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Frontotemporal dementia

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https://www.readbyqxmd.com/read/28229125/the-clinical-neuroanatomical-and-neuropathologic-phenotype-of-tbk1-associated-frontotemporal-dementia-a-longitudinal-case-report
#1
Carolin A M Koriath, Martina Bocchetta, Emilie Brotherhood, Ione O C Woollacott, Penny Norsworthy, Javier Simón-Sánchez, Cornelis Blauwendraat, Katrina M Dick, Elizabeth Gordon, Sophie R Harding, Nick C Fox, Sebastian Crutch, Jason D Warren, Tamas Revesz, Tammaryn Lashley, Simon Mead, Jonathan D Rohrer
INTRODUCTION: Mutations in the TANK-binding kinase 1 (TBK1) gene have recently been shown to cause frontotemporal dementia (FTD). However, the phenotype of TBK1-associated FTD is currently unclear. METHODS: We performed a single case longitudinal study of a patient who was subsequently found to have a novel A705fs mutation in the TBK1 gene. He was assessed annually over a 7-year period with a series of clinical, cognitive, and magnetic resonance imaging assessments...
2017: Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
https://www.readbyqxmd.com/read/28225838/-mental-simulation-in-frontotemporal-dementia
#2
Daniel G Politis, Wanda Y Rubinstein, María E Tabernero
: There is a common network for perception and execution of actions necessary for the acquisition of Theory of Mind, and the mirror neuron system could be the neural substrate. OBJECTIVE: To study the presence of apraxia and their relationship to Theory of Mind in patients with behavioral variant of Frontotemporal Dementia. METHODS: 24 patients were assessed, and the cognitive praxis assessment battery and theory of mind were administered...
May 2016: Vertex: Revista Argentina de Psiquiatriá
https://www.readbyqxmd.com/read/28222300/free-and-cued-selective-reminding-test-accuracy-for-alzheimer-s-and-neurodegenerative-disease-differential-diagnosis-a-large-scale-biomarker-characterized-monocenter-cohort-study-clinad
#3
Marc Teichmann, Stéphane Epelbaum, Dalila Samri, Marcel Levy Nogueira, Agnès Michon, Harald Hampel, Foudil Lamari, Bruno Dubois
INTRODUCTION: The International Working Group recommended the Free and Cued Selective Reminding Test (FCSRT) as a sensitive detector of the amnesic syndrome of the hippocampal type in typical Alzheimer's disease (AD). But does it differentiate AD from other neurodegenerative diseases? METHODS: We assessed the FCSRT and cerebrospinal fluid (CSF) AD biomarkers in 992 cases. Experts, blinded to biomarker data, attributed in 650 cases a diagnosis of typical AD, frontotemporal dementia, posterior cortical atrophy, Lewy body disease, progressive supranuclear palsy, corticobasal syndrome, primary progressive aphasias, "subjective cognitive decline," or depression...
February 18, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28219620/brain-structural-correlates-of-executive-and-social-cognition-profiles-in-behavioral-variant-frontotemporal-dementia-and-elderly-bipolar-disorder
#4
Sandra Baez, Clara Pinasco, María Roca, Jesica Ferrari, Blas Couto, Indira García-Cordero, Agustín Ibañez, Francy Cruz, Pablo Reyes, Diana Matallana, Facundo Manes, Marcelo Cetcovich, Teresa Torralva
An early stage of behavioral variant frontotemporal dementia (bvFTD) often displays a mix of behavioral disturbances and personality changes hindering a differential diagnosis from elderly bipolar disorder (BD), making this process a big challenge. However, no studies have compared these pathologies from neuropsychological and neuroanatomical perspectives. The aim of the present study was to compare the executive functions (EF) and social cognition profiles as well as the structural neuroimaging of bvFTD and elderly patients with BD...
February 17, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28217760/sleep-in-alzheimer-s-disease-beyond-amyloid
#5
Jerrah Holth, Tirth Patel, David M Holtzman
Sleep disorders are prevalent in Alzheimer's disease (AD) and a major cause of institutionalization. Like AD pathology, sleep abnormalities can appear years before cognitive decline and may be predictive of dementia. A bidirectional relationship between sleep and amyloid β (Aβ) has been well established with disturbed sleep and increased wakefulness leading to increased Aβ production and decreased Aβ clearance; whereas Aβ deposition is associated with increased wakefulness and sleep disturbances. Aβ fluctuates with the sleep wake cycle and is higher during wakefulness and lower during sleep...
