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Fronto temporal dementia

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https://www.readbyqxmd.com/read/28714891/structural-and-functional-neuroimaging-of-visual-hallucinations-in-lewy-body-disease-a-systematic-literature-review
#1
REVIEW
Stefania Pezzoli, Annachiara Cagnin, Oliver Bandmann, Annalena Venneri
Patients with Lewy body disease (LBD) frequently experience visual hallucinations (VH), well-formed images perceived without the presence of real stimuli. The structural and functional brain mechanisms underlying VH in LBD are still unclear. The present review summarises the current literature on the neural correlates of VH in LBD, namely Parkinson's disease (PD), and dementia with Lewy bodies (DLB). Following a systematic literature search, 56 neuroimaging studies of VH in PD and DLB were critically reviewed and evaluated for quality assessment...
July 15, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28664294/rare-causes-of-early-onset-dystonia-parkinsonism-with-cognitive-impairment-a-de-novo-psen-1-mutation
#2
Miryam Carecchio, Marina Picillo, Lorella Valletta, Antonio E Elia, Tobias B Haack, Autilia Cozzolino, Annalisa Vitale, Barbara Garavaglia, Arcangela Iuso, Caterina F Bagella, Sabina Pappatà, Paolo Barone, Holger Prokisch, Luigi Romito, Valeria Tiranti
Mutations in PSEN1 are responsible for familial Alzheimer's disease (FAD) inherited as autosomal dominant trait, but also de novo mutations have been rarely reported in sporadic early-onset dementia cases. Parkinsonism in FAD has been mainly described in advanced disease stages. We characterized a patient presenting with early-onset dystonia-parkinsonism later complicated by dementia and myoclonus. Brain MRI showed signs of iron accumulation in the basal ganglia mimicking neurodegeneration with brain iron accumulation (NBIA) as well as fronto-temporal atrophy...
July 2017: Neurogenetics
https://www.readbyqxmd.com/read/28660843/rapidly-progressive-fronto-temporal-dementia-ftd-associated-with-frontotemporal-lobar-degeneration-ftld-in-the-presence-of-fused-in-sarcoma-fus-protein-a-rare-sporadic-and-aggressive-form-of-ftd
#3
Nicholas I Bradfield, Catriona McLean, John Drago, David G Darby, David Ames
Fronto-temporal dementia (FTD) associated with Fused in Sarcoma (FUS) protein accumulation is an uncommon cause of FTD with a distinct syndrome of young age onset behavioral variant FTD, without a family history of FTD and caudate atrophy. We present a sporadic case of a 61-year-old patient with mixed features of both behavioral variant FTD with later semantic language dissolution associated with pathologically proven FUS. He was older than usual for FUS pathology, his course was rapidly progressive, and he had atypical language features...
June 29, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/28585384/modeling-the-c9orf72-repeat-expansion-mutation-using-human-induced-pluripotent-stem-cells
#4
Bhuvaneish T Selvaraj, Matthew R Livesey, Siddharthan Chandran
C9ORF72 repeat expansion is the most frequent causal genetic mutation giving rise to amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD). The relatively recent discovery of the C9ORF72 repeat expansion in 2011 and the complexity of the mutation have meant that animal models that successfully recapitulate human C9ORF72 repeat expansion-mediated disease are only now emerging. Concurrent advances in the use of patient-derived induced pluripotent stem cells (iPSCs) to model aspects of neurological disease offers an additional approach for the study of C9ORF72 mutation...
July 2017: Brain Pathology
https://www.readbyqxmd.com/read/28452647/a-case-of-fronto-temporal-dementia-ftd-masquerading-as-mood-disorder
#5
Sarah Pospos, Min Xi, Guanjie Chen, Ruiguo Zhang, Qingrong Tan, Andrius Baskys
A 56-year old Chinese female was referred to an academic medical center with atypical, treatment-resistant depression that continued for approximately 3 years after her sister's death. Comprehensive evaluation including neurocognitive testing, EEG, spinal tap, HIV testing and brain MRI revealed behavioral variant of fronto-temporal dementia (bvFTD) with significant frontal and temporal lobe atrophy.This patient's unusual clinical presentation emphasizes the overlap between depression and bvFTD, and underlines the importance of prompt, accurate diagnosis to minimize often-ineffective pharmacological interventions and caregiver burnout...
