/10.3233/JAD-190660DOI Listing
January 2019
1 Read

[18F]FDG-PET in a case of right temporal lobe variant of frontotemporal dementia.

Dement Neuropsychol 2019 Jul-Sep;13(3):350-351

University of São Paulo School of Medicine Hospital das Clínicas Department of Neurology São PauloSP Brazil Hospital das Clínicas, University of São Paulo School of Medicine, Department of Neurology, São Paulo, SP, Brazil.

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http://dx.doi.org/10.1590/1980-57642018dn13-030013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6753909PMC
September 2019
1 Read

Diagnostic imaging of dementia with Lewy bodies, frontotemporal lobar degeneration, and normal pressure hydrocephalus.

Authors:
Kazunari Ishii

Jpn J Radiol 2020 Jan 23;38(1):64-76. Epub 2019 Sep 23.

Department of Radiology, Kindai University Faculty of Medicine, 377-2 Ohnohigashi, Osakasayama, Osaka, 589-8511, Japan.

Neuroimaging can provide important biomarkers and is very useful for supporting dementia diagnosis. This review summarizes the neuroimaging findings of dementia with Lewy bodies (DLB), frontotemporal lobar degeneration (FTLD), and normal pressure hydrocephalus (NPH). In DLB, medial temporal atrophy is milder than that of Alzheimer's disease. Read More

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http://dx.doi.org/10.1007/s11604-019-00881-9DOI Listing
January 2020
4 Reads