January 2017: Neurobiology of Sleep and Circadian Rhythms
https://www.readbyqxmd.com/read/28216144/patient-ipsc-derived-neurons-for-disease-modeling-of-frontotemporal-dementia-with-mutation-in-chmp2b
#6
Yu Zhang, Benjamin Schmid, Nanett K Nikolaisen, Mikkel A Rasmussen, Blanca I Aldana, Mikkel Agger, Kirstine Calloe, Tina C Stummann, Hjalte M Larsen, Troels T Nielsen, Jinrong Huang, Fengping Xu, Xin Liu, Lars Bolund, Morten Meyer, Lasse K Bak, Helle S Waagepetersen, Yonglun Luo, Jørgen E Nielsen, Bjørn Holst, Christian Clausen, Poul Hyttel, Kristine K Freude
The truncated mutant form of the charged multivesicular body protein 2B (CHMP2B) is causative for frontotemporal dementia linked to chromosome 3 (FTD3). CHMP2B is a constituent of the endosomal sorting complex required for transport (ESCRT) and, when mutated, disrupts endosome-to-lysosome trafficking and substrate degradation. To understand the underlying molecular pathology, FTD3 patient induced pluripotent stem cells (iPSCs) were differentiated into forebrain-type cortical neurons. FTD3 neurons exhibited abnormal endosomes, as previously shown in patients...
February 6, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28214109/a-novel-mutation-in-trem2-gene-causing-nasu-hakola-disease-and-review-of-the-literature
#7
Efthimios Dardiotis, Vasileios Siokas, Eva Pantazi, Maria Dardioti, Dimitrios Rikos, Georgia Xiromerisiou, Aikaterini Markou, Dimitra Papadimitriou, Matthaios Speletas, Georgios M Hadjigeorgiou
Nasu-hakola disease (NHD) is a rare disease characterized by bone cysts and fractures, frontal lobe syndrome, and progressive presenile dementia. NHD may be the prototype of primary microglial disorders of the CNS or, as they have been coined, "microgliopathies". Mutations in TREM2 and TYROBP genes are known to cause NHD. Interestingly, recent evidence-associated rare genetic variants of TREM2 gene with increased risk of Alzheimer's disease, frontotemporal dementia, amyotrophic lateral sclerosis, and Parkinson's disease...
January 20, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28211814/are-major-dementias-triggered-by-poor-blood-flow-to-the-brain-theoretical-considerations
#8
Jack C de la Torre
There is growing evidence that chronic brain hypoperfusion plays a central role in the development of Alzheimer's disease (AD) long before dyscognitive symptoms or amyloid-β accumulation in the brain appear. This commentary proposes that dementia with Lewy bodies (DLB), frontotemporal dementia (FTD), and Creutzfeldt-Jakob disease (CJD) may also develop from chronic brain hypoperfusion following a similar but not identical neurometabolic breakdown as AD. The argument to support this conclusion is that chronic brain hypoperfusion, which is found at the early stages of the three dementias reviewed here, will reduce oxygen delivery and lower oxidative phosphorylation promoting a steady decline in the synthesis of the cell energy fuel adenosine triphosphate (ATP)...
February 15, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28210978/future-directions-in-imaging-neurodegeneration
#9
REVIEW
Joseph C Masdeu
Neuroimaging comprises a powerful set of instruments to diagnose various neurodegenerative disorders, clarifies their neurobiology, and monitors their treatment. Magnetic resonance imaging depicts volume changes, as well as abnormalities in functional and structural connectivity. Positron emission tomography (PET) allows for the quantification of regional cerebral metabolism, characteristically altered in Alzheimer's disease, amyotrophic lateral sclerosis, diffuse Lewy-body disease, and the frontotemporal dementias...
January 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28209520/deconstructing-empathy-neuroanatomical-dissociations-between-affect-sharing-and-prosocial-motivation-using-a-patient-lesion-model
#10
Suzanne M Shdo, Kamalini G Ranasinghe, Kelly A Gola, Clinton J Mielke, Paul V Sukhanov, Bruce L Miller, Katherine P Rankin
Affect sharing and prosocial motivation are integral parts of empathy that are conceptually and mechanistically distinct. We used a neurodegenerative disease (NDG) lesion model to more directly examine the neural correlates of these two aspects of real-world empathic responding. The study enrolled 275 participants, including 44 healthy older controls and 231 patients diagnosed with one of five neurodegenerative diseases (75 Alzheimer's disease, 58 behavioral variant frontotemporal dementia (bvFTD), 42 semantic variant primary progressive aphasia (svPPA), 28 progressive supranuclear palsy, and 28 non-fluent variant (nfvPPA)...