December 23, 2016: Clinical Gerontologist
https://www.readbyqxmd.com/read/28427413/adiponectin-levels-in-the-serum-and-cerebrospinal-fluid-of-amyotrophic-lateral-sclerosis-patients-possible-influence-on-neuroinflammation
#6
Patrizia Bossolasco, Raffaella Cancello, Alberto Doretti, Claudia Morelli, Vincenzo Silani, Lidia Cova
BACKGROUND: Adiponectin (APN) is a key player in energy homeostasis strictly associated with cerebrovascular and neurodegenerative diseases. Since APN also belongs to anti-inflammatory-acting adipokines and may influence both neuroinflammation and neurodegenerative processes, we decided to study the APN levels in amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. METHODS: We assessed APN levels by ELISA immunoassay in both the serum and cerebrospinal fluid of a cohort of familial and sporadic ALS patients, characterized by normal body mass index and absence of dysautonomic symptoms...
April 20, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28424054/an-unusual-delusion-of-duplication-in-a-patient-affected-by-dementia-with-lewy-bodies
#7
Paolo Solla, Gioia Mura, Antonino Cannas, Gianluca Floris, Davide Fonti, Gianni Orofino, Mauro Giovanni Carta, Francesco Marrosu
BACKGROUND: Dementia with Lewy bodies (DLB) is the second most frequent diagnosis of progressive degenerative dementia in older people. Delusions are common features in DLB and, among them, Capgras syndrome represents the most frequent disturbance, characterized by the recurrent and transient belief that a familiar person, often a close family member or caregiver, has been replaced by an identical-looking imposter. However, other delusional conditions near to misidentification syndromes can occur in DLB patients and may represent a major psychiatric disorder, although rarely studied systematically...
April 19, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28396624/granulostasis-protein-quality-control-of-rnp-granules
#8
REVIEW
Simon Alberti, Daniel Mateju, Laura Mediani, Serena Carra
Ribonucleoprotein (RNP) granules transport, store, or degrade messenger RNAs, thereby indirectly regulating protein synthesis. Normally, RNP granules are highly dynamic compartments. However, because of aging or severe environmental stress, RNP granules, in particular stress granules (SGs), convert into solid, aggregate-like inclusions. There is increasing evidence that such RNA-protein inclusions are associated with several age-related neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), fronto-temporal dementia (FTD) and Alzheimer's disease (AD)...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28219319/biomarkers-in-alzheimer-s-disease-recent-update
#9
Sushil Sharma, Walter Lipincott
Alzheimer disease (AD) is an age-related neurodegenerative disorder, characterized by loss of memory and cognitive function. It is the common cause of dementia in elderly and is a global health concern as the population of people aged 85 and older, is growing alarmingly. Although pharmacotherapy for the treatment of AD has improved, lot of work remains to treat this devastating disease. AD pathology begins even before the onset of clinical symptoms. Because therapies could be more effective if implemented early in the disease progression, it is highly prudent to discover reliable biomarkers, to detect its exact pathophysiology during pre-symptomatic stage...
February 20, 2017: Current Alzheimer Research
https://www.readbyqxmd.com/read/28137957/loss-of-function-mutations-in-the-atp13a2-park9-gene-cause-complicated-hereditary-spastic-paraplegia-spg78
#10
Alejandro Estrada-Cuzcano, Shaun Martin, Teodora Chamova, Matthis Synofzik, Dagmar Timmann, Tine Holemans, Albena Andreeva, Jennifer Reichbauer, Riet De Rycke, Dae-In Chang, Sarah van Veen, Jean Samuel, Ludger Schöls, Thorsten Pöppel, Danny Mollerup Sørensen, Bob Asselbergh, Christine Klein, Stephan Zuchner, Albena Jordanova, Peter Vangheluwe, Ivailo Tournev, Rebecca Schüle
Hereditary spastic paraplegias are heterogeneous neurodegenerative disorders characterized by progressive spasticity of the lower limbs due to degeneration of the corticospinal motor neurons. In a Bulgarian family with three siblings affected by complicated hereditary spastic paraplegia, we performed whole exome sequencing and homozygosity mapping and identified a homozygous p.Thr512Ile (c.1535C > T) mutation in ATP13A2. Molecular defects in this gene have been causally associated with Kufor-Rakeb syndrome (#606693), an autosomal recessive form of juvenile-onset parkinsonism, and neuronal ceroid lipofuscinosis (#606693), a neurodegenerative disorder characterized by the intracellular accumulation of autofluorescent lipopigments...
February 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28080965/feeling-learning-from-and-being-aware-of-inner-states-interoceptive-dimensions-in-neurodegeneration-and-stroke
#11
Indira García-Cordero, Lucas Sedeño, Laura de la Fuente, Andrea Slachevsky, Gonzalo Forno, Francisco Klein, Patricia Lillo, Jesica Ferrari, Clara Rodriguez, Julian Bustin, Teresa Torralva, Sandra Baez, Adrian Yoris, Sol Esteves, Margherita Melloni, Paula Salamone, David Huepe, Facundo Manes, Adolfo M García, Agustín Ibañez
Interoception is a complex process encompassing multiple dimensions, such as accuracy, learning and awareness. Here, we examined whether each of those dimensions relies on specialized neural regions distributed throughout the vast interoceptive network. To this end, we obtained relevant measures of cardiac interoception in healthy subjects and patients offering contrastive lesion models of neurodegeneration and focal brain damage: behavioural variant fronto-temporal dementia (bvFTD), Alzheimer's disease (AD) and fronto-insular stroke...
November 19, 2016: Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
https://www.readbyqxmd.com/read/27778099/brain-structural-profile-of-multiple-system-atrophy-patients-with-cognitive-impairment
#12
Eleonora Fiorenzato, Luca Weis, Klaus Seppi, Marco Onofrj, Pietro Cortelli, Stefano Zanigni, Caterina Tonon, Horacio Kaufmann, Timothy Michael Shepherd, Werner Poewe, Florian Krismer, Gregor Wenning, Angelo Antonini, Roberta Biundo
Current consensus diagnostic criteria for multiple system atrophy (MSA) consider dementia a non-supporting feature, although cognitive impairment and even frank dementia are reported in clinical practice. Mini-Mental State Examination (MMSE) is a commonly used global cognitive scale, and in a previous study, we established an MSA-specific screening cut-off score <27 to identify cognitive impairment. Finally, MSA neuroimaging findings suggest the presence of structural alterations in patients with cognitive deficits, although the extent of the anatomical changes is unclear...
October 24, 2016: Journal of Neural Transmission
https://www.readbyqxmd.com/read/27698649/progressively-disrupted-intrinsic-functional-connectivity-of-basolateral-amygdala-in-very-early-alzheimer-s-disease
#13
Marion Ortner, Lorenzo Pasquini, Martina Barat, Panagiotis Alexopoulos, Timo Grimmer, Stefan Förster, Janine Diehl-Schmid, Alexander Kurz, Hans Förstl, Claus Zimmer, Afra Wohlschläger, Christian Sorg, Henning Peters
Very early Alzheimer's disease (AD) - i.e., AD at stages of mild cognitive impairment (MCI) and mild dementia - is characterized by progressive structural and neuropathologic changes, such as atrophy or tangle deposition in medial temporal lobes, including hippocampus and entorhinal cortex and also adjacent amygdala. While progressively disrupted intrinsic connectivity of hippocampus with other brain areas has been demonstrated by many studies, amygdala connectivity was rarely investigated in AD, notwithstanding its known relevance for emotion processing and mood disturbances, which are both important in early AD...
2016: Frontiers in Neurology
https://www.readbyqxmd.com/read/27647788/person-based-versus-generalized-impulsivity-disinhibition-in-frontotemporal-dementia-and-alzheimer-disease
#14
Pongsatorn Paholpak, Andrew R Carr, Joseph P Barsuglia, Robin J Barrows, Elvira Jimenez, Grace J Lee, Mario F Mendez
BACKGROUND: While much disinhibition in dementia results from generalized impulsivity, in behavioral variant frontotemporal dementia (bvFTD) disinhibition may also result from impaired social cognition. OBJECTIVE: To deconstruct disinhibition and its neural correlates in bvFTD vs. early-onset Alzheimer's disease (eAD). METHODS: Caregivers of 16 bvFTD and 21 matched-eAD patients completed the Frontal Systems Behavior Scale disinhibition items...
September 19, 2016: Journal of Geriatric Psychiatry and Neurology
https://www.readbyqxmd.com/read/27630558/anatomical-correlates-of-non-verbal-perception-in-dementia-patients
#15
Pin-Hsuan Lin, Hsiu-Hui Chen, Nai-Ching Chen, Wen-Neng Chang, Chi-Wei Huang, Ya-Ting Chang, Shih-Wei Hsu, Che-Wei Hsu, Chiung-Chih Chang
PURPOSE: Patients with dementia who have dissociations in verbal and non-verbal sound processing may offer insights into the anatomic basis for highly related auditory modes. METHODS: To determine the neuronal networks on non-verbal perception, 16 patients with Alzheimer's dementia (AD), 15 with behavior variant fronto-temporal dementia (bv-FTD), 14 with semantic dementia (SD) were evaluated and compared with 15 age-matched controls. Neuropsychological and auditory perceptive tasks were included to test the ability to compare pitch changes, scale-violated melody and for naming and associating with environmental sound...