February 13, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28205215/multicenter-validation-of-an-mmse-moca-conversion-table
#11
David Bergeron, Kelsey Flynn, Louis Verret, Stéphane Poulin, Rémi W Bouchard, Christian Bocti, Tamàs Fülöp, Guy Lacombe, Serge Gauthier, Ziad Nasreddine, Robert Jr Laforce
BACKGROUND: Accumulating evidence points to the superiority of the MoCA over the MMSE as a cognitive screening tool. To facilitate the transition from the MMSE to the MoCA in clinical and research settings, authors have developed MMSE-MoCA conversion tables. However, it is unknown whether a conversion table generated from Alzheimer's disease (AD) patients would apply to patients with other dementia subtypes like vascular dementia or frontotemporal dementia. Furthermore, the reliability and accuracy of MMSE-MoCA conversion tables has not been properly evaluated...
February 15, 2017: Journal of the American Geriatrics Society
https://www.readbyqxmd.com/read/28199994/association-between-montreal-cognitive-assessment-sub-item-scores-and-corresponding-cognitive-test-performance-in-patients-with-frontotemporal-dementia-and-related-disorders
#12
Kristy K L Coleman, Brenda L Coleman, Julia D MacKinley, Stephen H Pasternak, Elizabeth C Finger
The Montreal Cognitive Assessment (MoCA), a brief screening test developed to detect patients with mild cognitive impairment, is used in clinical settings across North America [Nasreddine et al.: J Am Geriatr Soc 2005;53:695-699]. The MoCA has been demonstrated to be sensitive to cognitive deficits in frontotemporal dementias (FTD) and related disorders [Coleman et al.: Alzheimer Dis Assoc Disord 2016;30:258-263]. Given attentional impairments in patients with FTD, whether and to what extent the abbreviated items on the MoCA may predict performance on corresponding assessments is not known...
February 16, 2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28197542/the-proline-arginine-dipeptide-from-hexanucleotide-repeat-expanded-c9orf72-inhibits-the-proteasome
#13
Rahul Gupta, Matthews Lan, Jelena Mojsilovic-Petrovic, Won Hoon Choi, Nathaniel Safren, Sami Barmada, Min Jae Lee, Robert Kalb
An intronic hexanucleotide repeat expansion (HRE) mutation in the C9ORF72 gene is the most common cause of familial ALS and frontotemporal dementia (FTD) and is found in ∼7% of individuals with apparently sporadic disease. Several different diamino acid peptides can be generated from the HRE by noncanonical translation (repeat-associated non-ATG translation, or RAN translation), and some of these peptides can be toxic. Here, we studied the effects of two arginine containing RAN translation products [proline/arginine repeated 20 times (PR20) and glycine/arginine repeated 20 times (GR20)] in primary rat spinal cord neuron cultures grown on an astrocyte feeder layer...
January 2017: ENeuro
https://www.readbyqxmd.com/read/28196768/cerebrospinal-fluid-markers-detect-alzheimer-s-disease-in-nonamnestic-dementia
#14
Carly Oboudiyat, Tamar Gefen, Eleni Varelas, Sandra Weintraub, Emily Rogalski, Eileen H Bigio, M-Marsel Mesulam
INTRODUCTION: The accuracy of cerebrospinal fluid (CSF) biomarkers for detecting Alzheimer's disease (AD) pathology has not been fully validated in autopsied nonamnestic dementias. METHODS: We retrospectively evaluated CSF β-amyloid 1-42, phosphorylated-tau, and amyloid-tau index as predictors of Alzheimer pathology in patients with primary progressive aphasia, frontotemporal dementia, and progressive supranuclear palsy. RESULTS: Nineteen nonamnestic autopsied cases with relevant CSF values were included...
February 11, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28192553/clinical-evidence-of-disease-anticipation-in-families-segregating-a-c9orf72-repeat-expansion
#15
Sara Van Mossevelde, Julie van der Zee, Ilse Gijselinck, Kristel Sleegers, Jan De Bleecker, Anne Sieben, Rik Vandenberghe, Tim Van Langenhove, Jonathan Baets, Olivier Deryck, Patrick Santens, Adrian Ivanoiu, Christiana Willems, Veerle Bäumer, Marleen Van den Broeck, Karin Peeters, Maria Mattheijssens, Peter De Jonghe, Patrick Cras, Jean-Jacques Martin, Marc Cruts, Peter P De Deyn, Sebastiaan Engelborghs, Christine Van Broeckhoven
Importance: Patients carrying a C9orf72 repeat expansion leading to frontotemporal dementia and/or amyotrophic lateral sclerosis have highly variable ages at onset of disease, suggesting the presence of modifying factors. Objective: To provide clinical-based evidence for disease anticipation in families carrying a C9orf72 repeat expansion by analyzing age at onset, disease duration, and age at death in successive generations. Design, Setting, and Participants: This cohort study was performed from June 16, 2000, to June 1, 2016, in 36 extended Belgian families in which a C9orf72 repeat expansion was segregating...