2016: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/27555109/a-multimodal-structural-and-functional-neuroimaging-study-of-amnestic-mild-cognitive-impairment
#16
Srikala Bharath, Himanshu Joshi, John P John, Rakesh Balachandar, Shilpa Sadanand, Jitendra Saini, Keshav J Kumar, Mathew Varghese
Examination of brain structural and functional abnormalities in amnestic mild cognitive impairment (aMCI) has the potential to enhance our understanding of the initial pathophysiological changes in dementia. We examined gray matter volumes and white matter microstructural integrity, as well as resting state functional connectivity (rsFC) in patients with aMCI (N = 48) in comparison to elderly cognitively healthy comparison subjects (N = 48). Brain volumetric comparisons were carried out using voxel-based morphometric analysis of T1-weighted images using the FMRIB Software Library...
February 2017: American Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/27498041/different-fdg-pet-metabolic-patterns-at-single-subject-level-in-the-behavioral-variant-of-fronto-temporal-dementia
#17
Chiara Cerami, Alessandra Dodich, Giada Lettieri, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Luigi Gianolli, Stefano F Cappa, Daniela Perani
BACKGROUND: The diagnosis of probable behavioral variant of fronto-temporal dementia (bvFTD) according to current criteria requires the imaging evidence of frontal and/or anterior temporal atrophy or hypoperfusion/hypometabolism. Different variants of this pattern of brain involvement may, however, be found in individual cases, supporting the presence of heterogeneous phenotypes. OBJECTIVE: We examined in a case-by-case approach the FDG-PET metabolic patterns of patients fulfilling clinical criteria for probable bvFTD, assessing the presence and frequency of specific FDG-PET features...
October 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/27476503/dynamic-expression-of-the-mouse-orthologue-of-the-human-amyotropic-lateral-sclerosis-associated-gene-c9orf72-during-central-nervous-system-development-and-neuronal-differentiation
#18
Ross Ferguson, Eleni Serafeimidou-Pouliou, Vasanta Subramanian
The hexanucleotide repeat in the first intron of the C9orf72 gene is the most significant cause of amyotropic lateral sclerosis as well as some forms of fronto-temporal dementia. The C9orf72 protein has been previously reported to be expressed in post-mortem human brain as well as in late embryonic and some postnatal stages in mice. Herein, we present a detailed study of the distribution of C9orf72 protein in the embryonic, postnatal and adult mouse brain, spinal cord as well as during the differentiation of P19 embryonal carcinoma cells to neurons including motor neurons...
December 2016: Journal of Anatomy
https://www.readbyqxmd.com/read/27445778/identifying-dynamic-functional-connectivity-changes-in-dementia-with-lewy-bodies-based-on-product-hidden-markov-models
#19
Marion Sourty, Laurent Thoraval, Daniel Roquet, Jean-Paul Armspach, Jack Foucher, Frédéric Blanc
Exploring time-varying connectivity networks in neurodegenerative disorders is a recent field of research in functional MRI. Dementia with Lewy bodies (DLB) represents 20% of the neurodegenerative forms of dementia. Fluctuations of cognition and vigilance are the key symptoms of DLB. To date, no dynamic functional connectivity (DFC) investigations of this disorder have been performed. In this paper, we refer to the concept of connectivity state as a piecewise stationary configuration of functional connectivity between brain networks...
2016: Frontiers in Computational Neuroscience
https://www.readbyqxmd.com/read/27298765/face-shape-and-face-identity-processing-in-behavioral-variant-fronto-temporal-dementia-a-specific-deficit-for-familiarity-and-name-recognition-of-famous-faces
#20
François-Laurent De Winter, Dorien Timmers, Beatrice de Gelder, Marc Van Orshoven, Marleen Vieren, Miriam Bouckaert, Gert Cypers, Jo Caekebeke, Laura Van de Vliet, Karolien Goffin, Koen Van Laere, Stefan Sunaert, Rik Vandenberghe, Mathieu Vandenbulcke, Jan Van den Stock
Deficits in face processing have been described in the behavioral variant of fronto-temporal dementia (bvFTD), primarily regarding the recognition of facial expressions. Less is known about face shape and face identity processing. Here we used a hierarchical strategy targeting face shape and face identity recognition in bvFTD and matched healthy controls. Participants performed 3 psychophysical experiments targeting face shape detection (Experiment 1), unfamiliar face identity matching (Experiment 2), familiarity categorization and famous face-name matching (Experiment 3)...
2016: NeuroImage: Clinical
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