February 13, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28184974/-causes-of-death-in-amyotrophic-lateral-sclerosis-results-from-the-rhineland-palatinate-als-registry
#16
J Wolf, A Safer, J C Wöhrle, F Palm, W A Nix, M Maschke, A J Grau
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is associated with an increased mortality. Knowledge of possible causes of death could lead to an individualization of the palliative treatment concept and result in a differentiated palliative treatment pathway. Currently, only few systematic data are available on the heterogeneity of causes of death associated with ALS. OBJECTIVE: Analysis of the various causes of death in a prospective population-based German cohort of ALS patients...
February 9, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/28181891/-chronic-traumatic-encephalopathy-an-old-acquaintance-in-athletes
#17
E G B Vijverberg, A C M Pijnenburg, P Scheltens, Y A L Pijnenburg
- Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease caused by repetitive head injuries like those seen in sports such as boxing, American football and soccer.- The clinical features of CTE are a range of cognitive, psychiatric and motor symptoms, and histopathology involves deposits of hyperphosphorylated tau protein and the presence of TAR DNA-binding protein (TDP-43) with relatively little beta-amyloid.- CTE is difficult to differentiate clinically from Alzheimer's disease, frontotemporal dementia and psychiatric disorders because of the major symptom overlap between these conditions...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28176002/dysfunction-of-the-blood-cerebrospinal-fluid-barrier-and-n-methyl-d-aspartate-glutamate-receptor-antibodies-in-dementias
#18
Mandy Busse, Ralf Kunschmann, Henrik Dobrowolny, Jessica Hoffmann, Bernhard Bogerts, Johann Steiner, Thomas Frodl, Stefan Busse
N-Methyl-D-aspartate glutamate receptor (NMDA-R) antibodies (Abs) could play a role in neurodegenerative disorders. Since, in these diseases, NMDA-R Abs were detected in serum, but only sporadic in cerebrospinal fluid (CSF), the origin and impact of the Abs are still unresolved. We examined the presence of NMDA-R Abs in serum and CSF using a cell-based immunofluorescence assay as well as the function of the blood-CSF-barrier (B-CSF-B) by determination of Q albumin (ratio of albumin in CSF and serum) in patients with mild cognitive impairment (MCI; N = 59) and different types of dementia, Alzheimer's disease (AD; N = 156), subcortical ischemic vascular dementia (SIVD; N = 61), and frontotemporal dementia (FTD; N = 34)...
February 8, 2017: European Archives of Psychiatry and Clinical Neuroscience
https://www.readbyqxmd.com/read/28173118/early-microgliosis-precedes-neuronal-loss-and-behavioural-impairment-in-mice-with-a-frontotemporal-dementia-causing-chmp2b-mutation
#19
Emma L Clayton, Renzo Mancuso, Troels Tolstrup Nielsen, Sarah Mizielinska, Holly Holmes, Nicholas Powell, Frances Norona, Jytte Overgaard Larsen, Carmelo Milioto, Katherine M Wilson, Mark F Lythgoe, Sebastian Ourselin, Jörgen E Nielsen, Peter Johannsen, Ida Holm, John Collinge, Peter L Oliver, Diego Gomez-Nicola, Adrian M Isaacs
No abstract text is available yet for this article.
January 16, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28165465/apical-dendrite-degeneration-a-novel-cellular-pathology-for-betz-cells-in-als
#20
Barış Genç, Javier H Jara, Amiko K B Lagrimas, Peter Pytel, Raymond P Roos, M Marsel Mesulam, Changiz Geula, Eileen H Bigio, P Hande Özdinler
Apical dendrites of Betz cells are important sites for the integration of cortical input, however their health has not been fully assessed in ALS patients. We investigated the primary motor cortices isolated from post-mortem normal control subjects, patients with familial ALS (fALS), sporadic ALS (sALS), ALS with frontotemporal dementia (FTD-ALS), and Alzheimer's disease (AD), and found profound apical dendrite degeneration of Betz cells in both fALS and sALS, as well as FTD-ALS patients. In contrast, Betz cells of AD patients and normal controls retain cellular integrity in the motor cortex, and CA1 pyramidal neurons show abnormalities predominantly within their soma, rather than the apical dendrite...
February 6, 2017: Scientific Reports
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